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Updated: Jun 22, 2026

Fractionation for Resolution of Soluble and Insoluble Huntingtin Species
07:08

Fractionation for Resolution of Soluble and Insoluble Huntingtin Species

Published on: February 27, 2018

Rhesは,ストライア特異のタンパク質で,突然変異のハンティングチン細胞毒性を媒介する

Srinivasa Subramaniam1, Katherine M Sixt, Roxanne Barrow

  • 1Solomon H. Snyder Department of Neuroscience, Johns Hopkins University School of Medicine, 725 North Wolfe Street, Baltimore, MD 21205, USA.

Science (New York, N.Y.)
|June 6, 2009
PubMed
まとめ

ハンチントン病 (HD) の病理学は,変異したハンティングチン (mHtt) と,ストライアトム特異のタンパク質Rhes.との相互作用により,ストライアトムに局限されています. Rhes結合はmHttのスモイル化を誘導し,細胞死を引き起こし,HDの選択的な脳領域の影響を説明します.

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Huntington Disease l: Introduction01:21

Huntington Disease l: Introduction

Huntington disease or HD is a progressive, fatal neurodegenerative disorder inherited in an autosomal dominant pattern.PathophysiologyIt is caused by expansion of the CAG trinucleotide repeat in the HTT gene on chromosome 4 (4p16.3), producing an abnormal huntingtin protein with an expanded polyglutamine tract. This misfolded protein disrupts cellular function, leading to neuronal death. Normal alleles have ≤26 repeats, 27–35 are intermediate (risk of expansion), 36–39 show reduced penetrance,...
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After folding, the ER assesses the quality of secretory and membrane proteins. The correctly folded proteins are cleared by the calnexin cycle for transport to their final destination, while misfolded proteins are held back in the ER lumen. The ER chaperones attempt to unfold and refold the misfolded proteins but sometimes fail to achieve the correct native conformation. Such terminally misfolded proteins are then exported to the cytosol by ER-associated degradation or ERAD pathway for...