β-hydroxy-β-methylglutaric acid (HMG) is a precursor to cholesterol and coenzyme Q10 (CoQ10) synthesis. It is a product of hydroxymethylglutaryl-coenzyme A (HMGCoA). HMGCoA- reductase is a rate limiting enzyme in cholesterol production. Medications that interfere with this enzyme may result in elevated HMG and subsequent low levels of cholesterol and CoQ10. CoQ10 is important for cellular energy production in the mitochondrial respiratory chain.
- Rundek T, Naini A, Sacco R, Coates K, DiMauro S. Atorvastatin Decreases the Coenzyme Q10 Level in the Blood of Patients at Risk for Cardiovascular Disease and Stroke. Arch Neurol. 2004;61(6):889-892.
- Qu H, Guo M, Chai H, Wang Wt, Gao Zy, Shi Dz. Effects of Coenzyme Q10 on statin-Induced Myopathy: An Updated Meta- Analysis of Randomized Controlled Trials. J Am Heart Assoc. 2018;7(19):e009835.
- Wortmann SB, Kluijtmans LA, Engelke UFH, Wevers RA, Morava E. The 3-methylglutaconic acidurias: what's new? J Inher Metab Dis. 2012;35(1):13-22.
Urinary β-hydroxy-β-methylglutaric acid is often elevated in patients taking statin medications and red yeast rice. CoQ10 supplementation has been shown to help ameliorate statin-associated myopathies.
There are also inborn errors of metabolism which can elevate HMG. These affect the HMGCoA reductase enzyme with varying degrees of onset and clinical manifestations such as neurodevelopmental disorders and cardiomyopathy.
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