2-Oxoisovalerate

ChemistryAlso known as: 2-oxo-isovaleric acidAlso known as: α-ketoisovaleric acidAlso known as: KIVAN/A

Measures levels of an organic acid in serum or plasma from branched-chain amino acid breakdown.

Normal Range

Consult your provider

N/A

Abnormal Levels

Consult your provider

Specimen Type

Blood

Why This Biomarker Matters

Elevated levels indicate possible metabolic disorders affecting amino acid processing. Early diagnosis enables dietary and medical management to prevent neurological damage.

Overview

2-Oxoisovalerate is an organic acid intermediate produced during branched-chain amino acid metabolism. Its presence in urine at elevated levels may indicate disorders of amino acid metabolism, including maple syrup urine disease and other inherited metabolic conditions. This urine test is used for newborn screening and evaluating patients with symptoms like poor feeding, lethargy, or developmental delay. Detection allows early intervention to prevent serious health complications.

Technical Information (LOINC Codes)

Standardized laboratory codes for this biomarker

35868-9Primary

2-Oxoisovalerate [Moles/volume] in Serum or Plasma

umol/L

29866-1

2-Oxoisovalerate [Presence] in Urine

Available Lab Tests

Order tests that measure this biomarker

Urinary Organic Acids

$586.08

Results in 7-14 business days