All Phenotypes Vangl2<+> MGI Mouse

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Phenotypes associated with this allele

Allele Symbol
Allele Name
Allele ID

Vangl2⁺
wild type
MGI:1857643

Summary

59 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
ht1	Vangl2^Lp-2J/Vangl2⁺	B6.Cg-Vangl2^Lp-2J/GrsrJ	MGI:4462712
ht2	Vangl2^Lp/Vangl2⁺	C3H.A(Cg)-Vangl2^Lp	MGI:5661923
ht3	Vangl2^M1Yzcm/Vangl2⁺	C57BL/6J-Vangl2^M1Yzcm	MGI:5468814
ht4	Vangl2^em1(IMPC)Mbp/Vangl2⁺	C57BL/6N-Vangl2^em1(IMPC)Mbp/MbpMmucd	MGI:7317092
ht5	Vangl2^M1Yzcm/Vangl2⁺	either: C57BL/6J-Vangl2^m1Yzcm or (involves: C3H/HeJ * C57BL/6J)	MGI:5468816
ht6	Vangl2^Lp-m1Jus/Vangl2⁺	involves: 101/Rl * C3H/Rl * C57BL/6J	MGI:3708848
ht7	Vangl2^Lp/Vangl2⁺	involves: 129 * LPT/LeJ	MGI:5661427
ht8	Vangl2^Lp/Vangl2⁺	involves: A	MGI:3778628
ht9	Vangl2^Lp/Vangl2⁺	involves: C3H/HeH * C57BL/6 * LPT/Le * NMRI * SWR	MGI:5661722
ht10	Vangl2^M1Yzcm/Vangl2⁺	involves: C3H/HeJ * C57BL/6J	MGI:5468815
ht11	Vangl2^Lp/Vangl2⁺	involves: C57BL/6 * CBA/Ca * LPT/LeJ	MGI:3778629
ht12	Vangl2^Lp/Vangl2⁺	involves: C57BL/6 * LPT/LeJ	MGI:4360075
ht13	Vangl2^Lp/Vangl2⁺	involves: CBA * LPT/Le	MGI:5661926
ht14	Vangl2^Lp/Vangl2⁺	LPT/LeJ	MGI:3608686
cx15	Dact1^tm1.1Bnrc/Dact1⁺ Vangl2^Lp/Vangl2⁺	B6.Cg-Vangl2^Lp Dact1^tm1.1Bnrc	MGI:4360076
cx16	Dact1^tm1.1Bnrc/Dact1^tm1.1Bnrc Vangl2^Lp/Vangl2⁺	B6.Cg-Vangl2^Lp Dact1^tm1.1Bnrc	MGI:4360077
cx17	Celsr1^Crsh/Celsr1⁺ Vangl2^Lp/Vangl2⁺	C3H.Cg-Vangl2^Lp Celsr1^Crsh	MGI:5661740
cx18	Scrib^Crc/Scrib⁺ Vangl2^Lp/Vangl2⁺	C3H.Cg-Vangl2^Lp Scrib^Crc	MGI:5661815
cx19	Celsr1^Crsh/Celsr1⁺ Scrib^Crc/Scrib⁺ Vangl2^Lp/Vangl2⁺	C3H.Cg-Vangl2^Lp Scrib^Crc Celsr1^Crsh	MGI:5661822
cx20	Celsr1^Crsh/Celsr1⁺ Vangl2^Lp/Vangl2⁺	involves: 101/H * BALB/c * C3H/HeH * CBA * LPT/Le	MGI:5661720
cx21	Dact1^tm1.1Bnrc/Dact1⁺ Vangl2^Lp-m1Jus/Vangl2⁺	involves: 101/Rl * 129 * C3H/Rl * C57BL/6	MGI:4360080
cx22	Dact1^tm1.1Bnrc/Dact1^tm1.1Bnrc Vangl2^Lp-m1Jus/Vangl2⁺	involves: 101/Rl * 129 * C3H/Rl * C57BL/6	MGI:4360079
cx23	Sfrp1^tm1Aksh/Sfrp1^tm1Aksh Sfrp2^tm1Aksh/Sfrp2⁺ Sfrp5^tm1Aksh/Sfrp5^tm1Aksh Vangl2^Lp/Vangl2⁺	involves: 129 * A * C57BL/6 * LPT/LeJ	MGI:3795555
cx24	Sfrp1^tm1Aksh/Sfrp1^tm1Aksh Sfrp5^tm1Aksh/Sfrp5^tm1Aksh Vangl2^Lp/Vangl2⁺	involves: 129 * A * C57BL/6 * LPT/LeJ	MGI:3795556
cx25	Sfrp1^tm1Aksh/Sfrp1^tm1Aksh Sfrp2^tm1Aksh/Sfrp2^tm1Aksh Sfrp5^tm1Aksh/Sfrp5⁺ Vangl2^Lp/Vangl2⁺	involves: 129 * A * C57BL/6 * LPT/LeJ	MGI:3795553
cx26	Fzd7^tm1.1Nat/Fzd7^tm1.1Nat Vangl2^Lp/Vangl2⁺	involves: 129 * C57BL/6 * LPT/LeJ	MGI:5444709
cx27	Fzd2^tm1.1Nat/Fzd2⁺ Fzd7^tm1.1Nat/Fzd7^tm1.1Nat Vangl2^Lp/Vangl2⁺	involves: 129 * C57BL/6 * LPT/LeJ	MGI:5444707
cx28	Fzd2^tm1.1Nat/Fzd2⁺ Vangl2^Lp/Vangl2⁺	involves: 129 * C57BL/6 * LPT/LeJ	MGI:5444719
cx29	Ankrd6^tm1Pche/Ankrd6^tm1Pche Vangl2^Lp/Vangl2⁺	involves: 129 * LPT/LeJ	MGI:5661425
cx30	Vangl2^Lp/Vangl2⁺ Ptk7^{Gt(Betageo)1Matl}/Ptk7⁺	involves: 129P2/Ola * C57BL/6 * LPT/LeJ	MGI:3047813
cx31	Vangl1^Gt(XL802)Byg/Vangl1⁺ Vangl2^ska17/Vangl2⁺	involves: 129P2/OlaHsd * 129S6/SvEvTac * C57BL/6J	MGI:4941950
cx32	Vangl1^Gt(XL802)Byg/Vangl1^Gt(XL802)Byg Vangl2^Lp/Vangl2⁺	involves: 129P2/OlaHsd * A	MGI:4819599
cx33	Fat4^tm1.1Hmc/Fat4^tm1.1Hmc Vangl2^Lp/Vangl2⁺	involves: 129P2/OlaHsd * A * FVB/N	MGI:3846578
cx34	Cthrc1^tm1Hssk/Cthrc1^tm1Hssk Vangl2^Lp/Vangl2⁺	involves: 129P2/OlaHsd * C57BL/6	MGI:3815076
cx35	Cthrc1^tm1Hssk/Cthrc1⁺ Vangl2^Lp/Vangl2⁺	involves: 129P2/OlaHsd * C57BL/6	MGI:3815075
cx36	Vangl1^Gt(XL802)Byg/Vangl1^Gt(XL802)Byg Vangl2^tm1.2Yy/Vangl2⁺	involves: 129P2/OlaHsd * C57BL/6 * CBA	MGI:4819600
cx37	Cthrc1^tm1Hssk/Cthrc1⁺ Vangl2^Lp/Vangl2⁺	involves: 129P2/OlaHsd * C57BL/6 * CD-1	MGI:3815079
cx38	Vangl1^Gt(XL802)Byg/Vangl1⁺ Vangl2^Lp/Vangl2⁺	involves: 129P2/OlaHsd * C57BL/6J * LPT/LeJ	MGI:3778824
cx39	Fzd1^tm1.1Nat/Fzd1⁺ Vangl2^Lp/Vangl2⁺	involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * CBA * LPT/LeJ	MGI:4838143
cx40	Fzd2^tm1.1Nat/Fzd2^tm1.1Nat Vangl2^Lp/Vangl2⁺	involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * CBA * LPT/LeJ	MGI:4838145
cx41	Fzd1^tm1.1Nat/Fzd1⁺ Fzd2^tm1.1Nat/Fzd2⁺ Vangl2^Lp/Vangl2⁺	involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * CBA * LPT/LeJ	MGI:4838144
cx42	Cobl^C101/Cobl^C101 Vangl2^Lp/Vangl2⁺	involves: 129S2/SvPas * LPT/LeJ	MGI:2677880
cx43	Vangl2^Lp/Vangl2⁺ Mkks^{Gt(OST367255)Lex}/Mkks⁺	involves: 129S5/SvEvBrd * C57BL/6J * LPT/LeJ	MGI:3608972
cx44	Dvl3^tm1Awb/Dvl3⁺ Vangl2^Lp/Vangl2⁺	involves: 129S6/SvEvTac * A * Black Swiss	MGI:3831924
cx45	Dvl3^tm1Awb/Dvl3^tm1Awb Vangl2^Lp/Vangl2⁺	involves: 129S6/SvEvTac * A * Black Swiss	MGI:3831926
cx46	Sestd1^tm1.1Bnrc/Sestd1⁺ Vangl2^Lp/Vangl2⁺	involves: 129S6/SvEvTac * A * C57BL/6NCr	MGI:5520978
cx47	Sestd1^tm1.1Bnrc/Sestd1^tm1.1Bnrc Vangl2^Lp/Vangl2⁺	involves: 129S6/SvEvTac * A * C57BL/6NCr	MGI:5520977
cx48	Dvl2^tm1Awb/Dvl2^tm1Awb Vangl2^Lp/Vangl2⁺	involves: 129S6/SvEvTac * LPT/LeJ	MGI:3608687
cx49	Dvl1^tm1Awb/Dvl1⁺ Dvl2^tm1Awb/Dvl2^tm1Awb Vangl2^Lp/Vangl2⁺	involves: 129S6/SvEvTac * LPT/LeJ	MGI:3715984
cx50	Dvl2^tm1Awb/Dvl2^tm1Awb Tg(Dvl2/EGFP)2Awb/? Vangl2^Lp/Vangl2⁺	involves: 129S6/SvEvTac * LPT/LeJ	MGI:3715990
cx51	Bbs1^Gt1Nk/Bbs1⁺ Vangl2^Lp/Vangl2⁺	involves: 129S7/SvEvBrd * LPT/LeJ	MGI:3608975
cx52	Ptk7^chz/Ptk7⁺ Vangl2^Lp/Vangl2⁺	involves: A * BALB/cAnN * C3H/HeH	MGI:4830332
cx53	Sec24b^Y613X/Sec24b⁺ Vangl2^Lp/Vangl2⁺	involves: A * C3H/He * C57BL/6	MGI:4440638
cx54	Rpl10a^em2Mbar/Rpl10a⁺ Vangl2^Lp/Vangl2⁺	involves: A * C57BL/6	MGI:7385102
cx55	Grhl3^ct/? Vangl2^Lp/Vangl2⁺	involves: A * GFF	MGI:3794074
cx56	Lrp6^skax26/Lrp6⁺ Vangl2^Lp/Vangl2⁺	involves: A/J * 129S6/SvEvTac * C57BL/6 * C57BL/6J	MGI:5571494
cx57	Scrib^crn2/Scrib⁺ Vangl2^Lp/Vangl2⁺	involves: A/J * FVB/N	MGI:5056406
cx58	Scrib^Crc/Scrib⁺ Vangl2⁺/Vangl2^Lp	involves: BALB/c * C57BL/6 * CBA/Ca * LPT/LeJ * NMRI * SWR	MGI:3797053
cx59	Prickle1^tm1Nue/Prickle1⁺ Vangl2^Lp/Vangl2⁺	involves: C57BL/6J * CBA * LPT/LeJ	MGI:4356109

