Phenotypes associated with this allele

• Allele Symbol: Tg(Msx2-cre)5Rem
• Allele Name: transgene insertion 5, Robert E Maxson
• Allele ID: MGI:2159109
• Summary: 34 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
cn1	Tg(CAG-Bgeo,-Fgf4)1Mrt/? Tg(Msx2-cre)5Rem/?	involves: 129P2/OlaHsd	MGI:3832333
cn2	Fgf4^tm1.2Mrt/Fgf4^tm1.3Mrt Tg(Msx2-cre)5Rem/0	involves: 129P2/OlaHsd	MGI:2176793
cn3	Fgf8^tm1.3Mrt/Fgf8^tm1.4Mrt Tg(CAG-Bgeo,-Fgf4)1Mrt/? Tg(Msx2-cre)5Rem/?	involves: 129P2/OlaHsd	MGI:3832340
cn4	Notch1^tm2Rko/Notch1^tm2Rko Notch3^Gt(PST033)Byg/Notch3^Gt(PST033)Byg Tg(Msx2-cre)5Rem/0	involves: 129P2/OlaHsd * 129X1/SvJ	MGI:3525186
cn5	Notch1^tm2Rko/Notch1^tm2Rko Notch2^tm1Rko/Notch2^tm1Rko Notch3^Gt(PST033)Byg/Notch3^Gt(PST033)Byg Tg(Msx2-cre)5Rem/0	involves: 129P2/OlaHsd * 129X1/SvJ	MGI:3525189
cn6	Mib1^tm2Kong/Mib1^tm2Kong Tg(Msx2-cre)5Rem/0	involves: 129P2/OlaHsd * C57BL/6	MGI:3804816
cn7	Fgf8^tm1.3Mrt/Fgf8^tm1.4Mrt Tg(Msx2-cre)5Rem/0	involves: 129P2/OlaHsd * C57BL/6 * CBA * CD-1 * FVB/N	MGI:2176849
cn8	Wnt3^tm1Amc/Wnt3^tm2Amc Tg(Msx2-cre)5Rem/0	involves: 129S1/Sv * 129S7/SvEvBrd * 129X1/SvJ	MGI:2450906
cn9	Ctnnb1^tm1Max/Ctnnb1^tm2Kem Tg(Msx2-cre)5Rem/0	involves: 129S1/Sv * 129X1/SvJ	MGI:2450904
cn10	Kif3a^tm1Gsn/Kif3a^tm2Gsn Tg(Msx2-cre)5Rem/?	involves: 129S1/Sv * 129X1/SvJ	MGI:3710951
cn11	Porcn^tm1.1Lcm/Y Tg(Msx2-cre)5Rem/0	involves: 129S1/Sv * 129X1/SvJ * C57BL/6	MGI:5049889
cn12	Gt(ROSA)26Sor^tm2(Sp8)Lma/Gt(ROSA)26Sor^+ Sp8^tm1Smb/Sp8^tm1Smb Tg(Msx2-cre)5Rem/0	involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * CBA	MGI:5694217
cn13	Ctnnb1^tm2Kem/Ctnnb1^tm2Kem Gt(ROSA)26Sor^tm1(Fgf8)Lma/Gt(ROSA)26Sor^+ Tg(Msx2-cre)5Rem/0	involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * CBA	MGI:5694212
cn14	Ctnnb1^tm1Mmt/Ctnnb1^tm1Mmt Sp8^tm1Smb/Sp8^tm1Smb Tg(Msx2-cre)5Rem/0	involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * CBA	MGI:5694218
cn15	Ctnnb1^tm1Mmt/Ctnnb1^tm1Mmt Tg(Msx2-cre)5Rem/0	involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * CBA	MGI:5694219
cn16	Ctnnb1^tm2Kem/Ctnnb1^tm2Kem Tg(Msx2-cre)5Rem/0	involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * CBA	MGI:5694213
cn17	Sp8^tm1Smb/Sp8^tm1Smb Tg(Msx2-cre)5Rem/0	involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * CBA	MGI:5694216
cn18	Ofd1^tm2.1Bfra/Y Tg(Msx2-cre)5Rem/0	involves: 129S2/SvPas	MGI:4882104
cn19	Ctnnb1^tm2Kem/Ctnnb1^tm2Kem Rac1^tm1Djk/Rac1^+ Tg(Msx2-cre)5Rem/0	involves: 129S4/SvJae	MGI:3834608
cn20	Psen1^tm2Shn/Psen1^tm2Shn Psen2^tm1Haa/Psen2^+ Tg(Msx2-cre)5Rem/0	involves: 129S4/SvJae	MGI:3525175
cn21	Rac1^tm1Djk/Rac1^tm1.1Djk Tg(Msx2-cre)5Rem/0	involves: 129S4/SvJae	MGI:3834607
cn22	Gt(ROSA)26Sor^tm1(Dkk1)Flng/Gt(ROSA)26Sor^+ Rac1^tm1Djk/Rac1^+ Tg(Msx2-cre)5Rem/0	involves: 129S4/SvJae * 129X1/SvJ	MGI:3834606
cn23	Psen1^tm2Shn/Psen1^tm2Shn Psen2^tm1Haa/Psen2^tm1Haa Tg(Msx2-cre)5Rem/0	involves: 129S4/SvJae * C57BL/6 * CBA	MGI:3525177
cn24	Gt(ROSA)26Sor^tm1(Fgf8)Lma/Gt(ROSA)26Sor^+ Shh^tm2(cre/ERT2)Cjt/Shh^tm2(cre/ERT2)Cjt Tg(Msx2-cre)5Rem/0	involves: 129S6/SvEvTac * C57BL/6 * CBA	MGI:5694215
cn25	Msx1^tm1Rem/Msx1^tm1Rem Tg(Msx2-cre)5Rem/0	involves: 129S6/SvEvTac * C57BL/6 * SJL	MGI:3769673
cn26	Msx2^tm1Rem/Msx2^tm1Rem Tg(Msx2-cre)5Rem/0	involves: 129S6/SvEvTac * C57BL/6 * SJL	MGI:3769674
cn27	Gt(ROSA)26Sor^tm1(Dkk1)Flng/Gt(ROSA)26Sor^tm1(Dkk1)Flng Tg(Msx2-cre)5Rem/0	involves: 129X1/SvJ	MGI:3834605
cn28	Notch1^tm2Rko/Notch1^tm2Rko Notch2^tm1Rko/Notch2^tm1Rko Tg(Msx2-cre)5Rem/0	involves: 129X1/SvJ * C57BL/6 * CBA	MGI:3525181
cn29	Ctnnb1^tm1Mmt/Ctnnb1^tm1Mmt Gt(ROSA)26Sor^tm2(Sp8)Lma/Gt(ROSA)26Sor^+ Tg(Msx2-cre)5Rem/0	involves: 129X1/SvJ * C57BL/6 * CBA	MGI:5694224
cn30	Gt(ROSA)26Sor^tm1(Fgf8)Lma/Gt(ROSA)26Sor^+ Tg(Msx2-cre)5Rem/0	involves: C57BL/6 * CBA	MGI:5694210
cn31	Gt(ROSA)26Sor^tm2(Sp8)Lma/Gt(ROSA)26Sor^+ Tg(Msx2-cre)5Rem/0	involves: C57BL/6 * CBA	MGI:5694223
cn32	Wls^tm1Xzg/Wls^tm1Xzg Tg(Msx2-cre)5Rem/0	Not Specified	MGI:5426937
cn33	Notch2^tm1Rko/Notch2^tm1Rko Tg(Msx2-cre)5Rem/0	Not Specified	MGI:3525174
cn34	Notch1^tm2Rko/Notch1^tm2Rko Tg(Msx2-cre)5Rem/0	Not Specified	MGI:3525169

