GM2 Gangliosidoses Model

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Wild Mouse 750
Wild Mouse 750
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huPBMC-NCGhuHSC-NCG-XhuHSC-NCG-hIL15huHSC-NCG
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Wild Mouse 765
Wild Mouse 765

GM2 gangliosidoses, a subset of lysosomal storage disorders, is caused by a deficiency of the glycohydrolase, β-N-acetylhexosaminidase, and includes the closely related Tay–Sachs and Sandhoff diseases. The enzyme deficiency prevents the normal, stepwise degradation of ganglioside, which accumulates unchecked within the cellular lysosome, particularly in neurons.


● Genetically Engineered GM2 gangliosidoses Mouse Model

Strain No.
 Strain Name Strain Type Description
T017692 Hexb-KO Knockout Homozygous Hexb-KO mice show tremor, muscle weakness and an ataxic gait at 15 weeks old, with GM2 ganglioside storage and positive staining of polysaccharide substrate in the brain