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isocitrate dehydrogenase (NADP(+)) 2

Summary

Gene Symbol

IDH2

Aliases

IDH-2

Organism

Homo sapiens (human)

External Links

NCBI Gene

3418

HGNC

5383

KEGG Gene ID

hsa:3418

PubChem

3418

Alliance of Genome Resources

HGNC:5383

Annotation

Keyword

3D-structure
Acetylation
Alternative splicing
Disease variant
Glyoxylate bypass
Magnesium
Manganese
Metal-binding
Mitochondrion
NADP
Oxidoreductase
Reference proteome
Transit peptide
Tricarboxylic acid cycle

Proteins

Displaying **all 2** entries
UniProt	Protein Name
B4DSZ6	NADP(+)-specific ICDH Oxalosuccinate decarboxylase
P48735	ICD-M IDP NADP(+)-specific ICDH Oxalosuccinate decarboxylase

Annotation information from UniProt.

Gene Ontology (GO)

Displaying entry **6 - 6** of 6 in total

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GO Term	Evidence Code	PMID
glyoxylate cycle	IEA

GO Hierarchy

biological process

metabolic process [inference]

nitrogen compound metabolic process [inference]

cellular nitrogen compound metabolic process [inference]

nucleobase-containing compound metabolic process [inference]

nucleobase-containing small molecule metabolic process [inference]

nucleoside phosphate metabolic process [inference]

nucleotide metabolic process [inference]

purine nucleotide metabolic process [inference]

NADP metabolic process

pyridine nucleotide metabolic process [inference]

nicotinamide nucleotide metabolic process [inference]

NADP metabolic process

purine-containing compound metabolic process [inference]

purine nucleotide metabolic process [inference]

NADP metabolic process

pyridine-containing compound metabolic process [inference]

pyridine nucleotide metabolic process [inference]

nicotinamide nucleotide metabolic process [inference]

NADP metabolic process

organonitrogen compound metabolic process [inference]

purine-containing compound metabolic process [inference]

purine nucleotide metabolic process [inference]

NADP metabolic process

pyridine-containing compound metabolic process [inference]

pyridine nucleotide metabolic process [inference]

nicotinamide nucleotide metabolic process [inference]

NADP metabolic process

cellular metabolic process [inference]

cellular aldehyde metabolic process [inference]

glyoxylate metabolic process [inference]

glyoxylate cycle

organic acid metabolic process [inference]

oxoacid metabolic process [inference]

carboxylic acid metabolic process [inference]

monocarboxylic acid metabolic process [inference]

glyoxylate metabolic process [inference]

glyoxylate cycle

dicarboxylic acid metabolic process [inference]

2-oxoglutarate metabolic process

tricarboxylic acid metabolic process [inference]

isocitrate metabolic process

cellular aromatic compound metabolic process [inference]

nucleobase-containing compound metabolic process [inference]

nucleobase-containing small molecule metabolic process [inference]

nucleoside phosphate metabolic process [inference]

nucleotide metabolic process [inference]

purine nucleotide metabolic process [inference]

NADP metabolic process

pyridine nucleotide metabolic process [inference]

nicotinamide nucleotide metabolic process [inference]

NADP metabolic process

purine-containing compound metabolic process [inference]

purine nucleotide metabolic process [inference]

NADP metabolic process

phosphorus metabolic process [inference]

phosphate-containing compound metabolic process [inference]

nucleoside phosphate metabolic process [inference]

nucleotide metabolic process [inference]

purine nucleotide metabolic process [inference]

NADP metabolic process

pyridine nucleotide metabolic process [inference]

nicotinamide nucleotide metabolic process [inference]

NADP metabolic process

organophosphate metabolic process [inference]

nucleoside phosphate metabolic process [inference]

nucleotide metabolic process [inference]

purine nucleotide metabolic process [inference]

NADP metabolic process

pyridine nucleotide metabolic process [inference]

nicotinamide nucleotide metabolic process [inference]

NADP metabolic process

cellular nitrogen compound metabolic process [inference]

nucleobase-containing compound metabolic process [inference]

nucleobase-containing small molecule metabolic process [inference]

nucleoside phosphate metabolic process [inference]

nucleotide metabolic process [inference]

purine nucleotide metabolic process [inference]

NADP metabolic process

pyridine nucleotide metabolic process [inference]

nicotinamide nucleotide metabolic process [inference]

NADP metabolic process

purine-containing compound metabolic process [inference]

purine nucleotide metabolic process [inference]

