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PDHA1 [mitochondrial matrix]
Stable Identifier
R-HSA-69966
Type
Protein [EntityWithAccessionedSequence]
Species
Homo sapiens
Compartment
mitochondrial matrix
Synonyms
pyruvate dehydrogenase E1 alpha apoprotein, ODPA_HUMAN, Pyruvate dehydrogenase E1 component alpha subunit, somatic form, mitochondrial precursor , PDHE1-A type I
Locations in the PathwayBrowser
Expand all
Metabolism (Homo sapiens)
Aerobic respiration and respiratory electron transport (Homo sapiens)
Pyruvate metabolism (Homo sapiens)
PDH complex synthesizes acetyl-CoA from PYR (Homo sapiens)
DLAT trimer transfers acetyl to CoA (Homo sapiens)
PDH complex [mitochondrial matrix] (Homo sapiens)
PDH E1 [mitochondrial matrix] (Homo sapiens)
PDHA1,2 [mitochondrial matrix] (Homo sapiens)
PDHA1 [mitochondrial matrix] (Homo sapiens)
DLD dimer dehydrogenates dihydrolipoyl (Homo sapiens)
PDH complex [mitochondrial matrix] (Homo sapiens)
PDH E1 [mitochondrial matrix] (Homo sapiens)
PDHA1,2 [mitochondrial matrix] (Homo sapiens)
PDHA1 [mitochondrial matrix] (Homo sapiens)
PDH E1 decarboxylates PYR, transferring acetyl to DLAT (Homo sapiens)
PDH E1 [mitochondrial matrix] (Homo sapiens)
PDHA1,2 [mitochondrial matrix] (Homo sapiens)
PDHA1 [mitochondrial matrix] (Homo sapiens)
PDH complex [mitochondrial matrix] (Homo sapiens)
PDH E1 [mitochondrial matrix] (Homo sapiens)
PDHA1,2 [mitochondrial matrix] (Homo sapiens)
PDHA1 [mitochondrial matrix] (Homo sapiens)
p-lipo-PDH [mitochondrial matrix] (Homo sapiens)
PDH E1 [mitochondrial matrix] (Homo sapiens)
PDHA1,2 [mitochondrial matrix] (Homo sapiens)
PDHA1 [mitochondrial matrix] (Homo sapiens)
phosphorylated pyruvate dehydrogenase E1 complex [mitochondrial matrix] (Homo sapiens)
PDH E1 [mitochondrial matrix] (Homo sapiens)
PDHA1,2 [mitochondrial matrix] (Homo sapiens)
PDHA1 [mitochondrial matrix] (Homo sapiens)
Regulation of pyruvate metabolism (Homo sapiens)
Regulation of pyruvate dehydrogenase (PDH) complex (Homo sapiens)
PDK isozymes phosphorylate PDHC subunit E1 (Homo sapiens)
PDH complex [mitochondrial matrix] (Homo sapiens)
PDH E1 [mitochondrial matrix] (Homo sapiens)
PDHA1,2 [mitochondrial matrix] (Homo sapiens)
PDHA1 [mitochondrial matrix] (Homo sapiens)
p-lipo-PDH [mitochondrial matrix] (Homo sapiens)
PDH E1 [mitochondrial matrix] (Homo sapiens)
PDHA1,2 [mitochondrial matrix] (Homo sapiens)
PDHA1 [mitochondrial matrix] (Homo sapiens)
phosphorylated pyruvate dehydrogenase E1 complex [mitochondrial matrix] (Homo sapiens)
PDH E1 [mitochondrial matrix] (Homo sapiens)
PDHA1,2 [mitochondrial matrix] (Homo sapiens)
PDHA1 [mitochondrial matrix] (Homo sapiens)
PDP1,2 dephosphorylate p-lipo-PDH (Homo sapiens)
PDH complex [mitochondrial matrix] (Homo sapiens)
PDH E1 [mitochondrial matrix] (Homo sapiens)
PDHA1,2 [mitochondrial matrix] (Homo sapiens)
PDHA1 [mitochondrial matrix] (Homo sapiens)
