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Protein

Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial

Gene

HADH

Organism
Sus scrofa (Pig)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Plays an essential role in the mitochondrial beta-oxidation of short chain fatty acids. Exerts it highest activity toward 3-hydroxybutyryl-CoA.1 Publication

Catalytic activityi

(S)-3-hydroxyacyl-CoA + NAD+ = 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
Binding sitei57NAD1 Publication1
Binding sitei73Coenzyme ABy similarity1
Binding sitei80Coenzyme ABy similarity1
Binding sitei122NAD1 Publication1
Binding sitei127NAD1 Publication1
Binding sitei149Coenzyme ABy similarity1
Binding sitei149NAD1 Publication1
Sitei170Important for catalytic activityBy similarity1
Binding sitei173NADBy similarity1
Binding sitei305NADBy similarity1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi34 – 39NAD1 Publication6

GO - Molecular functioni

GO - Biological processi

Keywordsi

Molecular functionOxidoreductase
Biological processFatty acid metabolism, Lipid metabolism
LigandNAD

Enzyme and pathway databases

ReactomeiR-SSC-77310 Beta oxidation of lauroyl-CoA to decanoyl-CoA-CoA
R-SSC-77346 Beta oxidation of decanoyl-CoA to octanoyl-CoA-CoA
R-SSC-77348 Beta oxidation of octanoyl-CoA to hexanoyl-CoA
R-SSC-77350 Beta oxidation of hexanoyl-CoA to butanoyl-CoA
R-SSC-77352 Beta oxidation of butanoyl-CoA to acetyl-CoA
SABIO-RKiP00348
UniPathwayiUPA00659

Names & Taxonomyi

Protein namesi
Recommended name:
Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial (EC:1.1.1.35)
Short name:
HCDH
Alternative name(s):
Medium and short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase
Short-chain 3-hydroxyacyl-CoA dehydrogenase
Gene namesi
Name:HADH
Synonyms:HAD, HADHSC, SCHAD
OrganismiSus scrofa (Pig)
Taxonomic identifieri9823 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaLaurasiatheriaCetartiodactylaSuinaSuidaeSus
Proteomesi

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 – 12MitochondrionBy similarityAdd BLAST12
ChainiPRO_000000740813 – 314Hydroxyacyl-coenzyme A dehydrogenase, mitochondrialAdd BLAST302

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei80N6-succinyllysineBy similarity1
Modified residuei81N6-acetyllysine; alternateBy similarity1
Modified residuei81N6-succinyllysine; alternateBy similarity1
Modified residuei87N6-acetyllysine; alternateBy similarity1
Modified residuei87N6-succinyllysine; alternateBy similarity1
Modified residuei125N6-acetyllysineBy similarity1
Modified residuei136N6-acetyllysine; alternateBy similarity1
Modified residuei136N6-succinyllysine; alternateBy similarity1
Modified residuei179N6-acetyllysineBy similarity1
Modified residuei185N6-acetyllysine; alternateBy similarity1
Modified residuei185N6-succinyllysine; alternateBy similarity1
Modified residuei192N6-acetyllysine; alternateBy similarity1
Modified residuei192N6-succinyllysine; alternateBy similarity1
Modified residuei202N6-acetyllysine; alternateBy similarity1
Modified residuei202N6-succinyllysine; alternateBy similarity1
Modified residuei206N6-succinyllysineBy similarity1
Modified residuei212N6-acetyllysine; alternateBy similarity1
Modified residuei212N6-succinyllysine; alternateBy similarity1
Modified residuei241N6-acetyllysine; alternateBy similarity1
Modified residuei241N6-succinyllysine; alternateBy similarity1
Modified residuei312N6-acetyllysine; alternateBy similarity1
Modified residuei312N6-succinyllysine; alternateBy similarity1

Post-translational modificationi

Succinylation at Lys-81, adjacent to a coenzyme A binding site. Desuccinylated by SIRT5 (By similarity).By similarity

