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Protein

3-hydroxyisobutyrate dehydrogenase, mitochondrial

Gene

HIBADH

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Catalytic activityi

3-hydroxy-2-methylpropanoate + NAD+ = 2-methyl-3-oxopropanoate + NADH.1 Publication

Pathwayi: L-valine degradation

This protein is involved in the pathway L-valine degradation, which is part of Amino-acid degradation.1 Publication
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 sitei108NAD1 Publication1
Binding sitei134NAD1 Publication1
Active sitei209By similarity1
Binding sitei284NAD1 Publication1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi40 – 68NAD1 PublicationAdd BLAST29
Nucleotide bindingi103 – 104NAD1 Publication2

GO - Molecular functioni

GO - Biological processi

  • branched-chain amino acid catabolic process Source: Reactome
  • valine catabolic process Source: UniProtKB
Complete GO annotation...

Keywords - Molecular functioni

Oxidoreductase

Keywords - Biological processi

Branched-chain amino acid catabolism

Keywords - Ligandi

NAD

Enzyme and pathway databases

BioCyciZFISH:HS02854-MONOMER.
ReactomeiR-HSA-70895. Branched-chain amino acid catabolism.
SABIO-RKP31937.
UniPathwayiUPA00362.

Names & Taxonomyi

Protein namesi
Recommended name:
3-hydroxyisobutyrate dehydrogenase, mitochondrial (EC:1.1.1.31)
Short name:
HIBADH
Gene namesi
Name:HIBADH
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 7

Organism-specific databases

HGNCiHGNC:4907. HIBADH.

Subcellular locationi

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Mitochondrion

Pathology & Biotechi

Organism-specific databases

DisGeNETi11112.
OpenTargetsiENSG00000106049.
PharmGKBiPA29280.

Polymorphism and mutation databases

BioMutaiHIBADH.
DMDMi12643395.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 36Mitochondrion1 PublicationAdd BLAST36
ChainiPRO_000000715837 – 3363-hydroxyisobutyrate dehydrogenase, mitochondrialAdd BLAST300

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei60N6-acetyllysine; alternateBy similarity1
Modified residuei60N6-succinyllysine; alternateBy similarity1
Modified residuei76N6-acetyllysine; alternateBy similarity1
Modified residuei76N6-succinyllysine; alternateBy similarity1
Modified residuei95N6-succinyllysineBy similarity1
Modified residuei121N6-acetyllysineBy similarity1
Modified residuei141N6-succinyllysineBy similarity1
Modified residuei145N6-acetyllysineBy similarity1
Modified residuei149N6-acetyllysine; alternateBy similarity1
Modified residuei149N6-succinyllysine; alternateBy similarity1
Modified residuei238N6-acetyllysine; alternateBy similarity1
Modified residuei238N6-succinyllysine; alternateBy similarity1
Modified residuei242N6-acetyllysine; alternateBy similarity1
Modified residuei242N6-succinyllysine; alternateBy similarity1
Modified residuei297N6-succinyllysineBy similarity1
Modified residuei321N6-acetyllysine; alternateBy similarity1
Modified residuei321N6-succinyllysine; alternateBy similarity1

Keywords - PTMi

Acetylation

Proteomic databases

EPDiP31937.
PaxDbiP31937.
PeptideAtlasiP31937.
PRIDEiP31937.

2D gel databases

SWISS-2DPAGEP31937.

PTM databases

iPTMnetiP31937.
PhosphoSitePlusiP31937.
SwissPalmiP31937.

Expressioni

Tissue specificityi

Detected in skin fibroblasts.1 Publication

Gene expression databases

BgeeiENSG00000106049.
CleanExiHS_HIBADH.
ExpressionAtlasiP31937. baseline and differential.
GenevisibleiP31937. HS.

Organism-specific databases

HPAiHPA019522.
HPA021002.

Interactioni

Subunit structurei

Homodimer.By similarity

Protein-protein interaction databases

BioGridi116289. 10 interactors.
IntActiP31937. 1 interactor.
STRINGi9606.ENSP00000265395.

Structurei

Secondary structure

1336
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Beta strandi42 – 45Combined sources4
Helixi51 – 60Combined sources10
Beta strandi65 – 68Combined sources4
Helixi73 – 79Combined sources7
Turni80 – 82Combined sources3
Helixi89 – 95Combined sources7
Beta strandi97 – 101Combined sources5
Helixi106 – 114Combined sources9
Helixi119 – 121Combined sources3
Beta strandi128 – 131Combined sources4
Helixi137 – 149Combined sources13
Beta strandi153 – 156Combined sources4
Beta strandi159 – 161Combined sources3
Helixi162 – 168Combined sources7
Beta strandi171 – 177Combined sources7
Helixi179 – 181Combined sources3
Helixi182 – 189Combined sources8
Turni190 – 192Combined sources3
Beta strandi193 – 201Combined sources9
Helixi204 – 232Combined sources29
Helixi237 – 245Combined sources9
Helixi252 – 256Combined sources5
Turni261 – 263Combined sources3
Beta strandi265 – 267Combined sources3
Helixi268 – 271Combined sources4
Beta strandi275 – 278Combined sources4
Helixi279 – 295Combined sources17
Helixi301 – 314Combined sources14
Turni315 – 317Combined sources3
Helixi323 – 325Combined sources3
Helixi326 – 330Combined sources5

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2GF2X-ray2.38A/B/C/D41-335[»]
2I9PX-ray2.55A/B/C/D41-336[»]
ProteinModelPortaliP31937.
SMRiP31937.
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiP31937.

