Center for Bioinformatics
Oxidoreductases | Transferases | Hydrolases | Lyases | Isomerases | Ligases

Basic Information

Enzyme Number

4.1.1.28

Official Name

aromatic-L-amino-acid decarboxylase

Name from literature

aromatic amino acid decarboxylase

Pathway from literature

renal dopamine formation

Pathway from KEGG

Amino Acid Metabolism; Tryptophan metabolism; map00380

Amino Acid Metabolism; Histidine metabolism; map00340

Amino Acid Metabolism; Phenylalanine metabolism; map00360

Biosynthesis of Secondary Metabolites; Alkaloid biosynthesis I; map00950

Biosynthesis of Secondary Metabolites; Indole and ipecac alkaloid biosynthesis; map00901

Amino Acid Metabolism; Tyrosine metabolism; map00350

Organisms

Human (9606)

Genome localization

7p12.2[1644 ],

Comments

A pyridoxal-phosphate protein. The enzyme also acts on some other aromatic L-amino acids, including L-tryptophan.

Rate-limiting Description

"These results demonstrate that the A1 allele of DRD2 gene is associated with increased striatal activity of aromatic L-amino acid decarboxylase, the final enzyme in the biosynthesis of dopamine and the rate-limiting enzyme for trace amine (e.g." (15900211)

"In the 1950s it was found that an artificial aminoacid, 3,4-threo-dihydroxyphenylserine (DOPS), was converted to norepinephrine (NE) in a single step by the enzyme L-aromatic amino acid decarboxylase (AADC), bypassing the need for the rate limiting enzyme dopamine beta hydroxylase." (17017570)

Regulatory Information

Upstream transcription factor

2908

Regulatory type

Detail

feedback;

"When the aromatic L-amino acid decarboxylase (AADC) gene was added as a third gene, in an attempt to increase the conversion of L-DOPA to dopamine, feedback inhibition by the end product, dopamine, on TH activity resulted." (11436352)

key enzyme;

"We explore here the hypothesis that gene transfer of aromatic acid decarboxylase (AADC), a key enzyme in the pathway, will make neuronal cells more efficiently convert L-dopa into dopamine." (12938190)

transcriptional level;

"The neuronal promoter of the human aromatic L-amino acid decarboxylase (AADC) gene contains a perfectly palindromic element (TB) that conforms to the structure of a POU domain protein binding site of the MORE+2 type." (14741405#11168419)

transcriptional factor;NR3C1(2908)

"Isolation and identification of L-dopa decarboxylase as a protein that binds to and enhances transcriptional activity of the androgen receptor using the repressed transactivator yeast two-hybrid system." (12864730)

Gene ontology

Gene ontology

GO:0030170 (F) pyridoxal phosphate binding [P20711 ];
GO:0004058 (F) aromatic-L-amino-acid decarboxylase activity [P20711 ];
GO:0019752 (P) carboxylic acid metabolic process [P20711 ];
GO:0042423 (P) catecholamine biosynthetic process [P20711 ];
GO:0005515 (F) protein binding [P20711 ];

Disease relevance

Disease

Defects in DDC are the cause of aromatic L-amino-acid decarboxylase deficiency (AADCD) [MIM:608643]. AADCD deficiency is an inborn error in neurotransmitter metabolism that leads to combined serotonin and catecholamine deficiency. It causes developmental and psychomotor delay, poor feeding, lethargy, ptosis, intermittent hypothermia, gastrointestinal disturbances. The onset is early in infancy and inheritance is autosomal recessive [P20711 ];

Links

SwissProt

P20711

Entrez Gene

1644

HPRD

00145



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  Last Modified: 2009-03-24  
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