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The importance of gut motility in the metabolic control of propionic acidemia

✍ Scribed by Chitra Prasad; Samuel Nurko; Jacob Borovoy; Mark S. Korson

Book ID: 116682539
Publisher: Elsevier Science
Year: 2004
Tongue: English
Weight: 129 KB
Volume: 144
Category: Article
ISSN: 1097-6833
DOI: 10.1016/j.jpeds.2003.12.044

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Propionic acidemia is an inborn error of metabolism caused by a deficiency of propionyl-CoA carboxylase, a heteropolymeric mitochondrial enzyme involved in the catabolism of branched chain amino acids, odd-numbered chain length fatty acids, cholesterol, and other metabolites. The enzyme is composed