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Protein

3-hydroxyisobutyrate dehydrogenase, mitochondrial

Gene

Hibadh

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

Functioni

Catalytic activityi

3-hydroxy-2-methylpropanoate + NAD+ = 2-methyl-3-oxopropanoate + NADH.

Pathwayi: L-valine degradation

This protein is involved in the pathway L-valine degradation, which is part of Amino-acid degradation.
View all proteins of this organism that are known to be involved in the pathway L-valine degradation and in Amino-acid degradation.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei107NADBy similarity1
Binding sitei133NAD; via amide nitrogenBy similarity1
Active sitei208By similarity1
Binding sitei283NADBy similarity1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi39 – 68NADBy similarityAdd BLAST30
Nucleotide bindingi102 – 103NADBy similarity2

GO - Molecular functioni

GO - Biological processi

Keywordsi

Molecular functionOxidoreductase
Biological processBranched-chain amino acid catabolism
LigandNAD

Enzyme and pathway databases

ReactomeiR-MMU-70895 Branched-chain amino acid catabolism
UniPathwayiUPA00362

Names & Taxonomyi

Protein namesi
Recommended name:
3-hydroxyisobutyrate dehydrogenase, mitochondrial (EC:1.1.1.31)
Short name:
HIBADH
Gene namesi
Name:Hibadh
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 6

Organism-specific databases

MGIiMGI:1889802 Hibadh

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 – 35MitochondrionBy similarityAdd BLAST35
ChainiPRO_000000715936 – 3353-hydroxyisobutyrate dehydrogenase, mitochondrialAdd BLAST300

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei59N6-acetyllysine; alternateCombined sources1
Modified residuei59N6-succinyllysine; alternateCombined sources1
Modified residuei75N6-acetyllysine; alternateCombined sources1
Modified residuei75N6-succinyllysine; alternateCombined sources1
Modified residuei78N6-acetyllysine; alternateCombined sources1
Modified residuei78N6-succinyllysine; alternateCombined sources1
Modified residuei94N6-succinyllysineCombined sources1
Modified residuei120N6-acetyllysineCombined sources1
Modified residuei140N6-succinyllysineCombined sources1
Modified residuei144N6-acetyllysineCombined sources1
Modified residuei148N6-acetyllysine; alternateCombined sources1
Modified residuei148N6-succinyllysine; alternateCombined sources1
Modified residuei237N6-acetyllysine; alternateCombined sources1
Modified residuei237N6-succinyllysine; alternateCombined sources1
Modified residuei241N6-acetyllysine; alternateCombined sources1
Modified residuei241N6-succinyllysine; alternateCombined sources1
Modified residuei296N6-succinyllysineCombined sources1
Modified residuei320N6-acetyllysine; alternateCombined sources1
Modified residuei320N6-succinyllysine; alternateCombined sources1

Keywords - PTMi

Acetylation

Proteomic databases

EPDiQ99L13
MaxQBiQ99L13
PaxDbiQ99L13
PeptideAtlasiQ99L13
PRIDEiQ99L13

2D gel databases

REPRODUCTION-2DPAGEiQ99L13

PTM databases

iPTMnetiQ99L13
PhosphoSitePlusiQ99L13
SwissPalmiQ99L13

Expressioni

Gene expression databases

BgeeiENSMUSG00000029776
CleanExiMM_HIBADH
GenevisibleiQ99L13 MM

Interactioni

Subunit structurei

Homodimer.By similarity

Protein-protein interaction databases

BioGridi208456, 1 interactor
IntActiQ99L13, 3 interactors
MINTiQ99L13
STRINGi10090.ENSMUSP00000031788

Structurei

3D structure databases

ProteinModelPortaliQ99L13
SMRiQ99L13
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG0409 Eukaryota
COG2084 LUCA
GeneTreeiENSGT00530000063270
HOGENOMiHOG000219610
HOVERGENiHBG050424
InParanoidiQ99L13
KOiK00020
OMAiWPSEVNN
OrthoDBiEOG091G0CUJ
PhylomeDBiQ99L13
TreeFamiTF314043

Family and domain databases

Gene3Di1.10.1040.10, 1 hit
InterProiView protein in InterPro
IPR002204 3-OH-isobutyrate_DH-rel_CS
IPR008927 6-PGluconate_DH-like_C_sf
IPR013328 6PGD_dom2
IPR006115 6PGDH_NADP-bd
IPR011548 HIBADH
IPR015815 HIBADH-related
IPR036291 NAD(P)-bd_dom_sf
IPR029154 NADP-bd
PfamiView protein in Pfam
PF14833 NAD_binding_11, 1 hit
PF03446 NAD_binding_2, 1 hit
PIRSFiPIRSF000103 HIBADH, 1 hit
SUPFAMiSSF48179 SSF48179, 1 hit
SSF51735 SSF51735, 1 hit
TIGRFAMsiTIGR01692 HIBADH, 1 hit
PROSITEiView protein in PROSITE
PS00895 3_HYDROXYISOBUT_DH, 1 hit

Sequencei

Sequence statusi: Complete.

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

Q99L13-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MAASLGFRGA ASGLWYWSGR RRPVGSLAAV CSRSMASKTP VGFIGLGNMG
60 70 80 90 100
NPMAKNLMKH GYPLILYDVF PDVCKEFKEA GEQVASSPAE VAEKADRIIT
110 120 130 140 150
MLPSSMNAVE VYSGANGILK KVKKGSLLID SSTIDPSVSK ELAKEVEKMG
160 170 180 190 200
AVFMDAPVSG GVGAARSGNL TFMVGGVEDE FAAAQELLEC MGSNVVYCGA
210 220 230 240 250
VGTGQSAKIC NNMLLAISMI GTAEAMNLGI RSGLDPKLLA KILNMSSGRC
260 270 280 290 300
WSSDTYNPVP GVMHGVPSSN NYQGGFGTTL MAKDLGLAQD SATSTKTPIL
310 320 330
LGSLAHQIYR MMCSKGYSKK DFSSVFQYLR EEEPF
Length:335
Mass (Da):35,440
Last modified:June 1, 2001 - v1
Checksum:i5E9ECB03997DB110
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti2A → S in BAC37162 (PubMed:16141072).Curated1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
BC003914 mRNA Translation: AAH03914.1
AK078175 mRNA Translation: BAC37162.1
CCDSiCCDS20151.1
RefSeqiNP_663542.1, NM_145567.1
UniGeneiMm.286458

Genome annotation databases

EnsembliENSMUST00000031788; ENSMUSP00000031788; ENSMUSG00000029776
GeneIDi58875
KEGGimmu:58875
UCSCiuc009byw.1 mouse

Similar proteinsi

Entry informationi

Entry namei3HIDH_MOUSE
AccessioniPrimary (citable) accession number: Q99L13
Secondary accession number(s): Q8BJY2
Entry historyiIntegrated into UniProtKB/Swiss-Prot: June 27, 2003
Last sequence update: June 1, 2001
Last modified: May 23, 2018
This is version 139 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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