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Protein

3-hydroxyisobutyrate dehydrogenase, mitochondrial

Gene

Hibadh

Organism
Rattus norvegicus (Rat)
Status
Reviewed-Annotation score: Annotation score: 4 out of 5-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)LengthDescriptionGraphical viewFeature identifierActions
Binding sitei107 – 1071NADBy similarity
Binding sitei133 – 1331NAD; via amide nitrogenBy similarity
Active sitei208 – 2081Curated
Binding sitei283 – 2831NADBy similarity

Regions

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Nucleotide bindingi39 – 6830NADBy similarityAdd
BLAST
Nucleotide bindingi102 – 1032NADBy similarity

GO - Molecular functioni

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Oxidoreductase

Keywords - Biological processi

Branched-chain amino acid catabolism

Keywords - Ligandi

NAD

Enzyme and pathway databases

ReactomeiR-RNO-70895. Branched-chain amino acid catabolism.
SABIO-RKP29266.
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 lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeRattus
Proteomesi
  • UP000002494 Componenti: Chromosome 4

Organism-specific databases

RGDi708399. Hibadh.

Subcellular locationi

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Mitochondrion

Pathology & Biotechi

Mutagenesis

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Mutagenesisi68 – 681D → R: Decrease of activity with NAD, increase of activity with NADP. 1 Publication
Mutagenesisi208 – 2081K → A, H, N or R: Complete loss of activity. 1 Publication
Mutagenesisi212 – 2121N → Q: Decrease in activity. 1 Publication

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Transit peptidei1 – 3535MitochondrionBy similarityAdd
BLAST
Chaini36 – 3353003-hydroxyisobutyrate dehydrogenase, mitochondrialPRO_0000007160Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei59 – 591N6-acetyllysine; alternateBy similarity
Modified residuei59 – 591N6-succinyllysine; alternateBy similarity
Modified residuei75 – 751N6-acetyllysine; alternateBy similarity
Modified residuei75 – 751N6-succinyllysine; alternateBy similarity
Modified residuei94 – 941N6-succinyllysineBy similarity
Modified residuei120 – 1201N6-acetyllysineBy similarity
Modified residuei140 – 1401N6-succinyllysineBy similarity
Modified residuei144 – 1441N6-acetyllysineBy similarity
Modified residuei148 – 1481N6-acetyllysine; alternateBy similarity
Modified residuei148 – 1481N6-succinyllysine; alternateBy similarity
Modified residuei237 – 2371N6-acetyllysine; alternateBy similarity
Modified residuei237 – 2371N6-succinyllysine; alternateBy similarity
Modified residuei241 – 2411N6-acetyllysine; alternateBy similarity
Modified residuei241 – 2411N6-succinyllysine; alternateBy similarity
Modified residuei296 – 2961N6-succinyllysineBy similarity
Modified residuei320 – 3201N6-acetyllysine; alternateBy similarity
Modified residuei320 – 3201N6-succinyllysine; alternateBy similarity

Keywords - PTMi

Acetylation

Proteomic databases

PaxDbiP29266.
PRIDEiP29266.

PTM databases

iPTMnetiP29266.
PhosphoSiteiP29266.

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 interaction.
STRINGi10116.ENSRNOP00000011069.

Structurei

3D structure databases

ProteinModelPortaliP29266.
SMRiP29266. Positions 40-334.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG0409. Eukaryota.
COG2084. LUCA.
GeneTreeiENSGT00530000063270.
HOGENOMiHOG000219610.
HOVERGENiHBG050424.
InParanoidiP29266.
KOiK00020.
OMAiNIVHCGE.
OrthoDBiEOG091G0CUJ.
PhylomeDBiP29266.
TreeFamiTF314043.

Family and domain databases

Gene3Di1.10.1040.10. 1 hit.
3.40.50.720. 1 hit.
InterProiIPR002204. 3-OH-isobutyrate_DH-rel_CS.
IPR008927. 6-PGluconate_DH_C-like.
IPR013328. 6PGD_dom_2.
IPR006115. 6PGDH_NADP-bd.
IPR011548. HIBADH.
IPR015815. HIBADH-related.
IPR016040. NAD(P)-bd_dom.
IPR029154. NADP-bd.
[Graphical view]
PfamiPF14833. NAD_binding_11. 1 hit.
PF03446. NAD_binding_2. 1 hit.
[Graphical view]
PIRSFiPIRSF000103. HIBADH. 1 hit.
SUPFAMiSSF48179. SSF48179. 1 hit.
SSF51735. SSF51735. 1 hit.
TIGRFAMsiTIGR01692. HIBADH. 1 hit.
PROSITEiPS00895. 3_HYDROXYISOBUT_DH. 1 hit.
[Graphical view]

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.

Cross-referencesi

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.

3D structure databases

ProteinModelPortaliP29266.
SMRiP29266. Positions 40-334.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

IntActiP29266. 1 interaction.
STRINGi10116.ENSRNOP00000011069.

PTM databases

iPTMnetiP29266.
PhosphoSiteiP29266.

Proteomic databases

PaxDbiP29266.
PRIDEiP29266.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

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

Organism-specific databases

CTDi11112.
RGDi708399. Hibadh.

Phylogenomic databases

eggNOGiKOG0409. Eukaryota.
COG2084. LUCA.
GeneTreeiENSGT00530000063270.
HOGENOMiHOG000219610.
HOVERGENiHBG050424.
InParanoidiP29266.
KOiK00020.
OMAiNIVHCGE.
OrthoDBiEOG091G0CUJ.
PhylomeDBiP29266.
TreeFamiTF314043.

Enzyme and pathway databases

UniPathwayiUPA00362.
ReactomeiR-RNO-70895. Branched-chain amino acid catabolism.
SABIO-RKP29266.

Miscellaneous databases

PROiP29266.

Gene expression databases

BgeeiENSRNOG00000008063.
GenevisibleiP29266. RN.

Family and domain databases

Gene3Di1.10.1040.10. 1 hit.
3.40.50.720. 1 hit.
InterProiIPR002204. 3-OH-isobutyrate_DH-rel_CS.
IPR008927. 6-PGluconate_DH_C-like.
IPR013328. 6PGD_dom_2.
IPR006115. 6PGDH_NADP-bd.
IPR011548. HIBADH.
IPR015815. HIBADH-related.
IPR016040. NAD(P)-bd_dom.
IPR029154. NADP-bd.
[Graphical view]
PfamiPF14833. NAD_binding_11. 1 hit.
PF03446. NAD_binding_2. 1 hit.
[Graphical view]
PIRSFiPIRSF000103. HIBADH. 1 hit.
SUPFAMiSSF48179. SSF48179. 1 hit.
SSF51735. SSF51735. 1 hit.
TIGRFAMsiTIGR01692. HIBADH. 1 hit.
PROSITEiPS00895. 3_HYDROXYISOBUT_DH. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry namei3HIDH_RAT
AccessioniPrimary (citable) accession number: P29266
Secondary accession number(s): A1L107
Entry historyi
Integrated into UniProtKB/Swiss-Prot: December 1, 1992
Last sequence update: January 24, 2001
Last modified: September 7, 2016
This is version 142 of the entry and version 3 of the sequence. [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

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.