[HTML][HTML] Phosphorylation of threonine 3: implications for Huntingtin aggregation and neurotoxicity

CT Aiken, JS Steffan, CM Guerrero, H Khashwji… - Journal of Biological …, 2009 - ASBMB
Huntingtin (Htt) is a widely expressed protein that causes tissue-specific degeneration when
mutated to contain an expanded polyglutamine (poly (Q)) domain. Although Htt is large, 350
kDa, the appearance of amino-terminal fragments of Htt in extracts of postmortem brain
tissue from patients with Huntington disease (HD), and the fact that an amino-terminal
fragment, Htt exon 1 protein (Httex1p), is sufficient to cause disease in models of HD, points
to the importance of the amino-terminal region of Htt in the disease process. The first exon of …