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UniProtKB - P51553 (IDH3G_HUMAN)

Entry version 210 (02 Jun 2021)
Sequence version 1 (01 Oct 1996)
Previous versions | rss
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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

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the 'correct annotation' for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>-Experimental evidence at protein leveli <p>This indicates the type of evidence that supports the existence of the protein. Note that the 'protein existence' evidence does not give information on the accuracy or correctness of the sequence(s) displayed.<p><a href='/help/protein_existence' target='_top'>More...</a></p>

<p>This section provides any useful information about the protein, mostly biological knowledge.<p><a href='/help/function_section' target='_top'>More...</a></p>Functioni

Regulatory subunit which plays a role in the allosteric regulation of the enzyme catalyzing the decarboxylation of isocitrate (ICT) into alpha-ketoglutarate. The heterodimer composed of the alpha (IDH3A) and beta (IDH3B) subunits and the heterodimer composed of the alpha (IDH3A) and gamma (IDH3G) subunits, have considerable basal activity but the full activity of the heterotetramer (containing two subunits of IDH3A, one of IDH3B and one of IDH3G) requires the assembly and cooperative function of both heterodimers.

1 Publication

<p>This subsection of the 'Function' section provides information relevant to cofactors. A cofactor is any non-protein substance required for a protein to be catalytically active. Some cofactors are inorganic, such as the metal atoms zinc, iron, and copper in various oxidation states. Others, such as most vitamins, are organic.<p><a href='/help/cofactor' target='_top'>More...</a></p>Cofactori

Mg2+2 Publications, Mn2+1 PublicationNote: Divalent metal cations; Mn2+ or Mg2+. Activity higher in presence of Mn2+ than of Mg2+. Binds 1 Mg2+ or Mn2+ ion per subunit.1 Publication

<p>This subsection of the <a href="http://www.uniprot.org/help/function%5Fsection">Function</a> section describes regulatory mechanisms for enzymes, transporters or microbial transcription factors, and reports the components which regulate (by activation or inhibition) the reaction.<p><a href='/help/activity_regulation' target='_top'>More...</a></p>Activity regulationi

The heterotetramer and the heterodimer composed of IDH3A and IDH3G subunits can be allosterically activated by citrate (CIT) or/and ADP, and the two activators can act independently or synergistically. The heterodimer composed of IDH3A and IDH3B subunits cannot be allosterically regulated and the allosteric regulation of the heterotetramer is through the IDH3G subunit and not the IDH3B subunit. The IDH3G subunit contains the allosteric site which consists of a CIT-binding site and an ADP-binding site, and the binding of CIT and ADP causes conformational changes at the allosteric site which are transmitted to the active site in the catalytic subunit (IDH3A) through a cascade of conformational changes at the heterodimer interface, leading to stabilization of the isocitrate-binding at the active site and thus activation of the enzyme. ATP can activate the heterotetramer and the heterodimer composed of IDH3A and IDH3G subunits at low concentrations but inhibits their activities at high concentrations, whereas ATP exhibits only inhibitory effect on the heterodimer composed of IDH3A and IDH3B subunits.2 Publications

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the <a href="http://www.uniprot.org/help/function%5Fsection">Function</a> section describes the interaction between a single amino acid and another chemical entity. Priority is given to the annotation of physiological ligands.<p><a href='/help/binding' target='_top'>More...</a></p>Binding sitei120Citrate; allosteric activator1 Publication1
Binding sitei133Citrate; allosteric activator1 Publication1
Binding sitei136Substrate1 Publication1
Binding sitei167Substrate1 Publication1
<p>This subsection of the <a href="http://www.uniprot.org/help/function%5Fsection">Function</a> section indicates at which position the protein binds a given metal ion. The nature of the metal is indicated in the 'Description' field.<p><a href='/help/metal' target='_top'>More...</a></p>Metal bindingi254Magnesium or manganese; shared with catalytic subunit1 Publication1
Binding sitei254Substrate1 Publication1
Binding sitei312ADP; allosteric activator1 Publication1
Binding sitei313ADP; allosteric activator1 Publication1
Binding sitei324ADP; allosteric activator1 Publication1

<p>The <a href="http://www.geneontology.org/">Gene Ontology (GO)</a> project provides a set of hierarchical controlled vocabulary split into 3 categories:<p><a href='/help/gene_ontology' target='_top'>More...</a></p>GO - Molecular functioni

  • ATP binding Source: UniProtKB-KW
  • isocitrate dehydrogenase (NAD+) activity Source: UniProtKB
  • magnesium ion binding Source: UniProtKB
  • NAD binding Source: InterPro
Complete GO annotation on QuickGO ...