Allelic Composition	Vangl2^Lp-2J/Vangl2⁺
Genetic Background	B6.Cg-Vangl2^Lp-2J/GrsrJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Vangl2^Lp-2J mutation (1 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

mortality/aging

neonatal lethality, incomplete penetrance ( J:161923 )

• some newborns with severe spina bifida die shortly after birth

preweaning lethality, incomplete penetrance ( J:161923 )

• fewer than 50% of offspring from heterozygous intercrosses are heterozygotes

limbs/digits/tail

abnormal tail morphology ( J:161923 )

• a short, curled tail is the most common phenotype seen for this mutation with incomplete penetrance

short tail ( J:161923 )

curly tail ( J:161923 )

embryo

spina bifida ( J:161923 )

• observed to varying degrees of severity in some, but not all, affected mice and some with mild spina bifida have lived to adulthood and reproduced

nervous system

spina bifida ( J:161923 )

• observed to varying degrees of severity in some, but not all, affected mice and some with mild spina bifida have lived to adulthood and reproduced

reproductive system

vagina atresia ( J:161923 )

• some heterozygotes have closed vaginas

behavior/neurological

behavior/neurological phenotype ( J:161923 )

N	• no abnormal head movement

hearing/vestibular/ear

hearing/vestibular/ear phenotype ( J:161923 )

N	• auditory brainstem response is normal in 2 female and 2 male heterozygotes

Allelic Composition	Vangl2^Lp/Vangl2⁺
Genetic Background	C3H.A(Cg)-Vangl2^Lp

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Vangl2^Lp mutation (2 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

embryo

delayed neural tube closure ( J:216413 )

• Background Sensitivity: neural tube closure delay is less severe (1.2-somites delay) on the C3H/HeH background than on the mixed CBA and LPT/Le background (1.9-somites delay)

limbs/digits/tail

curly tail ( J:216413 )

• Background Sensitivity: looped tail phenotype becomes less penetrant on the C3H/HeH background, seen in 55% of mutants

nervous system

delayed neural tube closure ( J:216413 )

• Background Sensitivity: neural tube closure delay is less severe (1.2-somites delay) on the C3H/HeH background than on the mixed CBA and LPT/Le background (1.9-somites delay)

Allelic Composition	Vangl2^M1Yzcm/Vangl2⁺
Genetic Background	C57BL/6J-Vangl2^M1Yzcm

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Vangl2^M1Yzcm mutation (0 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

limbs/digits/tail

curly tail ( J:193593 )

Allelic Composition	Vangl2^em1(IMPC)Mbp/Vangl2⁺
Genetic Background	C57BL/6N-Vangl2^em1(IMPC)Mbp/MbpMmucd

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Vangl2^em1(IMPC)Mbp mutation (1 available); any Vangl2 mutation (34 available)

Data Sources

IMPC Data for Vangl2

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

embryo

abnormal placenta morphology ( J:211773 )

IMPC - UCD

homeostasis/metabolism

decreased circulating phosphate level ( J:211773 )

IMPC - UCD

vision/eye

persistence of hyaloid vascular system ( J:211773 )

IMPC - UCD

microphthalmia ( J:211773 )

IMPC - UCD

abnormal retina morphology ( J:211773 )

IMPC - UCD

Allelic Composition	Vangl2^M1Yzcm/Vangl2⁺
Genetic Background	either: C57BL/6J-Vangl2^m1Yzcm or (involves: C3H/HeJ * C57BL/6J)

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Vangl2^M1Yzcm mutation (0 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

nervous system

abnormal neural tube morphology ( J:193593 )

• in 4 of 16 mice at between E12.5 and E18.5

abnormal embryonic neuroepithelium morphology ( J:193593 )

• exposed neuroepithelium at E12.5

enlarged floor plate ( J:193593 )

• enlarged at E12.5

open neural tube ( J:193593 )

• from the midbrain to tail in some mice

growth/size/body

decreased fetal size ( J:193593 )

• in some mice at E18.5

fetal growth retardation ( J:193593 )

• in some mice at E18.5

vision/eye

failure of eyelid fusion ( J:193593 )

• in some mice

embryo

abnormal neural tube morphology ( J:193593 )

• in 4 of 16 mice at between E12.5 and E18.5

abnormal embryonic neuroepithelium morphology ( J:193593 )

• exposed neuroepithelium at E12.5

enlarged floor plate ( J:193593 )

• enlarged at E12.5

open neural tube ( J:193593 )

• from the midbrain to tail in some mice

Allelic Composition	Vangl2^Lp-m1Jus/Vangl2⁺
Genetic Background	involves: 101/Rl * C3H/Rl * C57BL/6J

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Vangl2^Lp-m1Jus mutation (1 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

limbs/digits/tail

curly tail ( J:68823 )

• severely kinked or looped tail; partial penetration

kinked tail ( J:68823 )

• severely kinked or looped tail; partial penetration

Allelic Composition	Vangl2^Lp/Vangl2⁺
Genetic Background	involves: 129 * LPT/LeJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Vangl2^Lp mutation (2 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

reproductive system

vagina atresia ( J:216288 )

• seen in less than 10% of females

female infertility ( J:216288 )

• seen in less than 10% of females

Allelic Composition	Vangl2^Lp/Vangl2⁺
Genetic Background	involves: A

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Vangl2^Lp mutation (2 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

behavior/neurological

behavior/neurological phenotype ( J:5888 )