NADP metabolic process

pyridine-containing compound metabolic process [inference]

pyridine nucleotide metabolic process [inference]

nicotinamide nucleotide metabolic process [inference]

NADP metabolic process

heterocycle metabolic process [inference]

nucleobase-containing compound metabolic process [inference]

nucleobase-containing small molecule metabolic process [inference]

nucleoside phosphate metabolic process [inference]

nucleotide metabolic process [inference]

purine nucleotide metabolic process [inference]

NADP metabolic process

pyridine nucleotide metabolic process [inference]

nicotinamide nucleotide metabolic process [inference]

NADP metabolic process

purine-containing compound metabolic process [inference]

purine nucleotide metabolic process [inference]

NADP metabolic process

pyridine-containing compound metabolic process [inference]

pyridine nucleotide metabolic process [inference]

nicotinamide nucleotide metabolic process [inference]

NADP metabolic process

primary metabolic process [inference]

carbohydrate metabolic process

glyoxylate cycle

tricarboxylic acid cycle

nucleobase-containing compound metabolic process [inference]

nucleobase-containing small molecule metabolic process [inference]

nucleoside phosphate metabolic process [inference]

nucleotide metabolic process [inference]

purine nucleotide metabolic process [inference]

NADP metabolic process

pyridine nucleotide metabolic process [inference]

nicotinamide nucleotide metabolic process [inference]

NADP metabolic process

small molecule metabolic process [inference]

alcohol metabolic process [inference]

secondary alcohol metabolic process [inference]

isocitrate metabolic process

organic acid metabolic process [inference]

oxoacid metabolic process [inference]

carboxylic acid metabolic process [inference]

monocarboxylic acid metabolic process [inference]

glyoxylate metabolic process [inference]

glyoxylate cycle

dicarboxylic acid metabolic process [inference]

2-oxoglutarate metabolic process

tricarboxylic acid metabolic process [inference]

isocitrate metabolic process

nucleobase-containing small molecule metabolic process [inference]

nucleoside phosphate metabolic process [inference]

nucleotide metabolic process [inference]

purine nucleotide metabolic process [inference]

NADP metabolic process

pyridine nucleotide metabolic process [inference]

nicotinamide nucleotide metabolic process [inference]

NADP metabolic process

organic substance metabolic process [inference]

carbohydrate metabolic process

glyoxylate cycle

cellular aldehyde metabolic process [inference]

glyoxylate metabolic process [inference]

glyoxylate cycle

organic acid metabolic process [inference]

oxoacid metabolic process [inference]

carboxylic acid metabolic process [inference]

monocarboxylic acid metabolic process [inference]

glyoxylate metabolic process [inference]

glyoxylate cycle

dicarboxylic acid metabolic process [inference]

2-oxoglutarate metabolic process

tricarboxylic acid metabolic process [inference]

isocitrate metabolic process

organophosphate metabolic process [inference]

nucleoside phosphate metabolic process [inference]

nucleotide metabolic process [inference]

purine nucleotide metabolic process [inference]

NADP metabolic process

pyridine nucleotide metabolic process [inference]

nicotinamide nucleotide metabolic process [inference]

NADP metabolic process

organic cyclic compound metabolic process [inference]

nucleobase-containing compound metabolic process [inference]

nucleobase-containing small molecule metabolic process [inference]

nucleoside phosphate metabolic process [inference]

nucleotide metabolic process [inference]

purine nucleotide metabolic process [inference]

NADP metabolic process

pyridine nucleotide metabolic process [inference]

nicotinamide nucleotide metabolic process [inference]

NADP metabolic process

purine-containing compound metabolic process [inference]

purine nucleotide metabolic process [inference]

NADP metabolic process

pyridine-containing compound metabolic process [inference]

pyridine nucleotide metabolic process [inference]

nicotinamide nucleotide metabolic process [inference]

NADP metabolic process

organonitrogen compound metabolic process [inference]

purine-containing compound metabolic process [inference]

purine nucleotide metabolic process [inference]

NADP metabolic process

pyridine-containing compound metabolic process [inference]

pyridine nucleotide metabolic process [inference]

nicotinamide nucleotide metabolic process [inference]

NADP metabolic process

organic hydroxy compound metabolic process [inference]

alcohol metabolic process [inference]

secondary alcohol metabolic process [inference]

isocitrate metabolic process

cellular process [inference]

cellular metabolic process [inference]

cellular aldehyde metabolic process [inference]

glyoxylate metabolic process [inference]

glyoxylate cycle

organic acid metabolic process [inference]

oxoacid metabolic process [inference]

carboxylic acid metabolic process [inference]

monocarboxylic acid metabolic process [inference]

glyoxylate metabolic process [inference]

glyoxylate cycle

dicarboxylic acid metabolic process [inference]