Family & Domainsi

Sequence similaritiesi

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG0409. Eukaryota.
COG2084. LUCA.
GeneTreeiENSGT00530000063270.
HOGENOMiHOG000219610.
HOVERGENiHBG050424.
InParanoidiP31937.
KOiK00020.
OMAiNIVHCGE.
OrthoDBiEOG091G0CUJ.
PhylomeDBiP31937.
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.

P31937-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MAASLRLLGA ASGLRYWSRR LRPAAGSFAA VCSRSVASKT PVGFIGLGNM
60 70 80 90 100
GNPMAKNLMK HGYPLIIYDV FPDACKEFQD AGEQVVSSPA DVAEKADRII
110 120 130 140 150
TMLPTSINAI EAYSGANGIL KKVKKGSLLI DSSTIDPAVS KELAKEVEKM
160 170 180 190 200
GAVFMDAPVS GGVGAARSGN LTFMVGGVED EFAAAQELLG CMGSNVVYCG
210 220 230 240 250
AVGTGQAAKI CNNMLLAISM IGTAEAMNLG IRLGLDPKLL AKILNMSSGR
260 270 280 290 300
CWSSDTYNPV PGVMDGVPSA NNYQGGFGTT LMAKDLGLAQ DSATSTKSPI
310 320 330
LLGSLAHQIY RMMCAKGYSK KDFSSVFQFL REEETF
Length:336
Mass (Da):35,329
Last modified:January 24, 2001 - v2
Checksum:iDA3128774A91AF48
GO

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF529362 mRNA. Translation: AAQ09596.1.
AB050000 mRNA. Translation: BAF42045.1.
AK316605 mRNA. Translation: BAG38192.1.
AC007130 Genomic DNA. No translation available.
AC005091 Genomic DNA. No translation available.
CH236948 Genomic DNA. Translation: EAL24214.1.
CH471073 Genomic DNA. Translation: EAW93897.1.
BC032324 mRNA. Translation: AAH32324.1.
CCDSiCCDS5414.1.
RefSeqiNP_689953.1. NM_152740.3.
UniGeneiHs.406758.

Genome annotation databases

EnsembliENST00000265395; ENSP00000265395; ENSG00000106049.
GeneIDi11112.
KEGGihsa:11112.
UCSCiuc003szf.4. human.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF529362 mRNA. Translation: AAQ09596.1.
AB050000 mRNA. Translation: BAF42045.1.
AK316605 mRNA. Translation: BAG38192.1.
AC007130 Genomic DNA. No translation available.
AC005091 Genomic DNA. No translation available.
CH236948 Genomic DNA. Translation: EAL24214.1.
CH471073 Genomic DNA. Translation: EAW93897.1.
BC032324 mRNA. Translation: AAH32324.1.
CCDSiCCDS5414.1.
RefSeqiNP_689953.1. NM_152740.3.
UniGeneiHs.406758.

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2GF2X-ray2.38A/B/C/D41-335[»]
2I9PX-ray2.55A/B/C/D41-336[»]
ProteinModelPortaliP31937.
SMRiP31937.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi116289. 10 interactors.
IntActiP31937. 1 interactor.
STRINGi9606.ENSP00000265395.

PTM databases

iPTMnetiP31937.
PhosphoSitePlusiP31937.
SwissPalmiP31937.

Polymorphism and mutation databases

BioMutaiHIBADH.
DMDMi12643395.

2D gel databases

SWISS-2DPAGEP31937.

Proteomic databases

EPDiP31937.
PaxDbiP31937.
PeptideAtlasiP31937.
PRIDEiP31937.

Protocols and materials databases

DNASUi11112.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000265395; ENSP00000265395; ENSG00000106049.
GeneIDi11112.
KEGGihsa:11112.
UCSCiuc003szf.4. human.

Organism-specific databases

CTDi11112.
DisGeNETi11112.
GeneCardsiHIBADH.
HGNCiHGNC:4907. HIBADH.
HPAiHPA019522.
HPA021002.
MIMi608475. gene.
neXtProtiNX_P31937.
OpenTargetsiENSG00000106049.
PharmGKBiPA29280.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG0409. Eukaryota.
COG2084. LUCA.
GeneTreeiENSGT00530000063270.
HOGENOMiHOG000219610.
HOVERGENiHBG050424.
InParanoidiP31937.
KOiK00020.
OMAiNIVHCGE.
OrthoDBiEOG091G0CUJ.
PhylomeDBiP31937.
TreeFamiTF314043.

Enzyme and pathway databases

UniPathwayiUPA00362.
BioCyciZFISH:HS02854-MONOMER.
ReactomeiR-HSA-70895. Branched-chain amino acid catabolism.
SABIO-RKP31937.

Miscellaneous databases

ChiTaRSiHIBADH. human.
EvolutionaryTraceiP31937.
GeneWikii3-hydroxyisobutyrate_dehydrogenase.
GenomeRNAii11112.
PROiP31937.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000106049.
CleanExiHS_HIBADH.
ExpressionAtlasiP31937. baseline and differential.
GenevisibleiP31937. HS.

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_HUMAN
AccessioniPrimary (citable) accession number: P31937
Secondary accession number(s): Q546Z2, Q9UDN3
Entry historyi
Integrated into UniProtKB/Swiss-Prot: July 1, 1993
Last sequence update: January 24, 2001
Last modified: November 2, 2016
This is version 167 of the entry and version 2 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

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

Documents

  1. Human chromosome 7
    Human chromosome 7: entries, gene names and cross-references to MIM
  2. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  3. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  4. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  5. 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.