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HADHA [mitochondrial inner membrane]
Stable Identifier
R-HSA-77264
Type
Protein [EntityWithAccessionedSequence]
Species
Homo sapiens
Compartment
mitochondrial inner membrane
Synonyms
Trifunctional enzyme alpha subunit, Trifunctional enzyme alpha subunit, mitochondrial precursor, TP-alpha, 78 kDa gastrin-binding protein, Long-chain enoyl-CoA hydratase , Long chain 3-hydroxyacyl-CoA dehydrogenase
Locations in the PathwayBrowser
Expand all
Metabolism (Homo sapiens)
Metabolism of lipids (Homo sapiens)
Fatty acid metabolism (Homo sapiens)
Mitochondrial Fatty Acid Beta-Oxidation (Homo sapiens)
mitochondrial fatty acid beta-oxidation of saturated fatty acids (Homo sapiens)
Beta oxidation of decanoyl-CoA to octanoyl-CoA-CoA (Homo sapiens)
3-Oxodecanoyl-CoA+CoA-SH<=>Octanoyl-CoA (Homo sapiens)
Trifunctional Protein [mitochondrial matrix] (Homo sapiens)
HADHA [mitochondrial inner membrane] (Homo sapiens)
Beta oxidation of hexanoyl-CoA to butanoyl-CoA (Homo sapiens)
3-Oxohexanoyl-CoA+CoA-SH<=>Butanoyl-CoA (Homo sapiens)
Trifunctional Protein [mitochondrial matrix] (Homo sapiens)
HADHA [mitochondrial inner membrane] (Homo sapiens)
Beta oxidation of lauroyl-CoA to decanoyl-CoA-CoA (Homo sapiens)
3-Oxododecanoyl-CoA+CoA-SH<=>Decanoyl-CoA (Homo sapiens)
Trifunctional Protein [mitochondrial matrix] (Homo sapiens)
HADHA [mitochondrial inner membrane] (Homo sapiens)
Beta oxidation of myristoyl-CoA to lauroyl-CoA (Homo sapiens)
(S)-3-Hydroxytetradecanoyl-CoA+NAD<=>3-Oxotetradecanoyl-CoA+NADH+H (Homo sapiens)
Trifunctional Protein [mitochondrial matrix] (Homo sapiens)
HADHA [mitochondrial inner membrane] (Homo sapiens)
3-Oxotetradecanoyl-CoA+CoA-SH<=>Lauroyl-CoA (Homo sapiens)
Trifunctional Protein [mitochondrial matrix] (Homo sapiens)
HADHA [mitochondrial inner membrane] (Homo sapiens)
trans-Tetradec-2-enoyl-CoA+H2O<=>(S)-3-Hydroxytetradecanoyl-CoA (Homo sapiens)
Trifunctional Protein [mitochondrial matrix] (Homo sapiens)
HADHA [mitochondrial inner membrane] (Homo sapiens)
Beta oxidation of octanoyl-CoA to hexanoyl-CoA (Homo sapiens)
3-Oxooctanoyl-CoA+CoA-SH<=>Hexanoyl-CoA (Homo sapiens)
Trifunctional Protein [mitochondrial matrix] (Homo sapiens)
HADHA [mitochondrial inner membrane] (Homo sapiens)
Beta oxidation of palmitoyl-CoA to myristoyl-CoA (Homo sapiens)
(S)-3-Hydroxyhexadecanoyl-CoA+NAD<=>3-Oxopalmitoyl-CoA+NADH+H (Homo sapiens)
Trifunctional Protein [mitochondrial matrix] (Homo sapiens)
HADHA [mitochondrial inner membrane] (Homo sapiens)
3-Oxopalmitoyl-CoA+CoA-SH<=>myristoyl-CoA (Homo sapiens)
Trifunctional Protein [mitochondrial matrix] (Homo sapiens)
HADHA [mitochondrial inner membrane] (Homo sapiens)
