title: A defect in l-isoleucine metabolism associated with alpha-methyl-beta-hydroxybutyric and alpha-methylacetoacetic aciduria: quantitative in vivo and in vitro studies.
mesh_terms: D000090:Acetoacetates / Q000652:urine*; D000293:Adolescent; D000592:Amino Acid Metabolism, Inborn Errors / Q000235:genetics / Q000378:metabolism*; D001360:Avidin / Q000378:metabolism; D002087:Butyrates / Q000378:metabolism; D002250:Carbon Radioisotopes; D002648:Child; D002849:Chromatography, Gas; D003065:Coenzyme A / Q000378:metabolism; D005260:Female; D005998:Glycine / Q000031:analogs & derivatives / Q000378:metabolism; D006801:Humans; D006885:Hydroxybutyrates / Q000652:urine*; D007532:Isoleucine / Q000378:metabolism*; D007962:Leukocytes / Q000378:metabolism; D008297:Male; D013058:Mass Spectrometry; D011422:Propionates / Q000378:metabolism; D013997:Time Factors; D014316:Tritium
chemical_list: D000090:Acetoacetates; D002087:Butyrates; D002250:Carbon Radioisotopes; D006885:Hydroxybutyrates; D011422:Propionates; D007532:Isoleucine; D014316:Tritium; D001360:Avidin; D003065:Coenzyme A; D005998:Glycine