Genotype
MGI:3832333

cn1

• Allelic Composition: Tg(CAG-Bgeo,-Fgf4)1Mrt/?; Tg(Msx2-cre)5Rem/?
• Genetic Background: involves: 129P2/OlaHsd

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

growth/size/body

postnatal growth retardation ( J:104411 )

• slower growth rate

limbs/digits/tail

polysyndactyly ( J:104411 )

• additional posterior digit composed of partial or complete metacarpal/metatarsal and 1-2 phalanges

• metacarpal/metatarsal usually fused with equivalent bones of digit V

• additional phalange-like elements between digit V and supernumerary digit in rare cases

• half of hind limbs with a thin digit-like structure ventral to digit I, contains metatarsal and 1-2 phalanges

• distal phalange of extra ventral digit sometimes attached to the distal phalange of digit I

• absence of interdigital programmed cell death

abnormal calcaneum morphology ( J:104411 )

• proximal part of the calcaneus is significantly enlarged

fused tarsal bones ( J:104411 )

• occasionally

abnormal limb development ( J:104411 )

• stylopod and zeugopod are normal

broad limb buds ( J:104411 )

• slightly larger than controls at E11.5

abnormal digit development ( J:104411 )

• supernumerary digit on the posterior side of autopods

• earliest indications at E12.5

vision/eye

eyelid hyperplasia ( J:104411 )

• hyperplasia of the eyelids

reproductive system

abnormal reproductive system morphology ( J:104411 )

• malformation of the external genitalia

embryo

broad limb buds ( J:104411 )

• slightly larger than controls at E11.5

skeleton

abnormal calcaneum morphology ( J:104411 )

• proximal part of the calcaneus is significantly enlarged

fused tarsal bones ( J:104411 )

• occasionally

Genotype
MGI:2176793

cn2

• Allelic Composition: Fgf4^tm1.2Mrt/Fgf4^tm1.3Mrt; Tg(Msx2-cre)5Rem/0
• Genetic Background: involves: 129P2/OlaHsd

normal phenotype

no abnormal phenotype detected ( J:61886 )

