Metabolic Analysis Markers (Urine)

3-Hydroxyisovaleric Acid

A healthy result should fall into the range 0 - 29 mmol/mol creatinine.

3-Hydroxyisovaleric acid is a normal human metabolite excreted in the urine. It is a byproduct of the leucine degradation pathway. Production of 3-hydroxyisovaleric acid begins with the conversion of 3-methylcrotonyl-CoA into 3-methylglutaconyl-CoA in the mitochondria by the biotin-dependent enzyme methylcrotonyl-CoA carboxylase.

References:

http://www.hmdb.ca/metabolites/HMDB0000754

3-Hydroxyisovaleric Acid result calculator

insert the value from you 3-Hydroxyisovaleric Acid test result.

What does it mean if your 3-Hydroxyisovaleric Acid result is too high?

Biotin deficiencies, certain lifestyle habits (smoking), or specific genetic conditions can reduce methylcrotonyl-CoA carboxylase activity. This reduction can lead to a buildup of 3-methylcrotonyl-CoA, which is converted into 3-hydroxyisovaleryl-CoA by the enzyme enoyl-CoA hydratase. Increased concentrations of 3-methylcrotonyl-CoA and 3-hydroxyisovaleryl-CoA can lead to a disruption of the esterified CoA:free CoA ratio, and ultimately to mitochondrial toxicity. Detoxification of these metabolic end products occur via the transfer of the 3-hydroxyisovaleryl moiety to carnitine forming 3-hydroxyisovaleric acid-carnitine or 3HIA-carnitine, which is then transferred across the inner mitochondrial membrane where 3-hydroxyisovaleric acid is released as the free acid.

3-Hydroxyisovaleric acid has been found to be elevated in smokers and in subjects undergoing long-term anticonvulsant therapy with carbamazepine and/or phenytoin. These levels are elevated due to impairment of renal reclamation of biotin. Levels may also be increased from prolonged consumption of raw egg-whites. When present in sufficiently high levels, 3-hydroxyisovaleric acid can act as an acidogen and a metabotoxin. An acidogen is an acidic compound that induces acidosis, which has multiple adverse effects on many organ systems. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels.

Chronically high levels of 3-hydroxyisovaleric acid are associated with at least a dozen inborn errors of metabolism, including 3-hydroxy-3-methylglutaryl-CoA lyase deficiency, 3-methylglutaconic aciduria type I, biotinidase deficiency and isovaleric aciduria, dihydrolipoamide dehydrogenase deficiency, 3-methylcrotonyl-CoA carboxylase 1 deficiency, 3-hydroxy-3-methylglutaryl-CoA lyase deficiency, late-onset multiple carboxylase deficiency, holocarboxylase synthetase deficiency, and 3-methylcrotonyl-CoA carboxylase 2 deficiency.

Are you concerned about your lab report?
Select your plan, upload your lab reports and we’ll provide you with a meaningful interpretation.

Basic Interpretation
  • Instant interpretation
  • Use self upload form
  • 800+ biomarkers explained
  • Nutrition Recommendations
  • Cancel anytime
Full Interpretation
  • Instant interpretation
  • We upload 4 reports for you
  • 800+ biomarkers explained
  • Nutrition Recommendations
  • Cancel anytime
  • Online support
Own It For Life
  • Unlimited access
  • No subscription
  • Instant interpretations
  • We upload 10 reports for you
  • 800+ biomarkers explained
  • Nutrition Recommendations
  • Dedicated support

Test results don't lie. Learn what is happening inside your body.

Health is not exactly rocket science. You just have to know what your body needs and doesn't need. Your medical tests contain that information. Upload them to our service and we’ll help you understand, organize, and act on them.

Get Started

Medical results explained and interpreted.

WE SUPPORT 1000+ BIOMARKERS INCLUDING BIOMARKERS FROM YOUR FAVOURITE LABS

Healthmatters.io customer review

"I was really concerned about my lab results and my GP was not able to see me for another week. Through HealthMatters I was able to interpret my results instantly."

Healthmatters.io thankfull female customer

"I've been keeping all my lab results in a spreadsheet, covering approximately 20 years. The HealthMatters team helped me upload that info into the cloud. I can show this data to my family and doctors now easily."

Healthmatters.io thankful young female customer

"I had no idea what my lab results meant. Here I found all the biomarkers that I was looking for online. Finally, I have not only organized them all neatly in one place, but I am also grateful to be able to understand what they mean."



Pricing
Privacy and security of personal data. HIPAA and GDPR compliant badges.

Your Privacy And Security

At HealthMatters, we're committed to maintaining the security and confidentiality of your personal information. We've put security measures in place to help protect against the loss, misuse or alteration of information under our control. We use procedural, physical and electronic security methods designed to prevent people who aren't authorized from getting access to this information. Our internal code of conduct adds additional privacy protection. See our Privacy Policy for more information.