Cytochrome c oxidase-deficient patients have distinct subunit assembly profiles.
AuthorsHanson BJ, Carrozzo R, Piemonte F, Tessa A, Robinson BH, Capaldi RA
JournalJ Biol Chem
PubMed ID11278850
'Cytochrome c oxidase (COX) deficiency is the most common respiratory chain defect in childhood and is clinically heterogeneous. We report a study of six patients with COX deficiencies. Two of the patients had as yet undefined defects, three patients had Surf-1 mutations, and one patient had a 15-base pair deletion ... More
Human complex I defects can be resolved by monoclonal antibody analysis into distinct subunit assembly patterns.
AuthorsTriepels RH, Hanson BJ, van den Heuvel LP, Sundell L, Marusich MF, Smeitink JA, Capaldi RA
JournalJ Biol Chem
PubMed ID11112787
Complex I defects are one of the most frequent causes of mitochondrial respiratory chain disorders. Therefore, it is important to find new approaches for detecting and characterizing Complex I deficiencies. In this paper, we introduce a new set of monoclonal antibodies that react with 39-, 30-, 20-, 18-, 15-, and ... More
Cytochrome C oxidase activity and oxygen tolerance.
AuthorsCampian JL, Gao X, Qian M, Eaton JW
JournalJ Biol Chem
PubMed ID17303578
Most cultured cells and intact animals die under hyperoxic conditions. However, a strain of HeLa cells that proliferates under 80% O(2), termed "HeLa-80," has been derived from wildtype HeLa cells ("HeLa-20") by selection for resistance to stepwise increases of oxygen partial pressure. The tolerance of HeLa-80 cells to hyperoxia is ... More