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Protein

3-hydroxyisobutyrate dehydrogenase, mitochondrial

Gene

Hibadh

Organism
Rattus norvegicus (Rat)
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 sitei208Curated1
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-RNO-70895 Branched-chain amino acid catabolism
SABIO-RKiP29266
UniPathwayiUPA00362

Names & Taxonomyi

Protein namesi
Recommended name:
3-hydroxyisobutyrate dehydrogenase, mitochondrial (EC:1.1.1.31)
Short name:
HIBADH
Gene namesi
Name:Hibadh
OrganismiRattus norvegicus (Rat)
Taxonomic identifieri10116 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeRattus
Proteomesi
  • UP000002494 Componenti: Chromosome 4

Organism-specific databases

RGDi708399 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

Pathology & Biotechi

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi68D → R: Decrease of activity with NAD, increase of activity with NADP. 1 Publication1
Mutagenesisi208K → A, H, N or R: Complete loss of activity. 1 Publication1
Mutagenesisi212N → Q: Decrease in activity. 1 Publication1

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 35MitochondrionBy similarityAdd BLAST35
ChainiPRO_000000716036 – 3353-hydroxyisobutyrate dehydrogenase, mitochondrialAdd BLAST300

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei59N6-acetyllysine; alternateBy similarity1
Modified residuei59N6-succinyllysine; alternateBy similarity1
Modified residuei75N6-acetyllysine; alternateBy similarity1
Modified residuei75N6-succinyllysine; alternateBy similarity1
Modified residuei94N6-succinyllysineBy similarity1
Modified residuei120N6-acetyllysineBy similarity1
Modified residuei140N6-succinyllysineBy similarity1
Modified residuei144N6-acetyllysineBy similarity1
Modified residuei148N6-acetyllysine; alternateBy similarity1
Modified residuei148N6-succinyllysine; alternateBy similarity1
Modified residuei237N6-acetyllysine; alternateBy similarity1
Modified residuei237N6-succinyllysine; alternateBy similarity1
Modified residuei241N6-acetyllysine; alternateBy similarity1
Modified residuei241N6-succinyllysine; alternateBy similarity1
Modified residuei296N6-succinyllysineBy similarity1
Modified residuei320N6-acetyllysine; alternateBy similarity1
Modified residuei320N6-succinyllysine; alternateBy similarity1

Keywords - PTMi

Acetylation

Proteomic databases

PaxDbiP29266
PRIDEiP29266

PTM databases

iPTMnetiP29266
PhosphoSitePlusiP29266

Expressioni

Tissue specificityi

Higher level in kidney, liver, and heart than in muscle.

Gene expression databases

BgeeiENSRNOG00000008063
GenevisibleiP29266 RN

Interactioni

Subunit structurei

Homodimer.

Protein-protein interaction databases

IntActiP29266, 1 interactor
STRINGi10116.ENSRNOP00000011069

Structurei

3D structure databases

ProteinModelPortaliP29266
SMRiP29266
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG0409 Eukaryota
COG2084 LUCA
GeneTreeiENSGT00530000063270
HOGENOMiHOG000219610
HOVERGENiHBG050424
InParanoidiP29266
KOiK00020
OMAiWPSEVNN
OrthoDBiEOG091G0CUJ
PhylomeDBiP29266
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.

P29266-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MAASLGFRGA ASGLRYWSGR RRPVGSLAAV CSRSMASKTP VGFIGLGNMG
60 70 80 90 100
NPMAKNLIKH GYPLILYDVF PDVCKEFKEA GEQVASSPAD VAEKADRIIT
110 120 130 140 150
MLPSSMNSIE VYSGANGILK KVKKGSLLID SSTIDPSVSK ELAKEVEKMG
160 170 180 190 200
AVFMDAPVSG GVGAARSGNL TFMVGGVENE FAAAQELLGC MGSNVLYCGA
210 220 230 240 250
VGSGQSAKIC NNMLLAISMI GTAEAMNLGI RSGLDPKLLA KILNMSSGRC
260 270 280 290 300
WSSDTYNPVP GVMDGVPSSN NYQGGFGTTL MAKDLGLAQD SATSTKTPIL
310 320 330
LGSVAHQIYR MMCSKGYSKK DFSSVFQYLR EEETF
Length:335
Mass (Da):35,303
Last modified:January 24, 2001 - v3
Checksum:iD266A7838500295A
GO

Sequence cautioni

The sequence AAA50312 differs from that shown. Reason: Frameshift at position 10.Curated

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
J04628 mRNA Translation: AAA50312.1 Frameshift.
BC127442 mRNA Translation: AAI27443.1
PIRiA32867
RefSeqiNP_071579.1, NM_022243.1
UniGeneiRn.73

Genome annotation databases

EnsembliENSRNOT00000011069; ENSRNOP00000011069; ENSRNOG00000008063
GeneIDi63938
KEGGirno:63938
UCSCiRGD:708399 rat

Similar proteinsi

Entry informationi

Entry namei3HIDH_RAT
AccessioniPrimary (citable) accession number: P29266
Secondary accession number(s): A1L107
Entry historyiIntegrated into UniProtKB/Swiss-Prot: December 1, 1992
Last sequence update: January 24, 2001
Last modified: May 23, 2018
This is version 151 of the entry and version 3 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  2. SIMILARITY comments
    Index of protein domains and families

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