3-Hydroxy-3-methylglutaric is the precursor of coenzyme Q10 and cholesterol. Slightly increased values may be caused by gastrointestinal yeast overgrowth. A moderate increase in urine HMG may also indicate decreased synthesis of coenzyme Q10. Certain cholesterol-lowering drugs may inhibit the synthesis pathway and result in high HMG values. Very elevated values may be caused by the genetic disorder, HMG aciduria.
Low levels of hydroxymethylglutarate may occur if there are low levels of precursors (amino acids, pyruvate, butyrate), if there are nutritional enzyme inhibitions, or if a low-activity enzyme variant is inherited. The enzymes that synthesize hydroxymethylglutarate are located in the liver and pancreas and these enzymes have no known nutritional cofactors. Hydroxymethylglutarate may be synthesized from amino acid metabolism, from pyruvate metabolism or from butyrate metabolism. Hydroxymethylglutarate can be used in both fatty acid breakdown and is an essential step in ketogenesis. Low levels of hydroxymethylglutarate may inhibit CoQ10 synthesis.
- Consider CO-Q10 supplementation if indicated.
- Consider supporting the movement of hydroxymethylglutarate between the mitochondrial matrix and the cell cytoplasm with L-carnitine.
- If levels of adipate and suberate are also high, there may be inherited or acquired problems with liver beta-oxidation; consider sulforaphane to support beta-oxidation.
Understand and improve your laboratory results with our health dashboard.
Upload your lab reports and get your interpretation today.
Our technology helps to understand, combine, track, organize, and act on your medical lab test results.
- Precursor of CoQ10.
- Slightly elevated levels suggest yeast overgrowth.
- Elevated levels may also indicate decreased CoQ10 synthesis. Very elevated levels may be due to HMG aciduria.
--------------------------------------------------------------------
High 3-hydroxy-3-methylglutaric acid is seen in the genetic disease 3-hydroxy 3-methylglutaric aciduria.
Typical values observed in the genetic disease are 200-11,000 mmol/mol creatinine.
The cause of less significant increases in this urinary metabolite is unknown.
3-Hydroxy-3-methylglutaric aciduria may cause:
- vomiting,
- lethargy,
- hypotonia,
- and apnea,
...sometimes evolving to coma.
Laboratory tests reveal metabolic acidosis with severe hypoketotic hypoglycemia on fasting or during acute illness, hyperammonemia, and abnormal liver function.
Preliminary diagnosis is based on a pattern of organic acids in urine which includes:
- 3-hydroxy-3-methylglutaric,
- 3-hydroxyisovaleric,
- 3-methylglutaconic,
- 3-methylglutaric,
and 3-methylcrotonic acids.
Because yeast also produces this compound and yeast metabolites are frequently elevated along with this compound; slight increases may be yeast-related. Reduced activity of 3-hydroxy 3-methylglutaryl Co A reductase, a critical enzyme at the beginning of the cholesterol synthesis pathway, may also elevate this compound. Check cholesterol values when this compound is elevated up to 300 mmol/mol creatinine.
Slight elevations may result from coenzyme Q10 deficiency. Supplementation with coenzyme Q10 at 50 - 120 mg/day may be beneficial.
Interpret Your Lab Results
Upload your lab report, and we'll interpret and provide you with recommendations today.
Get StartedMonthly plan
Annual plan
Own it for life
Our exclusive data entry service is a convenient way to get your results into your private dashboard. Simply attach an image or a file of your lab test results, and one of our qualified data entry team members will add the results for you. We support all sorts of files, whether PDFs, JPGs, or Excel. This service is excellent whether you have a lot of reports to upload or are too busy to do the data entry yourself.
We strive to make the data entry process easy for you. Whether by offering dozens of templates to choose from that pre-populate the most popular laboratory panels or by giving you instant feedback on the entered values. Our data entry forms are an easy, fast, and convenient way to enter the reports yourself. There is no limit on how many lab reports you can upload.
$15 /month
billed every month
Most popular
Data entry included
$79 /year
$6.60/month billed annually
Data entry included
$250 /once
own it for life
Are You a Health Professional?
Get started with our professional plan
Welcome to Healthmatters Pro.
Save time on interpreting lab results with the largest database of biomarkers online. In-depth research on any test at your fingertips, all stored and tracked in one place. Learn more
Pro Monthly Plus
for health professionals
$75 per month
At HealthMatters, we're committed to maintaining the security and confidentiality of your personal information. We've put industry-leading security standards in place to help protect against the loss, misuse, or alteration of the information under our control. We use procedural, physical, and electronic security methods designed to prevent unauthorized people from getting access to this information. Our internal code of conduct adds additional privacy protection. All data is backed up multiple times a day and encrypted using SSL certificates. See our Privacy Policy for more details.
2-Hydroxybutyric acid, 2-Hydroxyhippuric acid, 2-Hydroxyisocaproic acid, 2-Hydroxyisovaleric acid, 2-Hydroxyphenylacetic acid, 2-Oxo-4-methiolbutyric acid, 2-Oxoglutaric acid, 2-Oxoisocaproic acid, 2-Oxoisovaleric, 3-Hydroxy-3-methylglutaric, 3-Hydroxybutyric acid, 3-Hydroxyglutaric acid, 3-Indoleacetic acid (IAA), 3-Methyl-2-oxovaleric acid, 3-Methylglutaconic, 3-Methylglutaric acid, 3-Oxoglutaric acid, 4-Cresol, 4-Hydroxybenzoic acid, 4-Hydroxybutyric acid, 4-Hydroxyhippuric acid, 4-Hydroxyphenylacetic acid, 4-Hydroxyphenyllactic acid, 5-Hydroxyindoleacetic acid (5-HIAA), 5-Hydroxymethyl-furoic acid, Acetoacetic acid, Aconitic acid, Adipic acid, Arabinose, Ascorbic acid (Vitamin C), Carboxycitric acid, Citramalic acid, Citric acid, Creatinine, DHPPA (dihydroxyphenylpropionic acid), Dihydroxyphenylacetic acid (DOPAC), Ethylmalonic acid, Fumaric acid, Furan-2,5-dicarboxylic acid, Furancarbonylglycine, GABA, Glutaric acid (Vitamin B2), Glyceric acid, Glycolic acid, Hippuric acid, Homogentisic acid, Homovanillic acid (HVA), HPHPA (3-(3-hydroxyphenyl)-3-hydroxypropionic acid), HVA/DOPAC, HVA/DOPAC Ratio, Kynurenic acid, Lactic acid, Malic acid, Malonic acid, Mandelic acid, Methylcitric acid (Vitamin H), Methylmalonic acid (Vitamin B12), Methylsuccinic acid, N-Acetylaspartic acid, N-Acetylcysteine acid, Orotic acid, Oxalic acid, Pantothenic acid (Vitamin B5), Phenyllactic acid, Phenylpyruvic acid, Phosphoric acid, Pyridoxic acid (Vitamin B6), Pyroglutamic acid, Pyruvic acid, Quinolinic acid, Quinolinic acid/5-HIAA, Sebacic acid, Suberic acid, Succinic acid, Tartaric acid, Thymine, Tricarballyic acid, Uracil