N	• Background Sensitivity: head wobble seen in mice on an LPT/LeJ background is lost in mice descended from KF Stein's albino stock

abnormal motor capabilities/coordination/movement ( J:133042 )

• reduction if the frequency of forepaw vibrations

impaired balance ( J:133042 )

• mice will often fall on their sides when running

abnormal head movements ( J:13059 , J:133042 )

• mice display head rocking or wobbling that in some cases tends to choreic activity (J:13059)

• choreatic movement of the head (J:133042)

head shaking ( J:5061 )

• all mice with a marked degree of head shaking also show brain abnormalities

abnormal tail movements ( J:133042 )

• absence of tail rattling behavior

abnormal locomotor behavior ( J:133042 )

• decrease in the frequency of wire mesh climbing

decreased vertical activity ( J:133042 )

• decreased frequency of rearing which involves shaking movements of the forepart of the body

nervous system

nervous system phenotype ( J:5888 )

N	• Background Sensitivity: no ventricle abnormalities are detected in mice descended from KF Stein's albino stock unlike mice on an LPT/LeJ background

spina bifida ( J:158452 )

• in 5% of mice

craniorachischisis ( J:162640 )

abnormal orientation of cochlear hair cell stereociliary bundles ( J:142392 , J:214064 )

• stereociliary bundles in apical regions are rotated compared to in wild-type mice (J:142392)

abnormal brain ventricle morphology ( J:5061 )

• large ventriculus impar

• however, the third and fourth ventricles appear unaffected

enlarged lateral ventricles ( J:5061 )

• seen in 7 of 9 mice examined although sometimes only unilaterally

abnormal corpus callosum morphology ( J:5061 )

• appears to be reduced in some places

abnormal dorsal striatum morphology ( J:5061 )

• slightly deformed at the medial margin and in the septal area with the nucleus lateralis septi clearly malformed

abnormal hippocampus morphology ( J:5061 )

• somewhat deformed and caudally displaced

limbs/digits/tail

short tail ( J:162640 )

curly tail ( J:13059 , J:162640 , J:201925 )

• partial penetrance of loops in the tail (J:13059)

• variable degree of contortion of the tail ranging from extreme pretzel-like twists to minor angular crooks or curves (J:13059)

• in 21 of 28 mice (J:201925)

reproductive system

vagina atresia ( J:13059 , J:162640 )

• about one third of females lack a vaginal opening (J:13059)

(J:162640)

female infertility ( J:162640 )

skeleton

abnormal xiphoid process morphology ( J:12147 )

• frequent bifurcation of the xiphoid process, extending beyond the cartilaginous tip, is seen

hearing/vestibular/ear

hearing/vestibular/ear phenotype ( J:13059 )

N	• despite abnormal head movements, mice are not deaf

abnormal orientation of cochlear hair cell stereociliary bundles ( J:142392 , J:214064 )

• stereociliary bundles in apical regions are rotated compared to in wild-type mice (J:142392)

embryo

spina bifida ( J:158452 )

• in 5% of mice

craniorachischisis ( J:162640 )

Allelic Composition	Vangl2^Lp/Vangl2⁺
Genetic Background	involves: C3H/HeH * C57BL/6 * LPT/Le * NMRI * SWR

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Vangl2^Lp mutation (2 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

embryo

craniorachischisis ( J:216413 )

• 54% of embryos develop craniorachischisis

nervous system

craniorachischisis ( J:216413 )

• 54% of embryos develop craniorachischisis

Allelic Composition	Vangl2^M1Yzcm/Vangl2⁺
Genetic Background	involves: C3H/HeJ * C57BL/6J

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Vangl2^M1Yzcm mutation (0 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

limbs/digits/tail

curly tail ( J:193593 )

skeleton

skeleton phenotype ( J:193593 )

N	• skeletons are normal

Allelic Composition	Vangl2^Lp/Vangl2⁺
Genetic Background	involves: C57BL/6 * CBA/Ca * LPT/LeJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Vangl2^Lp mutation (2 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

nervous system

abnormal neural tube closure ( J:20323 )

• when cultured starting at E8.5 more embryos with open neural tubes are detected suggesting an increase in susceptibility to failure of neural tube closure in stressful conditions

• however, initiation of closure of the cranial neural tube at the midbrain/forebrain boundary is similar to wild-type

delayed neural tube closure ( J:20323 )

• explants of E8.5 embryos show a delay in closure of about 4 - 6 hrs

• explants of E9.5 and E10.5 embryos show a delay of posterior neuropore closure

embryo

abnormal neural tube closure ( J:20323 )

• when cultured starting at E8.5 more embryos with open neural tubes are detected suggesting an increase in susceptibility to failure of neural tube closure in stressful conditions

• however, initiation of closure of the cranial neural tube at the midbrain/forebrain boundary is similar to wild-type

delayed neural tube closure ( J:20323 )

• explants of E8.5 embryos show a delay in closure of about 4 - 6 hrs

• explants of E9.5 and E10.5 embryos show a delay of posterior neuropore closure

Allelic Composition	Vangl2^Lp/Vangl2⁺
Genetic Background	involves: C57BL/6 * LPT/LeJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Vangl2^Lp mutation (2 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

embryo

abnormal embryonic tissue morphology ( J:152701 )

• at the 6 to 7 somite stage the posterior length to width ratio is significantly decreased compared to controls

• however, the whole embryo length to width ratio is not significantly different from controls

limbs/digits/tail

curly tail ( J:152701 )

Allelic Composition	Vangl2^Lp/Vangl2⁺
Genetic Background	involves: CBA * LPT/Le

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Vangl2^Lp mutation (2 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

embryo

delayed neural tube closure ( J:216413 )

• Background Sensitivity: neural tube closure delay is more severe on this background (1.9-somites delay) than on the C3H/HeH background (1.2-somites delay)

limbs/digits/tail

curly tail ( J:216413 )

• Background Sensitivity: looped tail phenotype is more penetrant, seen in 90% of mutants, than on the C3H/HeH background in which 55% of mutants have a looped

nervous system

delayed neural tube closure ( J:216413 )

• Background Sensitivity: neural tube closure delay is more severe on this background (1.9-somites delay) than on the C3H/HeH background (1.2-somites delay)

Allelic Composition	Vangl2^Lp/Vangl2⁺
Genetic Background	LPT/LeJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Vangl2^Lp mutation (2 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

hearing/vestibular/ear

hearing/vestibular/ear phenotype ( J:100861 )

N	• normal stereocilia orientation at E18.5 and in inner ears cultured from E14.5 for 6 days

limbs/digits/tail

kinked tail ( J:108512 )

• tails are kinked or looped

behavior/neurological

abnormal head movements ( J:5888 )

• Background Sensitivity: unlike mice descended from KF Stein's albino stock, head wobble is seen

nervous system

delayed neural tube closure ( J:108512 )

• neural tube remains open at 8- to 9-somite stage

abnormal brain ventricular system morphology ( J:5888 )

• distortions in the septal area

enlarged third ventricle ( J:5888 )

• occasionally slightly enlarged

abnormal lateral ventricle morphology ( J:5888 )

• usually bilaterally enlarged and distorted although the abnormality may be unilateral

enlarged lateral ventricles ( J:5888 )

abnormal hippocampus morphology ( J:5888 )

• distortions to the overall shape

embryo

delayed neural tube closure ( J:108512 )

• neural tube remains open at 8- to 9-somite stage

Allelic Composition	Dact1^tm1.1Bnrc/Dact1⁺ Vangl2^Lp/Vangl2⁺
Genetic Background	B6.Cg-Vangl2^Lp Dact1^tm1.1Bnrc

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dact1^tm1.1Bnrc mutation (0 available); any Dact1 mutation (21 available)
Vangl2^Lp mutation (2 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

limbs/digits/tail

curly tail ( J:152701 )

• penetrance of the loop tail phenotype is decreased to less than 65% compared to greater than 80% in Vangl2 single heterozygotes

Allelic Composition	Dact1^tm1.1Bnrc/Dact1^tm1.1Bnrc Vangl2^Lp/Vangl2⁺
Genetic Background	B6.Cg-Vangl2^Lp Dact1^tm1.1Bnrc

Find Mice

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

embryo

caudal body truncation ( J:152701 )

• penetrance is reduced compared to Dact1 single mutants

digestive/alimentary system

abnormal digestive system morphology ( J:152701 )

• penetrance is reduced compared to Dact1 single mutants

abnormal anus morphology ( J:152701 )

• penetrance is reduced compared to Dact1 single mutants

reproductive system

abnormal external female genitalia morphology ( J:152701 )

