Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.
Entry version 219 (29 Sep 2021)
Sequence version 2 (11 Jul 2002)
Previous versions | rss
Add a publicationFeedback
Protein

Isocitrate dehydrogenase [NADP] cytoplasmic

Gene

IDH1

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

<p>This subsection of the <a href="http://www.uniprot.org/help/function_section">Function</a> section describes the catalytic activity of an enzyme, i.e. a chemical reaction that the enzyme catalyzes.<p><a href='/help/catalytic_activity' target='_top'>More...</a></p>Catalytic activityi

<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+1 Publication, Mn2+1 PublicationNote: Binds 1 Mg2+ or Mn2+ ion per subunit.1 Publication

<p>This subsection of the 'Function' section describes biophysical and chemical properties, such as maximal absorption, kinetic parameters, pH dependence, redox potentials and temperature dependence.<p><a href='/help/biophysicochemical_properties' target='_top'>More...</a></p>Kineticsi

  1. KM=49 µM for NADP1 Publication
  2. KM=29 µM for magnesium chloride1 Publication
  3. KM=65 µM for isocitrate1 Publication

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the <a href="http://www.uniprot.org/help/function_section">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 sitei77SubstrateCombined sources1 Publication1
Binding sitei82NADPCombined sources2 Publications1
Binding sitei109SubstrateCombined sources1 Publication1
Binding sitei132SubstrateCombined sources1 Publication1
<p>This subsection describes interesting single amino acid sites on the sequence that are not defined in any other subsection. This subsection can be displayed in different sections ('Function', 'PTM / Processing', 'Pathology and Biotech') according to its content.<p><a href='/help/site' target='_top'>More...</a></p>Sitei139Critical for catalysis1
Binding sitei212Substrate; shared with neighboring subunitCombined sources1 Publication1
Sitei212Critical for catalysis1
<p>This subsection of the <a href="http://www.uniprot.org/help/function_section">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 bindingi252Magnesium or manganese; shared with neighboring subunitCombined sources2 Publications1
Binding sitei260NADPCombined sources2 Publications1
Metal bindingi275Magnesium or manganeseCombined sources2 Publications1
Metal bindingi279Magnesium or manganeseCombined sources2 Publications1
Binding sitei328NADP; via amide nitrogen and carbonyl oxygenCombined sources2 Publications1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the <a href="http://www.uniprot.org/help/function_section">Function</a> section describes a region in the protein which binds nucleotide phosphates. It always involves more than one amino acid and includes all residues involved in nucleotide-binding.<p><a href='/help/np_bind' target='_top'>More...</a></p>Nucleotide bindingi75 – 77NADPCombined sources2 Publications3
Nucleotide bindingi310 – 315NADPCombined sources2 Publications6

<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

GO - Biological processi

<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

Molecular functionOxidoreductase
Biological processGlyoxylate bypass, Tricarboxylic acid cycle
LigandMagnesium, Manganese, Metal-binding, NADP

Enzyme and pathway databases

BioCyc Collection of Pathway/Genome Databases

More...
BioCyci
MetaCyc:HS06502-MONOMER

BRENDA Comprehensive Enzyme Information System

More...
BRENDAi
1.1.1.42, 2681

Pathway Commons web resource for biological pathway data

More...
PathwayCommonsi
O75874

Reactome - a knowledgebase of biological pathways and processes

More...
Reactomei
R-HSA-2978092, Abnormal conversion of 2-oxoglutarate to 2-hydroxyglutarate
R-HSA-389542, NADPH regeneration
R-HSA-6798695, Neutrophil degranulation
R-HSA-9033241, Peroxisomal protein import

SABIO-RK: Biochemical Reaction Kinetics Database

More...
SABIO-RKi
O75874

SIGNOR Signaling Network Open Resource

More...
SIGNORi
O75874

<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_and_taxonomy_section">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 [NADP] cytoplasmic (EC:1.1.1.422 Publications)
Short name:
IDH
Alternative name(s):
Cytosolic NADP-isocitrate dehydrogenase
IDP
NADP(+)-specific ICDH
Oxalosuccinate decarboxylase
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">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:IDH1
Synonyms:PICD
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">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_and_taxonomy_section">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_and_taxonomy_section">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 lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">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_manual">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 2

