Aromatic L-amino acid decarboxylase
Aromatic L-amino acid decarboxylase[edit | edit source]
Aromatic L-amino acid decarboxylase (AADC), also known as DOPA decarboxylase, is an enzyme that catalyzes the decarboxylation of aromatic L-amino acids to produce their corresponding amines. This enzyme is crucial in the biosynthesis of several important neurotransmitters, including dopamine, serotonin, and histamine.
Function[edit | edit source]
Aromatic L-amino acid decarboxylase is a pyridoxal phosphate-dependent enzyme that plays a key role in the central nervous system and peripheral nervous system. It is responsible for converting L-DOPA to dopamine, 5-HTP to serotonin, and histidine to histamine. These neurotransmitters are vital for various physiological functions, including mood regulation, sleep, and the modulation of the immune response.
Structure[edit | edit source]
AADC is a homodimeric enzyme, meaning it consists of two identical subunits. Each subunit binds one molecule of pyridoxal phosphate, which is essential for its enzymatic activity. The enzyme's active site is located at the interface of the two subunits, where the decarboxylation reaction takes place.
Clinical significance[edit | edit source]
Deficiencies in AADC activity can lead to a rare genetic disorder known as AADC deficiency. This condition is characterized by a lack of dopamine and serotonin production, leading to severe developmental and neurological symptoms. Patients with AADC deficiency may experience developmental delays, movement disorders, and autonomic dysfunction.
Genetic aspects[edit | edit source]
The gene encoding AADC is located on chromosome 7 in humans. Mutations in this gene can lead to AADC deficiency. Genetic testing can identify these mutations, which is crucial for the diagnosis and management of the disorder.
Related pages[edit | edit source]
Gallery[edit | edit source]
Apple Day singing, Chepstow Drill Hall 2014
Aromatic L-amino acid decarboxylase[edit | edit source]
DOPA decarboxylase dimer structure
Crystal structure of aromatic L-amino acid decarboxylase
Mechanism of action of aromatic L-amino acid decarboxylase
Pathway of serotonin biosynthesis
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