UniProtKB - P51553 (IDH3G_HUMAN)
Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial
IDH3G
Functioni
Cofactori
Activity regulationi
Sites
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Binding sitei | 120 | Citrate; allosteric activator1 Publication | 1 | |
Binding sitei | 133 | Citrate; allosteric activator1 Publication | 1 | |
Binding sitei | 136 | Substrate1 Publication | 1 | |
Binding sitei | 167 | Substrate1 Publication | 1 | |
Metal bindingi | 254 | Magnesium or manganese; shared with catalytic subunit1 Publication | 1 | |
Binding sitei | 254 | Substrate1 Publication | 1 | |
Binding sitei | 312 | ADP; allosteric activator1 Publication | 1 | |
Binding sitei | 313 | ADP; allosteric activator1 Publication | 1 | |
Binding sitei | 324 | ADP; allosteric activator1 Publication | 1 |
GO - Molecular functioni
- ATP binding Source: UniProtKB-KW
- isocitrate dehydrogenase (NAD+) activity Source: UniProtKB
- magnesium ion binding Source: UniProtKB
- NAD binding Source: InterPro
GO - Biological processi
- carbohydrate metabolic process Source: ProtInc
- isocitrate metabolic process Source: UniProtKB
- tricarboxylic acid cycle Source: GO_Central
Keywordsi
Biological process | Tricarboxylic acid cycle |
Ligand | ATP-binding, Magnesium, Manganese, Metal-binding, Nucleotide-binding |
Enzyme and pathway databases
BioCyci | MetaCyc:ENSG00000067829-MONOMER |
PathwayCommonsi | P51553 |
Reactomei | R-HSA-1268020, Mitochondrial protein import R-HSA-71403, Citric acid cycle (TCA cycle) |
SABIO-RKi | P51553 |
Names & Taxonomyi
Protein namesi | Recommended name: Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrialAlternative name(s): Isocitric dehydrogenase subunit gamma NAD(+)-specific ICDH subunit gamma |
Gene namesi | Name:IDH3G |
Organismi | Homo sapiens (Human) |
Taxonomic identifieri | 9606 [NCBI] |
Taxonomic lineagei | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo |
Proteomesi |
|
Organism-specific databases
HGNCi | HGNC:5386, IDH3G |
MIMi | 300089, gene |
neXtProti | NX_P51553 |
VEuPathDBi | HostDB:ENSG00000067829.18 |
Subcellular locationi
Mitochondrion
- Mitochondrion 1 Publication
Mitochondrion
- mitochondrial matrix Source: Reactome
- mitochondrion Source: LIFEdb
Nucleus
- nucleolus Source: HPA
Keywords - Cellular componenti
MitochondrionPathology & Biotechi
Mutagenesis
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Mutagenesisi | 117 | N → A: No effect on the activation of the heterodimer composed of IDH3A and IDH3G subunits by citrate. 1 Publication | 1 | |
Mutagenesisi | 120 | T → A: Significantly impairs the activation of the heterodimer composed of IDH3A and IDH3G subunits by citrate. 1 Publication | 1 | |
Mutagenesisi | 130 | S → A: No significant effect on the activation of the heterodimer composed of IDH3A and IDH3G subunits by citrate. 1 Publication | 1 | |
Mutagenesisi | 133 | N → A: Significantly impairs the activation of the heterodimer composed of IDH3A and IDH3G subunits by citrate. 1 Publication | 1 | |
Mutagenesisi | 136 | R → A: Significantly impairs the activation of the heterodimer composed of IDH3A and IDH3G subunits by citrate. 1 Publication | 1 | |
Mutagenesisi | 167 | R → A: Significantly impairs the activation of the heterodimer composed of IDH3A and IDH3G subunits by citrate. 1 Publication | 1 | |
Mutagenesisi | 173 | E → A: No effect on the activation of the heterodimer composed of IDH3A and IDH3G subunits by citrate and ADP. 1 Publication | 1 | |
Mutagenesisi | 174 | Y → F: Significantly impairs the activation of the heterodimer composed of IDH3A and IDH3G subunits by citrate. 1 Publication | 1 | |
Mutagenesisi | 190 | K → A: Complete loss of the activation of the heterotetramer and the heterodimer composed of IDH3A and IDH3G subunits by citrate and ADP. 2 Publications | 1 | |