GO - Biological processi

  • carbohydrate metabolic process Source: ProtInc
  • isocitrate metabolic process Source: UniProtKB
  • tricarboxylic acid cycle Source: GO_Central
Complete GO annotation on QuickGO ...

<p>UniProtKB Keywords constitute a <a href="http://www.uniprot.org/keywords">controlled vocabulary</a> with a hierarchical structure. Keywords summarise the content of a UniProtKB entry and facilitate the search for proteins of interest.<p><a href='/help/keywords' target='_top'>More...</a></p>Keywordsi

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

Enzyme and pathway databases

BioCyc Collection of Pathway/Genome Databases

More...BioCyci		MetaCyc:ENSG00000067829-MONOMER

Pathway Commons web resource for biological pathway data

More...PathwayCommonsi		P51553

Reactome - a knowledgebase of biological pathways and processes

More...Reactomei		R-HSA-1268020, Mitochondrial protein import
R-HSA-71403, Citric acid cycle (TCA cycle)

SABIO-RK: Biochemical Reaction Kinetics Database

More...SABIO-RKi		P51553

<p>This section provides information about the protein and gene name(s) and synonym(s) and about the organism that is the source of the protein sequence.<p><a href='/help/names_and_taxonomy_section' target='_top'>More...</a></p>Names & Taxonomyi

<p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section provides an exhaustive list of all names of the protein, from commonly used to obsolete, to allow unambiguous identification of a protein.<p><a href='/help/protein_names' target='_top'>More...</a></p>Protein namesi
Recommended name:
Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial
Alternative name(s):
Isocitric dehydrogenase subunit gamma
NAD(+)-specific ICDH subunit gamma
<p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section indicates the name(s) of the gene(s) that code for the protein sequence(s) described in the entry. Four distinct tokens exist: 'Name', 'Synonyms', 'Ordered locus names' and 'ORF names'.<p><a href='/help/gene_name' target='_top'>More...</a></p>Gene namesi
Name:IDH3G
<p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section provides information on the name(s) of the organism that is the source of the protein sequence.<p><a href='/help/organism-name' target='_top'>More...</a></p>OrganismiHomo sapiens (Human)
<p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section shows the unique identifier assigned by the NCBI to the source organism of the protein. This is known as the 'taxonomic identifier' or 'taxid'.<p><a href='/help/taxonomic_identifier' target='_top'>More...</a></p>Taxonomic identifieri9606 [NCBI]
<p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section contains the taxonomic hierarchical classification lineage of the source organism. It lists the nodes as they appear top-down in the taxonomic tree, with the more general grouping listed first.<p><a href='/help/taxonomic_lineage' target='_top'>More...</a></p>Taxonomic lineageiEukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo
<p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section is present for entries that are part of a <a href="http://www.uniprot.org/proteomes">proteome</a>, i.e. of a set of proteins thought to be expressed by organisms whose genomes have been completely sequenced.<p><a href='/help/proteomes_manual' target='_top'>More...</a></p>Proteomesi
  • UP000005640 <p>A UniProt <a href="http://www.uniprot.org/manual/proteomes%5Fmanual">proteome</a> can consist of several components.<br></br>The component name refers to the genomic component encoding a set of proteins.<p><a href='/help/proteome_component' target='_top'>More...</a></p> Componenti: Chromosome X

Organism-specific databases

Human Gene Nomenclature Database

More...HGNCi		HGNC:5386, IDH3G

Online Mendelian Inheritance in Man (OMIM)

More...MIMi		300089, gene

neXtProt; the human protein knowledge platform

More...neXtProti		NX_P51553

Eukaryotic Pathogen, Vector and Host Database Resources

More...VEuPathDBi		HostDB:ENSG00000067829.18

<p>This section provides information on the location and the topology of the mature protein in the cell.<p><a href='/help/subcellular_location_section' target='_top'>More...</a></p>Subcellular locationi

Keywords - Cellular componenti

Mitochondrion

<p>This section provides information on the disease(s) and phenotype(s) associated with a protein.<p><a href='/help/pathology_and_biotech_section' target='_top'>More...</a></p>Pathology & Biotechi

