All Phenotypes Tg(Sox10-cre)1Wdr MGI Mouse

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Phenotypes associated with this allele

Allele Symbol
Allele Name
Allele ID

Tg(Sox10-cre)1Wdr
transgene insertion 1, William D Richardson
MGI:3586900

Summary

11 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
cn1	Gjb2^tm1Ugds/Gjb2^tm1Ugds Tg(Sox10-cre)1Wdr/0	involves: 129P2/OlaHsd * C57BL/6 * CBA	MGI:5571190
cn2	Gt(ROSA)26Sor^{tm4(ACTB-tdTomato,-EGFP)Luo}/Gt(ROSA)26Sor⁺ Polr1a^em1Knwea/Polr1a^{tm1c(EUCOMM)Hmgu} Tg(Sox10-cre)1Wdr/0	involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * C57BL/6N * CBA	MGI:7526470
cn3	Gt(ROSA)26Sor^{tm4(ACTB-tdTomato,-EGFP)Luo}/Gt(ROSA)26Sor⁺ Polr1a^{tm1a(EUCOMM)Hmgu}/Polr1a^{tm1c(EUCOMM)Hmgu} Tg(Sox10-cre)1Wdr/0	involves: 129S1/Sv * 129X1/SvJ * C57BL/6N * CBA	MGI:7526465
cn4	Gt(ROSA)26Sor^{tm4(ACTB-tdTomato,-EGFP)Luo}/Gt(ROSA)26Sor⁺ Polr1a^{tm1d(EUCOMM)Hmgu}/Polr1a^{tm1c(EUCOMM)Hmgu} Tg(Sox10-cre)1Wdr/0	involves: 129S1/Sv * 129X1/SvJ * C57BL/6N * CBA	MGI:7526466
cn5	Smarca4^tm1.2Pcn/Smarca4^tm1.2Pcn Tg(Sox10-cre)1Wdr/0	involves: 129S2/SvPas * C57BL/6 * CBA	MGI:6241343
cn6	Gjb2^tm1Ugds/Gjb2^tm1Ugds Tg(Sox10-cre)1Wdr/0	involves: 129S2/SvPasCrl * C57BL/6 * CBA	MGI:5297177
cn7	Bsnd^tm1Tjj/Bsnd^tm1Tjj Tg(Sox10-cre)1Wdr/0	involves: 129/Sv * 129X1/SvJ * C57BL/6	MGI:3826852
cn8	Kmt2d^tm2.1Kaig/Kmt2d^tm2.1Kaig Tg(Sox10-cre)1Wdr/0	involves: C57BL/6 * C57BL/6J * CBA	MGI:7333175
cn9	Zfp276^{tm1c(EUCOMM)Hmgu}/Zfp276^{tm1c(EUCOMM)Hmgu} Tg(Sox10-cre)1Wdr/0	involves: C57BL/6 * C57BL/6N * CBA	MGI:7619747
cn10	Tg(Sox10-cre)1Wdr/0 Twsg1^tm1Aptr/Twsg1^tm1Aptr	involves: C57BL/6 * CBA	MGI:5056120
cn11	Gt(ROSA)26Sor^{tm4(ACTB-tdTomato,-EGFP)Luo}/Gt(ROSA)26Sor⁺ Polr1a^{tm1c(EUCOMM)Hmgu}/Polr1a^{tm1c(EUCOMM)Hmgu} Tg(Sox10-cre)1Wdr/0	Not Specified	MGI:7526467

Allelic Composition	Gjb2^tm1Ugds/Gjb2^tm1Ugds Tg(Sox10-cre)1Wdr/0
Genetic Background	involves: 129P2/OlaHsd * C57BL/6 * CBA

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gjb2^tm1Ugds mutation (3 available); any Gjb2 mutation (22 available)
Tg(Sox10-cre)1Wdr mutation (2 available)

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

hearing/vestibular/ear

cochlear hair cell degeneration ( J:206835 )

• at 1 month of age, hair cells are partly degenerated and by 6 months, hair cells are absent

degeneration of organ of Corti supporting cells ( J:206835 )

• supporting cells in the auditory epithelium are seen in some areas but missing in others

organ of Corti degeneration ( J:206835 )

• cochleae display severe and rapid degeneration of the organ of Corti; degeneration is seen as early as 1 month of age and rapidly progresses from base to apex, such that by 6 months of age the auditory epithelium is flat throughout the cochlea

deafness ( J:206835 )

