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Protein

Branched-chain-amino-acid aminotransferase, mitochondrial

Gene

BCAT2

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Catalyzes the first reaction in the catabolism of the essential branched chain amino acids leucine, isoleucine, and valine. May also function as a transporter of branched chain alpha-keto acids.

Catalytic activityi

L-leucine + 2-oxoglutarate = 4-methyl-2-oxopentanoate + L-glutamate.
L-isoleucine + 2-oxoglutarate = (S)-3-methyl-2-oxopentanoate + L-glutamate.
L-valine + 2-oxoglutarate = 3-methyl-2-oxobutanoate + L-glutamate.

Cofactori

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei168Substrate1

GO - Molecular functioni

GO - Biological processi

Keywordsi

Molecular functionAminotransferase, Transferase
Biological processAmino-acid biosynthesis, Branched-chain amino acid biosynthesis
LigandPyridoxal phosphate

Enzyme and pathway databases

BRENDAi2.6.1.42 2681
ReactomeiR-HSA-70895 Branched-chain amino acid catabolism
SABIO-RKiO15382

Names & Taxonomyi

Protein namesi
Recommended name:
Branched-chain-amino-acid aminotransferase, mitochondrial (EC:2.6.1.42)
Short name:
BCAT(m)
Alternative name(s):
Placental protein 18
Short name:
PP18
Gene namesi
Name:BCAT2
Synonyms:BCATM, BCT2, ECA40
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 19

Organism-specific databases

EuPathDBiHostDB:ENSG00000105552.14
HGNCiHGNC:977 BCAT2
MIMi113530 gene
neXtProtiNX_O15382

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Cytoplasm, Mitochondrion

Pathology & Biotechi

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi342C → A: Reduces activity about 6-fold. 1 Publication1
Mutagenesisi345C → A: Slight reduction of activity. 1 Publication1

Organism-specific databases

DisGeNETi587
OpenTargetsiENSG00000105552
PharmGKBiPA25289

Chemistry databases

ChEMBLiCHEMBL3616354
DrugBankiDB04074 Alpha-ketoisovalerate
DB00142 L-Glutamic Acid
DB00167 L-Isoleucine
DB00149 L-Leucine
DB02635 N-[O-Phosphono-Pyridoxyl]-Isoleucine
DB00114 Pyridoxal Phosphate
DB02142 Pyridoxamine-5'-Phosphate
GuidetoPHARMACOLOGYi2893

Polymorphism and mutation databases

BioMutaiBCAT2

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 27MitochondrionAdd BLAST27
ChainiPRO_000000127128 – 392Branched-chain-amino-acid aminotransferase, mitochondrialAdd BLAST365

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei229N6-(pyridoxal phosphate)lysine1
Modified residuei321N6-acetyllysineCombined sources1

Keywords - PTMi

Acetylation

Proteomic databases

EPDiO15382
MaxQBiO15382
PaxDbiO15382
PeptideAtlasiO15382
PRIDEiO15382
ProteomicsDBi48623
48624 [O15382-2]

PTM databases

iPTMnetiO15382
PhosphoSitePlusiO15382

Expressioni

Tissue specificityi

Ubiquitous.

Gene expression databases

BgeeiENSG00000105552 Expressed in 214 organ(s), highest expression level in body of stomach
CleanExiHS_BCAT2
ExpressionAtlasiO15382 baseline and differential
GenevisibleiO15382 HS

Organism-specific databases

HPAiHPA054091

Interactioni

Subunit structurei

Homodimer.1 Publication

Protein-protein interaction databases

BioGridi107062, 17 interactors
IntActiO15382, 4 interactors
STRINGi9606.ENSP00000322991

Chemistry databases

BindingDBiO15382

Structurei

Secondary structure

1392
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

ProteinModelPortaliO15382
SMRiO15382
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiO15382

Family & Domainsi

Sequence similaritiesi

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG0975 Eukaryota
COG0115 LUCA
GeneTreeiENSGT00390000009532
HOGENOMiHOG000276704
HOVERGENiHBG050678
InParanoidiO15382
KOiK00826
PhylomeDBiO15382
TreeFamiTF300882

Family and domain databases

CDDicd01557 BCAT_beta_family, 1 hit
InterProiView protein in InterPro
IPR001544 Aminotrans_IV
IPR018300 Aminotrans_IV_CS
IPR036038 Aminotransferase-like
IPR005786 B_amino_transII
IPR033939 BCAT_family
PfamiView protein in Pfam
PF01063 Aminotran_4, 1 hit
PIRSFiPIRSF006468 BCAT1, 1 hit
SUPFAMiSSF56752 SSF56752, 1 hit
TIGRFAMsiTIGR01123 ilvE_II, 1 hit
PROSITEiView protein in PROSITE
PS00770 AA_TRANSFER_CLASS_4, 1 hit

Sequences (2+)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

This entry has 2 described isoforms and 5 potential isoforms that are computationally mapped.Show allAlign All

