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Isobutyrylglycine
Please note: the following summary based on Human Metabolic Database from www.hmdb.ca
Isobutyrylglycine is identified in large amount in urine of patients with isobutyryl-CoA dehydrogenase deficiency
Isobutyryl-CoA dehydrogenase deficiency is a disorder caused by the deficiency of isobutyryl-CoA dehydrogenase that is involved in the catabolism of the branched-chain amino acid valine (PMID 15505379).

SpecimenNormalAbnormalSeverityAssociated with diseases
urine0.50-3.80 mmol/mol creatinine4.00-48.00 mmol/mol creatinineETHYLMALONIC ENCEPHALOPATHY (EPEMA)
urine0.50-3.80 mmol/mol creatinine5.00-116.00 mmol/mol creatinine
urine0.50-3.80 mmol/mol creatinine0.00-200.00 mmol/mol creatinineGLUTARIC ACIDURIA II
urine - - increasedISOBUTYRYL-COA DEHYDROGENASE DEFICIENCY

Associated Dissorders based on hmdb
Please note: the following summary based on Human Metabolic Database From www.hmdb.ca
  1. Epema syndrome (ENCEPHALOPATHY, PETECHIAE, ETHYLMALONIC ACIDURIA) Metagene:274
  2. Glutaric aciduria ii Metagene:9
  3. Isobutyryl-coa dehydrogenase deficiency Metagene:509