• mice show ABR threshold shifts at 12, 16, and 24 kHz, indicating substantial hearing loss

nervous system

cochlear hair cell degeneration ( J:206835 )

• at 1 month of age, hair cells are partly degenerated and by 6 months, hair cells are absent

cochlear ganglion degeneration ( J:206835 )

• spiral ganglion neuron degeneration that progresses from base to apex such that by 6 months of age, all cochlear turns show severe degeneration

• mice treated with an adenovirus expressing BDNF show preservation of the spiral ganglion neurons in the base region but does not rescue the organ of Corti degeneration

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
autosomal recessive nonsyndromic deafness 1A		DOID:0110475	OMIM:220290	J:206835

Allelic Composition	Gt(ROSA)26Sor^{tm4(ACTB-tdTomato,-EGFP)Luo}/Gt(ROSA)26Sor⁺ Polr1a^em1Knwea/Polr1a^{tm1c(EUCOMM)Hmgu} Tg(Sox10-cre)1Wdr/0
Genetic Background	involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * C57BL/6N * CBA

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gt(ROSA)26Sor^{tm4(ACTB-tdTomato,-EGFP)Luo} mutation (10 available); any Gt(ROSA)26Sor mutation (993 available)
Polr1a^em1Knwea mutation (0 available); any Polr1a mutation (94 available)
Polr1a^{tm1c(EUCOMM)Hmgu} mutation (0 available); any Polr1a mutation (94 available)
Tg(Sox10-cre)1Wdr mutation (2 available)

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

craniofacial

midline cleft upper lip ( J:335489 )

• median lip notches in some E14-E15 embryos

cleft palate ( J:335489 )

• in some E14-E15 embryos

facial cleft ( J:335489 )

• in some E14-E15 embryos

digestive/alimentary system

cleft palate ( J:335489 )

• in some E14-E15 embryos

growth/size/body

midline cleft upper lip ( J:335489 )

• median lip notches in some E14-E15 embryos

cleft palate ( J:335489 )

• in some E14-E15 embryos

facial cleft ( J:335489 )

• in some E14-E15 embryos

Allelic Composition	Gt(ROSA)26Sor^{tm4(ACTB-tdTomato,-EGFP)Luo}/Gt(ROSA)26Sor⁺ Polr1a^{tm1a(EUCOMM)Hmgu}/Polr1a^{tm1c(EUCOMM)Hmgu} Tg(Sox10-cre)1Wdr/0
Genetic Background	involves: 129S1/Sv * 129X1/SvJ * C57BL/6N * CBA

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gt(ROSA)26Sor^{tm4(ACTB-tdTomato,-EGFP)Luo} mutation (10 available); any Gt(ROSA)26Sor mutation (993 available)
Polr1a^{tm1a(EUCOMM)Hmgu} mutation (1 available); any Polr1a mutation (94 available)
Polr1a^{tm1c(EUCOMM)Hmgu} mutation (0 available); any Polr1a mutation (94 available)
Tg(Sox10-cre)1Wdr mutation (2 available)

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

cardiovascular system

cardiovascular system phenotype ( J:335489 )

N	• normal outflow tract septation in E9.5 embryos

craniofacial

frontal bone hypoplasia ( J:335489 )

• in E16 and E17 embryos

temporal bone squamous part hypoplasia ( J:335489 )

• in E16 and E17 embryos

cleft chin ( J:335489 )

• mandibular cleft in E14-E17 embryos

short mandible ( J:335489 )

• in E17 embryos

nasal bone hypoplasia ( J:335489 )

• in E16 and E17 embryos

cleft palate ( J:335489 )

• in E16 and E17 embryos

facial cleft ( J:335489 )

• in E16 and E17 embryos

digestive/alimentary system

cleft palate ( J:335489 )

• in E16 and E17 embryos

growth/size/body

cleft chin ( J:335489 )

• mandibular cleft in E14-E17 embryos

nasal bone hypoplasia ( J:335489 )

• in E16 and E17 embryos

cleft palate ( J:335489 )

• in E16 and E17 embryos

facial cleft ( J:335489 )

• in E16 and E17 embryos

respiratory system

nasal bone hypoplasia ( J:335489 )

• in E16 and E17 embryos

skeleton

frontal bone hypoplasia ( J:335489 )

