Poveda-Huertes Daniel, Mulica Patrycja, Vögtle F Nora
Institute of Biochemistry and Molecular Biology, ZBMZ, University of Freiburg, Freiburg, Germany.
Faculty of Biology, University of Freiburg, Freiburg, Germany.
Cell Tissue Res. 2017 Jan;367(1):73-81. doi: 10.1007/s00441-016-2492-9. Epub 2016 Sep 5.
Mitochondria play a key role in several metabolic and cell biological pathways and have attracted increasing attention due to their implication in life-span, ageing and human diseases. Mitochondrial proteases have a special role in these multiple biological functions, as they are involved in the regulation of various processes, e.g., mitochondrial protein biogenesis and quality control, mitochondrial dynamics, mitophagy and programmed cell death. The mitochondrial presequence processing machinery serves the particular purpose of maturing the majority of incoming precursor proteins by presequence cleavage, to ensure a stable mature protein by trimming of intermediate N-termini and to remove free toxic targeting peptides.
线粒体在多种代谢和细胞生物学途径中发挥关键作用，并且由于其与寿命、衰老及人类疾病的关联而受到越来越多的关注。线粒体蛋白酶在这些多种生物学功能中具有特殊作用，因为它们参与各种过程的调节，例如线粒体蛋白质生物合成与质量控制、线粒体动力学、线粒体自噬和程序性细胞死亡。线粒体前序列加工机制具有特定功能，即通过前序列切割使大多数进入的前体蛋白成熟，通过修剪中间N末端确保稳定的成熟蛋白，并去除游离的有毒靶向肽。
