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Article

Possible role of autoantibodies in the pathophysiology of GM2 gangliosidoses

15 Jan 2004

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...

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PMID: 14722612
DOI: 10.1172/jci19639

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