Genotype
MGI:3832340

cn3

• Allelic Composition: Fgf8^tm1.3Mrt/Fgf8^tm1.4Mrt; Tg(CAG-Bgeo,-Fgf4)1Mrt/?; Tg(Msx2-cre)5Rem/?
• Genetic Background: involves: 129P2/OlaHsd

limbs/digits/tail

limbs/digits/tail phenotype ( J:104411 )

N • reversal of abnormalities resulting from loss of Fgf8 alone

N • deltoid tuberosity is normal

polysyndactyly ( J:104411 )

• phenotype due to conditional over expression of Fgf4 retained

fused tarsal bones ( J:104411 )

abnormal limb development ( J:104411 )

abnormal limb bud morphology ( J:104411 )

abnormal digit development ( J:104411 )

growth/size/body

postnatal growth retardation ( J:104411 )

vision/eye

abnormal eyelid morphology ( J:104411 )

reproductive system

abnormal reproductive system morphology ( J:104411 )

embryo

abnormal limb bud morphology ( J:104411 )

skeleton

fused tarsal bones ( J:104411 )

Genotype
MGI:3525186

cn4

• Allelic Composition: Notch1^tm2Rko/Notch1^tm2Rko; Notch3^{Gt(PST033)Byg}/Notch3^{Gt(PST033)Byg}; Tg(Msx2-cre)5Rem/0
• Genetic Background: involves: 129P2/OlaHsd * 129X1/SvJ

integument

abnormal hair follicle morphology ( J:94517 )

• displayed similar follicular morphologies as mutant mice homozygous for Notch1^tm1Shn expressing the Tg(Msx2-cre)5Rem transgene

Genotype
MGI:3525189

cn5

• Allelic Composition: Notch1^tm2Rko/Notch1^tm2Rko; Notch2^tm1Rko/Notch2^tm1Rko; Notch3^{Gt(PST033)Byg}/Notch3^{Gt(PST033)Byg}; Tg(Msx2-cre)5Rem/0
• Genetic Background: involves: 129P2/OlaHsd * 129X1/SvJ

endocrine/exocrine glands

absent sebaceous gland ( J:94517 )

• loss of sebaceous glands in embryo-deleted follicles

integument

absent sebaceous gland ( J:94517 )

• loss of sebaceous glands in embryo-deleted follicles

thick epidermis ( J:94517 )

• had frequent acanthotic and perakeratotic scales

scaly skin ( J:94517 )

• had frequent perakeratotic scales

Genotype
MGI:3804816

cn6

• Allelic Composition: Mib1^tm2Kong/Mib1^tm2Kong; Tg(Msx2-cre)5Rem/0
• Genetic Background: involves: 129P2/OlaHsd * C57BL/6

limbs/digits/tail

syndactyly ( J:130369 )

fused phalanges ( J:130369 )

• digits 2 through 4 are fused in adulthood

• mice exhibit fusion of soft tissues and nails of digits 2 and 3

abnormal digit development ( J:130369 )

• at E13.5, the interdigit space between digits 2 and 3 is narrower than in wild-type mice

abnormal interdigital cell death ( J:130369 )

• at E15.5, interdigit separation is absent due to a decrease in interdigital cell death

growth/size/body

epidermal cyst ( J:130369 )

• at P28, mice exhibit epidermal cysts below the interfollicular epidermis that are filled with cornified materials

decreased body size ( J:130369 )

skeleton

fused phalanges ( J:130369 )

• digits 2 through 4 are fused in adulthood

• mice exhibit fusion of soft tissues and nails of digits 2 and 3

integument

hairless ( J:130369 )

• mice are bald at 4 weeks of age

premature hair loss ( J:130369 )

• mice begin to lose their hair along the dorsal midline and bald at 4 weeks of age

decreased hair follicle number ( J:130369 )

• at 28 days, hair follicles are barely detected in the back skin

abnormal epidermal layer morphology ( J:130369 )

• at P28, the epidermal cell layer has numerous squame

epidermal cyst ( J:130369 )

• at P28, mice exhibit epidermal cysts below the interfollicular epidermis that are filled with cornified materials

thick epidermis ( J:130369 )

• at P28

cellular

abnormal interdigital cell death ( J:130369 )

• at E15.5, interdigit separation is absent due to a decrease in interdigital cell death

Genotype
MGI:2176849

cn7

• Allelic Composition: Fgf8^tm1.3Mrt/Fgf8^tm1.4Mrt; Tg(Msx2-cre)5Rem/0
• Genetic Background: involves: 129P2/OlaHsd * C57BL/6 * CBA * CD-1 * FVB/N

limbs/digits/tail

oligodactyly ( J:104411 )

• forelimbs missing digit II or III

• hindlimb missing digit I

• forelimb digits I and V missing phalanges

• hindlimb digits II and V missing phalanges

abnormal forelimb stylopod morphology ( J:104411 )

• mildly hypoplastic

absent deltoid tuberosity ( J:104411 )

• deltoid tuberosity missing

abnormal forelimb zeugopod morphology ( J:66266 , J:104411 )

• zeugopod elements are mildly hypoplastic (J:66266)

absent radius ( J:66266 )