• penetrance is reduced compared to Dact1 single mutants

abnormal external male genitalia morphology ( J:152701 )

• penetrance is reduced compared to Dact1 single mutants

renal/urinary system

abnormal kidney morphology ( J:152701 )

• penetrance is reduced compared to Dact1 single mutants

abnormal urinary bladder morphology ( J:152701 )

• penetrance is reduced compared to Dact1 single mutants

limbs/digits/tail

curly tail ( J:152701 )

• penetrance of the loop tail phenotype is decreased to less than 20% compared to greater than 80% in Vangl2 single heterozygotes

Allelic Composition	Celsr1^Crsh/Celsr1⁺ Vangl2^Lp/Vangl2⁺
Genetic Background	C3H.Cg-Vangl2^Lp Celsr1^Crsh

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Celsr1^Crsh mutation (2 available); any Celsr1 mutation (144 available)
Vangl2^Lp mutation (2 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

embryo

abnormal left-right axis patterning ( J:216413 )

• rightward skewing of the embryo, with shortening of the right-hand size compared with the left, suggesting a defect in embryo turning

craniorachischisis ( J:216413 )

• 100% of embryos exhibit craniorachischisis, occurring usually as an isolated defect (in 89% of mutants) but sometimes associated with an abdominal wall defect (in 11% of mutants)

growth/size/body

omphalocele ( J:216413 )

• abdominal wall defect in 11% of mutants, which is likely to be omphalocele/exomphalos

nervous system

craniorachischisis ( J:216413 )

• 100% of embryos exhibit craniorachischisis, occurring usually as an isolated defect (in 89% of mutants) but sometimes associated with an abdominal wall defect (in 11% of mutants)

vision/eye

abnormal eyelid morphology ( J:216413 )

• 3 of 5 mutants fail to close eyelids at E16.5, while others show partial eyelid closure

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
neural tube defect		DOID:0080074	OMIM:301410 OMIM:601634	J:216413

Allelic Composition	Scrib^Crc/Scrib⁺ Vangl2^Lp/Vangl2⁺
Genetic Background	C3H.Cg-Vangl2^Lp Scrib^Crc

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Scrib^Crc mutation (3 available); any Scrib mutation (54 available)
Vangl2^Lp mutation (2 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

embryo

abnormal developmental patterning ( J:216413 )

• skewed body axis in some mutants

craniorachischisis ( J:216413 )

• 81% of mutants exhibit craniorachischisis, most often isolated (in 50% of mutants) but sometimes associated with abdominal wall defect (in 31% of mutants) and a skewed body axis

growth/size/body

abnormal abdominal wall morphology ( J:216413 )

• 3% of mutants exhibit only an abdominal wall defect while 31% exhibit both abdominal wall defect and craniorachischisis

limbs/digits/tail

curly tail ( J:216413 )

• 6% of mutants exhibit a looped tail

mortality/aging

prenatal lethality, incomplete penetrance ( J:216413 )

• 13% of mutants are viable postnatally

nervous system

craniorachischisis ( J:216413 )

• 81% of mutants exhibit craniorachischisis, most often isolated (in 50% of mutants) but sometimes associated with abdominal wall defect (in 31% of mutants) and a skewed body axis

exencephaly ( J:216413 )

• 3% of mutants exhibit exencephaly

vision/eye

abnormal eyelid morphology ( J:216413 )

• all mutants exhibit failure of eyelid closure at E16.5; all these have craniorachischisis

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
neural tube defect		DOID:0080074	OMIM:301410 OMIM:601634	J:216413

Allelic Composition	Celsr1^Crsh/Celsr1⁺ Scrib^Crc/Scrib⁺ Vangl2^Lp/Vangl2⁺
Genetic Background	C3H.Cg-Vangl2^Lp Scrib^Crc Celsr1^Crsh

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Celsr1^Crsh mutation (2 available); any Celsr1 mutation (144 available)
Scrib^Crc mutation (3 available); any Scrib mutation (54 available)
Vangl2^Lp mutation (2 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

embryo

craniorachischisis ( J:216413 )

• 2 of 3 mutants exhibit craniorachischisis

limbs/digits/tail

abnormal tail morphology ( J:216413 )

• 1 of 3 mice exhibits hindbrain exencephaly and a tail defect

nervous system

craniorachischisis ( J:216413 )

• 2 of 3 mutants exhibit craniorachischisis

exencephaly ( J:216413 )

• 1 of 3 mice exhibits hindbrain exencephaly and a tail defect

Allelic Composition	Celsr1^Crsh/Celsr1⁺ Vangl2^Lp/Vangl2⁺
Genetic Background	involves: 101/H * BALB/c * C3H/HeH * CBA * LPT/Le

Find Mice

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

embryo

craniorachischisis ( J:216413 )

• 54% of embryos develop craniorachischisis

limbs/digits/tail

curly tail ( J:216413 )

• the mice that do not develop craniorachischisis exhibit a looped tail

nervous system

craniorachischisis ( J:216413 )

• 54% of embryos develop craniorachischisis

Allelic Composition	Dact1^tm1.1Bnrc/Dact1⁺ Vangl2^Lp-m1Jus/Vangl2⁺
Genetic Background	involves: 101/Rl * 129 * C3H/Rl * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dact1^tm1.1Bnrc mutation (0 available); any Dact1 mutation (21 available)
Vangl2^Lp-m1Jus mutation (1 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

limbs/digits/tail

curly tail ( J:152701 )

• penetrance of the loop tail phenotype is decreased to less than 65% compared to greater than 80% in Vangl2 single heterozygotes

Allelic Composition	Dact1^tm1.1Bnrc/Dact1^tm1.1Bnrc Vangl2^Lp-m1Jus/Vangl2⁺
Genetic Background	involves: 101/Rl * 129 * C3H/Rl * C57BL/6

Find Mice

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

embryo

caudal body truncation ( J:152701 )

• penetrance is reduced compared to Dact1 single mutants

digestive/alimentary system

abnormal digestive system morphology ( J:152701 )

• penetrance is reduced compared to Dact1 single mutants

abnormal anus morphology ( J:152701 )

• penetrance is reduced compared to Dact1 single mutants

reproductive system

abnormal external female genitalia morphology ( J:152701 )

• penetrance is reduced compared to Dact1 single mutants

abnormal external male genitalia morphology ( J:152701 )

• penetrance is reduced compared to Dact1 single mutants

renal/urinary system

abnormal kidney morphology ( J:152701 )

• penetrance is reduced compared to Dact1 single mutants

abnormal urinary bladder morphology ( J:152701 )

• penetrance is reduced compared to Dact1 single mutants

limbs/digits/tail

curly tail ( J:152701 )

• penetrance of the loop tail phenotype is decreased to less than 20% compared to greater than 80% in Vangl2 single heterozygotes

Allelic Composition	Sfrp1^tm1Aksh/Sfrp1^tm1Aksh Sfrp2^tm1Aksh/Sfrp2⁺ Sfrp5^tm1Aksh/Sfrp5^tm1Aksh Vangl2^Lp/Vangl2⁺
Genetic Background	involves: 129 * A * C57BL/6 * LPT/LeJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Sfrp1^tm1Aksh mutation (1 available); any Sfrp1 mutation (18 available)
Sfrp2^tm1Aksh mutation (1 available); any Sfrp2 mutation (28 available)
Sfrp5^tm1Aksh mutation (0 available); any Sfrp5 mutation (26 available)
Vangl2^Lp mutation (2 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

nervous system

spina bifida ( J:135313 )

• 62% of mice exhibit spina bifida

embryo

spina bifida ( J:135313 )

• 62% of mice exhibit spina bifida

Allelic Composition	Sfrp1^tm1Aksh/Sfrp1^tm1Aksh Sfrp5^tm1Aksh/Sfrp5^tm1Aksh Vangl2^Lp/Vangl2⁺
Genetic Background	involves: 129 * A * C57BL/6 * LPT/LeJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Sfrp1^tm1Aksh mutation (1 available); any Sfrp1 mutation (18 available)
Sfrp5^tm1Aksh mutation (0 available); any Sfrp5 mutation (26 available)
Vangl2^Lp mutation (2 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

nervous system

spina bifida ( J:135313 )

• 13% of mice exhibit spina bifida

embryo

spina bifida ( J:135313 )

• 13% of mice exhibit spina bifida

Allelic Composition	Sfrp1^tm1Aksh/Sfrp1^tm1Aksh Sfrp2^tm1Aksh/Sfrp2^tm1Aksh Sfrp5^tm1Aksh/Sfrp5⁺ Vangl2^Lp/Vangl2⁺
Genetic Background	involves: 129 * A * C57BL/6 * LPT/LeJ

