Intrinsic uncoupling of cytochrome c oxidase may cause the maternally inherited mitochondrial diseases MELAS and LHON.
FEBS Lett
; 433(1-2): 93-7, 1998 Aug 14.
Article
en En
| MEDLINE
| ID: mdl-9738940
ABSTRACT
Mutations in the human mtDNA gene encoding subunit III of cytochrome c oxidase (CO) have been reported to cause MELAS and LHON. Poracoccus denitrificans cells expressing substitutions homologous to these MELAS- and LHON-causing mutations had lower growth yield than wild type cells and lower efficiency of proton pumping by CO (e.g. lower H+/e ratio and lower deltapsi), but had similar CO activity. These results indicate that both substitutions (F263L > A212T) cause intrinsic uncoupling, which may be the direct cause of the diseases. These results also suggest that subunit III is involved in proton pumping.
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Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
ADN Mitocondrial
/
Atrofias Ópticas Hereditarias
/
Síndrome MELAS
/
Complejo IV de Transporte de Electrones
Límite:
Humans
Idioma:
En
Revista:
FEBS Lett
Año:
1998
Tipo del documento:
Article
País de afiliación:
Estados Unidos