Article
Potent inhibition of huntingtin aggregation and cytotoxicity by a disulfide bond-free single-domain intracellular antibody.
Proceedings of the National Academy of Sciences of the United States of America - 21 Dec 2004
Colby David W, Chu Yijia, Cassady John P, Duennwald Martin, Zazulak Helen, Webster Jack M, Messer Anne, Lindquist Susan, Ingram Vernon Martin, Wittrup K Dane
Abstract excerpt
Huntington's disease (HD) is a progressive neurodegenerative disorder caused by an expansion in the number of polyglutamine-encoding CAG repeats in the gene that encodes the huntingtin (htt) protein. A property of the mutant protein that is intimately involved in the development of the disease is the propensity of the glutamine-expanded protein to misfold and generate an N-terminal proteolytic htt fragment that...
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