2. Gene
  3. IDH3G - isocitrate dehydrogenase (NAD(+)) 3 non-catalytic subunit gamma Gene

IDH3G - isocitrate dehydrogenase (NAD(+)) 3 non-catalytic subunit gamma Gene

  • Gene
  • Protein
  • Disease
  • Agent
  • Ortholog
Homo sapiens

Also known as H-IDHG

Gene ID: 3421 | Gene type: protein coding

About IDH3G

Cytogenetic location: Xq28 Genomic coordinates (GRCh38): X:153,785,768-153,794,375 (from NCBI)

This gene has 11 transcripts (splice variants), 249 orthologues and 2 paralogues. Ubiquitous expression in kidney (RPKM 22.6), heart (RPKM 18.4) 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 gamma subunit of one isozyme of NAD(+)-dependent isocitrate dehydrogenase. This gene is a candidate gene for periventricular heterotopia. Several alternatively spliced transcript variants of this gene have been described, but only some of their full length natures have been determined. [provided by RefSeq, Jul 2008]

IDH3G Products(2)

mRNA Protein Name
NM_004135.4 NP_004126.1 isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial isoform a precursor
NM_174869.3 NP_777358.1 isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial isoform b precursor
Gene Ontology
  • Molecular Function
  • Biological Process
  • Cellular Component
Molecular Function GO Annotation Evidence Reference Source
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 Reference Source
involved in tricarboxylic acid cycle IDA
IDA: Inferred from direct assay
14555658 GOA
Cellular Component GO Annotation Evidence Reference 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

IDH3G Protein Structure

Iso_dh

Iso_dh: Isocitrate/isopropylmalate dehydrogenase (56 - 378)

  • 0
  • 100
  • 200
  • 300
  • 393 a.a.
Protein Preferred Names Protein Names

isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial

IDH-gamma

IDH3G Protein-protein interaction Information

Type
Protein Name Protein ID Interactor Interactor Species Interactor ID Detection Method Reference
Intra
IDH3G P51553 HSPD1 Homo sapiens P10809
Anti Tag CoIP
33961781
Intra
IDH3G P51553 HSPD1 Homo sapiens P10809
Anti Tag CoIP
28514442
Cross: Cross-species interaction Intra: Intraspecies interaction

Related Diseases

Diseases Alias
Juxtacortical Chondroma

Periosteal Chondroma
Intracranial Chondrosarcoma
D-2-Hydroxyglutaric Aciduria 2

D2HGA2

D-2-Hydroxyglutaric Aciduria, Type 2
Syndromic X-Linked Intellectual Disability Lubs Type

Lubs X-Linked Mental Retardation Syndrome

Mecp2 Duplication Syndrome

Mental Retardation, X-Linked, Syndromic, Lubs Type

Mental Retardation, X-Linked, With Recurrent Respiratory Infections

Mrxsl

X-Linked Intellectual Disability-Hypotonia-Recurrent Infections Syndrome

Mental Retardation, X-Linked Syndromic, Lubs Type
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
2-Hydroxyglutaric Aciduria

2-Hga

2-Hydroxyglutaric Acidemia

2-Hydroxyglutaricaciduria

Combined D-2- And L-2-Hydroxyglutaric Aciduria
Multiple Enchondromatosis, Maffucci Type

Maffucci Syndrome

Chondrodysplasia With Hemangioma

Chondroplasia Angiomatosis

Enchondromatosis With Hemangiomata

Hemangiomatosis Chondrodystrophica

Kast Syndrome

Multiple Angiomas And Endochondromas

Dyschondrodysplasia With Hemangiomas

Enchondromatosis Type Ii

Enchondromatosis With Multiple Cavernous Hemangiomas

Dyschondroplasia And Cavernous Hemangioma

Hemangiomata With Dyschondroplasia
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
Bioactive Molecules (1)
Cat. No. Product Name Effect
Purity Rare Diseases
HY-RS06535 IDH3G Human Pre-designed siRNA Set A Pre-designed Sets / Unkown

Orthologs Information

Species Symbol Source ID
Rattus norvegicus IDH3G RGD RGD:2863
Mus musculus IDH3G MGD MGI:1099463
Canis familiaris IDH3G VGNC VGNC:54831
Bos taurus IDH3G VGNC VGNC:54635