NDUFA4 Is a Subunit of Complex IV of the Mammalian Electron Transport Chain

被引：300

作者：

Balsa, Eduardo ^{[1
,2
]}

Marco, Ricardo ^{[1
]}

Pereles-Clemente, Ester ^{[1
]}

Szklarczyk, Radek ^{[3
]}

Calvo, Enrique ^{[1
]}

Landazuri, Manuel O. ^{[2
]}

Antonio Enriquez, Jose ^{[1
,4
]}

机构：

[1] Ctr Nacl Invest Cardiovasc Carlos III CNIC, Madrid 28029, Spain

[2] Univ Autonoma Madrid, Serv Inmunol, Hosp Univ La Princesa, Inst Invest Sanitaria Princesa IIS IP, Madrid 28006, Spain

[3] Radboud Univ Nijmegen, Nijmegen Ctr Mol Life Sci, Ctr Mol & Biomol Informat, Med Ctr, NL-6500 HB Nijmegen, Netherlands

[4] Univ Zaragoza, Fac Ciencias, Dept Bioquim & Biol Mol & Celular, Zaragoza 50013, Spain

来源：

CELL METABOLISM | 2012年 / 16卷 / 03期

关键词：

CYTOCHROME-C-OXIDASE; OXIDATIVE-PHOSPHORYLATION SYSTEM; PROTEIN COMPLEX; BOVINE; EUKARYOTES; UBIQUINONE; EPR;

D O I：

10.1016/j.cmet.2012.07.015

中图分类号：

Q2 [细胞生物学];

学科分类号：

071009 ; 090102 ;

摘要：

The oxidative phosphorylation system is one of the best-characterized metabolic pathways. In mammals, the protein components and X-ray structures are defined for all complexes except complex I. Here, we show that NDUFA4, formerly considered a constituent of NADH Dehydrogenase (CI), is instead a component of the cytochrome c oxidase (CIV). Deletion of NDUFA4 does not perturb Cl. Rather, proteomic, genetic, evolutionary, and biochemical analyses reveal that NDUFA4 plays a role in CIV function and biogenesis. The change in the attribution of the NDUFA4 protein requires renaming of the gene and reconsideration of the structure of CIV. Furthermore, NDUFA4 should be considered a candidate gene for CIV rather than Cl deficiencies in humans.

引用

页码：378 / 386

页数：9

共 38 条

[1] STRUCTURE AT 2.8-ANGSTROM RESOLUTION OF F1-ATPASE FROM BOVINE HEART-MITOCHONDRIA [J].

ABRAHAMS, JP ;

LESLIE, AGW ;

LUTTER, R ;

WALKER, JE .

NATURE, 1994, 370 (6491) :621-628

[2] Respiratory complex III is required to maintain complex I in mammalian mitochondria [J].

Acín-Pérez, R ;

Bayona-Bafaluy, MP ;

Fernández-Silva, P ;

Moreno-Loshuertos, R ;

Perez-Martos, A ;

Bruno, C ;

Moraes, CT ;

Enríquez, JA .

MOLECULAR CELL, 2004, 13 (06) :805-815

[3] Respiratory Active Mitochondrial Supercomplexes [J].

Acin-Perez, Rebeca ;

Fernandez-Silva, Patricio ;

Luisa Peleato, Maria ;

Perez-Martos, Acisclo ;

Enriquez, Jose Antonio .

MOLECULAR CELL, 2008, 32 (04) :529-539

[4] Gapped BLAST and PSI-BLAST: a new generation of protein database search programs [J].

Altschul, SF ;

Madden, TL ;

Schaffer, AA ;

Zhang, JH ;

Zhang, Z ;

Miller, W ;

Lipman, DJ .

NUCLEIC ACIDS RESEARCH, 1997, 25 (17) :3389-3402

[5] Mitochondrial NADH:ubiquinone oxidoreductase (complex I) in eukaryotes: A highly conserved subunit composition highlighted by mining of protein databases [J].

Cardol, Pierre .

BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS, 2011, 1807 (11) :1390-1397

[6] Analysis of the subunit composition of complex I from bovine heart mitochondria [J].

Carroll, J ;

Fearnley, IM ;

Shannon, RJ ;

Hirst, J ;

Walker, JE .

MOLECULAR & CELLULAR PROTEOMICS, 2003, 2 (02) :117-126

[7] Definition of the nuclear encoded protein composition of bovine heart mitochondrial complex I - Identification of two new subunits [J].

Carroll, J ;

Shannon, RJ ;

Fearnley, IM ;

Walker, JE ;

Hirst, J .

JOURNAL OF BIOLOGICAL CHEMISTRY, 2002, 277 (52) :50311-50317

[8] Bovine complex I is a complex of 45 different subunits [J].

Carroll, Joe ;

Fearnley, Ian M. ;

Skehel, J. Mark ;

Shannon, Richard J. ;

Hirst, Judy ;

Walker, John E. .

JOURNAL OF BIOLOGICAL CHEMISTRY, 2006, 281 (43) :32724-32727

[9] Function and structure of complex II of the respiratory chain [J].

Cecchini, G .

ANNUAL REVIEW OF BIOCHEMISTRY, 2003, 72 :77-109

[10] Cytochrome c oxidase is required for the assembly/stability of respiratory complex I in mouse fibroblasts [J].

Diaz, Francisca ;

Fukui, Hirokazu ;

Garcia, Sofia ;

Moraes, Carlos T. .

MOLECULAR AND CELLULAR BIOLOGY, 2006, 26 (13) :4872-4881

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