Keywords - PTMi

Acetylation

Proteomic databases

PaxDbiP00348
PeptideAtlasiP00348
PRIDEiP00348

Expressioni

Gene expression databases

BgeeiENSSSCG00000009150
GenevisibleiP00348 SS

Interactioni

Subunit structurei

Homodimer.1 Publication

Protein-protein interaction databases

STRINGi9823.ENSSSCP00000009757

Structurei

Secondary structure

1314
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Beta strandi29 – 33Combined sources5
Helixi37 – 48Combined sources12
Beta strandi52 – 56Combined sources5
Helixi60 – 81Combined sources22
Turni82 – 84Combined sources3
Helixi86 – 98Combined sources13
Beta strandi100 – 104Combined sources5
Helixi106 – 108Combined sources3
Beta strandi110 – 112Combined sources3
Beta strandi114 – 118Combined sources5
Helixi124 – 137Combined sources14
Beta strandi143 – 146Combined sources4
Beta strandi149 – 151Combined sources3
Helixi153 – 157Combined sources5
Helixi163 – 165Combined sources3
Beta strandi166 – 170Combined sources5
Turni175 – 177Combined sources3
Beta strandi180 – 185Combined sources6
Helixi191 – 203Combined sources13
Beta strandi207 – 213Combined sources7
Turni215 – 218Combined sources4
Helixi219 – 235Combined sources17
Helixi241 – 252Combined sources12
Helixi258 – 265Combined sources8
Helixi267 – 279Combined sources13
Turni280 – 283Combined sources4
Helixi285 – 287Combined sources3
Helixi291 – 298Combined sources8
Turni304 – 307Combined sources4
Beta strandi308 – 312Combined sources5

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
3HDHX-ray2.80A/B/C13-314[»]
ProteinModelPortaliP00348
SMRiP00348
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiP00348

Family & Domainsi

Sequence similaritiesi

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG2304 Eukaryota
COG1250 LUCA
GeneTreeiENSGT00880000137923
HOGENOMiHOG000141498
HOVERGENiHBG000832
InParanoidiP00348
KOiK00022
OMAiMAHPMGP
OrthoDBiEOG091G089P
TreeFamiTF300886

Family and domain databases

Gene3Di1.10.1040.10, 1 hit
InterProiView protein in InterPro
IPR022694 3-OHacyl-CoA_DH
IPR006180 3-OHacyl-CoA_DH_CS
IPR006176 3-OHacyl-CoA_DH_NAD-bd
IPR006108 3HC_DH_C
IPR008927 6-PGluconate_DH-like_C_sf
IPR013328 6PGD_dom2
IPR036291 NAD(P)-bd_dom_sf
PfamiView protein in Pfam
PF00725 3HCDH, 1 hit
PF02737 3HCDH_N, 1 hit
PIRSFiPIRSF000105 HCDH, 1 hit
SUPFAMiSSF48179 SSF48179, 1 hit
SSF51735 SSF51735, 1 hit
PROSITEiView protein in PROSITE
PS00067 3HCDH, 1 hit

Sequencei

Sequence statusi: Complete.

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

P00348-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MAFATRQLVR SLSSSSTAAA SAKKILVKHV TVIGGGLMGA GIAQVAAATG
60 70 80 90 100
HTVVLVDQTE DILAKSKKGI EESLRKVAKK KFAENPKAGD EFVEKTLSSI
110 120 130 140 150
STSTDAASVV HSTDLVVEAI VENLKVKSEL FKRLDKFAAE HTIFASNTSS
160 170 180 190 200
LQITSLANAT TRQDRFAGLH FFNPVPLMKL VEVVKTPMTS QKTLESLVDF
210 220 230 240 250
SKTLGKHPVS CKDTPGFIVN RLLVPYLIEA VRLYERGDAS KEDIDTAMKL
260 270 280 290 300
GAGYPMGPFE LLDYVGLDTT KFIIDGWHEM DSQNPLFQPS PAMNKLVAEN
310
KFGKKTGEGF YKYK
Length:314
Mass (Da):34,161
Last modified:May 30, 2000 - v2
Checksum:i596CBFD227214C3B
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti108S → A AA sequence (PubMed:7409145).Curated1
Sequence conflicti122E → EQLKVVGE AA sequence (PubMed:7409145).Curated1
Sequence conflicti172 – 174FNP → N AA sequence (PubMed:7409145).Curated3

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF027652 mRNA Translation: AAD20939.1
PIRiT46866
RefSeqiNP_999496.1, NM_214331.1
UniGeneiSsc.12507

Genome annotation databases

EnsembliENSSSCT00000049871; ENSSSCP00000053505; ENSSSCG00000009150
ENSSSCT00000057321; ENSSSCP00000038152; ENSSSCG00000009150
GeneIDi397604
KEGGissc:397604

Entry informationi

Entry nameiHCDH_PIG
AccessioniPrimary (citable) accession number: P00348
Secondary accession number(s): Q9XS66
Entry historyiIntegrated into UniProtKB/Swiss-Prot: July 21, 1986
Last sequence update: May 30, 2000
Last modified: May 23, 2018
This is version 151 of the entry and version 2 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  2. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  3. SIMILARITY comments
    Index of protein domains and families

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