Organism-specific databases

Human Gene Nomenclature Database

More...
HGNCi
HGNC:5382, IDH1

Online Mendelian Inheritance in Man (OMIM)

More...
MIMi
147700, gene

neXtProt; the human protein knowledge platform

More...
neXtProti
NX_O75874

Eukaryotic Pathogen, Vector and Host Database Resources

More...
VEuPathDBi
HostDB:ENSG00000138413

<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

Cytoplasm, Peroxisome

<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

<p>This subsection of the 'Pathology and Biotech' section provides information on the disease(s) associated with genetic variations in a given protein. The information is extracted from the scientific literature and diseases that are also described in the <a href="http://www.ncbi.nlm.nih.gov/sites/entrez?db=omim">OMIM</a> database are represented with a <a href="http://www.uniprot.org/diseases">controlled vocabulary</a> in the following way:<p><a href='/help/involvement_in_disease' target='_top'>More...</a></p>Involvement in diseasei

Glioma (GLM)2 Publications
The gene represented in this entry is involved in disease pathogenesis. Mutations affecting Arg-132 are tissue-specific, and suggest that this residue plays a unique role in the development of high-grade gliomas. Mutations of Arg-132 to Cys, His, Leu or Ser abolish magnesium binding and abolish the conversion of isocitrate to alpha-ketoglutarate. Instead, alpha-ketoglutarate is converted to R(-)-2-hydroxyglutarate. Elevated levels of R(-)-2-hydroxyglutarate are correlated with an elevated risk of malignant brain tumors.1 Publication
Disease descriptionGliomas are benign or malignant central nervous system neoplasms derived from glial cells. They comprise astrocytomas and glioblastoma multiforme that are derived from astrocytes, oligodendrogliomas derived from oligodendrocytes and ependymomas derived from ependymocytes.
Related information in OMIM
Genetic variations are associated with cartilaginous tumors such as enchondroma or chondrosarcoma. Mutations of Arg-132 to Cys, Gly or His abolish the conversion of isocitrate to alpha-ketoglutarate. Instead, alpha-ketoglutarate is converted to R(-)-2-hydroxyglutarate.1 Publication

Organism-specific databases

DisGeNET

More...
DisGeNETi
3417

MalaCards human disease database

More...
MalaCardsi
IDH1
MIMi137800, phenotype

Open Targets

More...
OpenTargetsi
ENSG00000138413

Orphanet; a database dedicated to information on rare diseases and orphan drugs

More...
Orphaneti
251579, Giant cell glioblastoma
251576, Gliosarcoma
163634, Maffucci syndrome
99646, Metaphyseal chondromatosis with D-2-hydroxyglutaric aciduria
296, Ollier disease

The Pharmacogenetics and Pharmacogenomics Knowledge Base

More...
PharmGKBi
PA29630

Miscellaneous databases

Pharos NIH Druggable Genome Knowledgebase

More...
Pharosi
O75874, Tclin

Chemistry databases

ChEMBL database of bioactive drug-like small molecules

More...
ChEMBLi
CHEMBL2007625

Drug and drug target database

More...
DrugBanki
DB09374, Indocyanine green acid form
DB01727, Isocitric Acid
DB14568, Ivosidenib
DB03461, Nicotinamide adenine dinucleotide phosphate

DrugCentral

More...
DrugCentrali
O75874

IUPHAR/BPS Guide to PHARMACOLOGY

More...
GuidetoPHARMACOLOGYi
2884

Genetic variation databases

BioMuta curated single-nucleotide variation and disease association database

More...
BioMutai
IDH1

<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 <a href="http://www.uniprot.org/help/ptm_processing_section">PTM / Processing</a> section indicates that the initiator methionine is cleaved from the mature protein.<p><a href='/help/init_met' target='_top'>More...</a></p>Initiator methionineiRemovedCombined sources
<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_00000835752 – 414Isocitrate dehydrogenase [NADP] cytoplasmicAdd BLAST413