2-oxoglutarate metabolic process

tricarboxylic acid metabolic process [inference]

isocitrate metabolic process

cellular aromatic compound metabolic process [inference]

nucleobase-containing compound metabolic process [inference]

nucleobase-containing small molecule metabolic process [inference]

nucleoside phosphate metabolic process [inference]

nucleotide metabolic process [inference]

purine nucleotide metabolic process [inference]

NADP metabolic process

pyridine nucleotide metabolic process [inference]

nicotinamide nucleotide metabolic process [inference]

NADP metabolic process

purine-containing compound metabolic process [inference]

purine nucleotide metabolic process [inference]

NADP metabolic process

phosphorus metabolic process [inference]

phosphate-containing compound metabolic process [inference]

nucleoside phosphate metabolic process [inference]

nucleotide metabolic process [inference]

purine nucleotide metabolic process [inference]

NADP metabolic process

pyridine nucleotide metabolic process [inference]

nicotinamide nucleotide metabolic process [inference]

NADP metabolic process

organophosphate metabolic process [inference]

nucleoside phosphate metabolic process [inference]

nucleotide metabolic process [inference]

purine nucleotide metabolic process [inference]

NADP metabolic process

pyridine nucleotide metabolic process [inference]

nicotinamide nucleotide metabolic process [inference]

NADP metabolic process

cellular nitrogen compound metabolic process [inference]

nucleobase-containing compound metabolic process [inference]

nucleobase-containing small molecule metabolic process [inference]

nucleoside phosphate metabolic process [inference]

nucleotide metabolic process [inference]

purine nucleotide metabolic process [inference]

NADP metabolic process

pyridine nucleotide metabolic process [inference]

nicotinamide nucleotide metabolic process [inference]

NADP metabolic process

purine-containing compound metabolic process [inference]

purine nucleotide metabolic process [inference]

NADP metabolic process

pyridine-containing compound metabolic process [inference]

pyridine nucleotide metabolic process [inference]

nicotinamide nucleotide metabolic process [inference]

NADP metabolic process

heterocycle metabolic process [inference]

nucleobase-containing compound metabolic process [inference]

nucleobase-containing small molecule metabolic process [inference]

nucleoside phosphate metabolic process [inference]

nucleotide metabolic process [inference]

purine nucleotide metabolic process [inference]

NADP metabolic process

pyridine nucleotide metabolic process [inference]

nicotinamide nucleotide metabolic process [inference]

NADP metabolic process

purine-containing compound metabolic process [inference]

purine nucleotide metabolic process [inference]

NADP metabolic process

pyridine-containing compound metabolic process [inference]

pyridine nucleotide metabolic process [inference]

nicotinamide nucleotide metabolic process [inference]

NADP metabolic process

Displaying **all 5** entries
GO Term	Evidence Code	PMID
peroxisome	IEA
mitochondrion	HDA HTP IBA IDA	20833797 34800366
mitochondrial matrix	TAS
cytosol	IEA
extracellular exosome	HDA	23533145

GO Hierarchy

cellular component

cellular anatomical entity [inference]

cytosol

membrane-enclosed lumen [inference]

organelle lumen [inference]

intracellular organelle lumen [inference]

mitochondrial matrix

organelle [inference]

membrane-bounded organelle [inference]

vesicle [inference]

extracellular vesicle [inference]

extracellular exosome

intracellular membrane-bounded organelle [inference]

mitochondrion

microbody [inference]

peroxisome

extracellular membrane-bounded organelle [inference]

extracellular vesicle [inference]

extracellular exosome

intracellular organelle [inference]

intracellular membrane-bounded organelle [inference]

mitochondrion

microbody [inference]

peroxisome

extracellular organelle [inference]

extracellular membrane-bounded organelle [inference]

extracellular vesicle [inference]

extracellular exosome

Displaying **all 3** entries
GO Term	Evidence Code	PMID
NAD binding	IEA
magnesium ion binding	ISS
isocitrate dehydrogenase (NADP+) activity	IBA IDA IMP ISS	22416140 23226729

GO Hierarchy

molecular function

catalytic activity [inference]

oxidoreductase activity [inference]

oxidoreductase activity, acting on CH-OH group of donors [inference]

oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor [inference]

isocitrate dehydrogenase [NAD(P)+] activity [inference]

isocitrate dehydrogenase (NADP+) activity

binding [inference]

small molecule binding [inference]

heterocyclic compound binding [inference]

nucleotide binding [inference]

purine nucleotide binding [inference]

adenyl nucleotide binding [inference]

NAD binding

ion binding [inference]

cation binding [inference]

metal ion binding [inference]

magnesium ion binding

organic cyclic compound binding [inference]

nucleoside phosphate binding [inference]

nucleotide binding [inference]

purine nucleotide binding [inference]

adenyl nucleotide binding [inference]

NAD binding

Gene Ontology information from NCBI Gene and UniProt.