trans-Hexadec-2-enoyl-CoA+H2O<=>(S)-3-Hydroxyhexadecanoyl-CoA (Homo sapiens)
Trifunctional Protein [mitochondrial matrix] (Homo sapiens)
HADHA [mitochondrial inner membrane] (Homo sapiens)
mitochondrial fatty acid beta-oxidation of unsaturated fatty acids (Homo sapiens)
Linoleoyl-CoA is converted to cis,cis-3,6- dodecadienoyl-CoA by three cycles of beta-oxidation (Homo sapiens)
Trifunctional Protein [mitochondrial matrix] (Homo sapiens)
HADHA [mitochondrial inner membrane] (Homo sapiens)
Trans,cis-lauro-2,6-dienoyl-CoA is converted to cis,cis-3,6- 4-cis-decenoyl-CoA by one cycle of beta-oxidation (Homo sapiens)
Trifunctional Protein [mitochondrial matrix] (Homo sapiens)
HADHA [mitochondrial inner membrane] (Homo sapiens)
Phospholipid metabolism (Homo sapiens)
Glycerophospholipid biosynthesis (Homo sapiens)
Acyl chain remodeling of CL (Homo sapiens)
MLCL is acylated to CL by HADH (IM) (Homo sapiens)
HADH octamer [mitochondrial inner membrane] (Homo sapiens)
HADHA [mitochondrial inner membrane] (Homo sapiens)
External Reference Information
External Reference
UniProt:P40939 HADHA
Gene Names
HADHA, HADH
Chain
transit peptide:1-36, chain:37-763
Reference Genes
BioGPS Gene:3030 HADHA
COSMIC (genes):HADHA HADHA
CTD Gene:3030 HADHA
dbSNP Gene:3030 HADHA
ENSEMBL:ENSG00000084754 HADHA
HGNC:4801 HADHA
KEGG Gene (Homo sapiens):3030 HADHA
Monarch:3030 HADHA
NCBI Gene:3030 HADHA
OMIM:600890 HADHA
UCSC:P40939 HADHA
Reference Transcript
RefSeq:NM_000182.4 HADHA
Other Identifiers
0001850136
11715522_a_at
11715523_a_at
11754123_s_at
16895471
208629_s_at
208630_at
208631_s_at
2545093
2545094
2545095
2545096
2545097
2545098
2545099
2545100
2545101
2545103
2545105
2545107
2545108
2545109
2545112
2545113
2545116
2545119
2545121
2545122
2545123
2545125
2545126
2545127
2545128
2545130
2545133
2545134
2545135
2545136
2792499
2792502
3030
3403815
3403816
36952_at
52313_at
8050908
A_24_P242688
A_24_P353964
D16480_at
GE61246
GE80899
GO:0003674
GO:0003824
GO:0003857
GO:0003985
GO:0004300
GO:0005515
GO:0005575
GO:0005622
GO:0005737
GO:0005739
GO:0005743
GO:0006629
GO:0006631
GO:0006635
GO:0008150
GO:0008152
GO:0009056
GO:0016020
GO:0016491
GO:0016507
GO:0016509
GO:0016740
GO:0016746
GO:0016829
GO:0032991
GO:0035965
GO:0042645
GO:0043226
GO:0044281
GO:0055114
HMNXSV003021582
Hs.75860.0.A1_3p_a_at
Hs.75860.0.A3_3p_at
ILMN_1712751
PH_hs_0028970
TC02001665.hg
g595266_3p_a_at
Participates
as a component of
HADH octamer [mitochondrial inner membrane] (Homo sapiens)
Trifunctional Protein [mitochondrial matrix] (Homo sapiens)
Inferred To
Mtpalpha [mitochondrial inner membrane]
(Drosophila melanogaster)
hadhab [mitochondrial inner membrane]
(Danio rerio)
