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IDH3A - isocitrate dehydrogenase (NAD(+)) 3 catalytic subunit alpha Gene

Also Known as RP90

Species: Homo sapiens

Gene Type: protein coding

Gene ID: 3419

About IDH3A

Cytogenetic location: 15q25.1 Genomic coordinates (GRCh38): 15:78,149,362-78,171,945 (from NCBI)

This gene has 24 transcripts (splice variants), 259 orthologues, 2 paralogues and is associated with 3 phenotypes. Ubiquitous expression in heart (RPKM 59.2), adrenal (RPKM 37.1) and 25 other tissues.

Summary

Isocitrate dehydrogenases catalyze the oxidative decarboxylation of isocitrate to 2-oxoglutarate. These Enzymes belong to two distinct subclasses, one of which utilizes NAD(+) as the electron acceptor and the Other NADP(+). Five isocitrate dehydrogenases have been reported: three NAD(+)-dependent isocitrate dehydrogenases, which localize to the mitochondrial matrix, and two NADP(+)-dependent isocitrate dehydrogenases, one of which is mitochondrial and the Other predominantly cytosolic. NAD(+)-dependent isocitrate dehydrogenases catalyze the allosterically regulated rate-limiting step of the tricarboxylic acid cycle. Each isozyme is a heterotetramer that is composed of two alpha subunits, one beta subunit, and one gamma subunit. The protein encoded by this gene is the alpha subunit of one isozyme of NAD(+)-dependent isocitrate dehydrogenase. [provided by RefSeq, Jul 2008]

IDH3A Products (1)

mRNA	Protein	Name
NM_005530.3	NP_005521.1	isocitrate dehydrogenase [NAD] subunit alpha, mitochondrial precursor

Gene Ontology

Molecular Function
Biological Process
Cellular Component

Molecular Function GO Annotation	Evidence	References	Source
enables isocitrate dehydrogenase (NAD+) activity	EXP EXP: Inferred from Experiment	4336621	GOA
enables isocitrate dehydrogenase (NAD+) activity	IDA IDA: Inferred from direct assay	28098230	GOA
enables magnesium ion binding	IDA IDA: Inferred from direct assay	28098230	GOA
enables protein binding	IPI IPI: Inferred from physical interaction	14555658	GOA

Biological Process GO Annotation	Evidence	References	Source
involved in tricarboxylic acid cycle	IDA IDA: Inferred from direct assay	14555658	GOA

Cellular Component GO Annotation	Evidence	References	Source
part of isocitrate dehydrogenase complex (NAD+)	IDA IDA: Inferred from direct assay	14555658	GOA
part of isocitrate dehydrogenase complex (NAD+)	IPI IPI: Inferred from physical interaction	14555658	GOA

EXP: Inferred from Experiment IDA: Inferred from direct assay IPI: Inferred from physical interaction IMP: Inferred from mutant phenotype IGI: Inferred from genetic interaction IEP: Inferred from expression pattern

IDH3A Protein Structure

Iso_dh

0
100
200
300
366 a.a.

Protein Preferred Names	Protein Names
isocitrate dehydrogenase [NAD] subunit alpha, mitochondrial	H-IDH alpha

IDH3A Protein-protein interaction Information

Type	Protein Name	Protein ID	Interactor	Interactor Species	Interactor ID	Detection Method	References
Intra	IDH3A	P50213	IDH3G	Homo sapiens	P51553	GMS	14555658
Intra	IDH3A	P50213	IDH3G	Homo sapiens	P51553	Ion Exchange Chrom	14555658
Intra	IDH3A	P50213	IDH3G	Homo sapiens	P51553	X-Ray Diffraction	28098230
Intra	IDH3A	P50213	IDH3G	Homo sapiens	P51553	Anti Tag CoIP	33961781
Intra	IDH3A	P50213	IDH3G	Homo sapiens	P51553	Anti Tag CoIP	28514442
Intra	IDH3A	P50213	IDH3G	Homo sapiens	P51553	Reverse Phase Chrom	14555658
Intra	IDH3A	P50213	SHMT1	Homo sapiens	P34896	Anti Bait CoIP	30613765

Cross: Cross-species interaction Intra: Intraspecies interaction

Related Diseases

Diseases	Alias
Retinitis Pigmentosa 90	RP90 Retinitis Pigmentosa, Type 90
Retinitis Pigmentosa	RP Rod-Cone Dystrophy Autosomal Recessive Retinitis Pigmentosa Non-Syndromic Retinitis Pigmentosa Pericentral Pigmentary Retinopathy Pigmentary Retinopathy Tapetoretinal Degeneration Rcd Retinitis Pigmentosa Autosomal Recessive ARRP Retinitis Pigmentosa, Autosomal Recessive Retinitis Pigmentosa 1
Cone-Rod Dystrophy 2	Cone-Rod Dystrophy CORD2 Cone-Rod Retinal Dystrophy Rcrd2 Cone-Rod Retinal Dystrophy 2 Crd2 Cord Crd Retinal Cone-Rod Dystrophy Cone-Rod Retinal Dystrophy-2 Retinal Cone-Rod Dystrophy 2 Tapetoretinal Degeneration Cone-Rod Degeneration Cone Rod Dystrophy Dystrophy, Cone-Rod Dystrophy, Cone-Rod, Type 2 Retinitis Pigmentosa Retinitis Pigmentosa 2 Progressive Cone-Rod Dystrophy
Juxtacortical Chondroma	Periosteal Chondroma
2-Hydroxyglutaric Aciduria	2-Hga 2-Hydroxyglutaric Acidemia 2-Hydroxyglutaricaciduria Combined D-2- And L-2-Hydroxyglutaric Aciduria
D-2-Hydroxyglutaric Aciduria 1	D-2-Hydroxyglutaric Aciduria D2HGA1 D-2-Hga D-2-Hydroxyglutaric Acidemia D2ha D2hga Aciduria, D-2-Hydroxyglutaric, Type 1 Combined D-2- And L-2-Hydroxyglutaric Aciduria

Diseases	Alias
Waldenstroem'S Macroglobulinemia	Waldenstroem'S Macroglobulinemia Waldenstroem'S Macroglobulinemia Macroglobulinemia Of Waldenstrom Lymphoplasmacytic Lymphoma With Igm Gammopathy Lymphoplasmacytic Lymphoma Waldenstroem'S Macroglobulinemia Waldenstroem'S Macroglobulinemia Macroglobulinemia Of Waldenstrom Lymphoplasmacytic Lymphoma With Igm Gammopathy Lymphoplasmacytic Lymphoma

Related Agents

Pre-clinical Phase

Pre-clinical Phase

Bioactive Molecules (3)

Cat. No.	Product Name	Effect	Purity	Rare Diseases
HY-RS06533	IDH3A Human Pre-designed siRNA Set A	Pre-designed Set	/	Unkown
HY-RS22567	Idh3a Mouse Pre-designed siRNA Set A	Pre-designed Set	/	Unkown
HY-RS29086	Idh3a Rat Pre-designed siRNA Set A	Pre-designed Set	/	Unkown

Orthologs Information

Species	Symbol	Source	ID
Bos taurus	IDH3A	VGNC	VGNC:53942
Felis catus	IDH3A	VGNC	VGNC:104352
Mus musculus	IDH3A	MGD	MGI:1915084
Rattus norvegicus	IDH3A	RGD	RGD:70889
Canis familiaris	IDH3A	VGNC	VGNC:53184
Macaca mulatta	IDH3A	VGNC	VGNC:84352
Others	IDH3A	NCBI