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Congenital deficiency of two polypeptide subunits of the iron-protein fragment of mitochondrial complex I.
Recently, we described a patient with severe lactic acidosis due to congenital complex I (NADH-ubiquinone oxidoreductase) deficiency. We now report further enzymatic and immunological characterizations. Both NADH and ferricyanide titrations of complex I activity (measured as NADH-ferricyanide reduct...
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| Publicado no: | J Clin Invest |
|---|---|
| Main Authors: | , , , , |
| Formato: | Artigo |
| Idioma: | Inglês |
| Publicado em: |
American Society for Clinical Investigation
1987
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| Assuntos: | |
| Acesso em linha: | https://ncbi.nlm.nih.govhttps://pmc.ncbi.nlm.nih.gov/articles/PMC424102/ https://ncbi.nlm.nih.govhttps://pubmed.ncbi.nlm.nih.gov/3100577/ https://ncbi.nlm.nih.govhttps://doi.org/10.1172/JCI112834 |
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