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Protein

Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial

Gene

IDH3G

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

Functioni

Catalytic activityi

Isocitrate + NAD+ = 2-oxoglutarate + CO2 + NADH.

Cofactori

Mg2+By similarity, Mn2+By similarityNote: Binds 1 Mg2+ or Mn2+ ion per subunit.By similarity

Enzyme regulationi

Activated by increasing ADP/ATP ratios and by Ca2+.By similarity

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei136SubstrateBy similarity1
Binding sitei167SubstrateBy similarity1
Sitei174Critical for catalysisBy similarity1
Sitei221Critical for catalysisBy similarity1
Metal bindingi254Magnesium or manganeseBy similarity1
Binding sitei254SubstrateBy similarity1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi56 – 84NADSequence analysisAdd BLAST29
Nucleotide bindingi309 – 316ATPSequence analysis8

GO - Molecular functioni

GO - Biological processi

  • carbohydrate metabolic process Source: ProtInc
  • isocitrate metabolic process Source: UniProtKB
  • tricarboxylic acid cycle Source: Reactome

Keywordsi

Molecular functionOxidoreductase
Biological processTricarboxylic acid cycle
LigandATP-binding, Magnesium, Manganese, Metal-binding, NAD, Nucleotide-binding

Enzyme and pathway databases

BioCyciMetaCyc:ENSG00000067829-MONOMER.
ReactomeiR-HSA-1268020. Mitochondrial protein import.
R-HSA-71403. Citric acid cycle (TCA cycle).
SABIO-RKiP51553.

Names & Taxonomyi

Protein namesi
Recommended name:
Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial (EC:1.1.1.41)
Alternative name(s):
Isocitric dehydrogenase subunit gamma
NAD(+)-specific ICDH subunit gamma
Gene namesi
Name:IDH3G
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome X

Organism-specific databases

HGNCiHGNC:5386. IDH3G.

Subcellular locationi

GO - Cellular componenti

Keywords - Cellular componenti

Mitochondrion

Pathology & Biotechi

Organism-specific databases

OpenTargetsiENSG00000067829.
PharmGKBiPA29634.

Chemistry databases

DrugBankiDB00157. NADH.

Polymorphism and mutation databases

BioMutaiIDH3G.
DMDMi1708404.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 39MitochondrionBy similarityAdd BLAST39
ChainiPRO_000001444940 – 393Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrialAdd BLAST354

Proteomic databases

EPDiP51553.
MaxQBiP51553.
PaxDbiP51553.
PeptideAtlasiP51553.
PRIDEiP51553.

PTM databases

iPTMnetiP51553.
PhosphoSitePlusiP51553.

Expressioni

Gene expression databases

BgeeiENSG00000067829.
CleanExiHS_IDH3G.
ExpressionAtlasiP51553. baseline and differential.
GenevisibleiP51553. HS.

Organism-specific databases

HPAiHPA000425.
HPA002017.

Interactioni

Subunit structurei

Heterooligomer of subunits alpha, beta, and gamma in the apparent ratio of 2:1:1.By similarity

Protein-protein interaction databases

BioGridi109647. 25 interactors.
IntActiP51553. 2 interactors.
MINTiMINT-3018829.
STRINGi9606.ENSP00000217901.

Structurei

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
5GREX-ray2.65B40-393[»]
5GRFX-ray2.50B40-393[»]
5GRHX-ray2.80B40-393[»]
5GRIX-ray2.31B40-393[»]
5GRLX-ray2.79B40-393[»]
ProteinModelPortaliP51553.
SMRiP51553.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG0784. Eukaryota.
COG0473. LUCA.
GeneTreeiENSGT00590000083091.
HOGENOMiHOG000021113.
HOVERGENiHBG052080.
InParanoidiP51553.
KOiK00030.
OMAiHLKLNDY.
OrthoDBiEOG091G094X.
PhylomeDBiP51553.
TreeFamiTF315033.

Family and domain databases

Gene3Di3.40.718.10. 1 hit.
InterProiView protein in InterPro
IPR019818. IsoCit/isopropylmalate_DH_CS.
IPR004434. Isocitrate_DH_NAD.
IPR024084. IsoPropMal-DH-like_dom.
PfamiView protein in Pfam
PF00180. Iso_dh. 1 hit.
SMARTiView protein in SMART
SM01329. Iso_dh. 1 hit.
TIGRFAMsiTIGR00175. mito_nad_idh. 1 hit.
PROSITEiView protein in PROSITE
PS00470. IDH_IMDH. 1 hit.

Sequences (2)i

Sequence statusi: Complete.

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

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: P51553-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MALKVATVAG SAAKAVLGPA LLCRPWEVLG AHEVPSRNIF SEQTIPPSAK
60 70 80 90 100
YGGRHTVTMI PGDGIGPELM LHVKSVFRHA CVPVDFEEVH VSSNADEEDI
110 120 130 140 150
RNAIMAIRRN RVALKGNIET NHNLPPSHKS RNNILRTSLD LYANVIHCKS
160 170 180 190 200
LPGVVTRHKD IDILIVRENT EGEYSSLEHE SVAGVVESLK IITKAKSLRI
210 220 230 240 250
AEYAFKLAQE SGRKKVTAVH KANIMKLGDG LFLQCCREVA ARYPQITFEN
260 270 280 290 300
MIVDNTTMQL VSRPQQFDVM VMPNLYGNIV NNVCAGLVGG PGLVAGANYG
310 320 330 340 350
HVYAVFETAT RNTGKSIANK NIANPTATLL ASCMMLDHLK LHSYATSIRK
360 370 380 390
AVLASMDNEN MHTPDIGGQG TTSEAIQDVI RHIRVINGRA VEA
Length:393
Mass (Da):42,794
Last modified:October 1, 1996 - v1
Checksum:iA0870F2B7D228A37
GO
Isoform 2 (identifier: P51553-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     361-393: MHTPDIGGQGTTSEAIQDVIRHIRVINGRAVEA → VRFPSHPTLLPRPVSPCSLL

