MitoSciences: Serine-pyruvate aminotransferase (AGXT)

UniProt Number:	P21549
Alternate Names:	Alanine-glyoxylate aminotransferase, AGT, AGT1, SPT, SPAT
Structure and Function:	In peroxisomes, AGXT uses PLP (pyridoxal 5'-phosphate) as a cofactor and catalyses the nearly irreversible transfer of the amino group from alanine to glyoxylate, to yield glycine and pyruvate. As such, this removal of glyoxylate is considered a detoxification reaction.
Disease Associations:	Defects in AGXT are the cause of hyperoxaluria primary type 1 (HP1) [MIM:259900]; also known as primary hyperoxaluria type I (PH1) and oxalosis I. HP1 is a rare autosomal recessive inborn error of glyoxylate metabolism characterized by increased excretion of oxalate and glycolate, and the progressive accumulation of insoluble calcium oxalate in the kidney and urinary tract. In some HP1 patients AGXT defect is associated with a mitochondrial matrix localization.