• in E16 and E17 embryos

temporal bone squamous part hypoplasia ( J:335489 )

• in E16 and E17 embryos

cleft chin ( J:335489 )

• mandibular cleft in E14-E17 embryos

short mandible ( J:335489 )

• in E17 embryos

nasal bone hypoplasia ( J:335489 )

• in E16 and E17 embryos

Allelic Composition	Gt(ROSA)26Sor^{tm4(ACTB-tdTomato,-EGFP)Luo}/Gt(ROSA)26Sor⁺ Polr1a^{tm1d(EUCOMM)Hmgu}/Polr1a^{tm1c(EUCOMM)Hmgu} Tg(Sox10-cre)1Wdr/0
Genetic Background	involves: 129S1/Sv * 129X1/SvJ * C57BL/6N * CBA

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gt(ROSA)26Sor^{tm4(ACTB-tdTomato,-EGFP)Luo} mutation (10 available); any Gt(ROSA)26Sor mutation (993 available)
Polr1a^{tm1c(EUCOMM)Hmgu} mutation (0 available); any Polr1a mutation (94 available)
Polr1a^{tm1d(EUCOMM)Hmgu} mutation (0 available); any Polr1a mutation (94 available)
Tg(Sox10-cre)1Wdr mutation (2 available)

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

cardiovascular system

cardiovascular system phenotype ( J:335489 )

• normal outflow tract septation in E9.5 embryos

craniofacial

frontal bone hypoplasia ( J:335489 )

• in E16 and E17 embryos

temporal bone squamous part hypoplasia ( J:335489 )

• in E16 and E17 embryos

cleft chin ( J:335489 )

• mandibular cleft in E14-E17 embryos

short mandible ( J:335489 )

• in E17 embryos

nasal bone hypoplasia ( J:335489 )

• in E16 and E17 embryos

cleft palate ( J:335489 )

• in E16 and E17 embryos

facial cleft ( J:335489 )

• in E16 and E17 embryos

digestive/alimentary system

cleft palate ( J:335489 )

• in E16 and E17 embryos

growth/size/body

cleft chin ( J:335489 )

• mandibular cleft in E14-E17 embryos

nasal bone hypoplasia ( J:335489 )

• in E16 and E17 embryos

cleft palate ( J:335489 )

• in E16 and E17 embryos

facial cleft ( J:335489 )

• in E16 and E17 embryos

respiratory system

nasal bone hypoplasia ( J:335489 )

• in E16 and E17 embryos

skeleton

frontal bone hypoplasia ( J:335489 )

• in E16 and E17 embryos

temporal bone squamous part hypoplasia ( J:335489 )

• in E16 and E17 embryos

cleft chin ( J:335489 )

• mandibular cleft in E14-E17 embryos

short mandible ( J:335489 )

• in E17 embryos

nasal bone hypoplasia ( J:335489 )

• in E16 and E17 embryos

Allelic Composition	Smarca4^tm1.2Pcn/Smarca4^tm1.2Pcn Tg(Sox10-cre)1Wdr/0
Genetic Background	involves: 129S2/SvPas * C57BL/6 * CBA

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Smarca4^tm1.2Pcn mutation (1 available); any Smarca4 mutation (110 available)
Tg(Sox10-cre)1Wdr mutation (2 available)

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

embryo

abnormal melanoblast morphology ( J:241206 )

• embryos show a striking reduction in the numbers of cranial and ventral trunk melanoblasts at E12.5

nervous system

abnormal melanoblast morphology ( J:241206 )

• embryos show a striking reduction in the numbers of cranial and ventral trunk melanoblasts at E12.5

Allelic Composition	Gjb2^tm1Ugds/Gjb2^tm1Ugds Tg(Sox10-cre)1Wdr/0
Genetic Background	involves: 129S2/SvPasCrl * C57BL/6 * CBA

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gjb2^tm1Ugds mutation (3 available); any Gjb2 mutation (22 available)
Tg(Sox10-cre)1Wdr mutation (2 available)

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

hearing/vestibular/ear

abnormal hair cell physiology ( J:178055 )

• cochlear cultures fail to propagate calcium signals or release ATP following photostimulation with caged IP3 unlike wild-type cultures

nervous system

abnormal hair cell physiology ( J:178055 )

• cochlear cultures fail to propagate calcium signals or release ATP following photostimulation with caged IP3 unlike wild-type cultures

