Article
ATAXIN-1 interacts with the repressor Capicua in its native complex to cause SCA1 neuropathology.
Cell - 29 Dec 2006
Lam Yung C, Bowman Aaron B, Jafar-Nejad Paymaan, Lim Janghoo, Richman Ronald, Fryer John D, Hyun Eric D, Duvick Lisa A, Orr Harry T, Botas Juan, Zoghbi Huda Y
Abstract excerpt
Spinocerebellar ataxia type 1 (SCA1) is one of several neurodegenerative diseases caused by expansion of a polyglutamine tract in the disease protein, in this case, ATAXIN-1 (ATXN1). A key question in the field is whether neurotoxicity is mediated by aberrant, novel interactions with the expanded protein or whether its wild-type functions are augmented to a deleterious degree. We examined soluble protein...
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