• radius is always absent in mutants

abnormal hindlimb stylopod morphology ( J:66266 , J:104411 )

• stylopod is severely reduced in hindlimbs (11/19 bilaterally, 4/19 unilaterally) but only mildly affected in forelimbs (J:66266)

abnormal hindlimb zeugopod morphology ( J:66266 , J:104411 )

• zeugopod elements are mildly hypoplastic (J:66266)

abnormal limb development ( J:104411 )

small limb buds ( J:66266 )

• limb buds are reduced in size detected at ~E10.25

abnormal digit development ( J:66266 )

• digit I is missing in hindlimbs (13/19 bilaterally, 5/19 unilaterally) and digit II or III is missing in the forelimbs (12/19 bilaterally, 5/19 unilaterally)

skeleton

absent deltoid tuberosity ( J:104411 )

• deltoid tuberosity missing

absent radius ( J:66266 )

• radius is always absent in mutants

embryo

small limb buds ( J:66266 )

• limb buds are reduced in size detected at ~E10.25

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
tetralogy of Fallot		DOID:6419	OMIM:187500	J:66266

Genotype
MGI:2450906

cn8

• Allelic Composition: Wnt3^tm1Amc/Wnt3^tm2Amc; Tg(Msx2-cre)5Rem/0
• Genetic Background: involves: 129S1/Sv * 129S7/SvEvBrd * 129X1/SvJ

limbs/digits/tail

abnormal apical ectodermal ridge morphology ( J:81795 )

• mutants display variable AER disruption in the distal margin of the hindlimb consistent with a variable hindlimb phenotype

thin apical ectodermal ridge ( J:81795 )

• at E10.5, the dorsoventral girth of the AER in the distal margin of the hindlimb is reduced to ~50%

• at E10.5, the dorsoventral girth of the forelimb AER is also reduced; however, the anterior/posterior length of the AER is generally unaffected

abnormal autopod morphology ( J:81795 )

• 22 of 44 mutant mice exhibit mild to severe autopod defects

abnormal forelimb morphology ( J:81795 )

• mutant mice display normal forelimbs except in two cases (2/44) where digit 5 was absent or fused to digit 4

abnormal hindlimb morphology ( J:81795 )

• mutant mice display hindlimb defects of variable penetrance and expressivity, ranging from completely normal to entirely absent hindlimbs

absent hindlimb ( J:81795 )

• 3 of 44 mutant mice show complete absence of hindlimbs

short hindlimb ( J:81795 )

• 17 of 44 mutant mice show extensive truncations that extend into more proximal segments of the hindlimb

embryo

increased ectoderm apoptosis ( J:81795 )

• mutants display extensive apoptosis throughout the hindlimb mesenchyme and adjacent ectoderm at the 35-42 somite stage; elevated apoptosis in the mesenchyme is more significant dorsally

abnormal apical ectodermal ridge morphology ( J:81795 )

• mutants display variable AER disruption in the distal margin of the hindlimb consistent with a variable hindlimb phenotype

thin apical ectodermal ridge ( J:81795 )

• at E10.5, the dorsoventral girth of the AER in the distal margin of the hindlimb is reduced to ~50%

• at E10.5, the dorsoventral girth of the forelimb AER is also reduced; however, the anterior/posterior length of the AER is generally unaffected

cellular

increased ectoderm apoptosis ( J:81795 )

• mutants display extensive apoptosis throughout the hindlimb mesenchyme and adjacent ectoderm at the 35-42 somite stage; elevated apoptosis in the mesenchyme is more significant dorsally

Genotype
MGI:2450904

cn9

• Allelic Composition: Ctnnb1^tm1Max/Ctnnb1^tm2Kem; Tg(Msx2-cre)5Rem/0
• Genetic Background: involves: 129S1/Sv * 129X1/SvJ

mortality/aging

neonatal lethality, complete penetrance ( J:81795 )

• mutant mice are live-born but die within 24 hrs of birth

limbs/digits/tail

abnormal apical ectodermal ridge morphology ( J:81795 )

• AER formation fails to initiate in the mutant hindlimbs whereas in the forelimbs, AER is properly initiated at 20 somites but eventually disappears in the anterior and posterior extremes by ~38 somites

abnormal forelimb morphology ( J:81795 )

• mutant forelimbs are present but truncated at the level of the humerus or ulna

abnormal humerus morphology ( J:81795 )

absent deltoid tuberosity ( J:81795 )

• in newborn mutants, the humerus appears largely unaffected but shows absence of the deltoid crest

abnormal ulna morphology ( J:81795 )

• in mutant newborns, the proximal ulna is present to variable extents

absent hindlimb ( J:81795 )

• all mutant pups completely lack hindlimbs

skeleton

abnormal humerus morphology ( J:81795 )

absent deltoid tuberosity ( J:81795 )

• in newborn mutants, the humerus appears largely unaffected but shows absence of the deltoid crest

abnormal ulna morphology ( J:81795 )

• in mutant newborns, the proximal ulna is present to variable extents

embryo

increased ectoderm apoptosis ( J:81795 )

