Ubiquitin-dependent and -independent mitochondrial protein quality controls: implications in ageing and neurodegenerative diseases
Ubiquitin-dependent and -independent mitochondrial protein quality controls: implications in ageing and neurodegenerative diseases
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DOI：
10.1111/j.1365-2958.2008.06502.x
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发表时间：
2008-12-01
影响因子：
3.6
通讯作者：
Germain, Doris
中科院分区：
文献类型：
--
作者：
Germain, Doris
泛素不依赖的蛋白质质量控制的基质蛋白质的peption是很好的特点，直到最近的peption被认为是一个'无泛素化'的细胞器。然而，许多研究表明泛素-蛋白酶体途径在线粒体完整性的调节和维持中具有多种作用。特别感兴趣的是，这篇评论的线粒体泛素依赖的蛋白质质量控制的发现，这条途径可能共享相似的内质网相关的降解（ERAD）途径，其作用是消除错误折叠的蛋白质从内质网的内腔。泛素依赖性和非依赖性蛋白质质量控制之间的潜在串扰及其在衰老和神经退行性疾病，特别是帕金森病中的影响进行了讨论。
The ubiquitin-independent protein quality control of matrix proteins of the mitochondrion is well characterized and until recently the mitochondrion was considered a 'ubiquitination-free' organelle. However, a number of studies now indicate multiple roles of the ubiquitin-proteasome pathway in the regulation and maintenance of mitochondrial integrity. Of particular interest to this review is the finding of a mitochondrial ubiquitin-dependent protein quality control and that this pathway may share similarity to the endoplasmic reticulum-associated degradation (ERAD) pathway that acts to eliminate misfolded proteins from the lumen of the endoplasmic reticulum. The potential cross-talk between the ubiquitin-dependent and -independent protein quality controls and their implications in ageing and neurodegenerative diseases, notably in Parkinson's disease, are discussed.