Isobutyrylglycine
ChemistryAlso known as: N-isobutyrylglycineN/A
Qualitatively detects isobutyrylglycine in urine to screen for amino acid metabolism disorders.
Normal Range
Consult your provider
N/A
Abnormal Levels
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Specimen Type
Blood
Why This Biomarker Matters
Early detection of amino acid metabolism disorders allows immediate intervention to prevent intellectual disability, organ damage, and metabolic crises.
Overview
Isobutyrylglycine is an organic acid produced during amino acid metabolism. Its qualitative detection in urine may indicate a rare metabolic disorder affecting protein breakdown. Presence of this compound can suggest isobutyryl-CoA dehydrogenase deficiency or related amino acid metabolism disorders. This test is typically ordered during newborn screening or when metabolic disorders are suspected.
Technical Information (LOINC Codes)
Standardized laboratory codes for this biomarker
29873-7PrimaryIsobutyrylglycine [Presence] in Urine
54304-1Isobutyrylglycine [Moles/volume] in Urine
umol/L
Available Lab Tests
Order tests that measure this biomarker