Proteinopathies and OXPHOS dysfunction in neurodegenerative diseases.
Proteinopathies and OXPHOS dysfunction in neurodegenerative diseases.
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DOI：
10.1083/jcb.201709172
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发表时间：
2017-12-04
期刊：
影响因子：
--
通讯作者：
Manfredi G
中科院分区：
文献类型：
--
作者：
Kawamata H;Manfredi G
Kawamata和Manfredi回顾了神经退行性疾病中错误折叠蛋白质的积累如何导致线粒体功能障碍的机制。线粒体参与神经系统中的基本过程，如能量和中间代谢、钙稳态和细胞凋亡。主要神经退行性疾病的病理特征是由于基因突变或异常蛋白质稳态导致的错误折叠蛋白质的积累。错误折叠的蛋白质与线粒体结合，形成寡聚体和聚集体。由于线粒体功能障碍，特别是氧化磷酸化系统（OXPHOS），发生在神经退行性疾病，它被假定，这种缺陷是由错误折叠的蛋白质的积累。然而，这一假说和线粒体中蛋白质病的病理作用仍然难以捉摸。在这项研究中，我们批判性地回顾了拟议的机制，使示例性的错误折叠蛋白与线粒体及其对OXPHOS的后果。
Kawamata and Manfredi review proposed mechanisms of how the accumulation of misfolded proteins in neurodegenerative diseases causes mitochondrial dysfunction. Mitochondria participate in essential processes in the nervous system such as energy and intermediate metabolism, calcium homeostasis, and apoptosis. Major neurodegenerative diseases are characterized pathologically by accumulation of misfolded proteins as a result of gene mutations or abnormal protein homeostasis. Misfolded proteins associate with mitochondria, forming oligomeric and fibrillary aggregates. As mitochondrial dysfunction, particularly of the oxidative phosphorylation system (OXPHOS), occurs in neurodegeneration, it is postulated that such defects are caused by the accumulation of misfolded proteins. However, this hypothesis and the pathological role of proteinopathies in mitochondria remain elusive. In this study, we critically review the proposed mechanisms whereby exemplary misfolded proteins associate with mitochondria and their consequences on OXPHOS.
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影响因子：
3.5
作者：
De Vos KJ;Mórotz GM;Stoica R;Tudor EL;Lau KF;Ackerley S;Warley A;Shaw CE;Miller CC
通讯作者：
Miller CC
DOI：
10.3233/jad-2011-101716
发表时间：
2011
期刊：
Journal of Alzheimer's disease : JAD
影响因子：
--
作者：
Alikhani N;Guo L;Yan S;Du H;Pinho CM;Chen JX;Glaser E;Yan SS
通讯作者：
Yan SS
α-突触核蛋白与TOM20结合，并抑制在帕金森氏病中进口的线粒体蛋白。
影响因子：
17.1
作者：
Di Maio R;Barrett PJ;Hoffman EK;Barrett CW;Zharikov A;Borah A;Hu X;McCoy J;Chu CT;Burton EA;Hastings TG;Greenamyre JT
通讯作者：
Greenamyre JT
影响因子：
5.3
作者：
Crouch, PJ;Blake, R;Trounce, IA
通讯作者：
Trounce, IA
影响因子：
11.4
作者：
Area-Gomez, Estela;Castillo, Maria Del Carmen Lara;Tambini, Marc D.;Guardia-Laguarta, Cristina;de Groof, Ad J. C.;Madra, Moneek;Ikenouchi, Junichi;Umeda, Masato;Bird, Thomas D.;Sturley, Stephen L.;Schon, Eric A.
通讯作者：
Schon, Eric A.