期刊
BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS
卷 1797, 期 6-7, 页码 1113-1118出版社
ELSEVIER
DOI: 10.1016/j.bbabio.2009.12.006
关键词
Cyclophilin; Mitochondria; ATP synthase; Permeability transition; Cyclosporin A
资金
- NIGMS NIH HHS [R01 GM069883-04, GM69883, R01 GM069883] Funding Source: Medline
- Telethon [GGP08107] Funding Source: Medline
Cyclophilins are a family of peptidyl-prolyl cis-trans isomerases whose enzymatic activity can be inhibited by cyclosporin A. Sixteen cyclophilins have been identified in humans, and cyclophilin D is a unique isoform that is imported into the mitochondrial matrix. Here we shall (i) review the best characterized functions of cyclophilin D in mitochondria, i.e. regulation of the permeability transition pore, an inner membrane channel that plays an important role in the execution of cell death; (ii) highlight new regulatory interactions that are emerging in the literature, including the modulation of the mitochondrial F1FO ATP synthase through an interaction with the lateral stalk of the enzyme complex; and (iii) discuss diseases where cyclophilin D plays a pathogenetic role that makes it a suitable target for pharmacologic intervention. (C) 2009 Elsevier B.V. All rights reserved.
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