• in mutant hindlimbs, where an AER never forms, extensive apoptosis occurs throughout the hindlimb mesenchyme and adjacent ectoderm at the 35-42 somite stage; elevated apoptosis in the mesenchyme is more significant dorsally

• in the forelimbs, however, where the AER disappears later in development, apoptosis is restricted to the distal mesenchyme and ectoderm

• no significant reduction of cell proliferation is observed in the mutant mesenchyme or ectoderm

abnormal apical ectodermal ridge morphology ( J:81795 )

• AER formation fails to initiate in the mutant hindlimbs whereas in the forelimbs, AER is properly initiated at 20 somites but eventually disappears in the anterior and posterior extremes by ~38 somites

behavior/neurological

abnormal suckling behavior ( J:81795 )

• newborn mutant mice fail to nurse

cellular

increased ectoderm apoptosis ( J:81795 )

• in mutant hindlimbs, where an AER never forms, extensive apoptosis occurs throughout the hindlimb mesenchyme and adjacent ectoderm at the 35-42 somite stage; elevated apoptosis in the mesenchyme is more significant dorsally

• in the forelimbs, however, where the AER disappears later in development, apoptosis is restricted to the distal mesenchyme and ectoderm

• no significant reduction of cell proliferation is observed in the mutant mesenchyme or ectoderm

Genotype
MGI:3710951

cn10

• Allelic Composition: Kif3a^tm1Gsn/Kif3a^tm2Gsn; Tg(Msx2-cre)5Rem/?
• Genetic Background: involves: 129S1/Sv * 129X1/SvJ

limbs/digits/tail

abnormal limb bud morphology ( J:117033 )

• results are identical to those seen in Ift88^tm1.1Bky/ Ift88^tm1Bky Tg(Msx2-cre)5Rem mice

embryo

abnormal limb bud morphology ( J:117033 )

• results are identical to those seen in Ift88^tm1.1Bky/ Ift88^tm1Bky Tg(Msx2-cre)5Rem mice

Genotype
MGI:5049889

cn11

• Allelic Composition: Porcn^tm1.1Lcm/Y; Tg(Msx2-cre)5Rem/0
• Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6

Tissue-specific Porcn^tm1.1Lcm deletion phenotypes include skeletal defects and skin abnormalities

limbs/digits/tail

decreased autopod size ( J:173672 )

♂ • truncated

syndactyly ( J:173672 )

♂

integument

abnormal skin morphology ( J:173672 )

♂ • at E18.5, mice exhibit focal and dermis defects compared with wild-type mice

abnormal dermal layer morphology ( J:173672 )

♂ • at E18.5

Genotype
MGI:5694217

cn12

• Allelic Composition: Gt(ROSA)26Sor^tm2(Sp8)Lma/Gt(ROSA)26Sor⁺; Sp8^tm1Smb/Sp8^tm1Smb; Tg(Msx2-cre)5Rem/0
• Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * CBA

limbs/digits/tail

abnormal limb morphology ( J:223057 )

abnormal autopod morphology ( J:223057 )

Genotype
MGI:5694212

cn13

• Allelic Composition: Ctnnb1^tm2Kem/Ctnnb1^tm2Kem; Gt(ROSA)26Sor^tm1(Fgf8)Lma/Gt(ROSA)26Sor⁺; Tg(Msx2-cre)5Rem/0
• Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * CBA

limbs/digits/tail

limbs/digits/tail phenotype ( J:223057 )

N • unlike in mice lacking Fgf8 over-expression, mice develop normal humeri with deltoid tuberosity and the radius is evident

abnormal autopod morphology ( J:223057 )

• autopod rudiments

abnormal ulna morphology ( J:223057 )

• the ulna is longer and thicker

abnormal hindlimb morphology ( J:223057 )

• near normal pelvic girdles and femurs with one or two ectopic cartilages

skeleton

abnormal ulna morphology ( J:223057 )

• the ulna is longer and thicker

Genotype
MGI:5694218

cn14

• Allelic Composition: Ctnnb1^tm1Mmt/Ctnnb1^tm1Mmt; Sp8^tm1Smb/Sp8^tm1Smb; Tg(Msx2-cre)5Rem/0
• Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * CBA

limbs/digits/tail

limbs/digits/tail phenotype ( J:223057 )

N • polysyndactyly and ectopic limb phenotypes are rescued

Genotype
MGI:5694219

cn15

• Allelic Composition: Ctnnb1^tm1Mmt/Ctnnb1^tm1Mmt; Tg(Msx2-cre)5Rem/0
• Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * CBA

limbs/digits/tail

polydactyly ( J:223057 )

ectopic limbs ( J:223057 )

• some mice exhibit ectopic limbs in the flank ectoderm and ventral ectoderm

Genotype
MGI:5694213

cn16

• Allelic Composition: Ctnnb1^tm2Kem/Ctnnb1^tm2Kem; Tg(Msx2-cre)5Rem/0
• Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * CBA

limbs/digits/tail

abnormal autopod morphology ( J:223057 )