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the <a href="http://www.uniprot.org/manual/pathology%5Fand%5Fbiotech%5Fsection">'Pathology and Biotech'</a> section describes the effect of the experimental mutation of one or more amino acid(s) on the biological properties of the protein.<p><a href='/help/mutagen' target='_top'>More...</a></p>Mutagenesisi117N → A: No effect on the activation of the heterodimer composed of IDH3A and IDH3G subunits by citrate. 1 Publication1
Mutagenesisi120T → A: Significantly impairs the activation of the heterodimer composed of IDH3A and IDH3G subunits by citrate. 1 Publication1
Mutagenesisi130S → A: No significant effect on the activation of the heterodimer composed of IDH3A and IDH3G subunits by citrate. 1 Publication1
Mutagenesisi133N → A: Significantly impairs the activation of the heterodimer composed of IDH3A and IDH3G subunits by citrate. 1 Publication1
Mutagenesisi136R → A: Significantly impairs the activation of the heterodimer composed of IDH3A and IDH3G subunits by citrate. 1 Publication1
Mutagenesisi167R → A: Significantly impairs the activation of the heterodimer composed of IDH3A and IDH3G subunits by citrate. 1 Publication1
Mutagenesisi173E → A: No effect on the activation of the heterodimer composed of IDH3A and IDH3G subunits by citrate and ADP. 1 Publication1
Mutagenesisi174Y → F: Significantly impairs the activation of the heterodimer composed of IDH3A and IDH3G subunits by citrate. 1 Publication1
Mutagenesisi190K → A: Complete loss of the activation of the heterotetramer and the heterodimer composed of IDH3A and IDH3G subunits by citrate and ADP. 2 Publications1
Mutagenesisi229D → A: Significantly impairs the activation of the heterodimer composed of IDH3A and IDH3G subunits by citrate and ADP. 1 Publication1
Mutagenesisi276Y → F: Significantly impairs the activation of the heterodimer composed of IDH3A and IDH3G subunits by citrate and ADP. 1 Publication1
Mutagenesisi311R → A: Significantly impairs the activation of the heterodimer composed of IDH3A and IDH3G subunits by citrate. 1 Publication1
Mutagenesisi312N → A: Significantly impairs the activation of the heterodimer composed of IDH3A and IDH3G subunits by ADP. 1 Publication1
Mutagenesisi313T → A: Significantly impairs the activation of the heterodimer composed of IDH3A and IDH3G subunits by ADP. 1 Publication1
Mutagenesisi315K → A: No significant effect on the activation of the heterodimer composed of IDH3A and IDH3G subunits by ADP. 1 Publication1
Mutagenesisi324N → A: Complete loss of the activation of the heterodimer composed of IDH3A and IDH3G subunits by ADP. 1 Publication1

Organism-specific databases

Open Targets

More...OpenTargetsi		ENSG00000067829

The Pharmacogenetics and Pharmacogenomics Knowledge Base

More...PharmGKBi		PA29634

Miscellaneous databases

Pharos NIH Druggable Genome Knowledgebase

More...Pharosi		P51553, Tbio

Chemistry databases

Drug and drug target database

More...DrugBanki		DB06757, Manganese
DB00157, NADH

Genetic variation databases

BioMuta curated single-nucleotide variation and disease association database

More...BioMutai		IDH3G

Domain mapping of disease mutations (DMDM)

More...DMDMi		1708404

<p>This section describes post-translational modifications (PTMs) and/or processing events.<p><a href='/help/ptm_processing_section' target='_top'>More...</a></p>PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the 'PTM / Processing' section describes the extent of a transit peptide.<p><a href='/help/transit' target='_top'>More...</a></p>Transit peptidei1 – 39MitochondrionBy similarityAdd BLAST39
<p>This subsection of the 'PTM / Processing' section describes the extent of a polypeptide chain in the mature protein following processing or proteolytic cleavage.<p><a href='/help/chain' target='_top'>More...</a></p>ChainiPRO_000001444940 – 393Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrialAdd BLAST354

Proteomic databases

Encyclopedia of Proteome Dynamics

More...EPDi		P51553

jPOST - Japan Proteome Standard Repository/Database

More...jPOSTi		P51553

MassIVE - Mass Spectrometry Interactive Virtual Environment

More...MassIVEi		P51553

MaxQB - The MaxQuant DataBase

More...MaxQBi		P51553

PaxDb, a database of protein abundance averages across all three domains of life

More...PaxDbi		P51553

PeptideAtlas

More...PeptideAtlasi		P51553

PRoteomics IDEntifications database

More...PRIDEi		P51553

ProteomicsDB: a multi-organism proteome resource

More...ProteomicsDBi		19639
56332 [P51553-1]

PTM databases

iPTMnet integrated resource for PTMs in systems biology context

More...iPTMneti		P51553

Comprehensive resource for the study of protein post-translational modifications (PTMs) in human, mouse and rat.

