Organic Acids

2-Hydroxyisovaleric acid

Optimal result: 0 – 0.4 mmol/mol

Vibrant Wellness

By Healthmatters Editorial Team

Peer-reviewed sources

Moderate increase may result from:

- lactic acidosis

- episodic ketosis

- thiamine/lipoic acid deficiency

Patients with slight to moderate elevations may use dietary supplements containing thiamine to improve clinical symptoms.

Significant elevations are associated with:

- A significant increase of branched-chain amino acid metabolites is associated with the genetic disorders, maple syrup urine disease (MSUD) and pyruvate dehydrogenase deficiency.

- Elevated levels have been linked to short bowel syndrome.

2-Hydroxyisovaleric acid (aka 2-Hydroxy-3-methylbutyric acid) is a branched-chain amino acid metabolite.

2-hydroxy-3-methylbutyric acid is a valine derivative that is valine in which the amino group has been replaced by a ‘hydroxy’ group.

A moderate increase of branched-chain amino acid metabolites in urine may result from lactic acidosis, episodic ketosis, or deficiencies of the vitamins, thiamine or lipoic acid. A significant increase of branched-chain amino acid metabolites is associated with the genetic disorders, maple syrup urine disease (MSUD) and pyruvate dehydrogenase deficiency. Patients with slight to moderate elevations may use dietary supplements containing thiamine to improve clinical symptoms.

2-Hydroxyisovaleric acid (aka 2-Hydroxy-3-methylbutyric acid) is a branched-chain amino acid metabolite.

2-hydroxy-3-methylbutyric acid is a valine derivative that is valine in which the amino group has been replaced by a ‘hydroxy’ group.

Organize and understand all your lab results

Start organizing — $15/mo →

For Health Professionals Healthmatters Pro Multiple clients’ results in one dashboard. See Pro plans →

2-Hydroxyisovaleric acid

Every lab result you’ve ever had, charted and explained.

Healthmatters is a tracker for your lab results. Upload a report from any lab, any country — every marker charted and explained.

Start with my report →

Clinical Sources & References

All our content is backed by peer-reviewed studies, academic research, and trusted medical sources. We’re committed to accuracy and transparency — see our editorial policy for details.

Article Review Log

Written by Healthmatters Editorial Team

Organize and understand all your lab results

Start organizing — $15/mo →

For Health Professionals Healthmatters Pro Multiple clients’ results in one dashboard. See Pro plans →

Healthmatters Lifetime Plan

Pay $250. Track your health forever

One payment. No renewals. Yours for life.

10,000+ biomarkers explained · trends over time

See other plans →

Secure checkout via Stripe · GDPR & HIPAA

We accept results from any lab

LabCorp Quest Diagnostics Genova Diagnostics DUTCH Test Doctor's Data Vibrant America ZRT Laboratory Cyrex Labs + any other lab

What members are saying

★★★★★ 10,000+ happy members

★★★★★

"Watching my numbers move over the years — instead of forgetting last time's results — completely changed how I manage my health."

Daniel

Member since 2022

★★★★★

"Living across several countries made keeping my lab results organized a real challenge. Healthmatters brought my results from the USA, Spain, and Germany together — 20 years of history, with long-term trends instead of scattered reports."

Ben

Member since 2021 · tracks across countries

★★★★★

"Standard lab ranges are too wide for how I practice — 'normal' isn't optimal. I set my own bands, so a client sees yellow before a marker goes officially out of range. That's the conversation I want to be having."

Dr. Alexandra

Functional medicine · 6 yrs on Pro