The cytochrome b p.278Y>C mutation causative of a multisystem disorder enhances superoxide production and alters supramolecular interactions of respiratory chain complexes

Human Molecular Genetics

Volumn 22, Issue 11, 2013, Pages 2141-2151

  Ghelli, Anna ^a   Tropeano, Concetta V ^a   Calvaruso, Maria Antonietta ^a   Marchesini, Alessandra ^a   Iommarini, Luisa ^a   Porcelli, Anna Maria ^a   Zanna, Claudia ^a   De Nardo, Vera ^b   Martinuzzi, Andrea ^b   Wibrand, Flemming ^c   Vissing, John ^c   Kurelac, Ivana ^a   Gasparre, Giuseppe ^a   Selamoglu, Nur ^d   Daldal, Fevzi ^d   Rugolo, Michela ^a  

^a UNIVERSITY OF BOLOGNA   (Italy)

^b SCIENTIFIC INSTITUTE   (Italy)

^c UNIVERSITY OF COPENHAGEN   (Denmark)

^d UNIVERSITY OF PENNSYLVANIA   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

ADENOSINE TRIPHOSPHATE; CYSTEINE; CYTOCHROME B; GLUTATHIONE; GLUTATHIONE DISULFIDE; OLIGOMYCIN; SUPEROXIDE; UBIQUINOL CYTOCHROME C REDUCTASE;

ARTICLE; BIOCHEMICAL COMPOSITION; CELL FRACTIONATION; CELL VIABILITY; CONTROLLED STUDY; CYBRID; DISEASE ASSOCIATION; GENE MUTATION; HOMEOSTASIS; HUMAN; HUMAN CELL; MITOCHONDRIAL MEMBRANE POTENTIAL; MOLECULAR INTERACTION; POLYACRYLAMIDE GEL ELECTROPHORESIS; PRIORITY JOURNAL; RESPIRATORY CHAIN; WESTERN BLOTTING;

ADENOSINE TRIPHOSPHATE; CELL LINE; CYTOCHROMES B; DNA, MITOCHONDRIAL; ELECTRON TRANSPORT; ELECTRON TRANSPORT COMPLEX I; ELECTRON TRANSPORT COMPLEX IV; ENERGY METABOLISM; ENZYME ACTIVATION; GLUTATHIONE; HOMEOSTASIS; HUMANS; MITOCHONDRIA; MUTATION; SUPEROXIDES;

EID: 84877896972     PISSN: 09646906     EISSN: 14602083     Source Type: Journal    
DOI: 10.1093/hmg/ddt067     Document Type: Article

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