4-aminobutyrate aminotransferase, mitochondrial
Also known as: ABAT, GABAT
Function
Catalyzes the conversion of gamma-aminobutyrate and L-beta-aminoisobutyrate to succinate semialdehyde and methylmalonate semialdehyde, respectively. Can also convert delta-aminovalerate and beta-alanine (By similarity).
Classification
- Family (Pfam)
- PF00202 Aminotran_3
- InterPro
- 4NH2But_aminotransferase_euk, Aminotrans_3, Aminotrans_3_PPA_site, PyrdxlP-dep_Trfase, PyrdxlP-dep_Trfase_major, PyrdxlP-dep_Trfase_small
- Functional cluster
- Mixed Regulatory & Membrane Proteins
Gene Ontology · 34
- GO:0032144 4-aminobutyrate transaminase complex
- GO:0005759 mitochondrial matrix
- GO:0005739 mitochondrion
- GO:0047298 (S)-3-amino-2-methylpropionate transaminase activity
- GO:0034386 4-aminobutyrate:2-oxoglutarate transaminase activity
- GO:0042802 identical protein binding
- GO:0051536 iron-sulfur cluster binding
- GO:0046872 metal ion binding
- GO:0030170 pyridoxal phosphate binding
- GO:0032145 succinate-semialdehyde dehydrogenase binding
- GO:0021549 cerebellum development
- GO:0007620 copulation
- GO:0035640 exploration behavior
- GO:0009449 gamma-aminobutyric acid biosynthetic process
- GO:0009450 gamma-aminobutyric acid catabolic process
- GO:0009448 gamma-aminobutyric acid metabolic process
- GO:0007626 locomotory behavior
- GO:0045776 negative regulation of blood pressure
- GO:0033602 negative regulation of dopamine secretion
- GO:0014053 negative regulation of gamma-aminobutyric acid secretion
- GO:0050877 nervous system process
- GO:1904450 positive regulation of aspartate secretion
- GO:0045964 positive regulation of dopamine metabolic process
- GO:0031652 positive regulation of heat generation
- GO:0097151 positive regulation of inhibitory postsynaptic potential
- GO:0032024 positive regulation of insulin secretion
- GO:1902722 positive regulation of prolactin secretion
- GO:0070474 positive regulation of uterine smooth muscle contraction
- GO:0042220 response to cocaine
- GO:0045471 response to ethanol
- GO:0001666 response to hypoxia
- GO:0010039 response to iron ion
- GO:0035094 response to nicotine
- GO:0009410 response to xenobiotic stimulus
Disease associations
- GABA aminotransaminase deficiency MONDO:0013166
Drugs targeting this protein · 1
- VIGABATRIN inhibitor
Related proteins · sequence + function similarity
- 4-aminobutyrate aminotransferase, mitochondrial 0.99
- 4-aminobutyrate aminotransferase, mitochondrial 0.98
- 4-aminobutyrate aminotransferase, mitochondrial 0.98
- 4-aminobutyrate aminotransferase, mitochondrial 0.98
- Glutamate decarboxylase 1 0.65
- Glutamate decarboxylase 4 0.64
- Glutamate decarboxylase 0.63
- Glutamate decarboxylase 5 0.62
- Glutamate decarboxylase 2 0.61
- Glutamate decarboxylase 3 0.61
- Glutamate decarboxylase 0.61
- O-phosphoseryl-tRNA(Sec) selenium transferase 0.61
Literature · 8 cited papers
- An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome. J. Proteomics · 2014
- Initial characterization of the human central proteome. BMC Syst. Biol. · 2011
- Cysteine-321 of human brain GABA transaminase is involved in intersubunit cross-linking. Mol. Cells · 2004
- The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. · 2004
- Complete sequencing and characterization of 21,243 full-length human cDNAs. Nat. Genet. · 2004
- 4-aminobutyrate aminotransferase (GABA-transaminase) deficiency. J. Inherit. Metab. Dis. · 1999
- Primary structure and tissue distribution of human 4-aminobutyrate aminotransferase. Eur. J. Biochem. · 1995
- Screening and sequence determination of a cDNA encoding the human brain 4-aminobutyrate aminotransferase. Gene · 1995
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