3-Methylcrotonylglycine

ChemistryAlso known as: 3-methylcrotonyl glycineAlso known as: 3-methylcrotonoyl glycineN/A

Detects 3-methylcrotonylglycine in serum or plasma, indicating leucine metabolism disorder.

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

Why This Biomarker Matters

Presence suggests branched-chain amino acid metabolism disorder requiring specialized dietary management and monitoring to prevent serious metabolic complications.

Overview

3-Methylcrotonylglycine is a metabolite detected in urine that indicates abnormal metabolism of the amino acid leucine. Elevated levels suggest possible 3-methylcrotonyl-CoA carboxylase deficiency, a genetic metabolic disorder affecting how your body processes branched-chain amino acids. This condition can cause developmental delay, low muscle tone, and metabolic acidosis if untreated. Early detection through newborn screening allows for dietary management. This test is part of comprehensive metabolic screening, especially in newborns.

Research & Evidence

10 publications

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Research data from MEDLINE/PubMed · 10 articles

Technical Information (LOINC Codes)

Standardized laboratory codes for this biomarker

41039-9Primary
3-Methylcrotonylglycine [Moles/volume] in Serum or Plasma
nmol/mL
29628-5
3-Methylcrotonylglycine [Presence] in Urine
40848-4
3-Methylcrotonylglycine [Mass/time] in 24 hour Urine
ug/(24.h)
40927-6
3-Methylcrotonylglycine [Moles/volume] in Blood
nmol/mL
72441-9
3-Methylcrotonylglycine [Moles/volume] in Amniotic fluid
umol/L
72442-7
3-Methylcrotonylglycine [Moles/volume] in Cerebral spinal fluid
umol/L

Available Lab Tests

Order tests that measure this biomarker

3-Methylcrotonylglycine: Normal Range, Testing & Clinical Significance | Healos | Healos