Mutagenesisi | 229 | D → A: Significantly impairs the activation of the heterodimer composed of IDH3A and IDH3G subunits by citrate and ADP. 1 Publication | 1 | |
Mutagenesisi | 276 | Y → F: Significantly impairs the activation of the heterodimer composed of IDH3A and IDH3G subunits by citrate and ADP. 1 Publication | 1 | |
Mutagenesisi | 311 | R → A: Significantly impairs the activation of the heterodimer composed of IDH3A and IDH3G subunits by citrate. 1 Publication | 1 | |
Mutagenesisi | 312 | N → A: Significantly impairs the activation of the heterodimer composed of IDH3A and IDH3G subunits by ADP. 1 Publication | 1 | |
Mutagenesisi | 313 | T → A: Significantly impairs the activation of the heterodimer composed of IDH3A and IDH3G subunits by ADP. 1 Publication | 1 | |
Mutagenesisi | 315 | K → A: No significant effect on the activation of the heterodimer composed of IDH3A and IDH3G subunits by ADP. 1 Publication | 1 | |
Mutagenesisi | 324 | N → A: Complete loss of the activation of the heterodimer composed of IDH3A and IDH3G subunits by ADP. 1 Publication | 1 |
Organism-specific databases
OpenTargetsi | ENSG00000067829 |
PharmGKBi | PA29634 |
Miscellaneous databases
Pharosi | P51553, Tbio |
Chemistry databases
DrugBanki | DB06757, Manganese DB00157, NADH |
Genetic variation databases
BioMutai | IDH3G |
DMDMi | 1708404 |
PTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Transit peptidei | 1 – 39 | MitochondrionBy similarityAdd BLAST | 39 | |
ChainiPRO_0000014449 | 40 – 393 | Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrialAdd BLAST | 354 |
Proteomic databases
EPDi | P51553 |
jPOSTi | P51553 |
MassIVEi | P51553 |
MaxQBi | P51553 |
PaxDbi | P51553 |
PeptideAtlasi | P51553 |
PRIDEi | P51553 |
ProteomicsDBi | 19639 56332 [P51553-1] |
PTM databases
iPTMneti | P51553 |
PhosphoSitePlusi | P51553 |
Expressioni
Gene expression databases
Bgeei | ENSG00000067829, Expressed in right hemisphere of cerebellum and 243 other tissues |
ExpressionAtlasi | P51553, baseline and differential |
Genevisiblei | P51553, HS |
Organism-specific databases
HPAi | ENSG00000067829, Low tissue specificity |
Interactioni
Subunit structurei
Heterooligomer of subunits alpha (IDH3A), beta (IDH3B), and gamma (IDH3G) in the apparent ratio of 2:1:1. The heterodimer containing one IDH3A and one IDH3B subunit and the heterodimer containing one IDH3A and one IDH3G subunit assemble into a heterotetramer (which contains two subunits of IDH3A, one of IDH3B and one of IDH3G) and further into the heterooctamer.
2 PublicationsBinary interactionsi
P51553
With | #Exp. | IntAct |
---|---|---|
IDH3A [P50213] | 5 | EBI-1210876,EBI-355999 |
Protein-protein interaction databases
BioGRIDi | 109647, 33 interactors |
ComplexPortali | CPX-553, Mitochondrial isocitrate dehydrogenase complex (NAD+) |
CORUMi | P51553 |
IntActi | P51553, 10 interactors |
STRINGi | 9606.ENSP00000217901 |
Miscellaneous databases
RNActi | P51553, protein |
Structurei
Secondary structure
3D structure databases
SMRi | P51553 |
ModBasei | Search... |
PDBe-KBi | Search... |
Family & Domainsi
Sequence similaritiesi
Keywords - Domaini
Transit peptidePhylogenomic databases
eggNOGi | KOG0784, Eukaryota |
GeneTreei | ENSGT00950000182989 |
InParanoidi | P51553 |
OMAi | GTSMFEP |
OrthoDBi | 868374at2759 |
PhylomeDBi | P51553 |
TreeFami | TF315033 |
Family and domain databases
InterProi | View protein in InterPro IPR019818, IsoCit/isopropylmalate_DH_CS IPR004434, Isocitrate_DH_NAD IPR024084, IsoPropMal-DH-like_dom |
Pfami | View protein in Pfam PF00180, Iso_dh, 1 hit |
SMARTi | View protein in SMART SM01329, Iso_dh, 1 hit |
TIGRFAMsi | TIGR00175, mito_nad_idh, 1 hit |
PROSITEi | View protein in PROSITE PS00470, IDH_IMDH, 1 hit |
s (2+)i Sequence
Sequence statusi: Complete.
