1.1.b

Organic acidurias

  • Isobutyryl-CoA dehydrogenase deficiency
  • 2-Methylbutyryl-CoA dehydrogenase deficiency (Short/branched-chain acyl-CoA dehydrogenase deficiency; 2-methylbutyrylglycinuria)
  • 3-Methylcrotonyl-CoA carboxylase 1 deficiency (3-methylcrotonylglycinuria type 1)
  • 3-Methylcrotonyl-CoA carboxylase 2 deficiency (3-methylcrotonylglycinuria type 2)
  • 3-methylglutaconyl-CoA hydratase deficiency (3-methylglutaconic aciduria type 1)
  • Mitochondrial short-chain enoyl-CoA hydratase 1 deficiency (Crotonase deficiency)
  • 3-hydroxyisobutyryl-CoA hydrolase deficiency (β-hydroxyisobutyryl-CoA deacylase deficiency)
  • 3-hydroxyisobutyrate dehydrogenase deficiency
  • Methylmalonate semialdehyde dehydrogenase deficiency
  • Propionic acidemia due to propionyl-CoA carboxylase subunit alpha deficiency
  • Propionic acidemia due to propionyl-CoA carboxylase subunit beta deficiency
  • Methylmalonic aciduria due to methylmalonyl-CoA epimerase deficiency
  • Methylmalonic aciduria due to methylmalonyl-CoA mutase deficiency
  • Glutaryl-CoA dehydrogenase deficiency (Glutaric acidemia type 1)
  • Succinate-hydroxymethylglutarate-CoA transferase deficiency (Glutaric acidemia type 3)
  • Malonyl-CoA decarboxylase deficiency(Malonic aciduria)

Subnetworks

AOA 1

AOA

Aminoacid and organic acids related disorders

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