Allelic Composition	Bsnd^tm1Tjj/Bsnd^tm1Tjj Tg(Sox10-cre)1Wdr/0
Genetic Background	involves: 129/Sv * 129X1/SvJ * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Bsnd^tm1Tjj mutation (0 available); any Bsnd mutation (16 available)
Tg(Sox10-cre)1Wdr mutation (2 available)

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

hearing/vestibular/ear

abnormal vestibuloocular dark reflex ( J:143314 )

• mice exhibit abnormal gain and phase of angular vestibulo ocular reflexes (VOR) in the dark compared to wild-type mice

• however, VORs in the light are normal

cochlear outer hair cell degeneration ( J:143314 )

• after 3 weeks, outer hair cells in the basal turn begin to degenerate

abnormal stria vascularis morphology ( J:143314 )

• as the stria vascularis degenerates it loses the normal interdigitation of marginal and intermediate cells

• however, all cell types are retained

stria vascularis degeneration ( J:143314 )

thin stria vascularis ( J:143314 )

• at 2 weeks, the width of the stria vascularis is reduced by 30% to 50% compared to in wild-type mice

decreased endocochlear potential ( J:143314 )

• between P20 and P30

increased or absent threshold for auditory brainstem response ( J:143314 )

• at 3 weeks, mice exhibit hearing loss of about 60 dB as measured by auditory brainstem response that is less severe than in Slc12a2^tm2Bhk homozygotes

absent distortion product otoacoustic emissions ( J:143314 )

• at 3 to 4 weeks

behavior/neurological

abnormal vestibuloocular dark reflex ( J:143314 )

• mice exhibit abnormal gain and phase of angular vestibulo ocular reflexes (VOR) in the dark compared to wild-type mice

• however, VORs in the light are normal

nervous system

cochlear outer hair cell degeneration ( J:143314 )

• after 3 weeks, outer hair cells in the basal turn begin to degenerate

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Bartter disease type 4a		DOID:0110145	OMIM:602522	J:143314

Allelic Composition	Kmt2d^tm2.1Kaig/Kmt2d^tm2.1Kaig Tg(Sox10-cre)1Wdr/0
Genetic Background	involves: C57BL/6 * C57BL/6J * CBA

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Kmt2d^tm2.1Kaig mutation (0 available); any Kmt2d mutation (169 available)
Tg(Sox10-cre)1Wdr mutation (2 available)

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

craniofacial

abnormal basicranium morphology ( J:294895 )

• cranial base phenotypes are identical to homozygous mice with recombination induced by the H2az2^{Tg(Wnt1-cre)11Rth} allele except in some cases pups do have a small strip of presphenoid bone formation

short frontal bone ( J:294895 )

mandible hypoplasia ( J:294895 )

short nasal bone ( J:294895 )

face hypoplasia ( J:294895 )

• frontonasal hypoplasia

skeleton

abnormal basicranium morphology ( J:294895 )

short frontal bone ( J:294895 )

mandible hypoplasia ( J:294895 )

short nasal bone ( J:294895 )

growth/size/body

short nasal bone ( J:294895 )

face hypoplasia ( J:294895 )

• frontonasal hypoplasia

respiratory system

short nasal bone ( J:294895 )

Allelic Composition	Zfp276^{tm1c(EUCOMM)Hmgu}/Zfp276^{tm1c(EUCOMM)Hmgu} Tg(Sox10-cre)1Wdr/0
Genetic Background	involves: C57BL/6 * C57BL/6N * CBA

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Sox10-cre)1Wdr mutation (2 available)
Zfp276^{tm1c(EUCOMM)Hmgu} mutation (0 available); any Zfp276 mutation (28 available)

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

nervous system

abnormal oligodendrocyte differentiation ( J:324574 )

• mice show a transient delay in oligodendrocytes (OL) maturation and myelination: cells expressing Myrf protein (an OL maturity marker) in transverse spinal cord sections are reduced to 56% of controls at P7, with similar decreases noted in cells expressing myelin genes Mbp and Plp1 and Gstpi protein; at P30 only a slight reduction in Gstpi numbers is still observed, whereas no differences in all other differentiation markers and myelin genes are detected at P30 or P60