: The displayed sequence is further processed into a mature form. Sequence processingi
This entry describes 2 produced by isoformsialternative splicing. AlignAdd to basketThis entry has 2 described isoforms and 7 potential isoforms that are computationally mapped.Show allAlign All
This isoform has been chosen as the sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry. canonicali
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
Computationally mapped potential isoform sequencesi
There are 7 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basketQ17RL8 | Q17RL8_HUMAN | HCG2004980, isoform CRA_e | PDZD4 hCG_2004980 | 775 | Annotation score: | ||
E9PF84 | E9PF84_HUMAN | Isocitrate dehydrogenase [NAD] subu... | IDH3G | 285 | Annotation score: | ||
E7EQB8 | E7EQB8_HUMAN | Isocitrate dehydrogenase [NAD] subu... | IDH3G | 340 | Annotation score: | ||
G5E9Q7 | G5E9Q7_HUMAN | Isocitrate dehydrogenase [NAD] subu... | IDH3G hCG_2004980 | 322 | Annotation score: | ||
H7C1W2 | H7C1W2_HUMAN | Isocitrate dehydrogenase [NAD] subu... | IDH3G | 199 | Annotation score: | ||
H0Y5Q7 | H0Y5Q7_HUMAN | Isocitrate dehydrogenase [NAD] subu... | IDH3G | 211 | Annotation score: | ||
V9GYN6 | V9GYN6_HUMAN | PDZ domain-containing protein 4 | PDZD4 | 122 | Annotation score: |
Experimental Info
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Sequence conflicti | 267 | F → L in AAH01902 (PubMed:15489334).Curated | 1 |
Alternative sequence
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Alternative sequenceiVSP_046771 | 361 – 393 | MHTPD…RAVEA → VRFPSHPTLLPRPVSPCSLL in isoform 2. CuratedAdd BLAST | 33 |
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 |
CCDSi | CCDS14730.1 [P51553-1] CCDS44019.1 [P51553-2] |
RefSeqi | NP_004126.1, NM_004135.3 [P51553-1] NP_777358.1, NM_174869.2 [P51553-2] |
Genome annotation databases
Ensembli | ENST00000217901; ENSP00000217901; ENSG00000067829 [P51553-1] ENST00000370092; ENSP00000359110; ENSG00000067829 [P51553-2] |
GeneIDi | 3421 |
KEGGi | hsa:3421 |
UCSCi | uc004fip.4, human [P51553-1] |
Keywords - Coding sequence diversityi
Alternative splicingSimilar proteinsi
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 |
CCDSi | CCDS14730.1 [P51553-1] CCDS44019.1 [P51553-2] |
RefSeqi | NP_004126.1, NM_004135.3 [P51553-1] NP_777358.1, NM_174869.2 [P51553-2] |
3D structure databases
Select the link destinations: PDBei RCSB PDBi PDBji Links Updated | PDB entry | Method | Resolution (Å) | Chain | Positions | PDBsum |
5GRE | X-ray | 2.65 | B | 40-393 | [»] | |
5GRF | X-ray | 2.50 | B | 40-393 | [»] | |
5GRH | X-ray | 2.80 | B | 40-393 | [»] | |
5GRI | X-ray | 2.31 | B | 40-393 | [»] | |
5GRL | X-ray | 2.79 | B | 40-393 | [»] | |
5YVT | X-ray | 2.40 | B | 40-393 | [»] | |
6L57 | X-ray | 2.30 | B | 40-393 | [»] | |
6L59 | X-ray | 2.25 | B | 40-393 | [»] | |
7CE3 | X-ray | 3.47 | B | 40-393 | [»] | |
SMRi | P51553 | |||||
ModBasei | Search... | |||||
PDBe-KBi | Search... |
Protein-protein interaction databases
BioGRIDi | 109647, 33 interactors |
ComplexPortali | CPX-553, Mitochondrial isocitrate dehydrogenase complex (NAD+) |
CORUMi | P51553 |
IntActi | P51553, 10 interactors |
STRINGi | 9606.ENSP00000217901 |
Chemistry databases
