2-Hydroxyisovalerate

ChemistryAlso known as: 2oh-isovaleric acidAlso known as: 2-hydroxyisovaleric acidN/A

Measures 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 suggest serious inherited metabolic disorders. Early detection enables immediate dietary management and treatment to prevent irreversible neurological damage.

Overview

2-Hydroxyisovalerate is an organic acid produced during branched-chain amino acid metabolism. Its presence in urine can indicate metabolic disorders such as maple syrup urine disease, where the body cannot properly break down certain amino acids found in protein. These inherited conditions can cause developmental delays, seizures, and neurological damage if undetected. This urine test is a key component of newborn screening programs and helps identify at-risk individuals early for immediate intervention.

Technical Information (LOINC Codes)

Standardized laboratory codes for this biomarker

69781-3Primary

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

umol/L

29882-8

2-Hydroxyisovalerate [Presence] in Urine

69780-5

2-Hydroxyisovalerate [Moles/volume] in Cerebral spinal fluid

umol/L

Available Lab Tests

Order tests that measure this biomarker

Urinary Organic Acids

$586.08

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