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In muscle mitochondria, activities of both short\u2010chain acyl\u2010CoA dehydrogenase (SCAD) and medium\u2010chain acyl\u2010CoA dehydrogenase (MCAD) were 35% of normal. Antibodies against purified SCAD, MCAD, and electron\u2010transfer flavoprotein were used for detection of cross\u2010reacting material (CRM) in the patient's mitochondria. Western blot analysis showed absence of SCAD\u2010CRM, reduced amounts of MCAD\u2010CRM, and normal amounts of electron\u2010transfer flavoprotein\u2010CRM. The patient, who was unresponsive to treatment with oral carnitine, improved dramatically with daily administration of 100 mg oral riboflavin. Increase in muscle bulk and strength and resolution of the organic aciduria were associated with normalization of SCAD activity and \u201creappearance\u201d of SCAD\u2010CRM. In contrast, both MCAD activity and MCAD\u2010CRM remained lower than normal. These results suggest that in some patients with multiple acyl\u2010CoA dehydrogenase deficiency riboflavin supplementation may be effective in restoring the activity of SCAD, and possibly of other mitochondrial flavin\u2010dependent enzymes.<\/jats:p>","DOI":"10.1002\/ana.410250510","type":"journal-article","created":{"date-parts":[[2005,1,1]],"date-time":"2005-01-01T14:11:23Z","timestamp":1104588683000},"page":"479-484","source":"Crossref","is-referenced-by-count":39,"title":["Normalization of short\u2010chain acylcoenzyme a dehydrogenase after riboflavin treatment in a girl with multiple acylcoenzyme a dehydrogenase\u2014deficient 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