DrugBanki | DB06757, Manganese DB00157, NADH |
PTM databases
iPTMneti | P51553 |
PhosphoSitePlusi | P51553 |
Genetic variation databases
BioMutai | IDH3G |
DMDMi | 1708404 |
Proteomic databases
EPDi | P51553 |
jPOSTi | P51553 |
MassIVEi | P51553 |
MaxQBi | P51553 |
PaxDbi | P51553 |
PeptideAtlasi | P51553 |
PRIDEi | P51553 |
ProteomicsDBi | 19639 56332 [P51553-1] |
Protocols and materials databases
Antibodypediai | 408, 254 antibodies |
DNASUi | 3421 |
Genome annotation databases
Ensembli | ENST00000217901; ENSP00000217901; ENSG00000067829 [P51553-1] ENST00000370092; ENSP00000359110; ENSG00000067829 [P51553-2] |
GeneIDi | 3421 |
KEGGi | hsa:3421 |
UCSCi | uc004fip.4, human [P51553-1] |
Organism-specific databases
CTDi | 3421 |
GeneCardsi | IDH3G |
HGNCi | HGNC:5386, IDH3G |
HPAi | ENSG00000067829, Low tissue specificity |
MIMi | 300089, gene |
neXtProti | NX_P51553 |
OpenTargetsi | ENSG00000067829 |
PharmGKBi | PA29634 |
VEuPathDBi | HostDB:ENSG00000067829.18 |
GenAtlasi | Search... |
Phylogenomic databases
eggNOGi | KOG0784, Eukaryota |
GeneTreei | ENSGT00950000182989 |
InParanoidi | P51553 |
OMAi | GTSMFEP |
OrthoDBi | 868374at2759 |
PhylomeDBi | P51553 |
TreeFami | TF315033 |
Enzyme and pathway databases
BioCyci | MetaCyc:ENSG00000067829-MONOMER |
PathwayCommonsi | P51553 |
Reactomei | R-HSA-1268020, Mitochondrial protein import R-HSA-71403, Citric acid cycle (TCA cycle) |
SABIO-RKi | P51553 |
Miscellaneous databases
BioGRID-ORCSi | 3421, 2 hits in 624 CRISPR screens |
ChiTaRSi | IDH3G, human |
GeneWikii | IDH3G |
GenomeRNAii | 3421 |
Pharosi | P51553, Tbio |
PROi | PR:P51553 |
RNActi | P51553, protein |
SOURCEi | Search... |
Gene expression databases
Bgeei | ENSG00000067829, Expressed in right hemisphere of cerebellum and 243 other tissues |
ExpressionAtlasi | P51553, baseline and differential |
Genevisiblei | P51553, HS |
Family and domain databases
InterProi | View protein in InterPro IPR019818, IsoCit/isopropylmalate_DH_CS IPR004434, Isocitrate_DH_NAD IPR024084, IsoPropMal-DH-like_dom |
Pfami | View protein in Pfam PF00180, Iso_dh, 1 hit |
SMARTi | View protein in SMART SM01329, Iso_dh, 1 hit |
TIGRFAMsi | TIGR00175, mito_nad_idh, 1 hit |
PROSITEi | View protein in PROSITE PS00470, IDH_IMDH, 1 hit |
ProtoNeti | Search... |
MobiDBi | Search... |
Entry informationi
Entry namei | IDH3G_HUMAN | |
Accessioni | P51553Primary (citable) accession number: P51553 Secondary accession number(s): E9PDD5, Q9BUU5 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | October 1, 1996 |
Last sequence update: | October 1, 1996 | |
Last modified: | April 7, 2021 | |
This is version 209 of the entry and version 1 of the sequence. See complete history. | ||
Entry statusi | Reviewed (UniProtKB/Swiss-Prot) | |
Annotation program | Chordata Protein Annotation Program | |
Disclaimer | Any 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, Reference proteomeDocuments
- Human chromosome X
Human chromosome X: entries, gene names and cross-references to MIM - MIM cross-references
Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot - PDB cross-references
Index of Protein Data Bank (PDB) cross-references - SIMILARITY comments
Index of protein domains and families