More...PhosphoSitePlusi		P51553

<p>This section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms.<p><a href='/help/expression_section' target='_top'>More...</a></p>Expressioni

Gene expression databases

Bgee dataBase for Gene Expression Evolution

More...Bgeei		ENSG00000067829, Expressed in right hemisphere of cerebellum and 244 other tissues

ExpressionAtlas, Differential and Baseline Expression

More...ExpressionAtlasi		P51553, baseline and differential

Genevisible search portal to normalized and curated expression data from Genevestigator

More...Genevisiblei		P51553, HS

Organism-specific databases

Human Protein Atlas

More...HPAi		ENSG00000067829, Low tissue specificity

<p>This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.<p><a href='/help/interaction_section' target='_top'>More...</a></p>Interactioni

<p>This subsection of the <a href="http://www.uniprot.org/help/interaction%5Fsection">'Interaction'</a> section provides information about the protein quaternary structure and interaction(s) with other proteins or protein complexes (with the exception of physiological receptor-ligand interactions which are annotated in the <a href="http://www.uniprot.org/help/function%5Fsection">'Function'</a> section).<p><a href='/help/subunit_structure' target='_top'>More...</a></p>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 Publications

<p>This subsection of the '<a href="http://www.uniprot.org/help/interaction%5Fsection">Interaction</a>' section provides information about binary protein-protein interactions. The data presented in this section are a quality-filtered subset of binary interactions automatically derived from the <a href="https://www.ebi.ac.uk/intact/">IntAct database</a>. It is updated at every <a href="http://www.uniprot.org/help/synchronization">UniProt release</a>.<p><a href='/help/binary_interactions' target='_top'>More...</a></p>Binary interactionsi

Protein-protein interaction databases

The Biological General Repository for Interaction Datasets (BioGRID)

More...BioGRIDi		109647, 33 interactors

ComplexPortal: manually curated resource of macromolecular complexes

More...ComplexPortali		CPX-553, Mitochondrial isocitrate dehydrogenase complex (NAD+)

CORUM comprehensive resource of mammalian protein complexes

More...CORUMi		P51553

Protein interaction database and analysis system

More...IntActi		P51553, 10 interactors

STRING: functional protein association networks

More...STRINGi		9606.ENSP00000217901

Miscellaneous databases

RNAct, Protein-RNA interaction predictions for model organisms.

More...RNActi		P51553, protein

<p>This section provides information on the tertiary and secondary structure of a protein.<p><a href='/help/structure_section' target='_top'>More...</a></p>Structurei

Secondary structure

1393
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

SWISS-MODEL Repository - a database of annotated 3D protein structure models

More...SMRi		P51553

Database of comparative protein structure models

More...ModBasei		Search...

Protein Data Bank in Europe - Knowledge Base

More...PDBe-KBi		Search...

<p>This section provides information on sequence similarities with other proteins and the domain(s) present in a protein.<p><a href='/help/family_and_domains_section' target='_top'>More...</a></p>Family & Domainsi

<p>This subsection of the 'Family and domains' section provides information about the sequence similarity with other proteins.<p><a href='/help/sequence_similarities' target='_top'>More...</a></p>Sequence similaritiesi

Belongs to the isocitrate and isopropylmalate dehydrogenases family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

evolutionary genealogy of genes: Non-supervised Orthologous Groups

More...eggNOGi		KOG0784, Eukaryota

Ensembl GeneTree

More...GeneTreei		ENSGT00950000182989

InParanoid: Eukaryotic Ortholog Groups

More...InParanoidi		P51553

Identification of Orthologs from Complete Genome Data

More...OMAi		GTSMFEP

Database of Orthologous Groups

More...OrthoDBi		868374at2759

Database for complete collections of gene phylogenies

More...PhylomeDBi		P51553

TreeFam database of animal gene trees

More...TreeFami		TF315033

Family and domain databases

Integrated resource of protein families, domains and functional sites

More...InterProi		View protein in InterPro
IPR019818, IsoCit/isopropylmalate_DH_CS
IPR004434, Isocitrate_DH_NAD
IPR024084, IsoPropMal-DH-like_dom

