Leber hereditary optic neuropathy mutations in the ND6 subunit of mitochondrial complex I affect ubiquinone reduction kinetics in a bacterial model of the enzyme

Biochemical Journal

Volumn 409, Issue 1, 2008, Pages 129-137

  Pätsi, Jukka ^a   Kervinen, Marko ^a   Finel, Moshe ^b   Hassinen, Ilmo E ^a  

^a UNIVERSITY OF OULU   (Finland)

^b UNIVERSITY OF HELSINKI   (Finland)

Author keywords

Enzyme inhibitor; Escherichia coli; Leber hereditary optic neuropathy; Mitochondrial disease; Mitochondrial DNA; NADH:ubiquinone oxidoreductase

Indexed keywords

CATALYSIS; DISEASES; DNA; ENZYME INHIBITION; ESCHERICHIA COLI;

ENZYME INHIBITORS; LEBER HEREDITARY OPTIC NEUROPATHY; MITOCHONDRIAL DISEASE; MITOCHONDRIAL DNA; UBIQUINONE OXIDOREDUCTASE;

ENZYME KINETICS;

BACTERIAL ENZYME; CARBON; MALIC ACID; OXIDOREDUCTASE; PROTEIN SUBUNIT; REDUCED NICOTINAMIDE ADENINE DINUCLEOTIDE; REDUCED NICOTINAMIDE ADENINE DINUCLEOTIDE DEHYDROGENASE (UBIQUINONE); RUTHENIUM DERIVATIVE; UBIQUINONE; MITOCHONDRIAL DNA;

AMINO ACID SUBSTITUTION; ARTICLE; CELL GROWTH; ELECTRON TRANSPORT; ENERGY CONSERVATION; ENZYME ACTIVITY; ENZYME SUBSTRATE COMPLEX; ESCHERICHIA COLI; GENE MUTATION; LEBER HEREDITARY OPTIC NEUROPATHY; MUTANT; NONHUMAN; OXIDATION; PATHOGENESIS; PRIORITY JOURNAL; RECEPTOR AFFINITY; SEQUENCE HOMOLOGY; BACTERIUM; CATALYSIS; CHEMISTRY; FEMALE; GENETICS; HUMAN; IC 50; KINETICS; MALE; METABOLISM; MITOCHONDRION; MUTAGENESIS; MUTATION;

BACTERIA (MICROORGANISMS); ESCHERICHIA COLI;

BACTERIA; CATALYSIS; DNA, MITOCHONDRIAL; ELECTRON TRANSPORT COMPLEX I; ESCHERICHIA COLI; FEMALE; HUMANS; INHIBITORY CONCENTRATION 50; KINETICS; MALE; MITOCHONDRIA; MUTAGENESIS; MUTATION; OPTIC ATROPHY, HEREDITARY, LEBER; UBIQUINONE;

EID: 38149079945     PISSN: 02646021     EISSN: None     Source Type: Journal    
DOI: 10.1042/BJ20070866     Document Type: Article

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