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Protein

Trifunctional enzyme subunit alpha, mitochondrial

Gene

HADHA

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

Functioni

Bifunctional subunit.

Catalytic activityi

(3S)-3-hydroxyacyl-CoA = trans-2(or 3)-enoyl-CoA + H2O.
A long-chain (S)-3-hydroxyacyl-CoA + NAD+ = a long-chain 3-oxoacyl-CoA + NADH.

Pathwayi: fatty acid beta-oxidation

This protein is involved in the pathway fatty acid beta-oxidation, which is part of Lipid metabolism.
View all proteins of this organism that are known to be involved in the pathway fatty acid beta-oxidation and in Lipid metabolism.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sitei151Important for catalytic activityBy similarity1
Sitei173Important for catalytic activityBy similarity1

GO - Molecular functioni

GO - Biological processi

Keywordsi

Molecular functionLyase, Multifunctional enzyme, Oxidoreductase
Biological processFatty acid metabolism, Lipid metabolism
LigandNAD

Enzyme and pathway databases

BioCyciMetaCyc:HS01481-MONOMER
ReactomeiR-HSA-1482798 Acyl chain remodeling of CL
R-HSA-77285 Beta oxidation of myristoyl-CoA to lauroyl-CoA
R-HSA-77288 mitochondrial fatty acid beta-oxidation of unsaturated fatty acids
R-HSA-77305 Beta oxidation of palmitoyl-CoA to myristoyl-CoA
R-HSA-77310 Beta oxidation of lauroyl-CoA to decanoyl-CoA-CoA
R-HSA-77346 Beta oxidation of decanoyl-CoA to octanoyl-CoA-CoA
R-HSA-77348 Beta oxidation of octanoyl-CoA to hexanoyl-CoA
R-HSA-77350 Beta oxidation of hexanoyl-CoA to butanoyl-CoA
SABIO-RKiP40939
UniPathwayi
UPA00659

Chemistry databases

SwissLipidsiSLP:000000247

Names & Taxonomyi

Protein namesi
Recommended name:
Trifunctional enzyme subunit alpha, mitochondrial
Alternative name(s):
78 kDa gastrin-binding protein
TP-alpha
Including the following 2 domains:
Long-chain enoyl-CoA hydratase (EC:4.2.1.17)
Long chain 3-hydroxyacyl-CoA dehydrogenase (EC:1.1.1.211)
Gene namesi
Name:HADHA
Synonyms:HADH
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 2

Organism-specific databases

EuPathDBiHostDB:ENSG00000084754.10
HGNCiHGNC:4801 HADHA
MIMi600890 gene
neXtProtiNX_P40939

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

Mitochondrion

Pathology & Biotechi

Involvement in diseasei

Mitochondrial trifunctional protein deficiency (MTPD)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA disease biochemically characterized by loss of all enzyme activities of the mitochondrial trifunctional protein complex. Variable clinical manifestations include hypoglycemia, cardiomyopathy, delayed psychomotor development, sensorimotor axonopathy, generalized weakness, hepatic dysfunction, respiratory failure. Sudden infant death may occur. Most patients die from heart failure.
See also OMIM:609015
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_021125282V → D in MTPD; mild phenotype with slowly progressive myopathy and sensorimotor polyneuropathy. 1 PublicationCorresponds to variant dbSNP:rs137852773EnsemblClinVar.1
Natural variantiVAR_021126305I → N in MTPD; mild phenotype with slowly progressive myopathy and sensorimotor polyneuropathy. 1 PublicationCorresponds to variant dbSNP:rs137852774EnsemblClinVar.1
Long-chain 3-hydroxyl-CoA dehydrogenase deficiency (LCHAD deficiency)2 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionThe clinical features are very similar to TFP deficiency. Biochemically, LCHAD deficiency is characterized by reduced long-chain 3-hydroxyl-CoA dehydrogenase activity, while the other enzyme activities of the TFP complex are normal or only slightly reduced.
See also OMIM:609016
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_021127342L → P in LCHAD deficiency. 1 PublicationCorresponds to variant dbSNP:rs137852772EnsemblClinVar.1
Maternal acute fatty liver of pregnancy (AFLP)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionSevere maternal illness occurring during pregnancies with affected fetuses. This disease is associated with LCHAD deficiency and characterized by sudden unexplained infant death or hypoglycemia and abnormal liver enzymes (Reye-like syndrome).
See also OMIM:609016

