Aromatic L-amino acid decarboxylase

Aromatic L-amino acid decarboxylase (AADC) is an enzyme that plays a crucial role in the biochemical processes of the human body. It is responsible for the decarboxylation of aromatic L-amino acids, which are essential for the synthesis of several important neurotransmitters and hormones.

Function

AADC is involved in the synthesis of several key neurotransmitters and hormones. It catalyzes the decarboxylation of aromatic L-amino acids, specifically L-DOPA to dopamine, 5-HTP to serotonin, and L-tryptophan to tryptamine. These neurotransmitters and hormones play vital roles in various physiological functions, including mood regulation, motor control, and the body's response to stress.

Structure

The AADC enzyme is a dimer composed of two identical subunits. Each subunit contains a pyridoxal phosphate (PLP) molecule, which is essential for the enzyme's catalytic activity. The PLP molecule forms a Schiff base with a lysine residue in the enzyme, which is crucial for the decarboxylation reaction.

Clinical significance

Deficiency in AADC is a rare genetic disorder known as Aromatic L-amino acid decarboxylase deficiency (AADC deficiency). This condition is characterized by severe developmental delays, movement disorders, and autonomic dysfunction. It is caused by mutations in the DDC gene, which encodes the AADC enzyme.

See also

• Decarboxylation
• Neurotransmitter
• Aromatic L-amino acid decarboxylase deficiency