遗传性痉挛性截瘫：呼吸窒息还是未激活的底物？
Hereditary spastic paraplegia: respiratory choke or unactivated substrate?
作者信息
Claypool Steven M, Koehler Carla M
机构信息
Department of Chemistry and Biochemistry, University of California, Los Angeles, 90095, USA.
出版信息
Cell. 2005 Oct 21;123(2):183-5. doi: 10.1016/j.cell.2005.10.006.
Defects in the mitochondrial AAA protease family member, paraplegin, result in an autosomal recessive form of hereditary spastic paraplegia (HSP). In this issue of Cell, Nolden et al. (2005) report a new molecular mechanism for HSP based on the requirement of paraplegin for the proteolysis of a specific mitochondrial ribosomal protein. The processing of this substrate is required for robust translation in mitochondria.
线粒体AAA蛋白酶家族成员paraplegin的缺陷会导致常染色体隐性遗传性痉挛性截瘫（HSP）。在本期《细胞》杂志中，诺尔登等人（2005年）基于paraplegin对特定线粒体核糖体蛋白进行蛋白水解的需求，报道了一种HSP的新分子机制。线粒体中高效翻译需要对该底物进行加工处理。
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