• absent autopod elements

absent deltoid tuberosity ( J:223057 )

decreased diameter of humerus ( J:223057 )

• thinner than in controls

absent radius ( J:223057 )

short ulna ( J:223057 )

• two-thirds of the ulna is missing

absent hindlimb ( J:223057 )

• largely absent with the exception of the small remnant of the pelvic girdle

skeleton

absent deltoid tuberosity ( J:223057 )

decreased diameter of humerus ( J:223057 )

• thinner than in controls

absent radius ( J:223057 )

short ulna ( J:223057 )

• two-thirds of the ulna is missing

Genotype
MGI:5694216

cn17

• Allelic Composition: Sp8^tm1Smb/Sp8^tm1Smb; Tg(Msx2-cre)5Rem/0
• Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * CBA

limbs/digits/tail

abnormal limb morphology ( J:223057 )

• loss of distal structures

• however, stylopod and zeugopod develop normally in the forelimbs

oligodactyly ( J:223057 )

• only one abnormal digit forms

absent fibula ( J:223057 )

• missing or severely truncated

short fibula ( J:223057 )

• missing or severely truncated

absent tibia ( J:223057 )

• missing or severely truncated

short tibia ( J:223057 )

• missing or severely truncated

skeleton

absent fibula ( J:223057 )

• missing or severely truncated

short fibula ( J:223057 )

• missing or severely truncated

absent tibia ( J:223057 )

• missing or severely truncated

short tibia ( J:223057 )

• missing or severely truncated

Genotype
MGI:4882104

cn18

• Allelic Composition: Ofd1^tm2.1Bfra/Y; Tg(Msx2-cre)5Rem/0
• Genetic Background: involves: 129S2/SvPas

limbs/digits/tail

limbs/digits/tail phenotype ( J:168038 )

♂N • mice exhibit normal limbs

Genotype
MGI:3834608

cn19

• Allelic Composition: Ctnnb1^tm2Kem/Ctnnb1^tm2Kem; Rac1^tm1Djk/Rac1⁺; Tg(Msx2-cre)5Rem/0
• Genetic Background: involves: 129S4/SvJae

limbs/digits/tail

abnormal forelimb morphology ( J:145305 )

• most forelimbs lack structures distal to the scapula

absent hindlimb ( J:145305 )

Genotype
MGI:3525175

cn20

• Allelic Composition: Psen1^tm2Shn/Psen1^tm2Shn; Psen2^tm1Haa/Psen2⁺; Tg(Msx2-cre)5Rem/0
• Genetic Background: involves: 129S4/SvJae

integument

abnormal hair follicle morphology ( J:94517 )

• showed a transient phenotype in embryo-deleted skin but developed a normal coat by P22 with a few abnormal looking follicles

Genotype
MGI:3834607

cn21

• Allelic Composition: Rac1^tm1Djk/Rac1^tm1.1Djk; Tg(Msx2-cre)5Rem/0
• Genetic Background: involves: 129S4/SvJae

limbs/digits/tail

short forelimb ( J:145305 )

• partial forelimb truncation

absent hindlimb ( J:145305 )

Genotype
MGI:3834606

cn22

• Allelic Composition: Gt(ROSA)26Sor^{tm1(Dkk1)Flng}/Gt(ROSA)26Sor⁺; Rac1^tm1Djk/Rac1⁺; Tg(Msx2-cre)5Rem/0
• Genetic Background: involves: 129S4/SvJae * 129X1/SvJ

limbs/digits/tail

short forelimb ( J:145305 )

• severe forelimb truncation

absent hindlimb ( J:145305 )

Genotype
MGI:3525177

cn23

• Allelic Composition: Psen1^tm2Shn/Psen1^tm2Shn; Psen2^tm1Haa/Psen2^tm1Haa; Tg(Msx2-cre)5Rem/0
• Genetic Background: involves: 129S4/SvJae * C57BL/6 * CBA

mortality/aging

premature death ( J:94517 )

• died after weaning, with the longest survivor dying at P30

digestive/alimentary system

esophagus hyperplasia ( J:94517 )

• hyperplasia of the esophagus most likely leading to premature death

endocrine/exocrine glands

absent sebocyte ( J:94517 )

• did not detect mature sebocytes in embryo-deleted hair follicles

growth/size/body

esophagus hyperplasia ( J:94517 )

• hyperplasia of the esophagus most likely leading to premature death

epidermal cyst ( J:94517 )

• by P22, keratinized cysts replaced embryo-deleted hair follicles

decreased body size ( J:94517 )

• smaller than controls by P12

integument

absent sebocyte ( J:94517 )

• did not detect mature sebocytes in embryo-deleted hair follicles

epidermal cyst ( J:94517 )

• by P22, keratinized cysts replaced embryo-deleted hair follicles

abnormal hair growth ( J:94517 )

• mutants had regions with normal hair and naked skin patches that were separated by regions covered with short hairs presumably the result of different timing of Cre expression

focal hair loss ( J:94517 )

• had naked skin patches

short hair ( J:94517 )

