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Literature summary extracted from

  • Allen, G.F.; Land, J.M.; Heales, S.J.
    A new perspective on the treatment of aromatic L-amino acid decarboxylase deficiency (2009), Mol. Genet. Metab., 97, 6-14.
    View publication on PubMed

Inhibitors

EC Number Inhibitors Comment Organism Structure
4.1.1.28 Benserazide competitive inhibitor of L-Dopa, but a non-competitive inhibitor of 5-hydroxytryptophan Homo sapiens
4.1.1.28 carbidopa
-
 Homo sapiens
4.1.1.28 NSD-1015
-
 Homo sapiens

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
4.1.1.28 5-Hydroxytryptophan Homo sapiens
-
 Serotonin + CO2
-
 ?
4.1.1.28 L-Dopa Homo sapiens
-
 dopamine + CO2
-
 ?
4.1.1.28 L-phenylalanine Homo sapiens
-
 2-phenylethylamine + CO2
-
 ?
4.1.1.28 L-Trp Homo sapiens
-
 Tryptamine + CO2
-
 ?
4.1.1.28 m-tyrosine Homo sapiens
-
 m-tyramine + CO2
-
 ?
4.1.1.28 p-tyrosine Homo sapiens
-
 p-tyramine + CO2
-
 ?

Organism

EC Number Organism UniProt Comment Textmining
4.1.1.28 Homo sapiens
-
-
-

Source Tissue

EC Number Source Tissue Comment Organism Textmining
4.1.1.28 brain
-
 Homo sapiens
-
4.1.1.28 striatum
-
 Homo sapiens
-

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
4.1.1.28 5-Hydroxytryptophan
-
 Homo sapiens Serotonin + CO2
-
 ?
4.1.1.28 L-Dopa
-
 Homo sapiens dopamine + CO2
-
 ?
4.1.1.28 L-phenylalanine
-
 Homo sapiens 2-phenylethylamine + CO2
-
 ?
4.1.1.28 L-Trp
-
 Homo sapiens Tryptamine + CO2
-
 ?
4.1.1.28 m-tyrosine
-
 Homo sapiens m-tyramine + CO2
-
 ?
4.1.1.28 p-tyrosine
-
 Homo sapiens p-tyramine + CO2
-
 ?

Synonyms

EC Number Synonyms Comment Organism
4.1.1.28 AADC
-
 Homo sapiens
4.1.1.28 DOPA decarboxylase
-
 Homo sapiens

Cofactor

EC Number Cofactor Comment Organism Structure
4.1.1.28 pyridoxal 5'-phosphate the essential cofactor stabilizes and enhances enzymatic activity Homo sapiens

General Information

EC Number General Information Comment Organism
4.1.1.28 malfunction AADC deficiency is an autosomal recessive disorder caused by mutations in the AADC gene leading to severely reduced AADC activity. The condition presents early in life and is characterized by axial hypotonia, hypokinesia, choreoathetosis, developmentaldelay and episodes of dystonia, limb hypertonia and oculogyric crises Homo sapiens