Keywords - Diseasei

Disease mutation

Organism-specific databases

DisGeNETi3030
MalaCardsiHADHA
MIMi609015 phenotype
609016 phenotype
OpenTargetsiENSG00000084754
Orphaneti243367 Acute fatty liver of pregnancy
5 Long chain 3-hydroxyacyl-CoA dehydrogenase deficiency
746 Mitochondrial trifunctional protein deficiency
PharmGKBiPA29175

Chemistry databases

DrugBankiDB00157 NADH

Polymorphism and mutation databases

BioMutaiHADHA
DMDMi20141376

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 36MitochondrionSequence analysisAdd BLAST36
ChainiPRO_000000740337 – 763Trifunctional enzyme subunit alpha, mitochondrialAdd BLAST727

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei46N6-acetyllysine; alternateBy similarity1
Modified residuei46N6-succinyllysine; alternateBy similarity1
Modified residuei60N6-acetyllysine; alternateBy similarity1
Modified residuei60N6-succinyllysine; alternateBy similarity1
Modified residuei129N6-acetyllysineBy similarity1
Modified residuei166N6-acetyllysine; alternateBy similarity1
Modified residuei166N6-succinyllysine; alternateBy similarity1
Modified residuei213N6-succinyllysineBy similarity1
Modified residuei214N6-acetyllysine; alternateBy similarity1
Modified residuei214N6-succinyllysine; alternateBy similarity1
Modified residuei230N6-succinyllysineBy similarity1
Modified residuei249N6-acetyllysine; alternateBy similarity1
Modified residuei249N6-succinyllysine; alternateBy similarity1
Modified residuei289N6-acetyllysineBy similarity1
Modified residuei295N6-acetyllysineCombined sources1
Modified residuei303N6-acetyllysine; alternateCombined sources1
Modified residuei303N6-succinyllysine; alternateBy similarity1
Modified residuei316PhosphoserineBy similarity1
Modified residuei326N6-acetyllysine; alternateBy similarity1
Modified residuei326N6-succinyllysine; alternateBy similarity1
Modified residuei334N6-acetyllysine; alternateBy similarity1
Modified residuei334N6-succinyllysine; alternateBy similarity1
Modified residuei350N6-acetyllysine; alternateBy similarity1
Modified residuei350N6-succinyllysine; alternateBy similarity1
Modified residuei353N6-acetyllysineBy similarity1
Modified residuei395PhosphothreonineCombined sources1
Modified residuei399Omega-N-methylarginineBy similarity1
Modified residuei406N6-acetyllysine; alternateCombined sources1
Modified residuei406N6-succinyllysine; alternateBy similarity1
Modified residuei411N6-acetyllysine; alternateBy similarity1
Modified residuei411N6-succinyllysine; alternateBy similarity1
Modified residuei415N6-succinyllysineBy similarity1
Modified residuei419PhosphoserineBy similarity1
Modified residuei440N6-succinyllysineBy similarity1
Modified residuei460N6-acetyllysine; alternateBy similarity1
Modified residuei460N6-succinyllysine; alternateBy similarity1
Modified residuei505N6-acetyllysine; alternateCombined sources1
Modified residuei505N6-succinyllysine; alternateBy similarity1
Modified residuei519N6-acetyllysine; alternateBy similarity1
Modified residuei519N6-succinyllysine; alternateBy similarity1
Modified residuei540N6-acetyllysineCombined sources1
Modified residuei569N6-acetyllysine; alternateBy similarity1
Modified residuei569N6-succinyllysine; alternateBy similarity1
Modified residuei634N6-succinyllysineBy similarity1
Modified residuei644N6-acetyllysine; alternateCombined sources1
Modified residuei644N6-succinyllysine; alternateBy similarity1
Modified residuei646N6-succinyllysineBy similarity1
Modified residuei650PhosphoserineBy similarity1
Modified residuei664N6-acetyllysine; alternateBy similarity1
Modified residuei664N6-succinyllysine; alternateBy similarity1
Modified residuei728N6-acetyllysine; alternateBy similarity1
Modified residuei728N6-succinyllysine; alternateBy similarity1
Modified residuei735N6-acetyllysineBy similarity1
Modified residuei756PhosphoserineCombined sources1
Modified residuei759N6-acetyllysine; alternateBy similarity1
Modified residuei759N6-succinyllysine; alternateBy similarity1

