Phenotypes associated with this allele

• Allele Symbol: Seh1l^tm1Lzha
• Allele Name: targeted mutation 1, Liang Zhang
• Allele ID: MGI:6712817
• Summary: 4 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
cn1	Seh1l^tm1Lzha/Seh1l^tm1Lzha Tg(Cspg4-cre/Esr1*)BAkik/0	involves: 129 * C57BL/6	MGI:6712831
cn2	Seh1l^tm1Lzha/Seh1l^tm1Lzha Tg(CAG-cre/Esr1*)5Amc/0	involves: 129 * C57BL/6 * CBA	MGI:6712827
cn3	Seh1l^tm1Lzha/Seh1l^tm1Lzha Tg(Sox10-icre/ERT2)388Wdr/0	involves: 129 * C57BL/6 * CBA	MGI:6712830
cn4	Seh1l^tm1Lzha/Seh1l^tm1Lzha Olig1^tm1(cre)Rth/Olig1^+	involves: 129S4/SvJae * C57BL/6	MGI:6712828

Genotype
MGI:6712831

cn1

• Allelic Composition: Seh1l^tm1Lzha/Seh1l^tm1Lzha; Tg(Cspg4-cre/Esr1*)BAkik/0
• Genetic Background: involves: 129 * C57BL/6

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

cellular

abnormal cell differentiation ( J:276253 )

• mice administered tamoxifen from P26 to P30 show impaired adult oligodendrocyte progenitor cell differentiation, with mice showing reduced mature CC1+ oligodendrocytes at P42

nervous system

abnormal myelination ( J:276253 )

• mice administered tamoxifen and then treated with lysolecithin to induce demyelination show less MBP protein in the corpus callosum, reduced number of CC1+-differentiating oligodendrocytes, and fewer myelinated axons in the lesions indicating decreased remyelination and oligodendrocyte regeneration; this is also seen in spinal cord

Genotype
MGI:6712827

cn2

• Allelic Composition: Seh1l^tm1Lzha/Seh1l^tm1Lzha; Tg(CAG-cre/Esr1*)5Amc/0
• Genetic Background: involves: 129 * C57BL/6 * CBA

nervous system

dysmyelination ( J:276253 )

• tamoxifen-treated mice show a translucent optic nerve indicating a severe deficiency of myelin formation

Genotype
MGI:6712830

cn3

• Allelic Composition: Seh1l^tm1Lzha/Seh1l^tm1Lzha; Tg(Sox10-icre/ERT2)388Wdr/0
• Genetic Background: involves: 129 * C57BL/6 * CBA

nervous system

decreased oligodendrocyte number ( J:276253 )

• mice administered tamoxifen at P3 show a reduction in mature oligodendrocytes at P14

dysmyelination ( J:276253 )

• mice administered tamoxifen at P3 show a reduction in myelination at P14

Genotype
MGI:6712828

cn4

• Allelic Composition: Seh1l^tm1Lzha/Seh1l^tm1Lzha; Olig1^tm1(cre)Rth/Olig1⁺
• Genetic Background: involves: 129S4/SvJae * C57BL/6

mortality/aging

postnatal lethality, complete penetrance ( J:276253 )

• mice die during the third postnatal week

behavior/neurological

tremors ( J:276253 )

• mice develop tremors from postnatal week 2

ataxia ( J:276253 )

• mice develop ataxia from postnatal week 2

seizures ( J:276253 )

• mice develop seizures from postnatal week 2

nervous system

seizures ( J:276253 )

• mice develop seizures from postnatal week 2

abnormal brain development ( J:276253 )

• oligodendrocyte progenitor cells do not differentiate into MBP+, CNP+ oligodendrocytes after tri-iodothyronine induction, indicating oligodendrocyte progenitor cell differentiation defects

decreased oligodendrocyte number ( J:276253 )

• reduction of mature oligodendrocytes

• however, oligodendrocyte progenitor cell markers are detected in the spinal cord and brain at P4, P7, and P14 and oligodendrocyte progenitor cell proliferation rates are normal and markers of mature neurons, astrocytes, and microglia are similar to wild-type

dysmyelination ( J:276253 )

• optic nerve is translucent indicating a deficiency of myelin formation

• myelinated axons are hardly detectable in the optic nerve, corpus callosum, or spinal cord at P14

cellular

abnormal cell differentiation ( J:276253 )

• oligodendrocyte progenitor cells do not differentiate into MBP+, CNP+ oligodendrocytes after tri-iodothyronine induction, indicating oligodendrocyte progenitor cell differentiation defects