• had regions of short hairs

abnormal hair follicle morphology ( J:94517 )

• an epithelial cluster formed an unusual flat boundary with the dermal papilla in P0 embryo-deleted mutant follicles

• at P4, the upper part of embryo-deleted follicles contained loosely packed cells with enlarged cytoplasm and small nuclei and at P8, these loosely packed cells extended farther down to the matrix

abnormal hair follicle inner root sheath morphology ( J:94517 )

• inner root sheath cells fail to accumulate by P7 but the outer root sheath was normal at P8

hair follicle degeneration ( J:94517 )

• all cell layers of embryo-deleted follicles except the outer root sheath and the Dermal Papilla appeared to have collapsed around the melanin-containing core at P8

• At P12 and P15, degenerating embryo-deleted follicles lost contact with their Dermal Papilla and the outer root sheath began to proliferate, stratify, and keratinize

epidermal hyperplasia ( J:94517 )

• exhibited epidermal hyperproliferation

thick epidermis ( J:94517 )

• embryo-deleted epidermis at P8 was acanthotic and hyperkeratotic

scaly skin ( J:94517 )

• naked skin patches became scaly

thick skin ( J:94517 )

• naked skin patches became thick

Genotype
MGI:5694215

cn24

• Allelic Composition: Gt(ROSA)26Sor^tm1(Fgf8)Lma/Gt(ROSA)26Sor⁺; Shh^{tm2(cre/ERT2)Cjt}/Shh^{tm2(cre/ERT2)Cjt}; Tg(Msx2-cre)5Rem/0
• Genetic Background: involves: 129S6/SvEvTac * C57BL/6 * CBA

limbs/digits/tail

abnormal limb development ( J:223057 )

• as in Gt(ROSA)26Sor^tm1Lma/Gt(ROSA)26Sor⁺ Shh^{tm2(cre/ERT2)Cjt}/Shh^{tm2(cre/ERT2)Cjt} mice

Genotype
MGI:3769673

cn25

• Allelic Composition: Msx1^tm1Rem/Msx1^tm1Rem; Tg(Msx2-cre)5Rem/0
• Genetic Background: involves: 129S6/SvEvTac * C57BL/6 * SJL

craniofacial

cleft palate ( J:128624 )

digestive/alimentary system

cleft palate ( J:128624 )

growth/size/body

cleft palate ( J:128624 )

Genotype
MGI:3769674

cn26

• Allelic Composition: Msx2^tm1Rem/Msx2^tm1Rem; Tg(Msx2-cre)5Rem/0
• Genetic Background: involves: 129S6/SvEvTac * C57BL/6 * SJL

craniofacial

abnormal neurocranium morphology ( J:128624 )

skeleton

abnormal neurocranium morphology ( J:128624 )

integument

alopecia ( J:128624 )

Genotype
MGI:3834605

cn27

• Allelic Composition: Gt(ROSA)26Sor^{tm1(Dkk1)Flng}/Gt(ROSA)26Sor^{tm1(Dkk1)Flng}; Tg(Msx2-cre)5Rem/0
• Genetic Background: involves: 129X1/SvJ

limbs/digits/tail

short forelimb ( J:145305 )

• partial forelimb truncation

absent hindlimb ( J:145305 )

Genotype
MGI:3525181

cn28

• Allelic Composition: Notch1^tm2Rko/Notch1^tm2Rko; Notch2^tm1Rko/Notch2^tm1Rko; Tg(Msx2-cre)5Rem/0
• Genetic Background: involves: 129X1/SvJ * C57BL/6 * CBA

mortality/aging

premature death ( J:94517 )

• die around P25

endocrine/exocrine glands

absent sebaceous gland ( J:94517 )

• loss of sebaceous glands in embryo-deleted follicles

integument

absent sebaceous gland ( J:94517 )

• loss of sebaceous glands in embryo-deleted follicles

progressive hair loss ( J:94517 )

• regions of bald skin (where Cre was expressed in E9.5 developing follicles) alternating with hair (where Cre was expressed at P1 in follicle cells), however this hair was progressively lost as mice age

absent hair follicle inner root sheath ( J:94517 )

• embryo-deleted follicles contain a melanin-producing core with large, loosely packed cells replacing the inner root sheath

hair follicle degeneration ( J:94517 )

• by P25, P1-deleted follicles are degenerating instead of being in telogen

epidermal hyperplasia ( J:94517 )

scaly skin ( J:94517 )

• regions of scaly skin

Genotype
MGI:5694224

cn29

• Allelic Composition: Ctnnb1^tm1Mmt/Ctnnb1^tm1Mmt; Gt(ROSA)26Sor^tm2(Sp8)Lma/Gt(ROSA)26Sor⁺; Tg(Msx2-cre)5Rem/0
• Genetic Background: involves: 129X1/SvJ * C57BL/6 * CBA

limbs/digits/tail

limbs/digits/tail phenotype ( J:223057 )

