Names & Taxonomy

Uniprot ID:
Q9BYV1
Entry Name:
AGT2_HUMAN
Status:
reviewed
Protein Names:
Alanine--glyoxylate aminotransferase 2, mitochondrial (AGT 2) (EC 2.6.1.44) ((R)-3-amino-2-methylpropionate--pyruvate transaminase) (EC 2.6.1.40) (Beta-ALAAT II) (Beta-alanine-pyruvate aminotransferase) (D-AIBAT)
Gene Names:
AGXT2 AGT2
Gene Names Primary:
AGXT2
Organism:
Homo sapiens (Human)

Structure

Length:
514
Sequence:
MTLIWRHLLRPLCLVTSAPRILEMHPFLSLGTSRTSVTKLSLHTKPRMPPCDFMPERYQSLGYNRVLEIHKEHLSPVVTAYFQKPLLLHQGHMEWLFDAEGSRYLDFFSGIVTVSVGHCHPKVNAVAQKQLGRLWHTSTVFFHPPMHEYAEKLAALLPEPLKVIFLVNSGSEANELAMLMARAHSNNIDIISFRGAYHGCSPYTLGLTNVGTYKMELPGGTGCQPTMCPDVFRGPWGGSHCRDSPVQTIRKCSCAPDCCQAKDQYIEQFKDTLSTSVAKSIAGFFAEPIQGVNGVVQYPKGFLKEAFELVRARGGVCIADEVQTGFGRLGSHFWGFQTHDVLPDIVTMAKGIGNGFPMAAVITTPEIAKSLAKCLQHFNTFGGNPMACAIGSAVLEVIKEENLQENSQEVGTYMLLKFAKLRDEFEIVGDVRGKGLMIGIEMVQDKISCRPLPREEVNQIHEDCKHMGLLVGRGSIFSQTFRIAPSMCITKPEVDFAVEVFRSALTQHMERRAK
Proteomes:
UP000005640

Subcellular location

Subcellular Location:
Mitochondrion

Function

Function:
Can metabolize asymmetric dimethylarginine (ADMA) via transamination to alpha-keto-delta-(NN-dimethylguanidino) valeric acid (DMGV). ADMA is a potent inhibitor of nitric-oxide (NO) synthase, and this activity provides mechanism through which the kidney regulates blood pressure.
Catalytic Activity:
L-alanine + glyoxylate = pyruvate + glycine.; (R)-3-amino-2-methylpropanoate + pyruvate = 2-methyl-3-oxopropanoate + L-alanine.
Cofactor:
COFACTOR: Name=pyridoxal 5'-phosphate; Xref=ChEBI:CHEBI:597326;
Cross Reference Drug Bank:
DB00145 DB00160 DB00119
Gene Ontology Go:
mitochondrial matrix
mitochondrion
(R)-3-amino-2-methylpropionate-pyruvate transaminase activity
alanine-glyoxylate transaminase activity
beta-alanine-pyruvate transaminase activity
identical protein binding
pyridoxal phosphate binding
cellular nitrogen compound metabolic process
glycine biosynthetic process, by transamination of glyoxylate
glyoxylate catabolic process
glyoxylate metabolic process
L-alanine catabolic process, by transamination
nucleobase-containing small molecule metabolic process
positive regulation of nitric oxide biosynthetic process
pyrimidine nucleobase metabolic process
pyrimidine nucleoside catabolic process
small molecule metabolic process
Gene Ontology Biological Process:
cellular nitrogen compound metabolic process
glycine biosynthetic process, by transamination of glyoxylate
glyoxylate catabolic process
glyoxylate metabolic process
L-alanine catabolic process, by transamination
nucleobase-containing small molecule metabolic process
positive regulation of nitric oxide biosynthetic process
pyrimidine nucleobase metabolic process
pyrimidine nucleoside catabolic process
small molecule metabolic process
Gene Ontology Molecular Function:
(R)-3-amino-2-methylpropionate-pyruvate transaminase activity
alanine-glyoxylate transaminase activity
beta-alanine-pyruvate transaminase activity
identical protein binding
pyridoxal phosphate binding
Gene Ontology Cellular Component:
mitochondrial matrix
mitochondrion
Keywords:
Acetylation
Alternative splicing
Aminotransferase
Complete proteome
Direct protein sequencing
Mitochondrion
Polymorphism
Pyridoxal phosphate
Reference proteome
Transferase
Transit peptide

Publication

PubMed ID:
15372022 15489334 20018850 23023372 24275569 24834905 24586340