Note: Gene prediction based on EST data.
Show »
Length:380
Mass (Da):41,468
Checksum:i2D5987CC70725245
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti267F → L in AAH01902 (PubMed:15489334).Curated1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_046771361 – 393MHTPD…RAVEA → VRFPSHPTLLPRPVSPCSLL in isoform 2. CuratedAdd BLAST33

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
Z68907 mRNA. Translation: CAA93143.1.
Z68129 Genomic DNA. Translation: CAA92214.1.
U52111 Genomic DNA. No translation available.
U40272 mRNA. Translation: AAD09357.1.
BC000933 mRNA. Translation: AAH00933.1.
BC001902 mRNA. Translation: AAH01902.1.
CCDSiCCDS14730.1. [P51553-1]
CCDS44019.1. [P51553-2]
RefSeqiNP_004126.1. NM_004135.3. [P51553-1]
NP_777358.1. NM_174869.2. [P51553-2]
UniGeneiHs.410197.

Genome annotation databases

EnsembliENST00000217901; ENSP00000217901; ENSG00000067829. [P51553-1]
ENST00000370092; ENSP00000359110; ENSG00000067829. [P51553-2]
GeneIDi3421.
KEGGihsa:3421.
UCSCiuc004fip.4. human. [P51553-1]

Keywords - Coding sequence diversityi

Alternative splicing

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
Z68907 mRNA. Translation: CAA93143.1.
Z68129 Genomic DNA. Translation: CAA92214.1.
U52111 Genomic DNA. No translation available.
U40272 mRNA. Translation: AAD09357.1.
BC000933 mRNA. Translation: AAH00933.1.
BC001902 mRNA. Translation: AAH01902.1.
CCDSiCCDS14730.1. [P51553-1]
CCDS44019.1. [P51553-2]
RefSeqiNP_004126.1. NM_004135.3. [P51553-1]
NP_777358.1. NM_174869.2. [P51553-2]
UniGeneiHs.410197.

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
5GREX-ray2.65B40-393[»]
5GRFX-ray2.50B40-393[»]
5GRHX-ray2.80B40-393[»]
5GRIX-ray2.31B40-393[»]
5GRLX-ray2.79B40-393[»]
ProteinModelPortaliP51553.
SMRiP51553.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi109647. 25 interactors.
IntActiP51553. 2 interactors.
MINTiMINT-3018829.
STRINGi9606.ENSP00000217901.

Chemistry databases

DrugBankiDB00157. NADH.

PTM databases

iPTMnetiP51553.
PhosphoSitePlusiP51553.

Polymorphism and mutation databases

BioMutaiIDH3G.
DMDMi1708404.

Proteomic databases

EPDiP51553.
MaxQBiP51553.
PaxDbiP51553.
PeptideAtlasiP51553.
PRIDEiP51553.

Protocols and materials databases

DNASUi3421.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000217901; ENSP00000217901; ENSG00000067829. [P51553-1]
ENST00000370092; ENSP00000359110; ENSG00000067829. [P51553-2]
GeneIDi3421.
KEGGihsa:3421.
UCSCiuc004fip.4. human. [P51553-1]

Organism-specific databases

CTDi3421.
GeneCardsiIDH3G.
HGNCiHGNC:5386. IDH3G.
HPAiHPA000425.
HPA002017.
MIMi300089. gene.
neXtProtiNX_P51553.
OpenTargetsiENSG00000067829.
PharmGKBiPA29634.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG0784. Eukaryota.
COG0473. LUCA.
GeneTreeiENSGT00590000083091.
HOGENOMiHOG000021113.
HOVERGENiHBG052080.
InParanoidiP51553.
KOiK00030.
OMAiHLKLNDY.
OrthoDBiEOG091G094X.
PhylomeDBiP51553.
TreeFamiTF315033.

Enzyme and pathway databases

BioCyciMetaCyc:ENSG00000067829-MONOMER.
ReactomeiR-HSA-1268020. Mitochondrial protein import.
R-HSA-71403. Citric acid cycle (TCA cycle).
SABIO-RKiP51553.

Miscellaneous databases

ChiTaRSiIDH3G. human.
GeneWikiiIDH3G.
GenomeRNAii3421.
PROiPR:P51553.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000067829.
CleanExiHS_IDH3G.
ExpressionAtlasiP51553. baseline and differential.
GenevisibleiP51553. HS.

Family and domain databases

Gene3Di3.40.718.10. 1 hit.
InterProiView protein in InterPro
IPR019818. IsoCit/isopropylmalate_DH_CS.
IPR004434. Isocitrate_DH_NAD.
IPR024084. IsoPropMal-DH-like_dom.
PfamiView protein in Pfam
PF00180. Iso_dh. 1 hit.
SMARTiView protein in SMART
SM01329. Iso_dh. 1 hit.
TIGRFAMsiTIGR00175. mito_nad_idh. 1 hit.
PROSITEiView protein in PROSITE
PS00470. IDH_IMDH. 1 hit.
ProtoNetiSearch...

Entry informationi

Entry nameiIDH3G_HUMAN
AccessioniPrimary (citable) accession number: P51553
Secondary accession number(s): E9PDD5, Q9BUU5
Entry historyiIntegrated into UniProtKB/Swiss-Prot: October 1, 1996
Last sequence update: October 1, 1996
Last modified: April 12, 2017
This is version 180 of the entry and version 1 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, Reference proteome

Documents

  1. Human chromosome X
    Human chromosome X: 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. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  4. 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.