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Phenotypes Associated with This Genotype
Genotype
MGI:5896534
Allelic
Composition
Nsdhltm1.1Hrm/Y
Tg(GFAP-cre)25Mes/0
Genetic
Background
involves: 129S6/SvEvTac * C57BL/6J * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Nsdhltm1.1Hrm mutation (0 available); any Nsdhl mutation (9 available)
Tg(GFAP-cre)25Mes mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• die between P12-P20

behavior/neurological
• by P8 to P10

nervous system
• fewer, shorter cells that appear disorganized
• thinner with fewer granule cell precursors at P5 and becoming progressively worse with age
• at P8 only a single layer of granule cell precursors is present in severely affected mice
• appears disorganized with fewer hippocampal neurons
• fewer neurons present in the dentate gyrus at P0
• fewer hippocampal neurons at P0 especially in the dentate gyrus
• reduced numbers and organization of granule neurons at P7
• at P7
• increase in pycnotic nuclei in the outer layer
• reduced numbers and organization of granule neurons at P7
• decreased cholesterol levels and increased levels of sterol intermediates in granule cell precursors at P3, P5, and P7
• cultured granule cell precursors produce fewer and thinner neurite extensions
• cell morphology is improved with the addition of cholesterol to the culture medium
• cerebellar Purkinje cells fail to form extensive dendritic trees unlike wild-type controls
• dendritic trees eventually degenerate
• progressively reduced proliferation of granule cell precursors in cerebella starting at P5
• increase in apoptosis of granule cell precursors in cerebella beginning at P7
• cultured granule cell precurors show reduced proliferation that is largely restored with the addition of cholesterol to the culture medium

cellular
• fewer, shorter cells that appear disorganized


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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last database update
03/25/2025
MGI 6.24
The Jackson Laboratory