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Altered diffusion tensor imaging measurements in aged transgenic Huntington disease rats.

Antonsen, BT; Jiang, Y; Johnson, G Allan; Leergaard, TB; Nguyen, HP; Qu, H; Sijbers, J; ... (9 authors) (Brain Struct Funct, 2013-05)

Rodent models of Huntington disease (HD) are valuable tools for investigating HD pathophysiology and evaluating new therapeutic approaches. Non-invasive characterization of HD-related phenotype changes is important for monitoring ...

Stbd1 is highly elevated in skeletal muscle of Pompe disease mice but suppression of its expression does not affect lysosomal glycogen accumulation.

Das, S; Fredrickson, Keri B; Kishnani, Priya Sunil; Sun, Baodong; Yi, Haiqing (Mol Genet Metab, 2013-07)

Previous studies strongly suggest that starch binding domain containing protein 1 (Stbd1) plays an important role in intracellular glycogen trafficking into lysosomes. We report here that Stbd1 expression is markedly increased ...