Isoform A (identifier: O15382-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide
        10         20         30         40         50
MAAAALGQIW ARKLLSVPWL LCGPRRYASS SFKAADLQLE MTQKPHKKPG
60 70 80 90 100
PGEPLVFGKT FTDHMLMVEW NDKGWGQPRI QPFQNLTLHP ASSSLHYSLQ
110 120 130 140 150
LFEGMKAFKG KDQQVRLFRP WLNMDRMLRS AMRLCLPSFD KLELLECIRR
160 170 180 190 200
LIEVDKDWVP DAAGTSLYVR PVLIGNEPSL GVSQPTRALL FVILCPVGAY
210 220 230 240 250
FPGGSVTPVS LLADPAFIRA WVGGVGNYKL GGNYGPTVLV QQEALKRGCE
260 270 280 290 300
QVLWLYGPDH QLTEVGTMNI FVYWTHEDGV LELVTPPLNG VILPGVVRQS
310 320 330 340 350
LLDMAQTWGE FRVVERTITM KQLLRALEEG RVREVFGSGT ACQVCPVHRI
360 370 380 390
LYKDRNLHIP TMENGPELIL RFQKELKEIQ YGIRAHEWMF PV
Length:392
Mass (Da):44,288
Last modified:May 2, 2002 - v2
Checksum:iEC07047264B190DA
GO
Isoform B (identifier: O15382-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     9-100: Missing.

Show »
Length:300
Mass (Da):33,777
Checksum:i656A626FEC867073
GO

Computationally mapped potential isoform sequencesi

There are 5 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
B3KSI3B3KSI3_HUMAN
Branched-chain-amino-acid aminotran...
BCAT2
352Annotation score:
M0QXF9M0QXF9_HUMAN
Branched-chain-amino-acid aminotran...
BCAT2
445Annotation score:
M0QZ10M0QZ10_HUMAN
Branched-chain-amino-acid aminotran...
BCAT2
405Annotation score:
M0QZP4M0QZP4_HUMAN
Branched-chain-amino-acid aminotran...
BCAT2
313Annotation score:
M0R2K7M0R2K7_HUMAN
Branched-chain-amino-acid aminotran...
BCAT2
108Annotation score:

Sequence cautioni

The sequence AAB53087 differs from that shown. Reason: Erroneous initiation.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti154V → L in AAB53087 (PubMed:8702755).Curated1
Sequence conflicti207T → N in AAB53087 (PubMed:8702755).Curated1
Sequence conflicti214 – 216DPA → EPT in AAB53087 (PubMed:8702755).Curated3
Sequence conflicti253L → F in AAB53087 (PubMed:8702755).Curated1
Sequence conflicti326A → P in AAB53087 (PubMed:8702755).Curated1
Sequence conflicti330G → A in AAB53087 (PubMed:8702755).Curated1
Sequence conflicti349R → G in AAB53087 (PubMed:8702755).Curated1
Sequence conflicti357L → F in AAB53087 (PubMed:8702755).Curated1
Sequence conflicti370L → F in AAB53087 (PubMed:8702755).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_048234186T → R2 PublicationsCorresponds to variant dbSNP:rs11548193Ensembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0002369 – 100Missing in isoform B. 1 PublicationAdd BLAST92

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U68418 mRNA Translation: AAB67672.1
AF268047 mRNA Translation: AAK38368.1
AF268048 mRNA Translation: AAK38369.1
AK314548 mRNA Translation: BAG37134.1
CH471177 Genomic DNA Translation: EAW52403.1
BC001900 mRNA Translation: AAH01900.1
BC004243 mRNA Translation: AAH04243.2
U62739 mRNA Translation: AAB53087.1 Different initiation.
CCDSiCCDS12735.1 [O15382-1]
CCDS54290.1 [O15382-2]
RefSeqiNP_001158245.1, NM_001164773.1 [O15382-2]
NP_001181.2, NM_001190.3 [O15382-1]
NP_001271254.1, NM_001284325.1
UniGeneiHs.512670

Genome annotation databases

EnsembliENST00000316273; ENSP00000322991; ENSG00000105552 [O15382-1]
ENST00000545387; ENSP00000440973; ENSG00000105552 [O15382-2]
GeneIDi587
KEGGihsa:587
UCSCiuc002pkr.4 human [O15382-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U68418 mRNA Translation: AAB67672.1
AF268047 mRNA Translation: AAK38368.1
AF268048 mRNA Translation: AAK38369.1
AK314548 mRNA Translation: BAG37134.1
CH471177 Genomic DNA Translation: EAW52403.1
BC001900 mRNA Translation: AAH01900.1
BC004243 mRNA Translation: AAH04243.2
U62739 mRNA Translation: AAB53087.1 Different initiation.
CCDSiCCDS12735.1 [O15382-1]
CCDS54290.1 [O15382-2]
RefSeqiNP_001158245.1, NM_001164773.1 [O15382-2]
NP_001181.2, NM_001190.3 [O15382-1]
NP_001271254.1, NM_001284325.1
UniGeneiHs.512670

