- 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. 

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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.