Pfam protein domain database

More...Pfami		View protein in Pfam
PF00180, Iso_dh, 1 hit

Simple Modular Architecture Research Tool; a protein domain database

More...SMARTi		View protein in SMART
SM01329, Iso_dh, 1 hit

TIGRFAMs; a protein family database

More...TIGRFAMsi		TIGR00175, mito_nad_idh, 1 hit

PROSITE; a protein domain and family database

More...PROSITEi		View protein in PROSITE
PS00470, IDH_IMDH, 1 hit

<p>This section displays by default the canonical protein sequence and upon request all isoforms described in the entry. It also includes information pertinent to the sequence(s), including <a href="http://www.uniprot.org/help/sequence%5Flength">length</a> and <a href="http://www.uniprot.org/help/sequences">molecular weight</a>. The information is filed in different subsections. The current subsections and their content are listed below:<p><a href='/help/sequences_section' target='_top'>More...</a></p>Sequences (2+)i

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences%5Fsection">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical%5Fand%5Fisoforms">canonical sequence</a> displayed by default in the entry is complete or not.<p><a href='/help/sequence_status' target='_top'>More...</a></p>Sequence statusi: Complete.

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences%5Fsection">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical%5Fand%5Fisoforms">canonical sequence</a> displayed by default in the entry is in its mature form or if it represents the precursor.<p><a href='/help/sequence_processing' target='_top'>More...</a></p>Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 2 <p>This subsection of the 'Sequence' section lists the alternative protein sequences (isoforms) that can be generated from the same gene by a single or by the combination of up to four biological events (alternative promoter usage, alternative splicing, alternative initiation and ribosomal frameshifting). Additionally, this section gives relevant information on each alternative protein isoform.<p><a href='/help/alternative_products' target='_top'>More...</a></p> isoformsi produced by alternative splicing. AlignAdd to basket

This entry has 2 described isoforms and 7 potential isoforms that are computationally mapped.Show allAlign All

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

This isoform has been chosen as the <div> <p><b>What is the canonical sequence?</b><p><a href='/help/canonical_and_isoforms' target='_top'>More...</a></p>canonicali 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
<p>The checksum is a form of redundancy check that is calculated from the sequence. It is useful for tracking sequence updates.</p> <p>It should be noted that while, in theory, two different sequences could have the same checksum value, the likelihood that this would happen is extremely low.</p> <p>However UniProtKB may contain entries with identical sequences in case of multiple genes (paralogs).</p> <p>The checksum is computed as the sequence 64-bit Cyclic Redundancy Check value (CRC64) using the generator polynomial: x<sup>64</sup> + x<sup>4</sup> + x<sup>3</sup> + x + 1. The algorithm is described in the ISO 3309 standard. </p> <p class="publication">Press W.H., Flannery B.P., Teukolsky S.A. and Vetterling W.T.<br /> <strong>Cyclic redundancy and other checksums</strong><br /> <a href="http://www.nrbook.com/b/bookcpdf.php">Numerical recipes in C 2nd ed., pp896-902, Cambridge University Press (1993)</a>)</p> 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

Show »
Length:380
Mass (Da):41,468
Checksum:i2D5987CC70725245
GO

<p>In eukaryotic reference proteomes, unreviewed entries that are likely to belong to the same gene are computationally mapped, based on gene identifiers from Ensembl, EnsemblGenomes and model organism databases.<p><a href='/help/gene_centric_isoform_mapping' target='_top'>More...</a></p>Computationally mapped potential isoform sequencesi

There are 7 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
Q17RL8Q17RL8_HUMAN
HCG2004980, isoform CRA_e
PDZD4 hCG_2004980
775Annotation score:

Annotation score:3 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the 'correct annotation' for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
E9PF84E9PF84_HUMAN
Isocitrate dehydrogenase [NAD] subu...
IDH3G
285Annotation score:

Annotation score:2 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the 'correct annotation' for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
G5E9Q7G5E9Q7_HUMAN
Isocitrate dehydrogenase [NAD] subu...
IDH3G hCG_2004980
322Annotation score:

Annotation score:2 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the 'correct annotation' for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
E7EQB8E7EQB8_HUMAN
Isocitrate dehydrogenase [NAD] subu...
IDH3G
340Annotation score:

