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Literature summary for 4.1.1.28 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

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

Natural Substrates/ Products (Substrates)

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

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
brain
-
Homo sapiens
-
striatum
-
Homo sapiens
-

Substrates and Products (Substrate)

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

Synonyms

Synonyms Comment Organism
AADC
-
Homo sapiens
DOPA decarboxylase
-
Homo sapiens

Cofactor

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

General Information

General Information Comment Organism
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