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Aromatic L-amino acid decarboxylase is active as a homodimer. Before addition of the pyridoxal phosphate cofactor, the apoenzyme exists in an open conformation. Upon cofactor binding, a large structural transformation occurs as the subunits pull closer and close the active site. This conformational change results in the active, closed ...
Biosynthesis and breakdown of serotonin and the catecholamines, and the metabolic block in aromatic L-amino acid decarboxylase deficiency, Wassenberg et al., 2017. [1] Babies with severe aromatic L-amino acid decarboxylase deficiency usually present to clinicians during the first few months of life. Symptoms can include: Hypotonia (floppiness)
l-DOPA is produced from the amino acid l-tyrosine by the enzyme tyrosine hydroxylase. l-DOPA can act as an l-tyrosine mimetic and be incorporated into proteins by mammalian cells in place of l-tyrosine, generating protease-resistant and aggregate-prone proteins in vitro and may contribute to neurotoxicity with chronic l-DOPA administration. [10]
Autosomal dominant Segawa syndrome (the autosomal recessive form of Segawa syndrome is caused by mutations in a different gene that encodes tyrosine hydroxylase), Dopa-responsive dystonia 5a, Autosomal dominant DYT/PARK-GCH1 (designation in accordance with the Nomenclature of Genetic Movement Disorders maintained by the International Parkinson ...
Once levodopa has entered the central nervous system, it is converted into dopamine by the enzyme aromatic l-amino acid decarboxylase (AAAD), also known as DOPA decarboxylase (DDC). Pyridoxal phosphate (vitamin B 6) is a required cofactor in this reaction, and may occasionally be administered along with levodopa, usually in the form of pyridoxine.
Entacapone is a widely used adjunct drug of levodopa therapy. When given with an inhibitor of dopa decarboxylase (carbidopa or benserazide), levodopa is optimally saved. This "triple therapy" is becoming a standard in the treatment of Parkinson's disease. Specific reactions catalyzed by COMT include: L-DOPA (levodopa) → 3-O-methyldopa
Malonic aciduria or malonyl-CoA decarboxylase deficiency (MCD) is an autosomal-recessive [1] metabolic disorder caused by a genetic mutation that disrupts the activity of Malonyl-CoA decarboxylase. This enzyme breaks down Malonyl-CoA (a fatty acid precursor and a fatty acid oxidation blocker) into acetyl-CoA and carbon dioxide .
Levodopa (or L-DOPA) has been the most widely used treatment for over 30 years. [3] L-DOPA is transformed into dopamine in the dopaminergic neurons by dopa-decarboxylase. [3] Since motor symptoms are produced by a lack of dopamine in the substantia nigra, the administration of L-DOPA temporarily diminishes the motor symptoms. [3]