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Coordinated and reversible reduction of enzymes involved in terminal oxidative metabolism in skeletal muscle mitochondria from a riboflavin-responsive, multiple acyl-CoA dehydrogenase deficiency patient.

Abstract:

In this case report we studied alterations in mitochondrial proteins in a patient suffering from recurrent profound muscle weakness, associated with ethylmalonic-adipic aciduria, who had benefited from high dose of riboflavin treatment. Morphological and biochemical alterations included muscle lipid accumulation, low muscle carnitine content, reduction in fatty acid beta-oxidation and reduced activity of complexes I and II of the respiratory chain. Riboflavin therapy partially or totally reve...

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Publication status:
Published

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Publisher copy:
10.1002/elps.200500687

Authors


Gianazza, E More by this author
Vergani, L More by this author
More by this author
Institution:
University of Oxford
Department:
Oxford, MSD, NDORMS
Brambilla, D More by this author
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Journal:
Electrophoresis
Volume:
27
Issue:
5-6
Pages:
1182-1198
Publication date:
2006-03-05
DOI:
EISSN:
1522-2683
ISSN:
0173-0835
URN:
uuid:e132f62e-bdac-4a1a-8676-0387da7c89fa
Source identifiers:
229927
Local pid:
pubs:229927

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