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BCKDHA [mitochondrial matrix]
Stable Identifier
R-HSA-70014
Type
Protein [EntityWithAccessionedSequence]
Species
Homo sapiens
Compartment
mitochondrial matrix
Synonyms
branched chain keto acid dehydrogenase E1, alpha polypeptide
Locations in the PathwayBrowser
Expand all
Metabolism (Homo sapiens)
Metabolism of amino acids and derivatives (Homo sapiens)
Branched-chain amino acid catabolism (Homo sapiens)
BCKDH synthesizes BCAA-CoA from KIC, KMVA, KIV (Homo sapiens)
BCKDH [mitochondrial matrix] (Homo sapiens)
BCKDHA:BCKDHB tetramer [mitochondrial matrix] (Homo sapiens)
BCKDHA [mitochondrial matrix] (Homo sapiens)
BCKDK phosphorylates BCKDH (Homo sapiens)
BCKDH [mitochondrial matrix] (Homo sapiens)
BCKDHA:BCKDHB tetramer [mitochondrial matrix] (Homo sapiens)
BCKDHA [mitochondrial matrix] (Homo sapiens)
p-BCKDH [mitochondrial matrix] (Homo sapiens)
BCKDHA:p-BCKDHB tetramer [mitochondrial matrix] (Homo sapiens)
BCKDHA [mitochondrial matrix] (Homo sapiens)
PPM1K dephosphorylates p-BCKDH (Homo sapiens)
BCKDH [mitochondrial matrix] (Homo sapiens)
BCKDHA:BCKDHB tetramer [mitochondrial matrix] (Homo sapiens)
BCKDHA [mitochondrial matrix] (Homo sapiens)
p-BCKDH [mitochondrial matrix] (Homo sapiens)
BCKDHA:p-BCKDHB tetramer [mitochondrial matrix] (Homo sapiens)
BCKDHA [mitochondrial matrix] (Homo sapiens)
Literature References
PubMed ID
Title
Journal
Year
7918575
Differential processing of human and rat E1 alpha precursors of the branched-chain alpha-keto acid dehydrogenase complex caused by an N-terminal proline in the rat sequence
Chuang, DT
,
Wynn, RM
,
Kochi, H
,
Cox, RP
Biochim Biophys Acta
1994
External Reference Information
External Reference
UniProt:P12694 BCKDHA
Gene Names
BCKDHA
Chain
transit peptide:1-45, chain:46-445
Reference Genes
BioGPS Gene:593 BCKDHA
COSMIC (genes):BCKDHA BCKDHA
CTD Gene:593 BCKDHA
dbSNP Gene:593 BCKDHA
ENSEMBL:ENSG00000248098 BCKDHA
ENSEMBL_homo_sapiens_GENE:ENSG00000248098.12 BCKDHA
HGNC:986 BCKDHA
KEGG Gene (Homo sapiens):593 BCKDHA
Monarch:593 BCKDHA
NCBI Gene:593 BCKDHA
OMIM:608348 BCKDHA
UCSC:P12694 BCKDHA
Reference Transcript
RefSeq:NM_000709.3 BCKDHA
RefSeq:NM_001164783.1 BCKDHA
Other Identifiers
11733731_s_at
16862504
202331_PM_at
202331_at
37704_at
3834206
3834209
3834210
3834212
3834213
3834217
3834218
3834219
3834220
3834221
3834222
3834223
3834224
3834225
3834226
593
8029065
A_23_P67188
A_33_P3302305
GE60352
GO:0003824
GO:0003863
GO:0005515
GO:0005739
GO:0005759
GO:0005947
GO:0006520
GO:0009083
GO:0016491
GO:0016624
GO:0016829
GO:0016831
GO:0043226
GO:0046872
HMNXSV003002234
ILMN_1735979
PH_hs_0020110
PH_hs_0040098
TC19002636.hg
Z14093_at
g11386134_3p_at
Participates
as a component of
BCKDHA:p-BCKDHB tetramer [mitochondrial matrix] (Homo sapiens)
BCKDHA:BCKDHB tetramer [mitochondrial matrix] (Homo sapiens)
Inferred To
Ghost homologue of BCKDHA [mitochondrial matrix]
(Xenopus tropicalis)
BCKDHA [mitochondrial matrix]
(Bos taurus)
BCKDHA [mitochondrial matrix]
(Caenorhabditis elegans)
BCKDHA [mitochondrial matrix]
(Dictyostelium discoideum)
BCKDHA [mitochondrial matrix]
(Gallus gallus)
BCKDHA [mitochondrial matrix]
(Mus musculus)
BCKDHA [mitochondrial matrix]
(Plasmodium falciparum)
BCKDHA [mitochondrial matrix]
(Rattus norvegicus)
BCKDHA [mitochondrial matrix]
(Sus scrofa)
TMEM91 [mitochondrial matrix]
(Canis familiaris)
wu:fd20d04 [mitochondrial matrix]
(Danio rerio)
BckdhA [mitochondrial matrix]
(Drosophila melanogaster)
Cross References
RefSeq
NP_001158255.1
,
NP_000700.1
OpenTargets
ENSG00000248098
HPA
ENSG00000248098-BCKDHA
GeneCards
P12694
Ensembl
ENSG00000248098
,
ENST00000457836
,
ENSP00000269980
,
ENST00000269980
,
ENSP00000416000
PRO
P12694
Pharos - Targets
P12694
Orphanet
15360
HMDB Protein
HMDBP00300
PDB
1V11
,
1WCI
,
2BFB
,
1U5B
,
2BFE
,
1OLS
,
1X7Z
,
2BEV
,
1X7X
,
1X80
,
2J9F
,
2BFD
,
2BFC
,
2BFF
,
1V1R
,
1OLX
,
1DTW
,
1V1M
,
2BEW
,
1X7Y
,
1OLU
,
1X7W
,
2BEU
,
1V16
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