Complete localization of disulfide bonds
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Christina G. SchΓΌtte; Thorsten Lemm; Konrad Sandhoff; Gereon J. Glombitza
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Article
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2008
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Cold Spring Harbor Laboratory Press
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English
β 628 KB
## Abstract Lysosomal degradation of ganglioside GM2 by hexosaminidase A requires the presence of a small, nonβenzymatic cofactor, the GM2βactivator protein (GM2AP). Lack of functional protein leads to the AB variant of GM2βgangliosidosis, a fatal lysosomal storage disease. Although its possible m