OrthoDB (Group)

Group level

Eukaryota

Group Name

isocitrate dehydrogenase

Functional Category

E: Amino acid transport and metabolism
J: Translation, ribosomal structure and biogenesis
T: Signal transduction mechanisms

Displaying **all 4** entries
Gene Ontology
ATP binding
kinase activity
nucleotide binding
phosphorylation

Displaying **all 4** entries
InterPro
Protein kinase domain
Protein kinase, ATP binding site
Protein kinase-like domain superfamily
Serine/threonine-protein kinase, active site

Human Protein Atlas

ENSG00000182054

Tissue with high expression from Human Protein Atlas. Tissues that are highly expressed are highlighted.

Data available from v23.0.proteinatlas.org. Image credit: Human Protein Atlas

KEGG BRITE Database

Orthology

K00031

Name

isocitrate dehydrogenase [EC:1.1.1.42]

References

Rhea

D-threo-isocitrate + NADP⁺

CO₂ + 2-oxoglutarate + NADPH

Enzyme

1.1.1.42

PMID

Disease

Disease Ontology

Displaying entries **1 - 10** of 14 in total

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DO ID	Disease Name	Source
DOID:0080005	bone remodeling disease	Alliance of Genome Resources
DOID:0111352	D-2-hydroxyglutaric aciduria 2	Alliance of Genome Resources
DOID:10534	stomach cancer	Alliance of Genome Resources
DOID:1324	lung cancer	Alliance of Genome Resources
DOID:1824	status epilepticus	Alliance of Genome Resources
DOID:3070	high grade glioma	Alliance of Genome Resources
DOID:3748	esophagus squamous cell carcinoma	Alliance of Genome Resources
DOID:3907	lung squamous cell carcinoma	Alliance of Genome Resources
DOID:3908	lung non-small cell carcinoma	Alliance of Genome Resources
DOID:3910	lung adenocarcinoma	Alliance of Genome Resources

The Human Phenotype Ontology

Displaying entries **21 - 30** of 43 in total

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HPO ID	HPO Term
HP:0002763	Abnormal cartilage morphology
HP:0002797	Osteolysis
HP:0002893	Pituitary adenoma
HP:0002897	Parathyroid adenoma
HP:0002983	Micromelia
HP:0003002	Breast carcinoma
HP:0004322	Short stature
HP:0004936	Venous thrombosis
HP:0005701	Multiple enchondromatosis
HP:0006765	Chondrosarcoma

Displaying **all 3** entries
Disease ID	Disease Name
OMIM:613657	d-2-hydroxyglutaric aciduria 2
ORPHA:296	Ollier disease
ORPHA:163634	Maffucci syndrome

PubChem Disease

GHR Health Conditions

MedGen Diseases

D-2-hydroxyglutaric aciduria 2

OMIM Phenotypes

D-2-HYDROXYGLUTARIC ACIDURIA 2; D2HGA2

KEGG Diseases

Gene-Disease-Co-Occurrences-in-Literature (PubChem)

Ortholog

Displaying entries **1 - 10** of 86 in total

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Species	Gene ID	OrthoDB	Alliance of Genome Resources	Orthologous MAtrix
Mus musculus	269951		MGI:96414	MOUSE53875
Bos taurus	327669			BOVIN16409
Rattus norvegicus	361596		RGD:1597139	RATNO15712
Xenopus laevis	380013		Xenbase:XB-GENE-976321
Danio rerio	386951		ZFIN:ZDB-GENE-031118-95
Xenopus tropicalis	448026		Xenbase:XB-GENE-976315
Pan troglodytes	453645	9598_0:003cfb		PANTR11142
Canis lupus familiaris	479043			CANLF12065
Macaca mulatta	701480			MACMU41774
Saccharomyces cerevisiae S288C	850871		SGD:S000004164

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Last updated: August 19, 2024