Mevalonate !

Mevalonate is the product of HMG-CoA reductase: a six-carbon compound that commits carbon to cholesterol and isoprenoids.

Updated 2026-11-01

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Key facts

It is (R)-mevalonic acid, six carbons; in the body it is ionised as mevalonate.
It is made in the rate-limiting step of cholesterol synthesis, the one statins block.
Three phosphorylations (3 ATP) and one decarboxylation turn it into isopentenyl pyrophosphate (C5).

A carboxylic acid with two OH groups (C3 and C5) and a methyl on C3; it can close into a lactone (mevalonolactone).
Only the R enantiomer is active in the pathway.

In mevalonate kinase deficiency mevalonate builds up and spills into the urine (mevalonic aciduria).
Its non-sterol products (dolichol, ubiquinone, prenyl groups) explain statin effects beyond cholesterol.

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Which enzyme makes mevalonate?

HMG-CoA reductase, from HMG-CoA and 2 NADPH.

How much ATP is spent going from mevalonate to isopentenyl pyrophosphate?

Three ATP: two phosphorylations and a decarboxylation that also uses ATP.

What builds up in the urine in mevalonic aciduria?

Mevalonic acid, because mevalonate kinase is missing.

Molecules are shown in their neutral form, as the databases deposit them; in the cell the acids and phosphates are ionised.

Educational information, not medical advice. For any symptom, see a health professional.

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Representative model, for educational purposes only.