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the 'PTM / Processing' section specifies the position and type of each modified residue excluding <a href="http://www.uniprot.org/manual/lipid">lipids</a>, <a href="http://www.uniprot.org/manual/carbohyd">glycans</a> and <a href="http://www.uniprot.org/manual/crosslnk">protein cross-links</a>.<p><a href='/help/mod_res' target='_top'>More...</a></p>Modified residuei2N-acetylserineCombined sources1
Modified residuei42PhosphotyrosineCombined sources1
Modified residuei81N6-acetyllysineBy similarity1
Modified residuei126N6-succinyllysineBy similarity1
Modified residuei224N6-acetyllysineBy similarity1
Modified residuei233N6-acetyllysineBy similarity1
Modified residuei243N6-acetyllysineBy similarity1
Modified residuei321N6-acetyllysineCombined sources1
Modified residuei389PhosphoserineBy similarity1
Modified residuei400N6-succinyllysineBy similarity1

<p>This subsection of the <a href="http://www.uniprot.org/help/ptm_processing_section">PTM/processing</a> section describes post-translational modifications (PTMs). This subsection <strong>complements</strong> the information provided at the sequence level or describes modifications for which <strong>position-specific data is not yet available</strong>.<p><a href='/help/post-translational_modification' target='_top'>More...</a></p>Post-translational modificationi

Acetylation at Lys-374 dramatically reduces catalytic activity.By similarity

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

Encyclopedia of Proteome Dynamics

More...
EPDi
O75874

jPOST - Japan Proteome Standard Repository/Database

More...
jPOSTi
O75874

MassIVE - Mass Spectrometry Interactive Virtual Environment

More...
MassIVEi
O75874

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

More...
PaxDbi
O75874

PeptideAtlas

More...
PeptideAtlasi
O75874

PRoteomics IDEntifications database

More...
PRIDEi
O75874

ProteomicsDB: a multi-organism proteome resource

More...
ProteomicsDBi
50234

2D gel databases

USC-OGP 2-DE database

More...
OGPi
O75874

REPRODUCTION-2DPAGE

More...
REPRODUCTION-2DPAGEi
IPI00027223

University College Dublin 2-DE Proteome Database

More...
UCD-2DPAGEi
O75874

PTM databases

GlyGen: Computational and Informatics Resources for Glycoscience

More...
GlyGeni
O75874, 1 site, 1 O-linked glycan (1 site)

iPTMnet integrated resource for PTMs in systems biology context

More...
iPTMneti
O75874

MetOSite database of methionine sulfoxide sites

More...
MetOSitei
O75874

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

More...
PhosphoSitePlusi
O75874

SwissPalm database of S-palmitoylation events

More...
SwissPalmi
O75874

<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
ENSG00000138413, Expressed in metanephros and 247 other tissues

ExpressionAtlas, Differential and Baseline Expression

More...
ExpressionAtlasi
O75874, baseline and differential

Genevisible search portal to normalized and curated expression data from Genevestigator

More...
Genevisiblei
O75874, HS

Organism-specific databases

Human Protein Atlas

More...
HPAi
ENSG00000138413, 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_section">'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_section">'Function'</a> section).<p><a href='/help/subunit_structure' target='_top'>More...</a></p>Subunit structurei

Homodimer.

2 Publications

<p>This subsection of the '<a href="http://www.uniprot.org/help/interaction_section">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

Hide details

GO - Molecular functioni

Protein-protein interaction databases

The Biological General Repository for Interaction Datasets (BioGRID)

More...
BioGRIDi
109643, 90 interactors

CORUM comprehensive resource of mammalian protein complexes

More...
CORUMi
O75874

Database of interacting proteins

More...
DIPi
DIP-59311N

Protein interaction database and analysis system

More...
IntActi
O75874, 19 interactors

STRING: functional protein association networks

More...
STRINGi
9606.ENSP00000390265

Chemistry databases

BindingDB database of measured binding affinities

More...
BindingDBi
O75874

Miscellaneous databases

RNAct, Protein-RNA interaction predictions for model organisms.

More...
RNActi
O75874, 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

1414
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
O75874

Database of comparative protein structure models

More...
ModBasei
Search...

Protein Data Bank in Europe - Knowledge Base

More...
PDBe-KBi
Search...