Find Mice

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

embryo

abnormal rostral-caudal body axis extension ( J:135313 )

• mice exhibit an anterior-posterior elongation defect that is enhanced in the trunk relative to that observed in Sfrp1^tm1Aksh/Sfrp1^tm1Aksh Sfrp2^tm1Aksh/Sfrp2^tm1Aksh Sfrp5^tm1Aksh/Sfrp5⁺ mice

• at late head-fold stage, mice exhibit abnormal convergence and extension

open neural tube ( J:135313 )

craniorachischisis ( J:135313 )

abnormal somite development ( J:135313 )

nervous system

open neural tube ( J:135313 )

craniorachischisis ( J:135313 )

Allelic Composition	Fzd7^tm1.1Nat/Fzd7^tm1.1Nat Vangl2^Lp/Vangl2⁺
Genetic Background	involves: 129 * C57BL/6 * LPT/LeJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Fzd7^tm1.1Nat mutation (1 available); any Fzd7 mutation (35 available)
Vangl2^Lp mutation (2 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

cardiovascular system

abnormal heart morphology ( J:189062 )

• 35% of embryos (n=20) display cardiac defects

ventricular septal defect ( J:189062 )

• most defects are VSDs

craniofacial

cleft palate ( J:189062 )

• in 4% of embryos (n=25)

digestive/alimentary system

cleft palate ( J:189062 )

• in 4% of embryos (n=25)

growth/size/body

cleft palate ( J:189062 )

• in 4% of embryos (n=25)

Allelic Composition	Fzd2^tm1.1Nat/Fzd2⁺ Fzd7^tm1.1Nat/Fzd7^tm1.1Nat Vangl2^Lp/Vangl2⁺
Genetic Background	involves: 129 * C57BL/6 * LPT/LeJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Fzd2^tm1.1Nat mutation (1 available); any Fzd2 mutation (34 available)
Fzd7^tm1.1Nat mutation (1 available); any Fzd7 mutation (35 available)
Vangl2^Lp mutation (2 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

cardiovascular system

abnormal heart morphology ( J:189062 )

• 37% of embryos (n=18) display cardiac defects

ventricular septal defect ( J:189062 )

• most defects are VSDs

craniofacial

cleft palate ( J:189062 )

• in 53% of embryos (n=17)

digestive/alimentary system

cleft palate ( J:189062 )

• in 53% of embryos (n=17)

growth/size/body

cleft palate ( J:189062 )

• in 53% of embryos (n=17)

Allelic Composition	Fzd2^tm1.1Nat/Fzd2⁺ Vangl2^Lp/Vangl2⁺
Genetic Background	involves: 129 * C57BL/6 * LPT/LeJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Fzd2^tm1.1Nat mutation (1 available); any Fzd2 mutation (34 available)
Vangl2^Lp mutation (2 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

cardiovascular system

abnormal heart morphology ( J:189062 )

• 50% of embryos (n=6) display cardiac defects compared to 0% of embryos heterozygous for either mutation alone

ventricular septal defect ( J:189062 )

• most defects are VSDs

Allelic Composition	Ankrd6^tm1Pche/Ankrd6^tm1Pche Vangl2^Lp/Vangl2⁺
Genetic Background	involves: 129 * LPT/LeJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ankrd6^tm1Pche mutation (0 available); any Ankrd6 mutation (118 available)
Vangl2^Lp mutation (2 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

hearing/vestibular/ear

abnormal cochlear hair cell morphology ( J:216288 )

• patterning and polarity defects

abnormal cochlear outer hair cell morphology ( J:216288 )

• coordinated orientation of the outer-most row of hair cells is disrupted

• 29.2% of the 4th row of hair cells have an orientation deviation of 30 degrees or larger from the planar cell polarity axis compared to only 0.9% of hairs in wild-type controls

reproductive system

vagina atresia ( J:216288 )

female infertility ( J:216288 )

• all females are sterile compared to less than 10% of mice heterozygous for Vangl2^Lp alone

nervous system

abnormal cochlear hair cell morphology ( J:216288 )

• patterning and polarity defects

abnormal cochlear outer hair cell morphology ( J:216288 )

• coordinated orientation of the outer-most row of hair cells is disrupted

• 29.2% of the 4th row of hair cells have an orientation deviation of 30 degrees or larger from the planar cell polarity axis compared to only 0.9% of hairs in wild-type controls

Allelic Composition	Vangl2^Lp/Vangl2⁺ Ptk7^{Gt(Betageo)1Matl}/Ptk7⁺
Genetic Background	involves: 129P2/Ola * C57BL/6 * LPT/LeJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ptk7^{Gt(Betageo)1Matl} mutation (0 available); any Ptk7 mutation (60 available)
Vangl2^Lp mutation (2 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

hearing/vestibular/ear

hearing/vestibular/ear phenotype ( J:91298 )

N	• hair cell stereociliary bundle morphology is mostly unaffected

nervous system

spina bifida ( J:91298 )

• spina bifida is seen in 94% of double heterozygotes

embryo

spina bifida ( J:91298 )

• spina bifida is seen in 94% of double heterozygotes

Allelic Composition	Vangl1^Gt(XL802)Byg/Vangl1⁺ Vangl2^ska17/Vangl2⁺
Genetic Background	involves: 129P2/OlaHsd * 129S6/SvEvTac * C57BL/6J

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Vangl1^Gt(XL802)Byg mutation (1 available); any Vangl1 mutation (85 available)
Vangl2^ska17 mutation (0 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

normal phenotype

no abnormal phenotype detected ( J:169664 )

• no tail, neural tube or eyelid fusion defects are detected

Allelic Composition	Vangl1^Gt(XL802)Byg/Vangl1^Gt(XL802)Byg Vangl2^Lp/Vangl2⁺
Genetic Background	involves: 129P2/OlaHsd * A

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Vangl1^Gt(XL802)Byg mutation (1 available); any Vangl1 mutation (85 available)
Vangl2^Lp mutation (2 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

reproductive system

vagina atresia ( J:162640 )

female infertility ( J:162640 )

nervous system

abnormal cochlear inner hair cell morphology ( J:162640 )

• inner hair cells are misoriented compared to in wild-type mice

abnormal cochlear outer hair cell morphology ( J:162640 )

• outer hair cells are misoriented compared to in wild-type mice

limbs/digits/tail

short tail ( J:162640 )

• compared with Vangl1^Gt(XL802)Byg/Vangl1^Gt(XL802)Byg Vangl2^tm1.2Yy/Vangl2⁺

hearing/vestibular/ear

abnormal cochlear inner hair cell morphology ( J:162640 )

• inner hair cells are misoriented compared to in wild-type mice

abnormal cochlear outer hair cell morphology ( J:162640 )

• outer hair cells are misoriented compared to in wild-type mice

Allelic Composition	Fat4^tm1.1Hmc/Fat4^tm1.1Hmc Vangl2^Lp/Vangl2⁺
Genetic Background	involves: 129P2/OlaHsd * A * FVB/N

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Fat4^tm1.1Hmc mutation (1 available); any Fat4 mutation (223 available)
Vangl2^Lp mutation (2 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

renal/urinary system

kidney cyst ( J:138247 )

• increase in the severity of cystic tubules compared to mice homozygous for Fat4^tm1.1Hmc alone

growth/size/body

kidney cyst ( J:138247 )

• increase in the severity of cystic tubules compared to mice homozygous for Fat4^tm1.1Hmc alone

Allelic Composition	Cthrc1^tm1Hssk/Cthrc1^tm1Hssk Vangl2^Lp/Vangl2⁺
Genetic Background	involves: 129P2/OlaHsd * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cthrc1^tm1Hssk mutation (1 available); any Cthrc1 mutation (32 available)
Vangl2^Lp mutation (2 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

hearing/vestibular/ear

abnormal cochlear hair cell morphology ( J:140886 )

• orientation is significantly disrupted

• organization of hair cells is normal

abnormal cochlear inner hair cell morphology ( J:140886 )

• disruptions are most severe for inner hair cells

nervous system

abnormal cochlear hair cell morphology ( J:140886 )

• orientation is significantly disrupted

• organization of hair cells is normal

abnormal cochlear inner hair cell morphology ( J:140886 )

• disruptions are most severe for inner hair cells

Allelic Composition	Cthrc1^tm1Hssk/Cthrc1⁺ Vangl2^Lp/Vangl2⁺
Genetic Background	involves: 129P2/OlaHsd * C57BL/6

Find Mice

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

nervous system

abnormal neural tube closure ( J:140886 )

