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The objective of this grant is to elucidate the regulation, downstream signaling, and structural dynamics of the glucan phosphatase laforin. Mammalian cells store readily-mobilized energy in the form of glycogen, a water-soluble, cytoplasmic carbohydrate/glucan. When certain aspects of glycogen metabolism malfunction, cells develop insoluble, poorly branched, hyperphosphorylated glucans called Lafora bodies (LBs) (1-6). Therefore, mammalian cells both actively synthesize soluble glycogen and inhibit the accumulation of insoluble glucans, i.e. LBs. Humans develop LBs as a consequence of an autosomal recessive neurodegenerative disorder known as Lafora disease.
|Effective start/end date||7/1/10 → 4/30/14|