HADHA [mitochondrial inner membrane]
(Canis familiaris)
HADHA [mitochondrial inner membrane]
(Gallus gallus)
HADHA [mitochondrial inner membrane]
(Sus scrofa)
Hadha [mitochondrial inner membrane]
(Mus musculus)
Hadha [mitochondrial inner membrane]
(Rattus norvegicus)
ech-1.2 [mitochondrial inner membrane]
(Caenorhabditis elegans)
Cross References
RefSeq
NP_000173.2
Target Pathogen
5b354852be737e166372ed75
OpenTargets
ENSG00000084754
IntEnz
1.1.1.211
HPA
ENSG00000084754-HADHA
GeneCards
P40939
Ensembl
ENST00000380649
,
ENSP00000370023
,
ENSG00000084754
,
ENSP00000496185
,
ENST00000646483
PRO
P40939
Orphanet
16781
PDB
5ZRV
,
5ZQZ
,
6DV2
HMDB Protein
HMDBP00389
Interactors (6)
Accession
#Entities
Entities
Confidence Score
Evidence (IntAct)
UniProt:Q9GZQ8 MAP1LC3B
3
MAP1LC3B(2-121) [cytosol]
(R-HSA-5682674)
MAP1LC3B [autophagosome membrane]
(R-HSA-5683641)
MAP1LC3B [cytosol]
(R-HSA-5205651)
0.591
4
UniProt:Q9Y4K3 TRAF6
8
TRAF6 [cytosol]
(R-HSA-166366)
K48polyUb-TRAF6 [cytosol]
(R-HSA-6783233)
K63polyUb-TRAF6 [cytosol]
(R-HSA-2685681)
K63polyUb-TRAF6 [plasma membrane]
(R-HSA-936980)
TRAF6 [plasma membrane]
(R-HSA-936955)
K63polyUb-TRAF6 [endosome membrane]
(R-HSA-450227)
TRAF6 [endosome membrane]
(R-HSA-450272)
PolyUb-TRAF6 [cytosol]
(R-HSA-6782845)
0.589
2
UniProt:P42858 HTT
1
HTT [nucleoplasm]
(R-HSA-9023590)
0.556
3
UniProt:P04233 CD74
13
CD74(81-104) [lysosomal membrane]
(R-HSA-2130446)
CD74(81-104) [lysosomal lumen]
(R-HSA-2213220)
CD74(1-117) [lysosomal membrane]
(R-HSA-2130632)
CD74 [transport vesicle membrane]
(R-HSA-2130624)
CD74 [clathrin-coated endocytic vesicle membrane]
(R-HSA-2130522)
CD74 [endocytic vesicle membrane]
(R-HSA-2130460)
CD74 [integral component of lumenal side of endoplasmic reticulum membrane]
(R-HSA-1181241)
CD74 [ER to Golgi transport vesicle membrane]
(R-HSA-2130456)
CD74 [Golgi membrane]
(R-HSA-2214388)
CD74 [trans-Golgi network membrane]
(R-HSA-2130493)
CD74(1-176) [lysosomal membrane]
(R-HSA-2130602)
CD74 [lysosomal membrane]
(R-HSA-2130320)
CD74 [plasma membrane]
(R-HSA-2130666)
0.547
2
cardiolipin [ChEBI:28494]
4
CL [endoplasmic reticulum membrane]
(R-ALL-1524081)
cardiolipin [plasma membrane]
(R-ALL-9647661)
cardiolipin [mitochondrial outer membrane]
(R-ALL-8954419)
cardiolipin [mitochondrial inner membrane]
(R-ALL-1524072)
0.544
2
UniProt:P05919 vpu
6
VPU (P05919) protein [cytosol]
(R-HIV-173223)
VPU (P05919) protein [extracellular region]
(R-HIV-175522)
VPU (P05919) protein [nucleoplasm]
(R-HIV-175200)
VPU (P05919) [endoplasmic reticulum membrane]
(R-HIV-180550)
VPU (P05919) [Golgi membrane]
(R-HIV-3149439)
VPU (P05919) [plasma membrane]
(R-HIV-3149423)
0.462
4
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