• Background Sensitivity: defects seen in the midbrain region at E13.5

hearing/vestibular/ear

hearing/vestibular/ear phenotype ( J:140886 )

N	• sensory hair cells in the cochlea are normal

embryo

abnormal neural tube closure ( J:140886 )

• Background Sensitivity: defects seen in the midbrain region at E13.5

Allelic Composition	Vangl1^Gt(XL802)Byg/Vangl1^Gt(XL802)Byg Vangl2^tm1.2Yy/Vangl2⁺
Genetic Background	involves: 129P2/OlaHsd * C57BL/6 * CBA

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Vangl1^Gt(XL802)Byg mutation (1 available); any Vangl1 mutation (85 available)
Vangl2^tm1.2Yy mutation (0 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

nervous system

abnormal cochlear outer hair cell morphology ( J:162640 )

• outer hair cells in rows 3 and 2 are misoriented compared to in wild-type mice

limbs/digits/tail

long tail ( J:162640 )

• compared with Vangl2^Lp/Vangl2^Lp Vangl2^tm1.2Yy/Vangl2⁺

hearing/vestibular/ear

abnormal cochlear outer hair cell morphology ( J:162640 )

• outer hair cells in rows 3 and 2 are misoriented compared to in wild-type mice

Allelic Composition	Cthrc1^tm1Hssk/Cthrc1⁺ Vangl2^Lp/Vangl2⁺
Genetic Background	involves: 129P2/OlaHsd * C57BL/6 * CD-1

Find Mice

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

nervous system

nervous system phenotype ( J:140886 )

N	• Background Sensitivity: no defects in neural tube closure are seen

Allelic Composition	Vangl1^Gt(XL802)Byg/Vangl1⁺ Vangl2^Lp/Vangl2⁺
Genetic Background	involves: 129P2/OlaHsd * C57BL/6J * LPT/LeJ

Find Mice

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

Vangl1^Gt(XL802)Byg/Vangl1⁺ Vangl2^Lp/Vangl2⁺ and Vangl2^Lp/Vangl2^Lp embryos exhibit aberrant right subclavian artery

mortality/aging

perinatal lethality, incomplete penetrance ( J:132697 )

• fewer than expected double heterozygotes are found at weaning (20% rather than the expected 50%) given the presence of craniorachischisis late embryonic lethality is probably the cause of the distorted ratio

nervous system

craniorachischisis ( J:132697 )

• seen in over 60% of double heterozygotes at E13.5 - E18.5

• phenotype is as severe as in mice homozygous for Vangl2^Lp alone

• no obvious neural tube defects are seen in surviving mice

abnormal cochlear hair cell stereociliary bundle morphology ( J:132697 )

• profoundly distorted

abnormal orientation of inner hair cell stereociliary bundles ( J:132697 )

• 20% of inner hair cell bundles are misoriented

abnormal orientation of outer hair cell stereociliary bundles ( J:132697 )

• some bundles in all 3 layers are misoriented especially at the apical turn (over 50% of bundles in OHC1, 65% in OHC2, over 80% in OHC3)

• vertices are randomly oriented with rotation angles of 40 - 180 degrees

cardiovascular system

cardiovascular system phenotype ( J:132697 )

N	• unlike mice homozygous for Vangl2^Lp alone, no outflow tract abnormalities are detected in double heterozygotes

retroesophageal right subclavian artery ( J:132697 )

• at E14.5, the right subclavian artery is positioned dorsal to the esophagus

hearing/vestibular/ear

abnormal cochlea morphology ( J:132697 )

• reduced in size at E18.5 in mice displaying craniorachischisis

abnormal cochlear hair cell stereociliary bundle morphology ( J:132697 )

• profoundly distorted

abnormal orientation of inner hair cell stereociliary bundles ( J:132697 )

• 20% of inner hair cell bundles are misoriented

abnormal orientation of outer hair cell stereociliary bundles ( J:132697 )

• some bundles in all 3 layers are misoriented especially at the apical turn (over 50% of bundles in OHC1, 65% in OHC2, over 80% in OHC3)

• vertices are randomly oriented with rotation angles of 40 - 180 degrees

limbs/digits/tail

curly tail ( J:132697 )

embryo

craniorachischisis ( J:132697 )

• seen in over 60% of double heterozygotes at E13.5 - E18.5

• phenotype is as severe as in mice homozygous for Vangl2^Lp alone

• no obvious neural tube defects are seen in surviving mice

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
neural tube defect		DOID:0080074	OMIM:301410 OMIM:601634	J:132697

Allelic Composition	Fzd1^tm1.1Nat/Fzd1⁺ Vangl2^Lp/Vangl2⁺
Genetic Background	involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * CBA * LPT/LeJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Fzd1^tm1.1Nat mutation (1 available); any Fzd1 mutation (29 available)
Vangl2^Lp mutation (2 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

embryo

open neural tube ( J:165556 )

• in 12 of 27 mice, more common in the rostral neural tube

cardiovascular system

ventricular septal defect ( J:165556 )

• muscular and/or membranous in mice with neural tube defects

nervous system

open neural tube ( J:165556 )

• in 12 of 27 mice, more common in the rostral neural tube

Allelic Composition	Fzd2^tm1.1Nat/Fzd2^tm1.1Nat Vangl2^Lp/Vangl2⁺
Genetic Background	involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * CBA * LPT/LeJ

Find Mice

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

nervous system

open neural tube ( J:165556 )

• in all mice, more common in the caudal neural tube

abnormal cochlear hair cell morphology ( J:165556 )

• cochlear hair cell defects (hair cell number and outer hair cell row 3 orientation) are modestly more severe than in Vangl2^Lp heterozygotes

abnormal cochlear hair cell number ( J:165556 )

abnormal orientation of outer hair cell stereociliary bundles ( J:165556 )

• most severe in outer hair cell row 3

hearing/vestibular/ear

abnormal cochlear hair cell morphology ( J:165556 )

• cochlear hair cell defects (hair cell number and outer hair cell row 3 orientation) are modestly more severe than in Vangl2^Lp heterozygotes

abnormal cochlear hair cell number ( J:165556 )

abnormal orientation of outer hair cell stereociliary bundles ( J:165556 )

• most severe in outer hair cell row 3

cardiovascular system

ventricular septal defect ( J:165556 )

• muscular and/or membranous in mice with neural tube defects

embryo

open neural tube ( J:165556 )

• in all mice, more common in the caudal neural tube

craniofacial

cleft palate ( J:165556 )

• in 50% of mice

digestive/alimentary system

cleft palate ( J:165556 )

• in 50% of mice

growth/size/body

cleft palate ( J:165556 )

• in 50% of mice

Allelic Composition	Fzd1^tm1.1Nat/Fzd1⁺ Fzd2^tm1.1Nat/Fzd2⁺ Vangl2^Lp/Vangl2⁺
Genetic Background	involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * CBA * LPT/LeJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Fzd1^tm1.1Nat mutation (1 available); any Fzd1 mutation (29 available)
Fzd2^tm1.1Nat mutation (1 available); any Fzd2 mutation (34 available)
Vangl2^Lp mutation (2 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

embryo

open neural tube ( J:165556 )

• in 4 of 7 mice, more common in the rostral neural tube

cardiovascular system

ventricular septal defect ( J:165556 )

• muscular and/or membranous in mice with neural tube defects

nervous system

open neural tube ( J:165556 )

• in 4 of 7 mice, more common in the rostral neural tube

Allelic Composition	Cobl^C101/Cobl^C101 Vangl2^Lp/Vangl2⁺
Genetic Background	involves: 129S2/SvPas * LPT/LeJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cobl^C101 mutation (0 available); any Cobl mutation (78 available)
Vangl2^Lp mutation (2 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

nervous system

exencephaly ( J:85744 )

• open neural tubes in the midbrain region

• observable by E9.5 and occurring with 20% penetrance, this phenotype was not observed in lone C101 homozygotes or lone Lp heterozygotes

Allelic Composition	Vangl2^Lp/Vangl2⁺ Mkks^{Gt(OST367255)Lex}/Mkks⁺
Genetic Background	involves: 129S5/SvEvBrd * C57BL/6J * LPT/LeJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Mkks^{Gt(OST367255)Lex} mutation (0 available); any Mkks mutation (27 available)
Vangl2^Lp mutation (2 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

mortality/aging

embryonic lethality during organogenesis, complete penetrance ( J:102697 )

• all double heterozygotes die by E13.5

hearing/vestibular/ear

abnormal outer hair cell stereociliary bundle morphology ( J:102697 )

