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Protein

Trifunctional enzyme subunit alpha, mitochondrial

Gene

Hadha

Organism
Mus musculus (Mouse)
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

  • fatty acid beta-oxidation Source: MGI
  • response to drug Source: Ensembl
  • response to insulin Source: MGI

Keywordsi

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

Enzyme and pathway databases

ReactomeiR-MMU-1482798 Acyl chain remodeling of CL
R-MMU-77285 Beta oxidation of myristoyl-CoA to lauroyl-CoA
R-MMU-77305 Beta oxidation of palmitoyl-CoA to myristoyl-CoA
R-MMU-77310 Beta oxidation of lauroyl-CoA to decanoyl-CoA-CoA
R-MMU-77346 Beta oxidation of decanoyl-CoA to octanoyl-CoA-CoA
R-MMU-77348 Beta oxidation of octanoyl-CoA to hexanoyl-CoA
R-MMU-77350 Beta oxidation of hexanoyl-CoA to butanoyl-CoA
UniPathwayiUPA00659

Names & Taxonomyi

Protein namesi
Recommended name:
Trifunctional enzyme subunit alpha, mitochondrial
Alternative name(s):
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
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi

Organism-specific databases

MGIiMGI:2135593 Hadha

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

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 36MitochondrionSequence analysisAdd BLAST36
ChainiPRO_000032263937 – 763Trifunctional enzyme subunit alpha, mitochondrialAdd BLAST727

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei46N6-acetyllysine; alternateCombined sources1
Modified residuei46N6-succinyllysine; alternateCombined sources1
Modified residuei60N6-acetyllysine; alternateCombined sources1
Modified residuei60N6-succinyllysine; alternateCombined sources1
Modified residuei129N6-acetyllysineCombined sources1
Modified residuei166N6-acetyllysine; alternateCombined sources1
Modified residuei166N6-succinyllysine; alternateCombined sources1
Modified residuei213N6-succinyllysineCombined sources1
Modified residuei214N6-acetyllysine; alternateCombined sources1
Modified residuei214N6-succinyllysine; alternateCombined sources1
Modified residuei230N6-succinyllysineCombined sources1
Modified residuei231PhosphoserineCombined sources1
Modified residuei249N6-acetyllysine; alternateCombined sources1
Modified residuei249N6-succinyllysine; alternateCombined sources1
Modified residuei289N6-acetyllysineCombined sources1
Modified residuei295N6-acetyllysineBy similarity1
Modified residuei303N6-acetyllysine; alternateCombined sources1
Modified residuei303N6-succinyllysine; alternateCombined sources1
Modified residuei316PhosphoserineCombined sources1
Modified residuei326N6-acetyllysine; alternateCombined sources1
Modified residuei326N6-succinyllysine; alternateCombined sources1
Modified residuei334N6-acetyllysine; alternateCombined sources1
Modified residuei334N6-succinyllysine; alternateCombined sources1
Modified residuei350N6-acetyllysine; alternateCombined sources1
Modified residuei350N6-succinyllysine; alternateCombined sources1
Modified residuei353N6-acetyllysineCombined sources1
Modified residuei395PhosphothreonineCombined sources1
Modified residuei399Omega-N-methylarginineCombined sources1
Modified residuei406N6-acetyllysine; alternateCombined sources1
Modified residuei406N6-succinyllysine; alternateCombined sources1
Modified residuei411N6-acetyllysine; alternateCombined sources1
Modified residuei411N6-succinyllysine; alternateCombined sources1
Modified residuei415N6-succinyllysineCombined sources1
Modified residuei419PhosphoserineBy similarity1
Modified residuei436N6-acetyllysine; alternateCombined sources1
Modified residuei436N6-succinyllysine; alternateCombined sources1
Modified residuei440N6-succinyllysineCombined sources1
Modified residuei460N6-acetyllysine; alternateCombined sources1
Modified residuei460N6-succinyllysine; alternateCombined sources1
Modified residuei505N6-acetyllysine; alternateCombined sources1
Modified residuei505N6-succinyllysine; alternateCombined sources1
Modified residuei519N6-acetyllysine; alternateCombined sources1
Modified residuei519N6-succinyllysine; alternateCombined sources1
Modified residuei540N6-acetyllysineCombined sources1
Modified residuei569N6-acetyllysine; alternateCombined sources1
Modified residuei569N6-succinyllysine; alternateCombined sources1
Modified residuei620N6-succinyllysineCombined sources1
Modified residuei634N6-succinyllysineCombined sources1
Modified residuei644N6-acetyllysine; alternateCombined sources1
Modified residuei644N6-succinyllysine; alternateCombined sources1
Modified residuei646N6-succinyllysineCombined sources1
Modified residuei647PhosphoserineCombined sources1
Modified residuei650PhosphoserineBy similarity1
Modified residuei664N6-acetyllysine; alternateCombined sources1
Modified residuei664N6-succinyllysine; alternateCombined sources1
Modified residuei728N6-acetyllysine; alternateCombined sources1
Modified residuei728N6-succinyllysine; alternateCombined sources1
Modified residuei735N6-acetyllysineCombined sources1
Modified residuei759N6-acetyllysine; alternateCombined sources1
Modified residuei759N6-succinyllysine; alternateCombined sources1

Post-translational modificationi

Acetylation of Lys-569 and Lys-728 is observed in liver mitochondria from fasted mice but not from fed mice.