Keywords - PTMi

Acetylation, Methylation, Phosphoprotein

Proteomic databases

EPDiP40939
MaxQBiP40939
PaxDbiP40939
PeptideAtlasiP40939
PRIDEiP40939
ProteomicsDBi55392

2D gel databases

REPRODUCTION-2DPAGEiIPI00031522
UCD-2DPAGEiP40939

PTM databases

iPTMnetiP40939
PhosphoSitePlusiP40939
SwissPalmiP40939

Expressioni

Gene expression databases

BgeeiENSG00000084754 Expressed in 242 organ(s), highest expression level in gastrocnemius
CleanExiHS_HADH
HS_HADHA
ExpressionAtlasiP40939 baseline and differential
GenevisibleiP40939 HS

Organism-specific databases

HPAiHPA015536
HPA056070

Interactioni

Subunit structurei

Octamer of 4 alpha (HADHA) and 4 beta (HADHB) subunits.1 Publication

Binary interactionsi

Protein-protein interaction databases

BioGridi109280, 115 interactors
IntActiP40939, 68 interactors
MINTiP40939
STRINGi9606.ENSP00000370023

Structurei

3D structure databases

ProteinModelPortaliP40939
SMRiP40939
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

In the N-terminal section; belongs to the enoyl-CoA hydratase/isomerase family.Curated
In the central section; belongs to the 3-hydroxyacyl-CoA dehydrogenase family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG1683 Eukaryota
COG1024 LUCA
COG1250 LUCA
GeneTreeiENSGT00880000137923
HOGENOMiHOG000261346
HOVERGENiHBG005557
InParanoidiP40939
KOiK07515
OMAiPFRYMDT
OrthoDBiEOG091G02LF
PhylomeDBiP40939
TreeFamiTF352288

Family and domain databases

InterProiView protein in InterPro
IPR006180 3-OHacyl-CoA_DH_CS
IPR006176 3-OHacyl-CoA_DH_NAD-bd
IPR006108 3HC_DH_C
IPR008927 6-PGluconate_DH-like_C_sf
IPR029045 ClpP/crotonase-like_dom_sf
IPR018376 Enoyl-CoA_hyd/isom_CS
IPR001753 Enoyl-CoA_hydra/iso
IPR012803 Fa_ox_alpha_mit
IPR036291 NAD(P)-bd_dom_sf
PfamiView protein in Pfam
PF00725 3HCDH, 2 hits
PF02737 3HCDH_N, 1 hit
PF00378 ECH_1, 1 hit
SUPFAMiSSF48179 SSF48179, 2 hits
SSF51735 SSF51735, 1 hit
SSF52096 SSF52096, 1 hit
TIGRFAMsiTIGR02441 fa_ox_alpha_mit, 1 hit
PROSITEiView protein in PROSITE
PS00067 3HCDH, 1 hit
PS00166 ENOYL_COA_HYDRATASE, 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 6 potential isoforms that are computationally mapped.Show allAlign All