• some outer hair cell stereociliary hair bundles have a flattened shape

abnormal orientation of outer hair cell stereociliary bundles ( J:102697 )

• some outer hair cell stereociliary hair bundles are misoriented

nervous system

abnormal outer hair cell stereociliary bundle morphology ( J:102697 )

• some outer hair cell stereociliary hair bundles have a flattened shape

abnormal orientation of outer hair cell stereociliary bundles ( J:102697 )

• some outer hair cell stereociliary hair bundles are misoriented

Allelic Composition	Dvl3^tm1Awb/Dvl3⁺ Vangl2^Lp/Vangl2⁺
Genetic Background	involves: 129S6/SvEvTac * A * Black Swiss

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dvl3^tm1Awb mutation (1 available); any Dvl3 mutation (38 available)
Vangl2^Lp mutation (2 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

nervous system

abnormal neural tube morphology ( J:142392 )

• some mice develop neural tube abnormalities such as craniorachischisis and exencephaly

incomplete rostral neuropore closure ( J:142392 )

• some mice develop defects in rostral neural tube closure

craniorachischisis ( J:142392 )

• in 5 of 22 mice

decreased cochlear outer hair cell number ( J:142392 )

• some mice exhibit a loss of outer hair cell rows compared to in wild-type mice along 5% to 20% of the cochlear length

increased cochlear hair cell number ( J:142392 )

• some mice exhibit additional rows of outer and inner cells in the apical region of the cochlea compared to in wild-type mice

abnormal orientation of cochlear hair cell stereociliary bundles ( J:142392 )

• the orientation of cochlear hair cell stereociliary bundles in mice with neural tube defects is disrupted in the base and middle of the cochlear ducts

• stereociliary bundles in apical regions are rotated compared to in wild-type mice

exencephaly ( J:142392 )

• in 2 of 22 mice

cardiovascular system

cardiovascular system phenotype ( J:142392 )

N	• hearts develop normally

hearing/vestibular/ear

decreased cochlear outer hair cell number ( J:142392 )

• some mice exhibit a loss of outer hair cell rows compared to in wild-type mice along 5% to 20% of the cochlear length

increased cochlear hair cell number ( J:142392 )

• some mice exhibit additional rows of outer and inner cells in the apical region of the cochlea compared to in wild-type mice

abnormal orientation of cochlear hair cell stereociliary bundles ( J:142392 )

• stereociliary bundles in apical regions are rotated compared to in wild-type mice

• the orientation of cochlear hair cell stereociliary bundles in mice with neural tube defects is disrupted in the base and middle of the cochlear ducts

inner ear hypoplasia ( J:142392 )

• in mice with neural tube defects

embryo

abnormal neural tube morphology ( J:142392 )

• some mice develop neural tube abnormalities such as craniorachischisis and exencephaly

incomplete rostral neuropore closure ( J:142392 )

• some mice develop defects in rostral neural tube closure

craniorachischisis ( J:142392 )

• in 5 of 22 mice

Allelic Composition	Dvl3^tm1Awb/Dvl3^tm1Awb Vangl2^Lp/Vangl2⁺
Genetic Background	involves: 129S6/SvEvTac * A * Black Swiss

Find Mice

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

nervous system

abnormal neural tube morphology ( J:142392 )

• some mice develop similar neural tube defects as in Dvl3^tm1Awb Vangl2^Lp double heterozygotes

craniorachischisis ( J:142392 )

• in 6 of 16 mice

decreased cochlear outer hair cell number ( J:142392 )

• some mice exhibit a loss of outer hair cell rows compared to in wild-type mice along 5% to 20% of the cochlear length

increased cochlear hair cell number ( J:142392 )

• some mice exhibit additional rows of outer and inner cells in the apical region of the cochlea compared to in wild-type mice

abnormal orientation of cochlear hair cell stereociliary bundles ( J:142392 )

• the orientation of many cochlear hair cell stereociliary bundles is disrupted in the base and middle of the cochlear ducts

• mice with neural tube defects exhibit more severe orientation defects than in mice with normal neural tube closure

• stereociliary bundles in apical regions are rotated compared to in wild-type mice

cardiovascular system

abnormal cardiac outflow tract development ( J:142392 )

• mice exhibit conotruncal defects

hearing/vestibular/ear

decreased cochlear outer hair cell number ( J:142392 )

• some mice exhibit a loss of outer hair cell rows compared to in wild-type mice along 5% to 20% of the cochlear length

increased cochlear hair cell number ( J:142392 )

• some mice exhibit additional rows of outer and inner cells in the apical region of the cochlea compared to in wild-type mice

abnormal orientation of cochlear hair cell stereociliary bundles ( J:142392 )

• the orientation of many cochlear hair cell stereociliary bundles is disrupted in the base and middle of the cochlear ducts

• mice with neural tube defects exhibit more severe orientation defects than in mice with normal neural tube closure

• stereociliary bundles in apical regions are rotated compared to in wild-type mice

inner ear hypoplasia ( J:142392 )

• in mice with neural tube defects

embryo

abnormal neural tube morphology ( J:142392 )

• some mice develop similar neural tube defects as in Dvl3^tm1Awb Vangl2^Lp double heterozygotes

craniorachischisis ( J:142392 )

• in 6 of 16 mice

Allelic Composition	Sestd1^tm1.1Bnrc/Sestd1⁺ Vangl2^Lp/Vangl2⁺
Genetic Background	involves: 129S6/SvEvTac * A * C57BL/6NCr

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Sestd1^tm1.1Bnrc mutation (0 available); any Sestd1 mutation (32 available)
Vangl2^Lp mutation (2 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

limbs/digits/tail

curly tail ( J:201925 )

• in 69 of 84 mice

Allelic Composition	Sestd1^tm1.1Bnrc/Sestd1^tm1.1Bnrc Vangl2^Lp/Vangl2⁺
Genetic Background	involves: 129S6/SvEvTac * A * C57BL/6NCr

Find Mice

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

reproductive system

abnormal reproductive system development ( J:201925 )

• genitourinary defects in 2 of 15 mice compared with 4 of 11 Sestd1^tm1.1Bnrc homozygotes

limbs/digits/tail

short tail ( J:201925 )

• in 1 of 15 mice

curly tail ( J:201925 )

• in 6 of 15 mice

renal/urinary system

abnormal urinary system development ( J:201925 )

• genitourinary defects in 2 of 15 mice compared with 4 of 11 Sestd1^tm1.1Bnrc homozygotes

Allelic Composition	Dvl2^tm1Awb/Dvl2^tm1Awb Vangl2^Lp/Vangl2⁺
Genetic Background	involves: 129S6/SvEvTac * LPT/LeJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dvl2^tm1Awb mutation (1 available); any Dvl2 mutation (35 available)
Vangl2^Lp mutation (2 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

embryo

abnormal embryo development ( J:108512 )

• severe reduction in the embryo length to width ratio is observed similar to in Dvl1^tm1Awb Dvl2^tm1Awb Vangl2^Lp triple homozygotes

craniorachischisis ( J:108512 )

• similar to that observed in Dvl1^tm1Awb Dvl2^tm1Awb Vangl2^Lp triple homozygotes

hearing/vestibular/ear

abnormal organ of Corti morphology ( J:100861 )

• shorter, wider chochlear ducts

increased cochlear hair cell number ( J:100861 )

• increased rows of hair cells within the third of the cochlea nearest the apex

abnormal orientation of cochlear hair cell stereociliary bundles ( J:100861 )

• misorientation of stereociliary bundles at E18.5

abnormal cochlear inner hair cell morphology ( J:108512 )

• occasionally misaligned inner hair cells are obverse

nervous system

craniorachischisis ( J:108512 )

• similar to that observed in Dvl1^tm1Awb Dvl2^tm1Awb Vangl2^Lp triple homozygotes

increased cochlear hair cell number ( J:100861 )

• increased rows of hair cells within the third of the cochlea nearest the apex

abnormal orientation of cochlear hair cell stereociliary bundles ( J:100861 )

• misorientation of stereociliary bundles at E18.5

abnormal cochlear inner hair cell morphology ( J:108512 )

• occasionally misaligned inner hair cells are obverse

mortality/aging

neonatal lethality, complete penetrance ( J:108512 )

• no mice are recovered at birth

Allelic Composition	Dvl1^tm1Awb/Dvl1⁺ Dvl2^tm1Awb/Dvl2^tm1Awb Vangl2^Lp/Vangl2⁺
Genetic Background	involves: 129S6/SvEvTac * LPT/LeJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dvl1^tm1Awb mutation (2 available); any Dvl1 mutation (35 available)
Dvl2^tm1Awb mutation (1 available); any Dvl2 mutation (35 available)
Vangl2^Lp mutation (2 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

mortality/aging

neonatal lethality, complete penetrance ( J:108512 )