Miscellaneous databases

Relative evolutionary importance of amino acids within a protein sequence

More...
EvolutionaryTracei
O75874

<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

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the 'Family and Domains' section describes a region of interest that cannot be described in other subsections.<p><a href='/help/region' target='_top'>More...</a></p>Regioni94 – 100Substrate bindingCombined sources1 Publication7

<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

Phylogenomic databases

evolutionary genealogy of genes: Non-supervised Orthologous Groups

More...
eggNOGi
KOG1526, Eukaryota

Ensembl GeneTree

More...
GeneTreei
ENSGT00390000012547

The HOGENOM Database of Homologous Genes from Fully Sequenced Organisms

More...
HOGENOMi
CLU_023296_1_1_1

InParanoid: Eukaryotic Ortholog Groups

More...
InParanoidi
O75874

Identification of Orthologs from Complete Genome Data

More...
OMAi
HGTVQRH

Database of Orthologous Groups

More...
OrthoDBi
511880at2759

Database for complete collections of gene phylogenies

More...
PhylomeDBi
O75874

TreeFam database of animal gene trees

More...
TreeFami
TF300428

Family and domain databases

Integrated resource of protein families, domains and functional sites

More...
InterProi
View protein in InterPro
IPR019818, IsoCit/isopropylmalate_DH_CS
IPR004790, Isocitrate_DH_NADP
IPR024084, IsoPropMal-DH-like_dom

The PANTHER Classification System

More...
PANTHERi
PTHR11822, PTHR11822, 1 hit

Pfam protein domain database

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

PIRSF; a whole-protein classification database

More...
PIRSFi
PIRSF000108, IDH_NADP, 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
TIGR00127, nadp_idh_euk, 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_length">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>Sequence (1+)i

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences_section">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical_and_isoforms">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_section">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical_and_isoforms">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 has 1 described isoform and 3 potential isoforms that are computationally mapped.Show allAlign All

O75874-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MSKKISGGSV VEMQGDEMTR IIWELIKEKL IFPYVELDLH SYDLGIENRD
60 70 80 90 100
ATNDQVTKDA AEAIKKHNVG VKCATITPDE KRVEEFKLKQ MWKSPNGTIR
110 120 130 140 150
NILGGTVFRE AIICKNIPRL VSGWVKPIII GRHAYGDQYR ATDFVVPGPG
160 170 180 190 200
KVEITYTPSD GTQKVTYLVH NFEEGGGVAM GMYNQDKSIE DFAHSSFQMA
210 220 230 240 250
LSKGWPLYLS TKNTILKKYD GRFKDIFQEI YDKQYKSQFE AQKIWYEHRL
260 270 280 290 300
IDDMVAQAMK SEGGFIWACK NYDGDVQSDS VAQGYGSLGM MTSVLVCPDG
310 320 330 340 350
KTVEAEAAHG TVTRHYRMYQ KGQETSTNPI ASIFAWTRGL AHRAKLDNNK
360 370 380 390 400
ELAFFANALE EVSIETIEAG FMTKDLAACI KGLPNVQRSD YLNTFEFMDK
410
LGENLKIKLA QAKL
Length:414
Mass (Da):46,659
Last modified:July 11, 2002 - v2
<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:i60428B0B6E5851DC
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 3 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
C9J4N6C9J4N6_HUMAN
Isocitrate dehydrogenase [NADP] cyt...
IDH1
157Annotation 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>
C9JJE5C9JJE5_HUMAN
Isocitrate dehydrogenase [NADP] cyt...
IDH1
62Annotation 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>
C9JLU6C9JLU6_HUMAN
Isocitrate dehydrogenase [NADP] cyt...
IDH1
73Annotation 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>

