Recent advances in p97/VCP/Cdc48 cellular functions

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• 作者：Kunitoshi Yamanaka ; Yohei Sasagawa¹ ; Teru Ogura ; ^{ogura@gpo.kumamoto-u.ac.jp}
• 关键词：AAA ; ATPases associated with diverse cellular activities ; VCP ; valosin-containing protein ; ER ; endoplasmic reticulum ; ERAD ; ER associated degradation ; IBMPFD ; inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia ; ALS
• 刊名：Biochimica et Biophysica Acta (BBA)/Molecular Cell Research
• 出版年：2012
• 出版时间：January 2012
• 年：2012
• 卷：1823
• 期：1
• 页码：130-137
• 全文大小：615 K

文摘

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. This article is part of a Special Issue entitled: AAA ATPases: structure and function.