Annotation score:2 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the 'correct annotation' for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
H7C1W2H7C1W2_HUMAN
Isocitrate dehydrogenase [NAD] subu...
IDH3G
199Annotation score:

Annotation score:2 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the 'correct annotation' for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
H0Y5Q7H0Y5Q7_HUMAN
Isocitrate dehydrogenase [NAD] subu...
IDH3G
211Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the 'correct annotation' for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
V9GYN6V9GYN6_HUMAN
PDZ domain-containing protein 4
PDZD4
122Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the 'correct annotation' for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the 'Sequence' section reports difference(s) between the canonical sequence (displayed by default in the entry) and the different sequence submissions merged in the entry. These various submissions may originate from different sequencing projects, different types of experiments, or different biological samples. Sequence conflicts are usually of unknown origin.<p><a href='/help/conflict' target='_top'>More...</a></p>Sequence conflicti267F → L in AAH01902 (PubMed:15489334).Curated1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the 'Sequence' section describes the sequence of naturally occurring alternative protein isoform(s). The changes in the amino acid sequence may be due to alternative splicing, alternative promoter usage, alternative initiation, or ribosomal frameshifting.<p><a href='/help/var_seq' target='_top'>More...</a></p>Alternative sequenceiVSP_046771361 – 393MHTPD…RAVEA → VRFPSHPTLLPRPVSPCSLL in isoform 2. CuratedAdd BLAST33

Sequence databases

Select the link destinations:

EMBL nucleotide sequence database

More...EMBLi

GenBank nucleotide sequence database

More...GenBanki

DNA Data Bank of Japan; a nucleotide sequence database

More...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

The Consensus CDS (CCDS) project

More...CCDSi		CCDS14730.1 [P51553-1]
CCDS44019.1 [P51553-2]

NCBI Reference Sequences

More...RefSeqi		NP_004126.1, NM_004135.3 [P51553-1]
NP_777358.1, NM_174869.2 [P51553-2]

Genome annotation databases

Ensembl eukaryotic genome annotation project

More...Ensembli		ENST00000217901; ENSP00000217901; ENSG00000067829 [P51553-1]
ENST00000370092; ENSP00000359110; ENSG00000067829 [P51553-2]

Database of genes from NCBI RefSeq genomes

More...GeneIDi		3421

KEGG: Kyoto Encyclopedia of Genes and Genomes

More...KEGGi		hsa:3421

UCSC genome browser

More...UCSCi		uc004fip.4, human [P51553-1]

Keywords - Coding sequence diversityi

Alternative splicing

<p>This section provides links to proteins that are similar to the protein sequence(s) described in this entry at different levels of sequence identity thresholds (100%, 90% and 50%) based on their membership in UniProt Reference Clusters (<a href="http://www.uniprot.org/help/uniref">UniRef</a>).<p><a href='/help/similar_proteins_section' target='_top'>More...</a></p>Similar proteinsi

<p>This section is used to point to information related to entries and found in data collections other than UniProtKB.<p><a href='/help/cross_references_section' target='_top'>More...</a></p>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]

3D structure databases

Select the link destinations:

Protein Data Bank Europe

More...PDBei

Protein Data Bank RCSB

More...RCSB PDBi

Protein Data Bank Japan

More...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[»]
5YVTX-ray2.40B40-393[»]
6L57X-ray2.30B40-393[»]
6L59X-ray2.25B40-393[»]
7CE3X-ray3.47B40-393[»]
SMRiP51553
ModBaseiSearch...
PDBe-KBiSearch...

Protein-protein interaction databases

BioGRIDi109647, 33 interactors
ComplexPortaliCPX-553, Mitochondrial isocitrate dehydrogenase complex (NAD+)
CORUMiP51553
IntActiP51553, 10 interactors
STRINGi9606.ENSP00000217901

Chemistry databases

DrugBankiDB06757, Manganese
DB00157, NADH

PTM databases

iPTMnetiP51553
PhosphoSitePlusiP51553

Genetic variation databases

BioMutaiIDH3G
DMDMi1708404

Proteomic databases

EPDiP51553
jPOSTiP51553
MassIVEiP51553
MaxQBiP51553
PaxDbiP51553
PeptideAtlasiP51553
PRIDEiP51553
ProteomicsDBi19639
56332 [P51553-1]