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 conflicti32F → I in CAB66637 (PubMed:11230166).Curated1
Sequence conflicti126K → E in CAB66637 (PubMed:11230166).Curated1
Sequence conflicti172F → S in CAD97653 (PubMed:17974005).Curated1
Sequence conflicti174E → G in CAD97653 (PubMed:17974005).Curated1
Sequence conflicti218K → I in AAD02918 (PubMed:9866202).Curated1
Sequence conflicti307A → S in AAH93020 (PubMed:15815621).Curated1
Sequence conflicti329P → L in AAD02918 (PubMed:9866202).Curated1
Sequence conflicti381K → R in AAD02918 (PubMed:9866202).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the 'Sequence' section describes natural variant(s) of the protein sequence.<p><a href='/help/variant' target='_top'>More...</a></p>Natural variantiVAR_036013132R → C in colorectal cancer and glioma samples; glioblastoma multiforme; somatic mutation; found in patients with cartilaginous tumors; abolishes magnesium binding and alters enzyme activity so that isocitrate is no longer converted to alpha-ketoglutarate but instead alpha-ketoglutarate is converted to R(-)-2-hydroxyglutarate; induces histone methylation; enhances expression of chondrocyte-related genes; disturbs the formation of cartilaginous matrix; inhibits osteogenic differentiation. 4 PublicationsCorresponds to variant dbSNP:rs121913499EnsemblClinVar.1
Natural variantiVAR_055454132R → G in a glioma sample; glioblastoma multiforme; somatic mutation; found in patients with cartilaginous tumors. 2 PublicationsCorresponds to variant dbSNP:rs121913499EnsemblClinVar.1
Natural variantiVAR_055455132R → H in a glioma sample; glioblastoma multiforme; somatic mutation; found in patients with cartilaginous tumors; abolishes magnesium binding and alters enzyme activity so that isocitrate is no longer converted to alpha-ketoglutarate but instead alpha-ketoglutarate is converted to R(-)-2-hydroxyglutarate. 3 PublicationsCorresponds to variant dbSNP:rs121913500EnsemblClinVar.1
Natural variantiVAR_055456132R → L in a glioma sample; glioblastoma multiforme; somatic mutation; abolishes magnesium binding and alters enzyme activity so that isocitrate is no longer converted to alpha-ketoglutarate but instead alpha-ketoglutarate is converted to R(-)-2-hydroxyglutarate. 2 PublicationsCorresponds to variant dbSNP:rs121913500EnsemblClinVar.1
Natural variantiVAR_055457132R → S in a glioma sample; glioblastoma multiforme; somatic mutation; abolishes magnesium binding and alters enzyme activity so that isocitrate is no longer converted to alpha-ketoglutarate but instead alpha-ketoglutarate is converted to R(-)-2-hydroxyglutarate. 2 PublicationsCorresponds to variant dbSNP:rs121913499EnsemblClinVar.1
Natural variantiVAR_049780178V → I. Corresponds to variant dbSNP:rs34218846EnsemblClinVar.1

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
AF020038 mRNA Translation: AAD02918.1
AF113917 mRNA Translation: AAD29284.1
AL136702 mRNA Translation: CAB66637.1
CR541695 mRNA Translation: CAG46496.1
BX537411 mRNA Translation: CAD97653.1
AC016697 Genomic DNA Translation: AAX93221.1
CH471063 Genomic DNA Translation: EAW70439.1
BC012846 mRNA Translation: AAH12846.1
BC093020 mRNA Translation: AAH93020.1
U62389 mRNA Translation: AAB17375.1

The Consensus CDS (CCDS) project

More...
CCDSi
CCDS2381.1

Protein sequence database of the Protein Information Resource

More...
PIRi
T46280

NCBI Reference Sequences

More...
RefSeqi
NP_001269315.1, NM_001282386.1
NP_001269316.1, NM_001282387.1
NP_005887.2, NM_005896.3

Genome annotation databases

Ensembl eukaryotic genome annotation project

More...
Ensembli
ENST00000345146; ENSP00000260985; ENSG00000138413
ENST00000415913; ENSP00000390265; ENSG00000138413
ENST00000446179; ENSP00000410513; ENSG00000138413

Database of genes from NCBI RefSeq genomes

More...
GeneIDi
3417

KEGG: Kyoto Encyclopedia of Genes and Genomes

More...
KEGGi
hsa:3417

<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

<p>This subsection of the <a href="http://www.uniprot.org/manual/cross_references_section">Cross-references</a> section provides links to various web resources that are relevant for a specific protein.<p><a href='/help/web_resource' target='_top'>More...</a></p>Web resourcesi

Wikipedia

Isocitrate dehydrogenase entry

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF020038 mRNA Translation: AAD02918.1
AF113917 mRNA Translation: AAD29284.1
AL136702 mRNA Translation: CAB66637.1
CR541695 mRNA Translation: CAG46496.1
BX537411 mRNA Translation: CAD97653.1
AC016697 Genomic DNA Translation: AAX93221.1
CH471063 Genomic DNA Translation: EAW70439.1
BC012846 mRNA Translation: AAH12846.1
BC093020 mRNA Translation: AAH93020.1
U62389 mRNA Translation: AAB17375.1
CCDSiCCDS2381.1
PIRiT46280
RefSeqiNP_001269315.1, NM_001282386.1
NP_001269316.1, NM_001282387.1
NP_005887.2, NM_005896.3

