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Protein

3-hydroxyisobutyrate dehydrogenase, mitochondrial

Gene

HIBADH

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -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

Keywordsi

Molecular functionOxidoreductase
Biological processBranched-chain amino acid catabolism
LigandNAD

Enzyme and pathway databases

ReactomeiR-HSA-70895 Branched-chain amino acid catabolism
SABIO-RKiP31937
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

EuPathDBiHostDB:ENSG00000106049.8
HGNCiHGNC:4907 HIBADH
MIMi608475 gene
neXtProtiNX_P31937

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

Organism-specific databases

DisGeNETi11112
OpenTargetsiENSG00000106049
PharmGKBiPA29280

Chemistry databases

DrugBankiDB00157 NADH

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
ProteomicsDBi54804

2D gel databases

SWISS-2DPAGEiP31937

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, 33 interactors
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

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
OMAiWPSEVNN
OrthoDBiEOG091G0CUJ
PhylomeDBiP31937
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.

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

Similar proteinsi

Entry informationi

Entry namei3HIDH_HUMAN
AccessioniPrimary (citable) accession number: P31937
Secondary accession number(s): Q546Z2, Q9UDN3
Entry historyiIntegrated into UniProtKB/Swiss-Prot: July 1, 1993
Last sequence update: January 24, 2001
Last modified: July 18, 2018
This is version 176 of the entry and version 2 of the sequence. See 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

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