p-lipo-PDH [mitochondrial matrix] (Homo sapiens)
PDH E1 [mitochondrial matrix] (Homo sapiens)
PDHA1,2 [mitochondrial matrix] (Homo sapiens)
PDHA1 [mitochondrial matrix] (Homo sapiens)
phosphorylated pyruvate dehydrogenase E1 complex [mitochondrial matrix] (Homo sapiens)
PDH E1 [mitochondrial matrix] (Homo sapiens)
PDHA1,2 [mitochondrial matrix] (Homo sapiens)
PDHA1 [mitochondrial matrix] (Homo sapiens)
Metabolism of proteins (Homo sapiens)
Mitochondrial protein degradation (Homo sapiens)
CLPXP binds mitochondrial matrix proteins (Homo sapiens)
CLPXP:substrate protein (mitochondrial matrix) [mitochondrial matrix] (Homo sapiens)
Substrate of CLPXP (mitrochondrial matrix) [mitochondrial matrix] (Homo sapiens)
PDHA1 [mitochondrial matrix] (Homo sapiens)
Substrate of CLPXP (mitrochondrial matrix) [mitochondrial matrix] (Homo sapiens)
PDHA1 [mitochondrial matrix] (Homo sapiens)
CLPXP degrades mitochondrial matrix proteins (Homo sapiens)
CLPXP:substrate protein (mitochondrial matrix) [mitochondrial matrix] (Homo sapiens)
Substrate of CLPXP (mitrochondrial matrix) [mitochondrial matrix] (Homo sapiens)
PDHA1 [mitochondrial matrix] (Homo sapiens)
Signal Transduction (Homo sapiens)
Signaling by Nuclear Receptors (Homo sapiens)
Signaling by Retinoic Acid (Homo sapiens)
PDK isozymes phosphorylate PDHC subunit E1 (Homo sapiens)
PDH complex [mitochondrial matrix] (Homo sapiens)
PDH E1 [mitochondrial matrix] (Homo sapiens)
PDHA1,2 [mitochondrial matrix] (Homo sapiens)
PDHA1 [mitochondrial matrix] (Homo sapiens)
p-lipo-PDH [mitochondrial matrix] (Homo sapiens)
PDH E1 [mitochondrial matrix] (Homo sapiens)
PDHA1,2 [mitochondrial matrix] (Homo sapiens)
PDHA1 [mitochondrial matrix] (Homo sapiens)
phosphorylated pyruvate dehydrogenase E1 complex [mitochondrial matrix] (Homo sapiens)
PDH E1 [mitochondrial matrix] (Homo sapiens)
PDHA1,2 [mitochondrial matrix] (Homo sapiens)
PDHA1 [mitochondrial matrix] (Homo sapiens)
External Reference Information
External Reference
UniProt:P08559 PDHA1
Gene Names
PDHA1, PHE1A
Chain
transit peptide:1-29, chain:30-390
Reference Genes
BioGPS Gene:5160 PDHA1
COSMIC (genes):PDHA1 PDHA1
CTD Gene:5160 PDHA1
dbSNP Gene:5160 PDHA1
ENSEMBL:ENSG00000131828 PDHA1
ENSEMBL_homo_sapiens_GENE:ENSG00000131828.14 PDHA1
HGNC:8806 PDHA1
KEGG Gene (Homo sapiens):5160 PDHA1
Monarch:5160 PDHA1
NCBI Gene:5160 PDHA1
OMIM:300502 PDHA1
UCSC:P08559 PDHA1
Reference Transcript
RefSeq:NM_000284.3 PDHA1
RefSeq:NM_001173454.1 PDHA1
RefSeq:NM_001173455.1 PDHA1
RefSeq:NM_001173456.1 PDHA1
Other Identifiers
11717482_at
11717483_a_at
11717484_x_at
11747772_x_at
11753896_a_at
1555864_PM_s_at
1555864_s_at
17101957
200979_PM_at
200979_at
200980_PM_s_at
200980_s_at
38638_at
3970867
3970868
3970869
3970872
3970873
3970874
3970875
3970878
3970881
3970885
3970887
3970889
3970891
3970893
3970894
3970895
3970936
3970938
5160
8166335
A_23_P251095
D90084_at