N • mice exhibit normal appendage development

Genotype
MGI:5694210

cn30

• Allelic Composition: Gt(ROSA)26Sor^tm1(Fgf8)Lma/Gt(ROSA)26Sor⁺; Tg(Msx2-cre)5Rem/0
• Genetic Background: involves: C57BL/6 * CBA

limbs/digits/tail

polydactyly ( J:223057 )

abnormal calcaneum morphology ( J:223057 )

• enlarged

abnormal limb development ( J:223057 )

• excessive limb growth

skeleton

abnormal calcaneum morphology ( J:223057 )

• enlarged

abnormal skeleton development ( J:223057 )

• ectopic skeletal elements in the fore- and hindlimbs

Genotype
MGI:5694223

cn31

• Allelic Composition: Gt(ROSA)26Sor^tm2(Sp8)Lma/Gt(ROSA)26Sor⁺; Tg(Msx2-cre)5Rem/0
• Genetic Background: involves: C57BL/6 * CBA

limbs/digits/tail

limbs/digits/tail phenotype ( J:223057 )

N • mice exhibit normal appendage development

Genotype
MGI:5426937

cn32

• Allelic Composition: Wls^tm1Xzg/Wls^tm1Xzg; Tg(Msx2-cre)5Rem/0
• Genetic Background: Not Specified

limbs/digits/tail

decreased autopod size ( J:184918 )

• truncated

oligodactyly ( J:184918 )

abnormal forelimb morphology ( J:184918 )

• dorsally flexed

abnormal forelimb stylopod morphology ( J:184918 )

• contains reduced and malformed muscle mass at E17.5

abnormal hindlimb morphology ( J:184918 )

• dorsally flexed

abnormal hindlimb zeugopod morphology ( J:184918 )

• shortened

short fibula ( J:184918 )

short tibia ( J:184918 )

abnormal limb development ( J:184918 )

• impaired patterning; more severe in the ventral limb than in dorsal limb

• at E10.5, mesenchymal cells exhibit decreased proliferation while apoptosis is increased in the distal limb mesenchyme compared with wild-type cells

• mice exhibit dysgenesis of limb soft tissue with progressive defects from proximal to distal compared with wild-type mice

abnormal apical ectodermal ridge morphology ( J:184918 )

• impaired development

thin apical ectodermal ridge ( J:184918 )

• thin and narrow at E10.5

short limbs ( J:184918 )

• more severe in hindlimbs than forelimbs

embryo

abnormal apical ectodermal ridge morphology ( J:184918 )

• impaired development

thin apical ectodermal ridge ( J:184918 )

• thin and narrow at E10.5

cellular

increased apoptosis ( J:184918 )

• of distal limb mesenchyme cells at E12.5

decreased cell proliferation ( J:184918 )

• of limb mesenchyme cells at E12.5

skeleton

short fibula ( J:184918 )

short tibia ( J:184918 )

abnormal tendon morphology ( J:184918 )

• in the forelimbs at E17.5

abnormal ligament morphology ( J:184918 )

• in the forelimbs at E17.5

delayed cranial suture closure ( J:184918 )

integument

abnormal hair follicle development ( J:184918 )

• limb hair follicle formation is blocked

abnormal dermal layer morphology ( J:184918 )

• limb dermis differentiation is blocked

muscle

abnormal muscle development ( J:184918 )

• in the forelimbs at E17.5

• impaired in the autopod

abnormal tendon morphology ( J:184918 )

• in the forelimbs at E17.5

Genotype
MGI:3525174

cn33

• Allelic Composition: Notch2^tm1Rko/Notch2^tm1Rko; Tg(Msx2-cre)5Rem/0
• Genetic Background: Not Specified

normal phenotype

no abnormal phenotype detected ( J:94517 )

• indistinguishable from wild-type

Genotype
MGI:3525169

cn34

• Allelic Composition: Notch1^tm2Rko/Notch1^tm2Rko; Tg(Msx2-cre)5Rem/0
• Genetic Background: Not Specified

endocrine/exocrine glands

small sebaceous gland ( J:94517 )

• severely reduced sebaceous glands in embryo-deleted skin

integument

small sebaceous gland ( J:94517 )

• severely reduced sebaceous glands in embryo-deleted skin

abnormal hair growth ( J:94517 )

• mosaic pattern of hair growth (regions of finer, shorter, and wavy hairs that showed twisted, knotted morphology resulting from Cre expression at E9.5 and regions of normal hairs resulting from Cre expression at P1) that was maintained throughout life

short hair ( J:94517 )

waved hair ( J:94517 )

• regions of wavy hair that have a twisted, knotted morphology

abnormal hair shaft morphology ( J:94517 )

• bulbous perturbations in the shaft

abnormal hair follicle morphology ( J:94517 )

• medulla layer of embryo-deleted follicles was disorganized, with two smaller cells, instead of one, packed inside the medulla with fewer, disorganized trichohyalin granules

disorganized inner root sheath cells ( J:94517 )

• the inner root sheath Henle's layer of embryo-deleted follicles is improperly organized with up to four cell tips in the mutant hair instead of two

• however, the cortex is normal