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Protein

Hydroxyacid-oxoacid transhydrogenase, mitochondrial

Gene

Adhfe1

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Catalyzes the cofactor-independent reversible oxidation of gamma-hydroxybutyrate (GHB) to succinic semialdehyde (SSA) coupled to reduction of 2-ketoglutarate (2-KG) to D-2-hydroxyglutarate (D-2-HG). L-3-hydroxybutyrate (L-3-OHB) is also a substrate for HOT when using 2-KG as hydrogen acceptor, resulting in the formation of D-2-HG (By similarity).By similarity

Catalytic activityi

(S)-3-hydroxybutanoate + 2-oxoglutarate = acetoacetate + (R)-2-hydroxyglutarate.
4-hydroxybutanoate + 2-oxoglutarate = succinic semialdehyde + (R)-2-hydroxyglutarate.

GO - Molecular functioni

GO - Biological processi

Keywordsi

Molecular functionOxidoreductase

Enzyme and pathway databases

ReactomeiR-MMU-880009 Interconversion of 2-oxoglutarate and 2-hydroxyglutarate
SABIO-RKQ8R0N6

Names & Taxonomyi

Protein namesi
Recommended name:
Hydroxyacid-oxoacid transhydrogenase, mitochondrial (EC:1.1.99.24)
Short name:
HOT
Alternative name(s):
Alcohol dehydrogenase iron-containing protein 1
Short name:
ADHFe1
Gene namesi
Name:Adhfe1
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 1

Organism-specific databases

MGIiMGI:1923437 Adhfe1

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
ChainiPRO_0000322997? – 465Hydroxyacid-oxoacid transhydrogenase, mitochondrial
Transit peptidei1 – ?MitochondrionSequence analysis

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei443N6-acetyllysineCombined sources1
Modified residuei450PhosphoserineCombined sources1

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

PaxDbiQ8R0N6
PeptideAtlasiQ8R0N6
PRIDEiQ8R0N6

PTM databases

iPTMnetiQ8R0N6
PhosphoSitePlusiQ8R0N6
SwissPalmiQ8R0N6

Expressioni

Tissue specificityi

Expressed in white and brown adipose tissues, liver, and kidney. Expression is differentiation-dependent during in vitro brown and white adipogenesis.1 Publication

Inductioni

Down-regulated of 40% in white adipose tissue of ob/ob obese mice.1 Publication

Gene expression databases

BgeeiENSMUSG00000025911
CleanExiMM_ADHFE1
ExpressionAtlasiQ8R0N6 baseline and differential
GenevisibleiQ8R0N6 MM

Interactioni

Protein-protein interaction databases

IntActiQ8R0N6, 3 interactors
MINTiQ8R0N6
STRINGi10090.ENSMUSP00000116627

Structurei

3D structure databases

ProteinModelPortaliQ8R0N6
SMRiQ8R0N6
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG3857 Eukaryota
COG1454 LUCA
GeneTreeiENSGT00390000003849
HOGENOMiHOG000243335
HOVERGENiHBG057032
InParanoidiQ8R0N6
KOiK11173
OMAiRAACQCP
OrthoDBiEOG091G064B
PhylomeDBiQ8R0N6
TreeFamiTF105710

Family and domain databases

InterProiView protein in InterPro
IPR001670 ADH_Fe/gldA
PfamiView protein in Pfam
PF00465 Fe-ADH, 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

Isoform 1 (identifier: Q8R0N6-1) [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
MAAAARARVT HLLRHLQSTA CQCPTHSHTY SQAPGPSGKT ADYAFEMAVS
60 70 80 90 100
NIRYGAGVTK EVGMDLQNMG AKNVCLMTDK NLSQLPPVQI VMDSLSKNGI
110 120 130 140 150
SFQVYDDVRV EPTDGSFMDA IEFAKKGAFD AYVAVGGGST MDTCKAANLY
160 170 180 190 200
ASSPHSEFLD YVNAPIGKGK PVTVPLKPLI AVPTTSGTGS ETTGVAIFDY
210 220 230 240 250
EHLKVKTGIA SRAIKPTLGL VDPLHTLHMP CQVVANSGFD VLCHALESYT
260 270 280 290 300
AIPYSMRSPC PSNPIQRPAY QGSNPISDIW AVHALQIVAK YLKRAVRNPD
310 320 330 340 350
DLEARSKMHL ASAFAGIGFG NAGVHLCHGM SYPISGLVKT YKAKEYNVDH
360 370 380 390 400
PLVPHGLSVV LTSPAVFTFT AQMFPERHLE TAGILGANIR TARIQDAGLV
410 420 430 440 450
LADALRKFLF DLNVDDGLAA LGYSKDDIPS LVKGTLPQER VTKLAPRAQS
460
EEDLSALFEA SMKLY
Length:465
Mass (Da):49,937
Last modified:July 5, 2004 - v2
Checksum:iCD1463685F788C2A
GO
Isoform 2 (identifier: Q8R0N6-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-46: Missing.

Show »
Length:419
Mass (Da):45,015
Checksum:i510F18E8678AB656
GO

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0319871 – 46Missing in isoform 2. 1 PublicationAdd BLAST46

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK038853 mRNA Translation: BAC30151.1
AK050178 mRNA Translation: BAC34108.1
BC026584 mRNA Translation: AAH26584.2
CCDSiCCDS14812.1 [Q8R0N6-1]
RefSeqiNP_780445.1, NM_175236.4 [Q8R0N6-1]
XP_006495649.1, XM_006495586.2 [Q8R0N6-2]
XP_017168188.1, XM_017312699.1
UniGeneiMm.28514

Genome annotation databases

EnsembliENSMUST00000144177; ENSMUSP00000116627; ENSMUSG00000025911 [Q8R0N6-1]
GeneIDi76187
KEGGimmu:76187
UCSCiuc007agl.2 mouse [Q8R0N6-1]

Keywords - Coding sequence diversityi

Alternative splicing

Similar proteinsi

Entry informationi

Entry nameiHOT_MOUSE
AccessioniPrimary (citable) accession number: Q8R0N6
Secondary accession number(s): Q78SV3, Q8BYP0
Entry historyiIntegrated into UniProtKB/Swiss-Prot: March 18, 2008
Last sequence update: July 5, 2004
Last modified: March 28, 2018
This is version 109 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

Complete proteome, Reference proteome
UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health