Zentrum für Molekulare Biologie der Universität Heidelberg (ZMBH), DKFZ-ZMBH Alliance, Im Neuenheimer Feld 282, D-69,120 Heidelberg, Germany.
Nat Rev Mol Cell Biol. 2010 Nov;11(11):777-88. doi: 10.1038/nrm2993. Epub 2010 Oct 14.
The aggregation of misfolded proteins is associated with the perturbation of cellular function, ageing and various human disorders. Mounting evidence suggests that protein aggregation is often part of the cellular response to an imbalanced protein homeostasis rather than an unspecific and uncontrolled dead-end pathway. It is a regulated process in cells from bacteria to humans, leading to the deposition of aggregates at specific sites. The sequestration of misfolded proteins in such a way is protective for cell function as it allows for their efficient solubilization and refolding or degradation by components of the protein quality-control network. The organized aggregation of misfolded proteins might also allow their asymmetric distribution to daughter cells during cell division.
错误折叠蛋白质的聚集与细胞功能紊乱、衰老和各种人类疾病有关。越来越多的证据表明,蛋白质聚集通常是细胞对蛋白质平衡失调的反应的一部分,而不是一种非特异性和不受控制的死胡同途径。从细菌到人,这是一个在细胞中受到调节的过程,导致聚集物沉积在特定的部位。以这种方式隔离错误折叠的蛋白质对细胞功能是有保护作用的,因为它允许它们通过蛋白质质量控制网络的成分有效地溶解、重折叠或降解。错误折叠蛋白质的有组织聚集也可能允许它们在细胞分裂过程中不对称地分配到子细胞中。