Ubiquitin proteasome system in Parkinson's disease: A keeper or a witness?

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Ubiquitin Proteasome System in Stress and Disease

1 Laboratory of Chromatin Structure and Functions, Engelhardt Institute of Molecular Biology RAS, Moscow 119991, Russia 2Department of Biology, Technion – Israel Institute of Technology, 32000 Haifa, Israel 3Department of Biochemistry and Molecular Biology, The George S. Wise Faculty of Life Sciences, Tel Aviv University, 69978 Tel Aviv, Israel 4Department of Microbiology & Immunology, Universi...

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The ubiquitin-proteasome system in Alzheimer's disease

Accumulation of proteins is a recurring event in many neurodegenerative diseases, including Alzheimer's disease (AD). Evidence has suggested that protein accumulation may result from a dysfunction in the ubiquitin proteasome system (UPS). Indeed, there is clear genetic and biochemical evidence of an involvement of the ubiquitin proteasome system in AD. This review summarizes the data supporting...

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Ubiquitin–proteasome system involvement in Huntington’s disease

Huntington's disease (HD) is a genetic autosomal dominant neurodegenerative disease caused by the expansion of a CAG repeat in the huntingtin (htt) gene. This triplet expansion encodes a polyglutamine stretch (polyQ) in the N-terminus of the high molecular weight (348-kDa) and ubiquitously expressed protein htt. Normal individuals have between 6 and 35 CAG triplets, while expansions longer than...

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Accumulation of ubiquitin conjugates in a polyglutamine disease model occurs without global ubiquitin/proteasome system impairment.

Aggregation-prone proteins have been suggested to overwhelm and impair the ubiquitin/proteasome system (UPS) in polyglutamine (polyQ) disorders, such as Huntington's disease (HD). Overexpression of an N-terminal fragment of mutant huntingtin (N-mutHtt), an aggregation-prone polyQ protein responsible for HD, obstructs the UPS in cellular models. Furthermore, based on the accumulation of polyubiq...

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The ubiquitin-proteasome-system.

NOTICE: this is the author’s version of a work that was accepted for publication in Biochimica et Biophysica Acta Molecular Cell Research. Changes resulting from the publishing process, such as peer review, editing, corrections, structural formatting, and other quality control mechanisms may not be reflected in this document. Changes may have been made to this work since it was submitted for pu...

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ژورنال

عنوان ژورنال: Experimental Neurology

سال: 2012

ISSN: 0014-4886

DOI: 10.1016/j.expneurol.2012.08.008