Keywords - PTMi

Acetylation, Methylation, Phosphoprotein

Proteomic databases

EPDiQ8BMS1
MaxQBiQ8BMS1
PaxDbiQ8BMS1
PeptideAtlasiQ8BMS1
PRIDEiQ8BMS1

2D gel databases

REPRODUCTION-2DPAGEiIPI00223092

PTM databases

iPTMnetiQ8BMS1
PhosphoSitePlusiQ8BMS1
SwissPalmiQ8BMS1

Expressioni

Gene expression databases

BgeeiENSMUSG00000025745
ExpressionAtlasiQ8BMS1 baseline and differential
GenevisibleiQ8BMS1 MM

Interactioni

Subunit structurei

Octamer of 4 alpha (HADHA) and 4 beta (HADHB) subunits.By similarity

Protein-protein interaction databases

BioGridi220648, 7 interactors
IntActiQ8BMS1, 10 interactors
MINTiQ8BMS1
STRINGi10090.ENSMUSP00000120976

Structurei

3D structure databases

ProteinModelPortaliQ8BMS1
SMRiQ8BMS1
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
InParanoidiQ8BMS1
KOiK07515
OMAiPFRYMDT
OrthoDBiEOG091G02LF
PhylomeDBiQ8BMS1
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

Sequencei

Sequence statusi: Complete.

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

Q8BMS1-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MVASRAIGSL SRFSAFRILR SRGCICRSFT TSSALLTRTH INYGVKGDVA
60 70 80 90 100
VIRINSPNSK VNTLNKEVQS EFIEVMNEIW ANDQIRSAVL ISSKPGCFVA
110 120 130 140 150
GADINMLSSC TTPQEATRIS QEGQRMFEKL EKSPKPVVAA ISGSCLGGGL
160 170 180 190 200
ELAIACQYRI ATKDRKTVLG VPEVLLGILP GAGGTQRLPK MVGVPAAFDM
210 220 230 240 250
MLTGRNIRAD RAKKMGLVDQ LVEPLGPGIK SPEERTIEYL EEVAVNFAKG
260 270 280 290 300
LADRKVSAKQ SKGLVEKLTT YAMTVPFVRQ QVYKTVEEKV KKQTKGLYPA
310 320 330 340 350
PLKIIDAVKA GLEQGSDAGY LAESQKFGEL ALTKESKALM GLYNGQVLCK
360 370 380 390 400
KNKFGAPQKN VQQLAILGAG LMGAGIAQVS VDKGLKTLLK DTTVTGLGRG
410 420 430 440 450
QQQVFKGLND KVKKKALTSF ERDSIFSNLI GQLDYKGFEK ADMVIEAVFE
460 470 480 490 500
DLGVKHKVLK EVESVTPEHC IFASNTSALP INQIAAVSKR PEKVIGMHYF
510 520 530 540 550
SPVDKMQLLE IITTDKTSKD TTASAVAVGL RQGKVIIVVK DGPGFYTTRC
560 570 580 590 600
LAPMMSEVMR ILQEGVDPKK LDALTTGFGF PVGAATLADE VGVDVAQHVA
610 620 630 640 650
EDLGKAFGER FGGGSVELLK QMVSKGFLGR KSGKGFYIYQ EGSKNKSLNS
660 670 680 690 700
EMDNILANLR LPAKPEVSSD EDVQYRVITR FVNEAVLCLQ EGILATPAEG
710 720 730 740 750
DIGAVFGLGF PPCLGGPFRF VDLYGAQKVV DRLRKYESAY GTQFTPCQLL
760
LDHANNSSKK FYQ
Length:763
Mass (Da):82,670
Last modified:March 1, 2003 - v1
Checksum:i73D203795D5C1141
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti196A → D in BAE41822 (PubMed:16141072).Curated1
Sequence conflicti459L → S in AAH37009 (PubMed:15489334).Curated1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK029017 mRNA Translation: BAC26245.1
AK170478 mRNA Translation: BAE41822.1
AK170683 mRNA Translation: BAE41956.1
BC027156 mRNA Translation: AAH27156.1
BC037009 mRNA Translation: AAH37009.1
BC046978 mRNA Translation: AAH46978.1
BC058569 mRNA Translation: AAH58569.1
CCDSiCCDS19155.1
RefSeqiNP_849209.1, NM_178878.2
UniGeneiMm.200497

Genome annotation databases

EnsembliENSMUST00000156859; ENSMUSP00000120976; ENSMUSG00000025745
GeneIDi97212
KEGGimmu:97212
UCSCiuc008wvc.1 mouse

Entry informationi

Entry nameiECHA_MOUSE
AccessioniPrimary (citable) accession number: Q8BMS1
Secondary accession number(s): Q3TCY3, Q5U5Y5, Q8QZU4
Entry historyiIntegrated into UniProtKB/Swiss-Prot: March 18, 2008
Last sequence update: March 1, 2003
Last modified: May 23, 2018
This is version 154 of the entry and version 1 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  2. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  3. SIMILARITY comments
    Index of protein domains and families

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