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
1T09X-ray2.70A/B1-414[»]
1T0LX-ray2.41A/B/C/D1-414[»]
3INMX-ray2.10A/B/C1-414[»]
3MAPX-ray2.80A/B1-414[»]
3MARX-ray3.41A/B1-414[»]
3MASX-ray3.20A/B1-414[»]
4I3KX-ray3.31A/B1-414[»]
4I3LX-ray3.29A/B1-414[»]
4KZOX-ray2.20A/B/C1-414[»]
4L03X-ray2.10A/B/C1-414[»]
4L04X-ray2.87A/B/C/D/E/F1-414[»]
4L06X-ray2.28A/B/C/D/E/F1-414[»]
4UMXX-ray1.88A/B1-414[»]
4UMYX-ray2.07A/B1-414[»]
4XRXX-ray3.20A/B1-414[»]
4XS3X-ray3.29A/B1-414[»]
5DE1X-ray2.25A/B2-414[»]
5GIRX-ray1.93C/D126-137[»]
5K10electron microscopy3.80A/B3-413[»]
5K11electron microscopy3.80A/B3-413[»]
5L57X-ray2.69A1-414[»]
5L58X-ray3.04A1-414[»]
5LGEX-ray2.70A/B/C/D1-414[»]
5SUNX-ray2.48A/B1-414[»]
5SVFX-ray2.34A/B/C/D1-414[»]
5TQHX-ray2.20A/B/C/D1-414[»]
5YFMX-ray2.40A/B/C1-414[»]
5YFNX-ray2.50A/B1-414[»]
6ADGX-ray3.00A/B/C1-414[»]
6B0ZX-ray2.33A/B/C/D1-414[»]
6BKXX-ray1.65A/B/C1-414[»]
6BKYX-ray2.17A/B/C/D/E/F1-414[»]
6BKZX-ray2.01A/B1-414[»]
6BL0X-ray2.17A/B/C1-414[»]
6BL1X-ray2.02A/B/C1-414[»]
6BL2X-ray1.92A/B/C1-414[»]
6IO0X-ray2.20A/B1-414[»]
6O2YX-ray2.80A/B/C1-414[»]
6O2ZX-ray2.50A/B1-414[»]
6PAYX-ray2.20A/B/C/D1-414[»]
6Q6FX-ray3.30A/B1-414[»]
6U4JX-ray2.11A/B1-414[»]
6VEIX-ray2.10A/B1-414[»]
6VG0X-ray2.66A/B/C1-414[»]
SMRiO75874
ModBaseiSearch...
PDBe-KBiSearch...

Protein-protein interaction databases

BioGRIDi109643, 90 interactors
CORUMiO75874
DIPiDIP-59311N
IntActiO75874, 19 interactors
STRINGi9606.ENSP00000390265

Chemistry databases

BindingDBiO75874
ChEMBLiCHEMBL2007625
DrugBankiDB09374, Indocyanine green acid form
DB01727, Isocitric Acid
DB14568, Ivosidenib
DB03461, Nicotinamide adenine dinucleotide phosphate
DrugCentraliO75874
GuidetoPHARMACOLOGYi2884

PTM databases

GlyGeniO75874, 1 site, 1 O-linked glycan (1 site)
iPTMnetiO75874
MetOSiteiO75874
PhosphoSitePlusiO75874
SwissPalmiO75874

Genetic variation databases

BioMutaiIDH1

2D gel databases

OGPiO75874
REPRODUCTION-2DPAGEiIPI00027223
UCD-2DPAGEiO75874

Proteomic databases

EPDiO75874
jPOSTiO75874
MassIVEiO75874
PaxDbiO75874
PeptideAtlasiO75874
PRIDEiO75874
ProteomicsDBi50234

Protocols and materials databases

ABCD curated depository of sequenced antibodies

More...
ABCDi
O75874, 4 sequenced antibodies

Antibodypedia a portal for validated antibodies

More...
Antibodypediai
34198, 959 antibodies

The DNASU plasmid repository

More...
DNASUi
3417

Genome annotation databases

EnsembliENST00000345146; ENSP00000260985; ENSG00000138413
ENST00000415913; ENSP00000390265; ENSG00000138413
ENST00000446179; ENSP00000410513; ENSG00000138413
GeneIDi3417
KEGGihsa:3417

