2-Methylbutyrylglycine

ChemistryAlso known as: 2-methylbutyryl glycineAlso known as: Isobutyryl glycineN/A

Detects presence of 2-methylbutyrylglycine in serum or plasma as a metabolic marker.

Normal Range
Consult your provider
N/A
Abnormal Levels
Consult your provider
Specimen Type
Blood

Why This Biomarker Matters

Abnormal levels may indicate a rare genetic metabolic disorder requiring specialized treatment. Early detection through screening allows for dietary management and prevention of serious neurological complications.

Overview

2-Methylbutyrylglycine is an organic compound found in urine that may indicate metabolic dysfunction, particularly related to amino acid and fatty acid breakdown. Elevated levels can suggest certain genetic metabolic disorders affecting how your body processes amino acids. This qualitative test is typically ordered when metabolic disease is suspected, particularly in newborn screening programs or when investigating unexplained developmental delays, muscle weakness, or other metabolic symptoms.

Technical Information (LOINC Codes)

Standardized laboratory codes for this biomarker

69790-4Primary
2-Methylbutyrylglycine [Moles/volume] in Serum or Plasma
umol/L
29884-4
2-Methylbutyrylglycine [Presence] in Urine

Available Lab Tests

Order tests that measure this biomarker