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