A pathogenic mutation in cytochrome c oxidase results in impaired proton pumping while retaining O2-reduction activity

Biochimica et Biophysica Acta - Bioenergetics

Volumn 1797, Issue 5, 2010, Pages 550-556

  Namslauer, Ida ^a   Lee, Hyun Ju ^b   Gennis, Robert B ^b   Brzezinski, Peter ^a  

^a STOCKHOLM UNIVERSITY   (Sweden)

^b UNIVERSITY OF ILLINOIS AT URBANA CHAMPAIGN   (United States)

Author keywords

Cytochrome aa3; Deficiency; Electron transfer; Mitochondria; Mitochondrial disease; MtDNA; Respiratory chain

Indexed keywords

CYTOCHROME C OXIDASE; GLUTAMIC ACID; HISTIDINE; MITOCHONDRIAL DNA; THREONINE; THYRONINE;

ABSORPTION SPECTROSCOPY; AMINO ACID SUBSTITUTION; ARTICLE; CONTROLLED STUDY; ELECTRON TRANSPORT; ENZYME ACTIVITY; ENZYME PURIFICATION; ENZYME STRUCTURE; GENE MUTATION; HUMAN; HUMAN CELL; HUMAN TISSUE; MUTATIONAL ANALYSIS; OXIDATION REDUCTION REACTION; PATHOGENESIS; PHENOTYPE; PRIORITY JOURNAL; PROTON TRANSPORT; RESPIRATORY FAILURE; RHODOBACTER SPHAEROIDES;

AMINO ACID SUBSTITUTION; CATALYSIS; CATALYTIC DOMAIN; COLONIC NEOPLASMS; DNA, MITOCHONDRIAL; ELECTRON TRANSPORT COMPLEX IV; HUMANS; KINETICS; MITOCHONDRIA; MODELS, MOLECULAR; MUTAGENESIS, SITE-DIRECTED; MUTATION; OXYGEN; PROTEIN CONFORMATION; PROTON PUMPS; PROTONS; RHODOBACTER SPHAEROIDES; STRUCTURE-ACTIVITY RELATIONSHIP;

BACTERIA (MICROORGANISMS); RHODOBACTER SPHAEROIDES;

EID: 77950371031     PISSN: 00052728     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.bbabio.2010.01.027     Document Type: Article

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