3-Methylglutaconate

ChemistryAlso known as: 3-methylglutaconic acidAlso known as: methylglutaconic acidN/A

Qualitatively detects 3-methylglutaconate organic acid in your urine.

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

Why This Biomarker Matters

Presence of this acid suggests a rare metabolic disorder affecting amino acid breakdown, requiring specialized diagnosis and treatment to prevent neurological and muscular complications.

Overview

3-methylglutaconate is an organic acid that may appear in urine when metabolism is disrupted. This qualitative test identifies its presence or absence. Elevated urinary levels may indicate 3-methylglutaconic aciduria, a rare metabolic disorder affecting the breakdown of leucine and other amino acids. This condition can affect muscle function and neurological development. The test is part of screening for inborn errors of metabolism, especially in newborns or children with developmental concerns.

Technical Information (LOINC Codes)

Standardized laboratory codes for this biomarker

33273-4Primary
3-Methylglutaconate [Moles/volume] in Serum or Plasma
nmol/L
29629-3
3-Methylglutaconate [Presence] in Urine
72440-1
3-Methylglutaconate [Moles/volume] in Cerebral spinal fluid
umol/L

Available Lab Tests

Order tests that measure this biomarker