GE79463
GE86300
GO:0003824
GO:0004738
GO:0004739
GO:0005515
GO:0005634
GO:0005730
GO:0005739
GO:0005759
GO:0005967
GO:0005975
GO:0006006
GO:0006086
GO:0006090
GO:0006091
GO:0006099
GO:0006790
GO:0016491
GO:0016624
GO:0034604
GO:0043226
GO:0043231
GO:0045254
GO:0055086
GO:1901135
GO:1902494
HMNXSV003001760
Hs.1023.0.S2_3p_at
ILMN_1772369
PH_hs_0000131
PH_hs_0035314
TC0X000092.hg
g4505684_3p_s_at
Participates
as a member of
Substrate of CLPXP (mitrochondrial matrix) [mitochondrial matrix] (Homo sapiens)
PDHA1,2 [mitochondrial matrix] (Homo sapiens)
Inferred To
PDHA2 [mitochondrial matrix]
(Gallus gallus)
PDA1 [mitochondrial matrix]
(Saccharomyces cerevisiae)
Pdha1 [mitochondrial matrix]
(Mus musculus)
Pdha [mitochondrial matrix]
(Drosophila melanogaster)
pdhA [mitochondrial matrix]
(Dictyostelium discoideum)
pda1 [mitochondrial matrix]
(Schizosaccharomyces pombe)
PDHA1 [mitochondrial matrix]
(Bos taurus)
PDHA1 [mitochondrial matrix]
(Caenorhabditis elegans)
PDHA1 [mitochondrial matrix]
(Canis familiaris)
PDHA1 [mitochondrial matrix]
(Sus scrofa)
Pdha1l1 [mitochondrial matrix]
(Rattus norvegicus)
Cross References
RefSeq
NP_001166927.1
,
NP_001166925.1
,
NP_000275.1
,
NP_001166926.1
OpenTargets
ENSG00000131828
GeneCards
P08559
HPA
ENSG00000131828-PDHA1
Ensembl
ENST00000422285
,
ENSP00000394382
,
ENSP00000406473
,
ENST00000540249
,
ENST00000355808
,
ENSP00000348062
,
ENST00000423505
,
ENSG00000131828
,
ENSP00000440761
PRO
P08559
Pharos - Targets
P08559
Orphanet
16631
HMDB Protein
HMDBP00046
PDB
6CFO
,
1NI4
,
6CER
,
3EXI
,
2OZL
,
3EXH
,
3EXF
,
3EXG
,
3EXE
Interactors (3)
Accession
#Entities
Entities
Confidence Score
Evidence (IntAct)
UniProt:P42229 STAT5A
4
STAT5A [nucleoplasm]
(R-HSA-9701901)
p-Y694-STAT5A [nucleoplasm]
(R-HSA-507995)
p-Y694-STAT5A [cytosol]
(R-HSA-507936)
STAT5A [cytosol]
(R-HSA-380747)
0.643
4
UniProt:P13569 CFTR
17
PolyUb-CFTR [clathrin-coated endocytic vesicle membrane]
(R-HSA-8869104)
PolyUb-CFTR [plasma membrane]
(R-HSA-8867595)
CFTR [lysosomal membrane]
(R-HSA-5627279)
CFTR [Golgi-associated vesicle membrane]
(R-HSA-5627079)
CFTR [endosome membrane]
(R-HSA-6782965)
PolyUb-CFTR [endosome membrane]
(R-HSA-6782976)
CFTR F508del [endoplasmic reticulum membrane]
(R-HSA-8866834)
ub-CFTR F508del [cytosol]
(R-HSA-8866838)
ub-CFTR F508del [endoplasmic reticulum membrane]
(R-HSA-8866842)
CFTR G542* [plasma membrane]
(R-HSA-5678986)
CFTR G551D [plasma membrane]
(R-HSA-5678977)
CFTR W1282* [plasma membrane]
(R-HSA-5678976)
CFTR F508del [plasma membrane]
(R-HSA-5678978)
CFTR N1303K [plasma membrane]
(R-HSA-5678983)
CFTR [plasma membrane]
(R-HSA-383188)
CFTR [endoplasmic reticulum membrane]
(R-HSA-8866837)
misfolded CFTR [endoplasmic reticulum membrane]
(R-HSA-8868730)
0.519
5
UniProt:Q16891 IMMT
1
IMMT (MIC60) [mitochondrial inner membrane]
(R-HSA-8949569)
0.499
4
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