Organism-specific databases

Comparative Toxicogenomics Database

More...
CTDi
3417
DisGeNETi3417

GeneCards: human genes, protein and diseases

More...
GeneCardsi
IDH1
HGNCiHGNC:5382, IDH1
HPAiENSG00000138413, Low tissue specificity
MalaCardsiIDH1
MIMi137800, phenotype
147700, gene
neXtProtiNX_O75874
OpenTargetsiENSG00000138413
Orphaneti251579, Giant cell glioblastoma
251576, Gliosarcoma
163634, Maffucci syndrome
99646, Metaphyseal chondromatosis with D-2-hydroxyglutaric aciduria
296, Ollier disease
PharmGKBiPA29630
VEuPathDBiHostDB:ENSG00000138413

GenAtlas: human gene database

More...
GenAtlasi
Search...

Phylogenomic databases

eggNOGiKOG1526, Eukaryota
GeneTreeiENSGT00390000012547
HOGENOMiCLU_023296_1_1_1
InParanoidiO75874
OMAiHGTVQRH
OrthoDBi511880at2759
PhylomeDBiO75874
TreeFamiTF300428

Enzyme and pathway databases

BioCyciMetaCyc:HS06502-MONOMER
BRENDAi1.1.1.42, 2681
PathwayCommonsiO75874
ReactomeiR-HSA-2978092, Abnormal conversion of 2-oxoglutarate to 2-hydroxyglutarate
R-HSA-389542, NADPH regeneration
R-HSA-6798695, Neutrophil degranulation
R-HSA-9033241, Peroxisomal protein import
SABIO-RKiO75874
SIGNORiO75874

Miscellaneous databases

BioGRID ORCS database of CRISPR phenotype screens

More...
BioGRID-ORCSi
3417, 12 hits in 1010 CRISPR screens

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

More...
ChiTaRSi
IDH1, human
EvolutionaryTraceiO75874

The Gene Wiki collection of pages on human genes and proteins

More...
GeneWikii
IDH1

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

More...
GenomeRNAii
3417
PharosiO75874, Tclin

Protein Ontology

More...
PROi
PR:O75874
RNActiO75874, protein

The Stanford Online Universal Resource for Clones and ESTs

More...
SOURCEi
Search...

Gene expression databases

BgeeiENSG00000138413, Expressed in metanephros and 247 other tissues
ExpressionAtlasiO75874, baseline and differential
GenevisibleiO75874, HS

Family and domain databases

InterProiView protein in InterPro
IPR019818, IsoCit/isopropylmalate_DH_CS
IPR004790, Isocitrate_DH_NADP
IPR024084, IsoPropMal-DH-like_dom
PANTHERiPTHR11822, PTHR11822, 1 hit
PfamiView protein in Pfam
PF00180, Iso_dh, 1 hit
PIRSFiPIRSF000108, IDH_NADP, 1 hit
SMARTiView protein in SMART
SM01329, Iso_dh, 1 hit
TIGRFAMsiTIGR00127, nadp_idh_euk, 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 nameiIDHC_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: O75874
Secondary accession number(s): Q567U4
, Q6FHQ6, Q7Z3V0, Q93090, Q9NTJ9, Q9UKW8
<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_and_isoforms">canonical sequence</a> are also displayed.<p><a href='/help/entry_history' target='_top'>More...</a></p>Entry historyiIntegrated into UniProtKB/Swiss-Prot: May 30, 2000
Last sequence update: July 11, 2002
Last modified: September 29, 2021
This is version 219 of the entry and version 2 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, Direct protein sequencing, Reference proteome

Documents

  1. Human chromosome 2
    Human chromosome 2: entries, gene names and cross-references to MIM
  2. Human entries with genetic variants
    List of human entries with genetic variants
  3. Human variants curated from literature reports
    Index of human variants curated from literature reports
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  6. SIMILARITY comments
    Index of protein domains and families
UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health

We'd like to inform you that we have updated our Privacy Notice to comply with Europe’s new General Data Protection Regulation (GDPR) that applies since 25 May 2018.

Do not show this banner again