Mitochondrial Cristae Shape Determines Respiratory Chain Supercomplexes Assembly and Respiratory Efficiency

被引:952
作者
Cogliati, Sara [1 ,2 ]
Frezza, Christian [1 ]
Soriano, Maria Eugenia [1 ,2 ]
Varanita, Tatiana [1 ,2 ]
Quintana-Cabrera, Ruben [1 ,2 ]
Corrado, Mauro [1 ,3 ]
Cipolat, Sara [1 ]
Costa, Veronica [1 ]
Casarin, Alberto [4 ]
Gomes, Ligia C. [1 ]
Perales-Clemente, Ester [5 ]
Salviati, Leonardo [4 ]
Fernandez-Silva, Patricio [6 ]
Enriquez, Jose A. [5 ]
Scorrano, Luca [1 ,2 ,3 ]
机构
[1] Venetian Inst Mol Med, Dulbecco Telethon Inst, I-35129 Padua, Italy
[2] Univ Padua, Dept Biol, I-35121 Padua, Italy
[3] IRCCS Fdn Santa Lucia, I-00143 Rome, Italy
[4] Univ Padua, Dept Woman & Child Hlth, Clin Genet Unit, I-35128 Padua, Italy
[5] Ctr Nacl Invest Cardiovasc Carlos III, Madrid 28029, Spain
[6] Univ Zaragoza, Fac Ciencias, Dept Bioquim & Biol Mol & Celular, E-50009 Zaragoza, Spain
关键词
DOMINANT OPTIC ATROPHY; ATP SYNTHASE; CYTOCHROME-C; OXIDATIVE-PHOSPHORYLATION; ENDOPLASMIC-RETICULUM; MAMMALIAN-CELLS; INNER MEMBRANE; COMPLEX-I; APOPTOSIS; PROTEIN;
D O I
10.1016/j.cell.2013.08.032
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
Respiratory chain complexes assemble into functional quaternary structures called supercomplexes (RCS) within the folds of the inner mitochondrial membrane, or cristae. Here, we investigate the relationship between respiratory function and mitochondrial ultrastructure and provide evidence that cristae shape determines the assembly and stability of RCS and hence mitochondrial respiratory efficiency. Genetic and apoptotic manipulations of cristae structure affect assembly and activity of RCS in vitro and in vivo, independently of changes to mitochondrial protein synthesis or apoptotic outer mitochondrial membrane permeabilization. We demonstrate that, accordingly, the efficiency of mitochondria-dependent cell growth depends on cristae shape. Thus, RCS assembly emerges as a link between membrane morphology and function.
引用
收藏
页码:160 / 171
页数:12
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