Article
Evidence that small molecule enhancement of β-hexosaminidase activity corrects the behavioral phenotype in Dutch APP(E693Q) mice through reduction of ganglioside-bound Aβ.
Molecular psychiatry - 1 Feb 2015
Knight E M, Williams H N, Stevens A C, Kim S H, Kottwitz J C, Morant A D, Steele J W, Klein W L, Yanagisawa K, Boyd R E, Lockhart D J, Sjoberg E R, Ehrlich M E, Wustman B A, Gandy S
Abstract excerpt
Certain mutant Alzheimer's amyloid-β (Aβ) peptides (that is, Dutch mutant APP(E693Q)) form complexes with gangliosides (GAβ). These mutant Aβ peptides may also undergo accelerated aggregation and accumulation upon exposure to GM2 and GM3. We hypothesized that increasing β-hexosaminidase (β-hex) activity would lead to a reduction in GM2 levels, which in turn, would cause a reduction in Aβ aggregation and...
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