Article

Possible role of autoantibodies in the pathophysiology of GM2 gangliosidoses

2004-01-15

Abstract excerpt

Mice containing a disruption of the Hexb gene have provided a useful model system for the study of the human lysosomal storage disorder known as Sandhoff disease (SD). Hexb -/-mice rapidly develop a progressive neurologic disease of ganglioside GM2 and GA2 storage. Our study revealed that the disease states in this model are associated with the appearance of antiganglioside autoantibodies. Both elevation of serum...

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Literature Corpus work: 1970cc8e-75d2-504d-9c94-484b06058f31
DOI: 10.1172/jci200419639

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