Cyclophilin D in mitochondrial pathophysiology

被引：145

作者：

Giorgio, Valentina ^{[1
,2
]}

Soriano, Maria Eugenia ^{[4
]}

Basso, Emy ^{[1
,2
]}

Bisetto, Elena ^{[3
]}

Lippe, Giovanna ^{[5
]}

Forte, Michael A. ^{[6
]}

Bernardi, Paolo ^{[1
,2
,4
]}

机构：

[1] Univ Padua, Dept Biomed Sci, I-35121 Padua, Italy

[2] Univ Padua, CNR Inst Neurosci, I-35121 Padua, Italy

[3] Univ Udine, Dept Biomed Sci & Technol, I-33100 Udine, Italy

[4] Venetian Inst Mol Med, Padua, Italy

[5] Univ Udine, Dept Food Sci, I-33100 Udine, Italy

[6] Oregon Hlth & Sci Univ, Vollum Inst, Portland, OR 97201 USA

来源：

BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS | 2010年 / 1797卷 / 6-7期

关键词：

Cyclophilin; Mitochondria; ATP synthase; Permeability transition; Cyclosporin A; PERMEABILITY TRANSITION PORE; ADENINE-NUCLEOTIDE TRANSLOCASE; CYCLOSPORINE-A-BINDING; CELL-DEATH; ISOMERASES IMMUNOPHILINS; HEART-MITOCHONDRIA; MUSCLE APOPTOSIS; INNER MEMBRANE; ANIMAL-MODEL; INHIBITION;

D O I：

10.1016/j.bbabio.2009.12.006

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

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.

引用

页码：1113 / 1118

页数：6

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