Isoform 1 (identifier: P40939-1) [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
MVACRAIGIL SRFSAFRILR SRGYICRNFT GSSALLTRTH INYGVKGDVA
60 70 80 90 100
VVRINSPNSK VNTLSKELHS EFSEVMNEIW ASDQIRSAVL ISSKPGCFIA
110 120 130 140 150
GADINMLAAC KTLQEVTQLS QEAQRIVEKL EKSTKPIVAA INGSCLGGGL
160 170 180 190 200
EVAISCQYRI ATKDRKTVLG TPEVLLGALP GAGGTQRLPK MVGVPAALDM
210 220 230 240 250
MLTGRSIRAD RAKKMGLVDQ LVEPLGPGLK PPEERTIEYL EEVAITFAKG
260 270 280 290 300
LADKKISPKR DKGLVEKLTA YAMTIPFVRQ QVYKKVEEKV RKQTKGLYPA
310 320 330 340 350
PLKIIDVVKT GIEQGSDAGY LCESQKFGEL VMTKESKALM GLYHGQVLCK
360 370 380 390 400
KNKFGAPQKD VKHLAILGAG LMGAGIAQVS VDKGLKTILK DATLTALDRG
410 420 430 440 450
QQQVFKGLND KVKKKALTSF ERDSIFSNLT GQLDYQGFEK ADMVIEAVFE
460 470 480 490 500
DLSLKHRVLK EVEAVIPDHC IFASNTSALP ISEIAAVSKR PEKVIGMHYF
510 520 530 540 550
SPVDKMQLLE IITTEKTSKD TSASAVAVGL KQGKVIIVVK DGPGFYTTRC
560 570 580 590 600
LAPMMSEVIR ILQEGVDPKK LDSLTTSFGF PVGAATLVDE VGVDVAKHVA
610 620 630 640 650
EDLGKVFGER FGGGNPELLT QMVSKGFLGR KSGKGFYIYQ EGVKRKDLNS
660 670 680 690 700
DMDSILASLK LPPKSEVSSD EDIQFRLVTR FVNEAVMCLQ EGILATPAEG
710 720 730 740 750
DIGAVFGLGF PPCLGGPFRF VDLYGAQKIV DRLKKYEAAY GKQFTPCQLL
760
ADHANSPNKK FYQ
Length:763
Mass (Da):83,000
Last modified:April 3, 2002 - v2
Checksum:i247FF7B4E48FB484
GO
Isoform 2 (identifier: P40939-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     61-82: VNTLSKELHSEFSEVMNEIWAS → HVSRLQDPSRSNTAITRSTENS
     83-763: Missing.

Note: No experimental confirmation available. May be produced at very low levels due to a premature stop codon in the mRNA, leading to nonsense-mediated mRNA decay.
Show »
Length:82
Mass (Da):9,054
Checksum:i0AE1BB06A4C4A924
GO

Computationally mapped potential isoform sequencesi

There are 6 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
H0YFD6H0YFD6_HUMAN
Trifunctional enzyme subunit alpha,...
HADHA
792Annotation score:
A0A2R8Y4F5A0A2R8Y4F5_HUMAN
Trifunctional enzyme subunit alpha,...
HADHA
728Annotation score:
A0A2R8YG21A0A2R8YG21_HUMAN
Trifunctional enzyme subunit alpha,...
HADHA
499Annotation score:
A0A2R8Y688A0A2R8Y688_HUMAN
Trifunctional enzyme subunit alpha,...
HADHA
357Annotation score:
A0A2R8Y4E0A0A2R8Y4E0_HUMAN
Trifunctional enzyme subunit alpha,...
HADHA
54Annotation score:
A0A2R8YDM1A0A2R8YDM1_HUMAN
Trifunctional enzyme subunit alpha,...
HADHA
37Annotation score:

