Reactome | glyoxylate + alanine => glycine + pyruvate [peroxisome]

glyoxylate + alanine => glycine + pyruvate [peroxisome]

Stable Identifier

R-HSA-389684

Type

Reaction [transition]

Species

Homo sapiens

Compartment

peroxisomal matrix

ReviewStatus

5/5

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glyoxylate + alanine => glycine + pyruvate [peroxisome]

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Alanine-glyoxylate transaminase (AGXT) catalyzes the irreversible reaction of glyoxylate and alanine to form glycine and pyruvate (Danpure and Jennings 1988). The active form of the enzyme is a homodimer (Zhang et al. 2003) with one molecule of pyridoxal phosphate bound to each subunit (Coulter-Mackie et al. 2005). Mutations in this enzyme are associated with primary hyperoxaluria type I. Mutant alleles encode both catalytically inactive proteins and active ones that are mis-localized to mitochondria (Purdue et al. 1990; Takada et al. 1990).

Literature References

PubMed ID	Title	Journal	Year
12899834	Crystal structure of alanine:glyoxylate aminotransferase and the relationship between genotype and enzymatic phenotype in primary hyperoxaluria type 1 Roe, SM, Bartlam, M, Pearl, LH, Danpure, CJ, Rao, Z, Zhang, X, Hou, Y	J Mol Biol	2003
2363689	Human peroxisomal L-alanine: glyoxylate aminotransferase. Evolutionary loss of a mitochondrial targeting signal by point mutation of the initiation codon Takada, Y, Kaneko, N, Danpure, CJ, Purdue, PE, Esumi, H	Biochem J	1990
15802217	Overexpression of human alanine:glyoxylate aminotransferase in Escherichia coli: renaturation from guanidine-HCl and affinity for pyridoxal phosphate co-factor Lian, Q, Coulter-Mackie, MB, Wong, SG	Protein Expr Purif	2005
1703535	Identification of mutations associated with peroxisome-to-mitochondrion mistargeting of alanine/glyoxylate aminotransferase in primary hyperoxaluria type 1 Takada, Y, Danpure, CJ, Purdue, PE	J Cell Biol	1990
3416563	Further studies on the activity and subcellular distribution of alanine:glyoxylate aminotransferase in the livers of patients with primary hyperoxaluria type 1 Jennings, PR, Danpure, CJ	Clin Sci (Lond)	1988