4-aminobutyrate aminotransferase, mitochondrial

Name4-aminobutyrate aminotransferase, mitochondrial
Synonyms
  • (S)-3-amino-2-methylpropionate transaminase
  • 2.6.1.19
  • GABA aminotransferase
  • GABA transaminase
  • GABA-AT
  • GABA-T
  • GABAT
  • Gamma-amino-N-butyrate transaminase
  • L-AIBAT
Gene NameABAT
OrganismHuman
Amino acid sequence
>lcl|BSEQ0010221|4-aminobutyrate aminotransferase, mitochondrial
MASMLLAQRLACSFQHSYRLLVPGSRHISQAAAKVDVEFDYDGPLMKTEVPGPRSQELMK
QLNIIQNAEAVHFFCNYEESRGNYLVDVDGNRMLDLYSQISSVPIGYSHPALLKLIQQPQ
NASMFVNRPALGILPPENFVEKLRQSLLSVAPKGMSQLITMACGSCSNENALKTIFMWYR
SKERGQRGFSQEELETCMINQAPGCPDYSILSFMGAFHGRTMGCLATTHSKAIHKIDIPS
FDWPIAPFPRLKYPLEEFVKENQQEEARCLEEVEDLIVKYRKKKKTVAGIIVEPIQSEGG
DNHASDDFFRKLRDIARKHGCAFLVDEVQTGGGCTGKFWAHEHWGLDDPADVMTFSKKMM
TGGFFHKEEFRPNAPYRIFNTWLGDPSKNLLLAEVINIIKREDLLNNAAHAGKALLTGLL
DLQARYPQFISRVRGRGTFCSFDTPDDSIRNKLILIARNKGVVLGGCGDKSIRFRPTLVF
RDHHAHLFLNIFSDILADFK
Number of residues500
Molecular Weight56438.405
Theoretical pI8.04
GO Classification
Functions
  • (S)-3-amino-2-methylpropionate transaminase activity
  • 4-aminobutyrate transaminase activity
  • succinate-semialdehyde dehydrogenase binding
  • metal ion binding
  • protein homodimerization activity
  • iron-sulfur cluster binding
  • pyridoxal phosphate binding
Processes
  • response to drug
  • positive regulation of insulin secretion
  • synaptic transmission
  • behavioral response to cocaine
  • copulation
  • neurotransmitter secretion
  • gamma-aminobutyric acid biosynthetic process
  • gamma-aminobutyric acid catabolic process
  • neurotransmitter catabolic process
  • response to hypoxia
  • positive regulation of heat generation
  • locomotory behavior
  • negative regulation of blood pressure
  • response to ethanol
  • response to nicotine
  • response to iron ion
Components
  • 4-aminobutyrate transaminase complex
  • mitochondrial matrix
  • extracellular exosome
  • neuron projection
  • mitochondrion
General FunctionSuccinate-semialdehyde dehydrogenase binding
Specific FunctionCatalyzes the conversion of gamma-aminobutyrate and L-beta-aminoisobutyrate to succinate semialdehyde and methylmalonate semialdehyde, respectively. Can also convert delta-aminovalerate and beta-alanine.
Pfam Domain Function
Transmembrane RegionsNot Available
GenBank Protein ID602705
UniProtKB IDP80404
UniProtKB Entry NameGABT_HUMAN
Cellular LocationMitochondrion matrix
Gene sequence
>lcl|BSEQ0010222|4-aminobutyrate aminotransferase, mitochondrial (ABAT)
ATGGCCTCCATGTTGCTCGCCCAGCGCCTGGCCTGCAGCTTCCAGCACAGCTACCGCCTG
CTGGTGCCTGGATCCAGACACATTAGTCAAGCTGCAGCCAAAGTCGACGTTGAATTTGAT
TATGATGGGCCTCTGATGAAGACGGAAGTCCCAGGGCCTAGATCTCAGGAGTTAATGAAA
CAGCTGAATATAATTCAGAATGCAGAGGCTGTGCATTTTTTCTGCAATTACGAAGAGAGC
CGAGGCAATTACCTGGTTGATGTGGACGGCAACCGAATGCTGGATCTTTATTCCCAGATC
TCCTCTGTCCCCATAGGTTACAGCCACCCCGCCCTGCTGAAACTCATCCAACAGCCTCAA
AATGCGAGCATGTTTGTCAACAGACCCGCCCTCGGAATCCTGCCTCCGGAGAACTTTGTG
GAGAAGCTCCGGCAGTCCTTGCTCTCGGTGGCTCCCAAAGGGATGTCCCAGCTCATCACC
ATGGCCTGCGGCTCCTGCTCCAATGAAAACGCCTTAAAGACCATCTTCATGTGGTACCGG
AGCAAGGAAAGAGGGCAGAGGGGCTTCTCCCAGGAGGAGCTGGAGACGTGCATGATTAAC
CAGGCCCCTGGCTGCCCCGACTACAGCATCCTCTCCTTCATGGGCGCGTTCCATGGGAGG
ACCATGGGTTGCTTAGCGACCACGCACTCTAAAGCCATTCACAAGATCGACATCCCTTCC
TTTGACTGGCCCATCGCACCGTTCCCACGGCTGAAATACCCTCTGGAAGAGTTTGTGAAA
GAGAACCAACAGGAGGAGGCCCGCTGTCTGGAAGAGGTGGAGGATCTGATTGTGAAATAT
CGGAAAAAGAAGAAGACGGTGGCCGGGATCATCGTGGAGCCCATCCAGTCCGAGGGTGGA
GACAACCACGCATCCGATGACTTCTTTCGGAAGCTGAGAGACATCGCCAGGAAGCATGGC
TGCGCCTTCTTGGTGGACGAGGTCCAGACCGGAGGAGGCTGCACGGGCAAGTTCTGGGCC
CATGAGCACTGGGGCCTGGATGACCCAGCAGACGTGATGACCTTCAGCAAGAAGATGATG
ACTGGGGGCTTCTTCCACAAGGAGGAGTTCAGGCCTAATGCTCCCTACCGGATCTTCAAC
ACCTGGCTGGGGGACCCGTCCAAGAACCTGTTGCTGGCTGAGGTCATCAACATCATCAAG
CGGGAGGACCTGCTAAATAATGCAGCCCATGCCGGGAAGGCCCTGCTCACAGGACTGCTG
GACCTCCAGGCCCGGTACCCCCAGTTCATCAGCAGGGTGAGAGGACGAGGCACCTTTTGC
TCCTTCGATACTCCCGATGATTCCATACGGAATAAGCTCATTTTAATTGCCAGAAACAAA
GGTGTGGTGTTGGGTGGCTGTGGTGACAAATCCATTCGTTTCCGTCCCACGCTGGTCTTC
AGGGATCACCACGCTCACCTGTTCCTCAATATTTTCAGTGACATCTTAGCAGACTTCAAG
TAA
GenBank Gene IDL32961
GeneCard IDNot Available
GenAtlas IDABAT
HGNC IDHGNC:23
Chromosome Location16
Locus16p13.2
References
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