Sequence cautioni

The sequence BAG63804 differs from that shown. Wrong choice of frame.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti146L → V in BAA03941 (PubMed:8135828).Curated1
Sequence conflicti152V → L in AAA56664 (PubMed:7918661).Curated1
Sequence conflicti171T → A in AAA56664 (PubMed:7918661).Curated1
Sequence conflicti178A → I in AAA56664 (PubMed:7918661).Curated1
Sequence conflicti197 – 198AL → VF in AAA56664 (PubMed:7918661).Curated2
Sequence conflicti206S → N in AAA56664 (PubMed:7918661).Curated1
Sequence conflicti211R → S in AAA56664 (PubMed:7918661).Curated1
Sequence conflicti576T → P in AAA56664 (PubMed:7918661).Curated1
Sequence conflicti694L → S in BAA03941 (PubMed:8135828).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_021125282V → D in MTPD; mild phenotype with slowly progressive myopathy and sensorimotor polyneuropathy. 1 PublicationCorresponds to variant dbSNP:rs137852773EnsemblClinVar.1
Natural variantiVAR_021126305I → N in MTPD; mild phenotype with slowly progressive myopathy and sensorimotor polyneuropathy. 1 PublicationCorresponds to variant dbSNP:rs137852774EnsemblClinVar.1
Natural variantiVAR_021127342L → P in LCHAD deficiency. 1 PublicationCorresponds to variant dbSNP:rs137852772EnsemblClinVar.1
Natural variantiVAR_048908358Q → K. Corresponds to variant dbSNP:rs2229420EnsemblClinVar.1
Natural variantiVAR_002273510E → Q in AFLP and LCHAD deficiency; loss of activity. 4 PublicationsCorresponds to variant dbSNP:rs137852769EnsemblClinVar.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_05901061 – 82VNTLS…EIWAS → HVSRLQDPSRSNTAITRSTE NS in isoform 2. 1 PublicationAdd BLAST22
Alternative sequenceiVSP_05901183 – 763Missing in isoform 2. 1 PublicationAdd BLAST681

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
D16480 mRNA Translation: BAA03941.1
U04627 mRNA Translation: AAA56664.1
AK302532 mRNA Translation: BAG63804.1 Sequence problems.
AK313027 mRNA Translation: BAG35861.1
AC010896 Genomic DNA Translation: AAY14643.1
AC011742 Genomic DNA Translation: AAX93141.1
CH471053 Genomic DNA Translation: EAX00703.1
BC009235 mRNA Translation: AAH09235.1
AB020811 Genomic DNA Translation: BAA76735.1
CCDSiCCDS1721.1 [P40939-1]
PIRiJC2108
RefSeqiNP_000173.2, NM_000182.4 [P40939-1]
UniGeneiHs.516032

Genome annotation databases

EnsembliENST00000380649; ENSP00000370023; ENSG00000084754 [P40939-1]
ENST00000646483; ENSP00000496185; ENSG00000084754 [P40939-2]
GeneIDi3030
KEGGihsa:3030
UCSCiuc002rgy.3 human [P40939-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
D16480 mRNA Translation: BAA03941.1
U04627 mRNA Translation: AAA56664.1
AK302532 mRNA Translation: BAG63804.1 Sequence problems.
AK313027 mRNA Translation: BAG35861.1
AC010896 Genomic DNA Translation: AAY14643.1
AC011742 Genomic DNA Translation: AAX93141.1
CH471053 Genomic DNA Translation: EAX00703.1
BC009235 mRNA Translation: AAH09235.1
AB020811 Genomic DNA Translation: BAA76735.1
CCDSiCCDS1721.1 [P40939-1]
PIRiJC2108
RefSeqiNP_000173.2, NM_000182.4 [P40939-1]
UniGeneiHs.516032

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
5ZQZelectron microscopy4.20A/C1-763[»]
5ZRVelectron microscopy7.70A/C/E/G1-763[»]
6DV2X-ray3.60G/H/I/J/K/L37-763[»]
ProteinModelPortaliP40939
SMRiP40939
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi109280, 115 interactors
IntActiP40939, 68 interactors
MINTiP40939
STRINGi9606.ENSP00000370023

Chemistry databases

DrugBankiDB00157 NADH
SwissLipidsiSLP:000000247

PTM databases

iPTMnetiP40939
PhosphoSitePlusiP40939
SwissPalmiP40939

Polymorphism and mutation databases

BioMutaiHADHA
DMDMi20141376

2D gel databases

REPRODUCTION-2DPAGEiIPI00031522
UCD-2DPAGEiP40939

Proteomic databases

EPDiP40939
MaxQBiP40939
PaxDbiP40939
PeptideAtlasiP40939
PRIDEiP40939
ProteomicsDBi55392

