Ethylmalonic and methylsuccinic aciduria in ethylmalonic encephalopathy arise from abnormal isoleucine metabolism

A comparison of [9,10‐³H]palmitic and [9,10‐³H]myristic acids for the detection of defects of fatty acid oxidation in intact cultured fibroblasts

N. J. Manning, S. E. Olpin, R. J. Pollitt et al. · Journal of Inherited Metabolic Disease · 1989 · 153 citations

Life Sciences, Biochemistry, Molecular Biology +12

Purification and characterization of 2-methyl-branched chain acyl coenzyme A dehydrogenase, an enzyme involved in the isoleucine and valine metabolism, from rat liver mitochondria.

Yasuyuki Ikeda, Kay Tanaka · Journal of Biological Chemistry · 1983 · 109 citations · Full text

Life Sciences, Biochemistry, Molecular Biology +16

Ethylmalonic Aciduria Is Associated with an Amino Acid Variant of Short Chain Acyl-Coenzyme A Dehydrogenase

Morten J. Corydon, N. Gregersen, W. Lehnert et al. · Pediatric Research · 1996 · 101 citations

Life Sciences, Biochemistry, Genetics +8

A new syndrome with ethylmalonic aciduria and normal fatty acid oxidation in fibroblasts

Alberto Burlina, Carlo Dionisi‐Vici, Michael J. Bennett et al. · The Journal of Pediatrics · 1994 · 75 citations

Life Sciences, Biochemistry, Cellular Metabolism +13

Demonstration of a new mammalian isoleucine catabolic pathway yielding an <i>R</i> series of metabolites

Orval Mamer, Susan Tjoa, C. R. Scriver et al. · Biochemical Journal · 1976 · 65 citations · Full text

Life Sciences, Biochemistry, Enzymology +15