Protocols and materials databases

Antibodypedia a portal for validated antibodies

More...Antibodypediai		408, 255 antibodies

The DNASU plasmid repository

More...DNASUi		3421

Genome annotation databases

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

Organism-specific databases

Comparative Toxicogenomics Database

More...CTDi		3421

GeneCards: human genes, protein and diseases

More...GeneCardsi		IDH3G
HGNCiHGNC:5386, IDH3G
HPAiENSG00000067829, Low tissue specificity
MIMi300089, gene
neXtProtiNX_P51553
OpenTargetsiENSG00000067829
PharmGKBiPA29634
VEuPathDBiHostDB:ENSG00000067829.18

GenAtlas: human gene database

More...GenAtlasi		Search...

Phylogenomic databases

eggNOGiKOG0784, Eukaryota
GeneTreeiENSGT00950000182989
InParanoidiP51553
OMAiGTSMFEP
OrthoDBi868374at2759
PhylomeDBiP51553
TreeFamiTF315033

Enzyme and pathway databases

BioCyciMetaCyc:ENSG00000067829-MONOMER
PathwayCommonsiP51553
ReactomeiR-HSA-1268020, Mitochondrial protein import
R-HSA-71403, Citric acid cycle (TCA cycle)
SABIO-RKiP51553

Miscellaneous databases

BioGRID ORCS database of CRISPR phenotype screens

More...BioGRID-ORCSi		3421, 2 hits in 624 CRISPR screens

ChiTaRS: a database of human, mouse and fruit fly chimeric transcripts and RNA-sequencing data

More...ChiTaRSi		IDH3G, human

The Gene Wiki collection of pages on human genes and proteins

More...GeneWikii		IDH3G

Database of phenotypes from RNA interference screens in Drosophila and Homo sapiens

More...GenomeRNAii		3421
PharosiP51553, Tbio

Protein Ontology

More...PROi		PR:P51553
RNActiP51553, protein

The Stanford Online Universal Resource for Clones and ESTs

More...SOURCEi		Search...

Gene expression databases

BgeeiENSG00000067829, Expressed in right hemisphere of cerebellum and 244 other tissues
ExpressionAtlasiP51553, baseline and differential
GenevisibleiP51553, HS

Family and domain databases

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

MobiDB: a database of protein disorder and mobility annotations

More...MobiDBi		Search...

<p>This section provides general information on the entry.<p><a href='/help/entry_information_section' target='_top'>More...</a></p>Entry informationi

<p>This subsection of the 'Entry information' section provides a mnemonic identifier for a UniProtKB entry, but it is not a stable identifier. Each reviewed entry is assigned a unique entry name upon integration into UniProtKB/Swiss-Prot.<p><a href='/help/entry_name' target='_top'>More...</a></p>Entry nameiIDH3G_HUMAN
<p>This subsection of the 'Entry information' section provides one or more accession number(s). These are stable identifiers and should be used to cite UniProtKB entries. Upon integration into UniProtKB, each entry is assigned a unique accession number, which is called 'Primary (citable) accession number'.<p><a href='/help/accession_numbers' target='_top'>More...</a></p>AccessioniPrimary (citable) accession number: P51553
Secondary accession number(s): E9PDD5, Q9BUU5
<p>This subsection of the 'Entry information' section shows the date of integration of the entry into UniProtKB, the date of the last sequence update and the date of the last annotation modification ('Last modified'). The version number for both the entry and the <a href="http://www.uniprot.org/help/canonical%5Fand%5Fisoforms">canonical sequence</a> are also displayed.<p><a href='/help/entry_history' target='_top'>More...</a></p>Entry historyiIntegrated into UniProtKB/Swiss-Prot: October 1, 1996
Last sequence update: October 1, 1996
Last modified: June 2, 2021
This is version 210 of the entry and version 1 of the sequence. See complete history.
<p>This subsection of the 'Entry information' section indicates whether the entry has been manually annotated and reviewed by UniProtKB curators or not, in other words, if the entry belongs to the Swiss-Prot section of UniProtKB (<strong>reviewed</strong>) or to the computer-annotated TrEMBL section (<strong>unreviewed</strong>).<p><a href='/help/entry_status' target='_top'>More...</a></p>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.

<p>This section contains any relevant information that doesn't fit in any other defined sections<p><a href='/help/miscellaneous_section' target='_top'>More...</a></p>Miscellaneousi

Keywords - Technical termi

3D-structure, 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
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