Protocols and materials databases

DNASUi3030
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000380649; ENSP00000370023; ENSG00000084754 [P40939-1]
ENST00000646483; ENSP00000496185; ENSG00000084754 [P40939-2]
GeneIDi3030
KEGGihsa:3030
UCSCiuc002rgy.3 human [P40939-1]

Organism-specific databases

CTDi3030
DisGeNETi3030
EuPathDBiHostDB:ENSG00000084754.10
GeneCardsiHADHA
HGNCiHGNC:4801 HADHA
HPAiHPA015536
HPA056070
MalaCardsiHADHA
MIMi600890 gene
609015 phenotype
609016 phenotype
neXtProtiNX_P40939
OpenTargetsiENSG00000084754
Orphaneti243367 Acute fatty liver of pregnancy
5 Long chain 3-hydroxyacyl-CoA dehydrogenase deficiency
746 Mitochondrial trifunctional protein deficiency
PharmGKBiPA29175
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG1683 Eukaryota
COG1024 LUCA
COG1250 LUCA
GeneTreeiENSGT00880000137923
HOGENOMiHOG000261346
HOVERGENiHBG005557
InParanoidiP40939
KOiK07515
OMAiPFRYMDT
OrthoDBiEOG091G02LF
PhylomeDBiP40939
TreeFamiTF352288

Enzyme and pathway databases

UniPathwayi
UPA00659

BioCyciMetaCyc:HS01481-MONOMER
ReactomeiR-HSA-1482798 Acyl chain remodeling of CL
R-HSA-77285 Beta oxidation of myristoyl-CoA to lauroyl-CoA
R-HSA-77288 mitochondrial fatty acid beta-oxidation of unsaturated fatty acids
R-HSA-77305 Beta oxidation of palmitoyl-CoA to myristoyl-CoA
R-HSA-77310 Beta oxidation of lauroyl-CoA to decanoyl-CoA-CoA
R-HSA-77346 Beta oxidation of decanoyl-CoA to octanoyl-CoA-CoA
R-HSA-77348 Beta oxidation of octanoyl-CoA to hexanoyl-CoA
R-HSA-77350 Beta oxidation of hexanoyl-CoA to butanoyl-CoA
SABIO-RKiP40939

Miscellaneous databases

ChiTaRSiHADHA human
GenomeRNAii3030
PROiPR:P40939
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000084754 Expressed in 242 organ(s), highest expression level in gastrocnemius
CleanExiHS_HADH
HS_HADHA
ExpressionAtlasiP40939 baseline and differential
GenevisibleiP40939 HS

Family and domain databases

InterProiView protein in InterPro
IPR006180 3-OHacyl-CoA_DH_CS
IPR006176 3-OHacyl-CoA_DH_NAD-bd
IPR006108 3HC_DH_C
IPR008927 6-PGluconate_DH-like_C_sf
IPR029045 ClpP/crotonase-like_dom_sf
IPR018376 Enoyl-CoA_hyd/isom_CS
IPR001753 Enoyl-CoA_hydra/iso
IPR012803 Fa_ox_alpha_mit
IPR036291 NAD(P)-bd_dom_sf
PfamiView protein in Pfam
PF00725 3HCDH, 2 hits
PF02737 3HCDH_N, 1 hit
PF00378 ECH_1, 1 hit
SUPFAMiSSF48179 SSF48179, 2 hits
SSF51735 SSF51735, 1 hit
SSF52096 SSF52096, 1 hit
TIGRFAMsiTIGR02441 fa_ox_alpha_mit, 1 hit
PROSITEiView protein in PROSITE
PS00067 3HCDH, 1 hit
PS00166 ENOYL_COA_HYDRATASE, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiECHA_HUMAN
AccessioniPrimary (citable) accession number: P40939
Secondary accession number(s): B2R7L4
, B4DYP2, Q16679, Q53T69, Q53TA2, Q96GT7, Q9UQC5
Entry historyiIntegrated into UniProtKB/Swiss-Prot: February 1, 1995
Last sequence update: April 3, 2002
Last modified: November 7, 2018
This is version 212 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 chromosome 2
    Human chromosome 2: entries, gene names and cross-references to MIM
  3. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  4. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  5. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  6. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  7. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
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