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Article

Effects of short-chain acyl-CoA dehydrogenase deficiency on development expression of metabolic enzyme genes in the mouse.

Biochemical and molecular medicine - 1 Apr 1996

Hinsdale M E, Hamm D A, Wood P A

Abstract excerpt

Patients with an acyl-CoA dehydrogenase deficiency share the disease features of hypoglycemia, hyperammonemia, tissue fatty change, hypoketonemia, carnitine deficiency, and organic acidemia due to apparent disruption of normal fatty acid, glucose, and urea metabolism. Most of the acute clinical episodes occur in young children. These episodes are precipitated by fasting and are often fatal, with the in vivo...

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PMID: 8733888
DOI: 10.1006/bmme.1996.0016

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OTC

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