Glyoxylate aminotransferase

Hyperoxaluria type 1 is due to functional efficiency of the liver specific peroxisomal enzyme alanine-glyoxylate aminotransferase. It leads to severe renal failure. The nervous system is not affected. 

One case where enzymatic involvement is documented is that of (R)-a-fluoro-/3-alanine (11.42), itself the major (>80%) metabolite of 5-fluoroura-cil (11.16) in humans. With rat liver homogenates, it was demonstrated that mitochondrial L-alanine-glyoxylate aminotransferase II (AlaAT-II, EC 2.6.1.44) catalyzed the defluorination of (R)- and (S)-a-fluoro-/3-alanine with catalytic efficiencies of 0.038 and 0.050 mM"1 s 1 at 37° and pH 7.0, respectively, [76], The primary product of the reaction was jS-aminoacrylate... 

This enzyme [EC 2.6.1.2], also known as glutamic-pyruvic transaminase and glutamic-alanine transaminase, catalyzes the pyridoxal-phosphate-dependent reaction of alanine with 2-ketoglutarate, resulting on the production of pyruvate and glutamate. 2-Aminobutanoate will also react, albeit slowly. There is another alanine aminotransferase [EC 2.6.1.12], better known as alanine-oxo-acid aminotransferase, which catalyzes the pyridoxal-phosphate-dependent reaction of alanine and a 2-keto acid to generate pyruvate and an amino acid. See also Alanine Glyoxylate Aminotransferase... 

This pyridoxal-phosphate-dependent enzyme [EC 2.6.1.4] catalyzes the reaction of glycine with a-ketoglu-tarate (or, 2-oxoglutarate) to produce glyoxylate and l-glutamate. See also GlycineiOxaloacetate Aminotransferase Glyoxylate Aminotransferase A. E. Braunstein (1973) The Enzymes, 3rd ed., 9, 379. 

AROMATIC AMINO ACID-GLYOXYLATE AMINOTRANSFERASE Aromatic amino acid hydroxylases, PHENYLALANINE HYDROXYLASE TYROSINE HYDROXYLASE TRYPTOPHAN HYDROXYLASE ARRHENIUS CONSTANT... 

D-bifunctional protein deficiency [5], 2-methyl acyl-CoA racemase (AMACR) deficiency [3] and sterol carrier protein (SCP-x) deficiency [6], the disorders of etherphospholipid biosynthesis (dihydroxyacetone phosphate acyltransferase and alkyl- dihydroxyacetone phosphate synthase deficiency) [2], the disorders of phytanic acid alpha-oxidation (Refsum disease) [15], and the disorders of glyoxylate detoxification with hyperoxaluria type 1 as caused by alanine glyoxylate aminotransferase deficiency as a sole representative. 

Located in the peroxisomes of liver, L-alanine-glyoxylate aminotransferase catalyzes the transamination of alanine and glyoxylate to form pyruvate and glycine. A rare inborn error of metabolism manifested as hyperoxaluria is due to a deficiency of this enzyme. 

Figure 8.4. Pathways of tryptophan metaholism. Tryptophan dioxygenase, EC 1.13.11.11 formylkynurenine formamidase, EC 3.5.1.9 kynurenine hydroxylase, EC 1.14.13.9 kynureninase, EC 3.7.1.3 3-hydroxyanthranilate oxidase, EC 1.10.3.5 picolinate carboxylase, EC 4.1.1.45 kynurenine oxoglutarate aminotransferase, EC 2.6.1.7 kynurenine glyoxylate aminotransferase, 2.6.1.63 tryptophan hydroxylase, EC 1.14.16.4 and 5-hydroxytryptophan decarboxylase, EC 4.1.1.26. Relative molecular masses (Mr) tryptophan, 204.2 serotonin, 176.2 kynurenine, 208.2 3-hydroxykynurenine, 223.2 kynurenic acid, 189.2 xanthurenic acid, 205.2 and quinolinic acid 167.1. CoA, coenzyme A.

Kanai M, Yoshioka A, Tanaka S et al (2010) Associations between glutathione S-transferase % Ilel05Val and glyoxylate aminotransferase ProllLeu and Ile340Met polymorphisms and early-onset oxaliplatin-induced neuropathy. Cancer Epidemiol 34 189-193... 

Amino acid AGXT Alanine-glyoxylate aminotransferase 2q37.3... 

Glycine (aminoacetic acid, aminoethanoic acid) is HjN. CHj.COOH, with = 2.4. It is transaminated by an ala-nine-glyoxylate aminotransferase. The glycine can then be incorporated into proteins and used for serine synthesis or simply be degraded. 

AGX1 Alanine glyoxylate aminotransferase, catalyzes the synthesis of glycine from glyoxylate, which is one of three pathways for glycine biosynthesis in yeast has similarity to mammalian and plant alanine glyoxylate aminotransferases... 

Ornithine aminotransferase Acetylornithine aminotr. Succinylornithine aminotr. Ala-glyoxylate aminotransferase... 

Lawyer, A.L. and I. Zelitch Inhibition of glutamate Glyoxylate aminotransferase activity in tobacco leaves and callus by glycidate, an inhibitor of photorespiration Plant Physiol. 61 (1978) 242-247. 

Aminopeptidase, cytosol Aminopeptidase, leucine Aminopropyltransferase, putrescine Aminotransferase Aminotransferase, alanine Aminotransferase, aspartate Aminotransferase, glutamate-glyoxylate Aminotransferase, ornithine-keto acid Aminotransferase, serine-glyoxylate Ammonia... 

CHAPPLE, C.C.S., GLOVER, J.R., ELLIS, B.E., Purification and characterization of methionine-glyoxylate aminotransferase from Brassica carinata and Brassica napus.. Plant Physiol, 1990,94,1887-1896. 

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