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
1EKFX-ray1.95A/B28-392[»]
1EKPX-ray2.50A/B28-392[»]
1EKVX-ray2.25A/B28-392[»]
1KT8X-ray1.90A/B28-392[»]
1KTAX-ray1.90A/B28-392[»]
2A1HX-ray1.80A/B28-392[»]
2HDKX-ray2.40A/B28-392[»]
2HG8X-ray1.80A/B28-392[»]
2HGWX-ray1.98A/B28-392[»]
2HGXX-ray1.80A/B28-392[»]
2HHFX-ray1.80A/B28-392[»]
5BWRX-ray2.20A/B28-392[»]
5BWTX-ray2.20A/B28-392[»]
5BWUX-ray2.17A/B28-392[»]
5BWVX-ray1.86A/B28-392[»]
5BWWX-ray1.82A/B28-392[»]
5BWXX-ray1.70A/B28-392[»]
5CR5X-ray1.61A/B28-392[»]
5HNEX-ray2.04A/B28-392[»]
5I5SX-ray2.06A/B28-392[»]
5I5TX-ray2.31A/B28-392[»]
5I5UX-ray2.40A/B28-392[»]
5I5VX-ray1.94A/B28-392[»]
5I5WX-ray2.40A/B28-392[»]
5I5XX-ray1.65A/B28-392[»]
5I5YX-ray1.81A/B28-392[»]
5I60X-ray2.12A/B28-392[»]
5MPRX-ray1.60A28-392[»]
ProteinModelPortaliO15382
SMRiO15382
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi107062, 17 interactors
IntActiO15382, 4 interactors
STRINGi9606.ENSP00000322991

Chemistry databases

BindingDBiO15382
ChEMBLiCHEMBL3616354
DrugBankiDB04074 Alpha-ketoisovalerate
DB00142 L-Glutamic Acid
DB00167 L-Isoleucine
DB00149 L-Leucine
DB02635 N-[O-Phosphono-Pyridoxyl]-Isoleucine
DB00114 Pyridoxal Phosphate
DB02142 Pyridoxamine-5'-Phosphate
GuidetoPHARMACOLOGYi2893

PTM databases

iPTMnetiO15382
PhosphoSitePlusiO15382

Polymorphism and mutation databases

BioMutaiBCAT2

Proteomic databases

EPDiO15382
MaxQBiO15382
PaxDbiO15382
PeptideAtlasiO15382
PRIDEiO15382
ProteomicsDBi48623
48624 [O15382-2]

Protocols and materials databases

DNASUi587
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000316273; ENSP00000322991; ENSG00000105552 [O15382-1]
ENST00000545387; ENSP00000440973; ENSG00000105552 [O15382-2]
GeneIDi587
KEGGihsa:587
UCSCiuc002pkr.4 human [O15382-1]

Organism-specific databases

CTDi587
DisGeNETi587
EuPathDBiHostDB:ENSG00000105552.14
GeneCardsiBCAT2
HGNCiHGNC:977 BCAT2
HPAiHPA054091
MIMi113530 gene
neXtProtiNX_O15382
OpenTargetsiENSG00000105552
PharmGKBiPA25289
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG0975 Eukaryota
COG0115 LUCA
GeneTreeiENSGT00390000009532
HOGENOMiHOG000276704
HOVERGENiHBG050678
InParanoidiO15382
KOiK00826
PhylomeDBiO15382
TreeFamiTF300882

Enzyme and pathway databases

BRENDAi2.6.1.42 2681
ReactomeiR-HSA-70895 Branched-chain amino acid catabolism
SABIO-RKiO15382

Miscellaneous databases

ChiTaRSiBCAT2 human
EvolutionaryTraceiO15382
GenomeRNAii587
PROiPR:O15382
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000105552 Expressed in 214 organ(s), highest expression level in body of stomach
CleanExiHS_BCAT2
ExpressionAtlasiO15382 baseline and differential
GenevisibleiO15382 HS

Family and domain databases

CDDicd01557 BCAT_beta_family, 1 hit
InterProiView protein in InterPro
IPR001544 Aminotrans_IV
IPR018300 Aminotrans_IV_CS
IPR036038 Aminotransferase-like
IPR005786 B_amino_transII
IPR033939 BCAT_family
PfamiView protein in Pfam
PF01063 Aminotran_4, 1 hit
PIRSFiPIRSF006468 BCAT1, 1 hit
SUPFAMiSSF56752 SSF56752, 1 hit
TIGRFAMsiTIGR01123 ilvE_II, 1 hit
PROSITEiView protein in PROSITE
PS00770 AA_TRANSFER_CLASS_4, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiBCAT2_HUMAN
AccessioniPrimary (citable) accession number: O15382
Secondary accession number(s): B2RB87
, O00269, Q96KG1, Q9BTB6, Q9BUU6
Entry historyiIntegrated into UniProtKB/Swiss-Prot: July 15, 1998
Last sequence update: May 2, 2002
Last modified: November 7, 2018
This is version 177 of the entry and version 2 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. Human chromosome 19
    Human chromosome 19: entries, gene names and cross-references to MIM
  6. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
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