Yamanaka Kunitoshi, Sasagawa Yohei, Ogura Teru
Department of Molecular Cell Biology, Institute of Molecular Embryology and Genetics, Kumamoto University, 2-2-1 Honjo, Kumamoto 860-0811, Japan.
Biochim Biophys Acta. 2012 Jan;1823(1):130-7. doi: 10.1016/j.bbamcr.2011.07.001. Epub 2011 Jul 12.
p97/VCP/Cdc48 is one of the best-characterized type II AAA (ATPases associated with diverse cellular activities) ATPases. p97 is suggested to be a ubiquitin-selective chaperone and its key function is to disassemble protein complexes. p97 is involved in a wide variety of cellular activities. Recently, novel functions, namely autophagy and mitochondrial quality control, for p97 have been uncovered. p97 was identified as a causative factor for inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia (IBMPFD) and more recently as a causative factor for amyotrophic lateral sclerosis (ALS). In this review, we will summarize and discuss recent progress and topics in p97 functions and the relationship to its associated diseases.
p97/VCP/Cdc48是特征最为明确的II型AAA（与多种细胞活动相关的ATP酶）ATP酶之一。p97被认为是一种泛素选择性伴侣蛋白，其关键功能是拆解蛋白质复合物。p97参与多种细胞活动。最近，已发现p97具有自噬和线粒体质量控制等新功能。p97被确定为与骨佩吉特病和额颞叶痴呆相关的包涵体肌病（IBMPFD）的致病因素，最近又被确定为肌萎缩侧索硬化症（ALS）的致病因素。在本综述中，我们将总结并讨论p97功能方面的最新进展和相关话题，以及它与相关疾病的关系。
