AGMAT
Function
Hydrolyzes linear guanidino acids to form urea and the corresponding amines. Displays specificity for substrates having a negatively charged head group and short chains including taurocyamine, guanidino propanoic and butanoic acids. May protect cells by detoxifying potentially harmful amounts of guanidino acids. Metabolizes L-arginine with low efficiency.
Pathway
Nitrogen metabolism; urea cycle; L-ornithine and urea from L-arginine: step 1/1.
Sequence Similarities
Belongs to the ureohydrolase superfamily. Arginase family.
Tissue Specificity
Highly expressed in liver and kidney. Also found in skeletal muscle, fetal liver, brain, testis, skin and the gastrointestinal tract. Within brain, expression is higher in the cerebral cortex with lower levels in the medulla and spinal cord.
Cellular localization
- Mitochondrion
Alternative names
GDAH, AGMAT