1.4.c

Disorders of ketone body metabolism

  • Mitochondrial 3-hydroxy-3-methylglutaryl-CoA synthase deficiency
  • Succinyl-CoA:3-oxoacid-CoA transferase deficiency
  • Mitochondrial acetoacetyl-CoA thiolase deficiency (β-ketothiolase deficiency; α-methylacetoacetic aciduria)
  • Cytosolic acetoacetyl-CoA thiolase deficiency
  • 3-hydroxy-3-methylglutaryl-CoA lyase deficiency (Hydroxymethylglutaric aciduria)
  • Monocarboxylate transporter 1 deficiency
  • Monocarboxylate transporter 1 superactivity

Subnetworks

cfao 1

C-FAO

Carbohydrate, fatty acid oxidation and ketone bodies disorders

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