... of the N-terminal region of huntingtin, encoded by exon-1 of the huntingtin gene, where CAG expansion leads to Huntington's disease, a fatal autosomal-dominant neurodegenerative condition. I also discuss how inhibition of tetramerization blocks the much slower (by many orders of magnitude) process...

... proteins. Studies using purified components have shown that the minimal requirements for S-acylation are an appropriate zDHHC enzyme–substrate pair and fatty acyl-CoA. However, additional proteins including GCP16 (also known as Golga7), Golga7b, huntingtin and selenoprotein K, have been suggested...

... a hypothesis to answer the question of why so many signaling molecules that work in the nucleus are also found in the primary cilium. Primary cilium Signaling scaffold molecule Transmembrane receptor Nuclear pore Huntingtin The primary cilium is a generally non-motile, membrane-bound...

..., termed the PX-associated B (PXB) domain, has led to the proposal that they function as endosome-associated scaffolds. Here, we used unbiased quantitative proteomics to define the SNX21 interactome. We reveal that the N-terminal extension of SNX21 interacts with huntingtin (Htt) whereas the PXB domain...

...Jinting Yan; Hui Zhang; Yang Liu; Feilong Zhao; Shu Zhu; Chengmei Xie; Tie-Shan Tang; Caixia Guo ABSTRACT Human Huntingtin ( HTT ), a Huntington's disease gene, is highly expressed in the mammalian brain and testis. Simultaneous knockout of mouse Huntingtin ( Htt ) in brain and testis impairs male...

... of proteins activate autophagy ( Tyedmers et al., 2010 ). The autophagy-lysosomal degradation machinery can also, to some extent, clear protein aggregates ( Korolchuk et al., 2010 ). Amyotrophic lateral sclerosis Huntington disease Huntingtin Optineurin Phosphorylation SOD1 TBK1 Ubiquitin...

...Patrick Lajoie; Erik L. Snapp Huntington's disease (HD) is caused by expanded glutamine repeats within the huntingtin (Htt) protein. Mutant Htt (mHtt) in the cytoplasm has been linked to induction of the luminal endoplasmic reticulum (ER) stress pathway, the unfolded protein response (UPR). How...

...Shouqing Luo; David C. Rubinsztein Huntington's disease is caused by a polyglutamine expansion in the huntingtin protein. Wild-type huntingtin, by contrast, appears to protect cells from pro-apoptotic insults. Here we describe a novel anti-apoptotic function for huntingtin. When cells are exposed...

..., maintenance and/or disappearance of F-actin-enriched pathological inclusion bodies. * Author for correspondence (e-mail: [email protected] ) 31 1 2008 © The Company of Biologists Limited 2008 2008 Actin Aggresome Autophagy Inclusion bodies Cytoskeleton Huntingtin Jasplakinolide...

...Kyung-Jin Lee; Antony Panzera; David Rogawski; Lois E. Greene; Evan Eisenberg The effect of normal cellular prion protein (PrP C ) on abnormal protein aggregation was examined by transfecting huntingtin fragments (Htt) into SN56 neuronal-derived cells depleted of PrP C by RNA interference. PrP C...

... with organelles such as nucleoli in heat-shocked cells or the large inclusions formed from fragments of mutant huntingtin protein. In heat-shocked cells, nucleoplasmic Hsp70 has reduced mobility relative to the cytoplasm, whereas the ATPase-deficient mutant of Hsp70, Hsp70(K71E), is almost completely immobilized...