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Protein turnover and inclusion body formation
被引：55
作者：
Mitra, Siddhartha
[1
,4
,5
]
Tsvetkov, Andrey S.
[1
,2
,3
,6
]
Finkbeiner, Steven
[1
,2
,3
,4
,5
,6
]
机构：
[1] Gladstone Inst Neurol Dis, San Francisco, CA USA
[2] Taube Koret Ctr Huntingtons Dis Res, San Francisco, CA USA
[3] Univ Calif San Francisco, Neurosci Program, Dept Neurol, San Francisco, CA 94143 USA
[4] Univ Calif San Francisco, Biomed Sci Program, San Francisco, CA 94143 USA
[5] Univ Calif San Francisco, Med Scientist Training Program, San Francisco, CA 94143 USA
[6] Univ Calif San Francisco, Neurosci Program, Dept Physiol, San Francisco, CA 94143 USA
来源：
关键词：
Huntington disease;
huntingtin;
polyglutamine;
autophagy;
neurodegeneration;
ubiquitin;
proteasome;
D O I：
10.4161/auto.5.7.9291
中图分类号：
Q2 [细胞生物学];
学科分类号：
071009 ;
090102 ;
摘要：
In a recent study, we investigated the relationship between inclusion body (IB) formation and the activity of the ubiquitin-proteasome system (UPS) in a primary neuron model of Huntington disease. We followed individual neurons over the course of days and monitored the level of mutant huntingtin (htt) (which causes Huntington disease), IB formation, UPS function, and neuronal toxicity. The accumulation of UPS substrates and neuronal toxicity increased with increasing levels of proteasome inhibition. The UPS was more impaired in neurons that subsequently formed IBs than in those that did not; however, after IBs formed, UPS function improved. These findings suggest that IB formation is a protective cellular response mediated in part by increased degradation of intracellular protein.
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页码：1037 / 1038
页数：2
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