• no mice are recovered at birth

nervous system

craniorachischisis ( J:108512 )

• similar to that observed in Dvl1^tm1Awb Dvl2^tm1Awb Vangl2^Lp triple homozygotes

embryo

craniorachischisis ( J:108512 )

• similar to that observed in Dvl1^tm1Awb Dvl2^tm1Awb Vangl2^Lp triple homozygotes

Allelic Composition	Dvl2^tm1Awb/Dvl2^tm1Awb Tg(Dvl2/EGFP)2Awb/? Vangl2^Lp/Vangl2⁺
Genetic Background	involves: 129S6/SvEvTac * LPT/LeJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dvl2^tm1Awb mutation (1 available); any Dvl2 mutation (35 available)
Tg(Dvl2/EGFP)2Awb mutation (0 available)
Vangl2^Lp mutation (2 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

normal phenotype

no abnormal phenotype detected ( J:108512 )

• mice are restored to fertile adults

Allelic Composition	Bbs1^Gt1Nk/Bbs1⁺ Vangl2^Lp/Vangl2⁺
Genetic Background	involves: 129S7/SvEvBrd * LPT/LeJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Bbs1^Gt1Nk mutation (1 available); any Bbs1 mutation (70 available)
Vangl2^Lp mutation (2 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

mortality/aging

embryonic lethality during organogenesis, incomplete penetrance ( J:102697 )

• 7% of double heterozygotes die by E13.5

hearing/vestibular/ear

abnormal outer hair cell stereociliary bundle morphology ( J:102697 )

• some outer hair cell stereociliary hair bundles have a flattened shape

abnormal orientation of outer hair cell stereociliary bundles ( J:102697 )

• some outer hair cell stereociliary hair bundles are misoriented

nervous system

abnormal outer hair cell stereociliary bundle morphology ( J:102697 )

• some outer hair cell stereociliary hair bundles have a flattened shape

abnormal orientation of outer hair cell stereociliary bundles ( J:102697 )

• some outer hair cell stereociliary hair bundles are misoriented

Allelic Composition	Ptk7^chz/Ptk7⁺ Vangl2^Lp/Vangl2⁺
Genetic Background	involves: A * BALB/cAnN * C3H/HeH

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ptk7^chz mutation (2 available); any Ptk7 mutation (60 available)
Vangl2^Lp mutation (2 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

embryo

spina bifida ( J:163834 )

• 23% exhibit spina bifida or craniorachischisis

craniorachischisis ( J:163834 )

• 23% exhibit spina bifida or craniorachischisis

nervous system

spina bifida ( J:163834 )

• 23% exhibit spina bifida or craniorachischisis

craniorachischisis ( J:163834 )

• 23% exhibit spina bifida or craniorachischisis

Allelic Composition	Sec24b^Y613X/Sec24b⁺ Vangl2^Lp/Vangl2⁺
Genetic Background	involves: A * C3H/He * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Sec24b^Y613X mutation (1 available); any Sec24b mutation (68 available)
Vangl2^Lp mutation (2 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

mortality/aging

lethality throughout fetal growth and development, incomplete penetrance ( J:158452 )

• over 50% of mice die between late embryogenesis and four weeks of age

postnatal lethality, incomplete penetrance ( J:158452 )

• over 50% of mice die between late embryogenesis and four weeks of age

nervous system

spina bifida ( J:158452 )

• in 68% of mice

embryo

spina bifida ( J:158452 )

• in 68% of mice

Allelic Composition	Rpl10a^em2Mbar/Rpl10a⁺ Vangl2^Lp/Vangl2⁺
Genetic Background	involves: A * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Rpl10a^em2Mbar mutation (0 available); any Rpl10a mutation (50 available)
Vangl2^Lp mutation (2 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

embryo

spina bifida ( J:328993 )

• low frequency as in Vangl2^Lp heterozygotes

nervous system

spina bifida ( J:328993 )

• low frequency as in Vangl2^Lp heterozygotes

Allelic Composition	Grhl3^ct/? Vangl2^Lp/Vangl2⁺
Genetic Background	involves: A * GFF

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Grhl3^ct mutation (2 available); any Grhl3 mutation (53 available)
Vangl2^Lp mutation (2 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

nervous system

meningomyelocele ( J:125330 )

• mice exhibit meningomyelocele

abnormal astrocyte morphology ( J:125330 )

• at E16.5 and E18.5 mice with meningomyelocele display increased astrocyte staining compared to in wild-type mice

abnormal spinal cord morphology ( J:125330 )

• in mice with meningomyelocele, the dorsal region of the neural tube lays laterally to the ventral horn and the dorsal white matter is moved into a ventrolateral position unlike in wild-type mice

• at E16.5, the number of neuronal cells in the meningomyelocele placode is decreased compared to in wild-type mice

behavior/neurological

abnormal motor capabilities/coordination/movement ( J:125330 )

• mice exhibit meningomyelocele with hypotonic hindlimbs with spontaneous and extensive movement of the hip and knee joints but only slight movement at the ankle joints

• mice do not display coordination between fore- and hindlimbs

• mice occasionally exhibit plantar steps that demonstrate the ability to support their body weight

growth/size/body

decreased fetal weight ( J:125330 )

• mice with meningomyelocele exhibit reduced fetal weight

skeleton

meningomyelocele ( J:125330 )

• mice exhibit meningomyelocele

embryo

meningomyelocele ( J:125330 )

• mice exhibit meningomyelocele

Allelic Composition	Lrp6^skax26/Lrp6⁺ Vangl2^Lp/Vangl2⁺
Genetic Background	involves: A/J * 129S6/SvEvTac * C57BL/6 * C57BL/6J

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Lrp6^skax26 mutation (0 available); any Lrp6 mutation (95 available)
Vangl2^Lp mutation (2 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

hearing/vestibular/ear

abnormal orientation of outer hair cell stereociliary bundles ( J:206979 )

• everely affected in OHC2 and OHC3 layers at all three regions analyzed except for OHC3 in the middle region of the organ of Corti

limbs/digits/tail

curly tail ( J:206979 )

• kinky/looped tail in most mice

kinked tail ( J:206979 )

• kinky/looped tail in most mice

embryo

spina bifida ( J:206979 )

• in some mice unlike single heterozygotes

nervous system

spina bifida ( J:206979 )

• in some mice unlike single heterozygotes

abnormal orientation of outer hair cell stereociliary bundles ( J:206979 )

• everely affected in OHC2 and OHC3 layers at all three regions analyzed except for OHC3 in the middle region of the organ of Corti

Allelic Composition	Scrib^crn2/Scrib⁺ Vangl2^Lp/Vangl2⁺
Genetic Background	involves: A/J * FVB/N

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Scrib^crn2 mutation (0 available); any Scrib mutation (54 available)
Vangl2^Lp mutation (2 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

nervous system

craniorachischisis ( J:174027 )

• show either craniorachischisis or loop tail

embryo

craniorachischisis ( J:174027 )

• show either craniorachischisis or loop tail

limbs/digits/tail

curly tail ( J:174027 )

• show either craniorachischisis or loop tail

Allelic Composition	Scrib^Crc/Scrib⁺ Vangl2⁺/Vangl2^Lp
Genetic Background	involves: BALB/c * C57BL/6 * CBA/Ca * LPT/LeJ * NMRI * SWR

Find Mice

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

nervous system

spina bifida ( J:72608 )

craniorachischisis ( J:72608 )

• 29 of 54 double heterozygotes exhibit craniorachisichisis and the remainder had either spina bifida or a looped tail

limbs/digits/tail

curly tail ( J:72608 )

• in a small minority of double heterozygotes

embryo

spina bifida ( J:72608 )

craniorachischisis ( J:72608 )

• 29 of 54 double heterozygotes exhibit craniorachisichisis and the remainder had either spina bifida or a looped tail

Allelic Composition	Prickle1^tm1Nue/Prickle1⁺ Vangl2^Lp/Vangl2⁺
Genetic Background	involves: C57BL/6J * CBA * LPT/LeJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prickle1^tm1Nue mutation (1 available); any Prickle1 mutation (34 available)
Vangl2^Lp mutation (2 available); any Vangl2 mutation (34 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

embryo

abnormal embryonic epiblast morphology ( J:151993 )

• nuclei in the epiblast are more spherical and randomly aligned along the apical basal axis

• expression analysis indicates that apical-basal cell polarity is disrupted similar to the phenotype of single homozygous Prickle1 mutant mice

Contributing Projects: Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
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