Metabolism of 1-13C-propionate in vivo in patients with disorders of propionate metabolism

Metabolism of propionate in human subjects was studied using bolus administration of 1-13C-propionate i.v. or orally. The study population consisted of five patients with propionic acidemia (PA), eight with methylmalonic acidemia (MMA; four responsive to vitamin B12), one each with multiple carboxyl...

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Veröffentlicht in:	Pediatric research 1991-07, Vol.30 (1), p.15-22
Hauptverfasser:	BARSHOP, B. A, YOSHIDA, I, AJAMI, A, SWEETMAN, L, WOLFF, J. A, SWEETMAN, F. R, PRODANOS, C, SMITH, M, NYHAN, W. L
Format:	Artikel
Sprache:	eng
Schlagworte:	Adolescent Amino Acid Metabolism, Inborn Errors - metabolism Biological and medical sciences Biotin - pharmacology Carbon Dioxide - metabolism Carboxy-Lyases - deficiency Carnitine - pharmacology Child Child, Preschool Female Half-Life Humans Infant Male Medical sciences Metabolic diseases Methylmalonic Acid - blood Methylmalonyl-CoA Decarboxylase Propionates - administration & dosage Propionates - blood Propionates - metabolism Transcobalamins - deficiency Vitamin B 12 - pharmacology
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