• however, overall numbers of Sox10+ oligodendroglial cells (OGCs), Pdgfra+ oligodendrocyte progenitor cells (OPCs), Gfap+ astrocytes, and Iba1+ microglia are not significantly changed in the spinal cord at P1-P60

abnormal myelination ( J:324574 )

• cells expressing myelin genes Mbp and Plp1 and Gstpi protein are significantly reduced in transverse spinal cord sections at P7; however, only a slight reduction in Gstpi numbers is still observed at P30

• analyses of spinal cord white matter regions showed an increased g-ratio and a drastically reduced number of myelinated axons at P7 but not at P30, indicating a transient delay in the myelination of axons in early postnatal stages

dysmyelination ( J:324574 )

• mice show strongly reduced expression of myelin genes in the spinal cord at P7

• in vitro, RNA-Seq analyses of primary OGC cultures derived from P0-P3 forebrains show significant downregulation of myelination-related genes, consistent with the delayed oligodendrocyte differentiation and hypomyelination phenotype seen in vivo

homeostasis/metabolism

decreased lipogenesis ( J:324574 )

• RNA-Seq analyses of primary OGC cultures derived from P0-P3 forebrains show significant downregulation of genes associated with fatty acid, cholesterol and sphingomyelin biosynthesis

Allelic Composition	Tg(Sox10-cre)1Wdr/0 Twsg1^tm1Aptr/Twsg1^tm1Aptr
Genetic Background	involves: C57BL/6 * CBA

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Sox10-cre)1Wdr mutation (2 available)
Twsg1^tm1Aptr mutation (0 available); any Twsg1 mutation (20 available)

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

craniofacial

abnormal frontonasal prominence morphology ( J:173627 )

• mice exhibit midline fusion of the frontonasal prominences compared with wild-type mice

fused first pharyngeal arch ( J:173627 )

small first pharyngeal arch ( J:173627 )

embryo

fused first pharyngeal arch ( J:173627 )

small first pharyngeal arch ( J:173627 )

nervous system

abnormal telencephalon development ( J:173627 )

• mice exhibit non-septation of the telencephalic vesicles compared with wild-type mice

Allelic Composition	Gt(ROSA)26Sor^{tm4(ACTB-tdTomato,-EGFP)Luo}/Gt(ROSA)26Sor⁺ Polr1a^{tm1c(EUCOMM)Hmgu}/Polr1a^{tm1c(EUCOMM)Hmgu} Tg(Sox10-cre)1Wdr/0
Genetic Background	Not Specified

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gt(ROSA)26Sor^{tm4(ACTB-tdTomato,-EGFP)Luo} mutation (10 available); any Gt(ROSA)26Sor mutation (993 available)
Polr1a^{tm1c(EUCOMM)Hmgu} mutation (0 available); any Polr1a mutation (94 available)
Tg(Sox10-cre)1Wdr mutation (2 available)

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

cardiovascular system

cardiovascular system phenotype ( J:335489 )

• normal outflow tract septation in E9.5 embryos

craniofacial

frontal bone hypoplasia ( J:335489 )

• in E16 and E17 embryos

temporal bone squamous part hypoplasia ( J:335489 )

• in E16 and E17 embryos

cleft chin ( J:335489 )

• mandibular cleft in E14-E17 embryos

short mandible ( J:335489 )

• in E17 embryos

nasal bone hypoplasia ( J:335489 )

• in E16 and E17 embryos

cleft palate ( J:335489 )

• in E16 and E17 embryos

facial cleft ( J:335489 )

• in E16 and E17 embryos

digestive/alimentary system

cleft palate ( J:335489 )

• in E16 and E17 embryos

growth/size/body

cleft chin ( J:335489 )

• mandibular cleft in E14-E17 embryos

nasal bone hypoplasia ( J:335489 )

• in E16 and E17 embryos

cleft palate ( J:335489 )

• in E16 and E17 embryos

facial cleft ( J:335489 )

• in E16 and E17 embryos

respiratory system

nasal bone hypoplasia ( J:335489 )

• in E16 and E17 embryos

skeleton

frontal bone hypoplasia ( J:335489 )

• in E16 and E17 embryos

temporal bone squamous part hypoplasia ( J:335489 )

• in E16 and E17 embryos

cleft chin ( J:335489 )

• mandibular cleft in E14-E17 embryos

short mandible ( J:335489 )

• in E17 embryos

nasal bone hypoplasia ( J:335489 )

• in E16 and E17 embryos

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