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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 entries **1 - 5** of 6 in total

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GO Term	Evidence Code	PMID
2-oxoglutarate metabolic process	ISS
glyoxylate cycle	IEA
carbohydrate metabolic process	NAS	8954790
isocitrate metabolic process	ISS
NADP metabolic process	IBA

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
cytosol	IEA
peroxisome	IEA
mitochondrial matrix	TAS
mitochondrion	HDA IBA IDA	20833797
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
magnesium ion binding	ISS
NAD binding	IEA
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.

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

RHEA:19629

D-threo-isocitrate + NADP⁺

CO₂ + 2-oxoglutarate + NADPH

Enzyme

1.1.1.42

PMID

Disease

Disease Ontology

Displaying entries **1 - 10** of **347** in total

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DO ID	Disease Name	Source
DOID:0014667	disease of metabolism	DisGeNET DisGeNET
DOID:0050083	Keshan disease	DisGeNET
DOID:0050486	exanthem	DisGeNET
DOID:0050557	congenital muscular dystrophy	DisGeNET
DOID:0050572	cone-rod dystrophy	DisGeNET
DOID:0050573	2-hydroxyglutaric aciduria	DisGeNET
DOID:0050575	D-2-hydroxyglutaric aciduria	DisGeNET DisGeNET
DOID:0050589	inflammatory bowel disease	DisGeNET
DOID:0050646	distal arthrogryposis	DisGeNET
DOID:0050700	cardiomyopathy	DisGeNET DisGeNET

The Human Phenotype Ontology

Displaying entries **1 - 10** of 43 in total

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HPO ID	HPO Term
HP:0000006	Autosomal dominant inheritance
HP:0000826	Precocious puberty
HP:0000853	Goiter
HP:0000926	Platyspondyly
HP:0000944	Abnormal metaphysis morphology
HP:0001028	Hemangioma
HP:0001250	Seizure
HP:0001252	Hypotonia
HP:0001263	Global developmental delay
HP:0001387	Joint stiffness

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

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	Alliance of Genome Resources	Orthologous MAtrix
Caenorhabditis elegans	181311	WB:WBGene00007942
Callorhinchus milii	103187483		CALMI03779
Danio rerio	386951	ZFIN:ZDB-GENE-031118-95
Astyanax mexicanus	103037962		ASTMX09028
Ictalurus punctatus	100304677		ICTPU28304
Electrophorus electricus	113573975		ELEEL13759
Esox lucius	105016647		ESOLU51196
Esox lucius	105025672		ESOLU55097
Salmo salar	100194639		SALSA44572
Salmo trutta	115152014		SALTR115336

GlyTouCan

GlyComb

GlycoPOST

UniCarb-DR

All Resources

Other Information

Tools

Search Tools

Summary

External Links

Annotation

KEGG BRITE Database

PubChem Disease

International Collaboration

Acknowledgements