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β-Hydroxybutyrate dehydrogenase (BDH1) !

This enzyme turns acetoacetate into β-hydroxybutyrate in the liver, and back again in tissues that burn ketones.

Updated 2026-11-01

Key facts

  • Acetoacetate + NADH ⇌ D-β-hydroxybutyrate + NAD⁺; reversible.
  • Sits on the inner mitochondrial membrane and needs phosphatidylcholine to work.
  • The β-hydroxybutyrate/acetoacetate ratio mirrors mitochondrial NADH/NAD⁺ (normally about 3:1).
Also called
D-3-hydroxybutyrate dehydrogenase
EC number
1.1.1.30
Genes
BDH1

Regulation

  • No major allosteric control: it follows the redox state (NADH/NAD⁺).
  • Alcohol and hypoxia raise NADH and shift towards β-hydroxybutyrate.

Inhibitors and toxins

  • No inhibitors in clinical use

Clinical notes

  • In diabetic and alcoholic ketoacidosis the ratio climbs to 10:1, and the nitroprusside strip (which measures acetoacetate) underestimates ketones.
  • During ketoacidosis treatment β-hydroxybutyrate turns back into acetoacetate and the strip can look more positive even as the patient improves.

Pathways

Test yourself

Think of the answer, then tap the question to see it.

What sets the ratio of β-hydroxybutyrate to acetoacetate?

The mitochondrial NADH/NAD⁺ ratio, because the reaction is reversible and near equilibrium.

Why can the urine strip become more positive while ketoacidosis is being treated?

β-Hydroxybutyrate, which the strip misses, is converted back into acetoacetate, which it detects.

Which membrane lipid does β-hydroxybutyrate dehydrogenase need?

Phosphatidylcholine of the inner mitochondrial membrane.

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

Sources: BDH1: D-beta-hydroxybutyrate dehydrogenase, mitochondrial (human) (UniProt, 2026); Synthesis of Ketone Bodies (Reactome, 2026); Utilization of Ketone Bodies (Reactome, 2026).

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Representative model, for educational purposes only.