IDH3G

Protein identityUniProt · HPA

UniProt accession

P51553

Gene

IDH3G

Ensembl

ENSG00000067829

Chromosome

X

Canonical length

393 aa

Protein class

Citric acid cycle related proteins, Metabolic proteins, Plasma proteins, Predicted intracellular proteins

Subcellular location

Nucleoli,Mitochondria

OverviewNCBI Gene

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]

Canonical amino-acid sequenceUniProt

393 residues, UniProt reviewed canonical sequence.

>P51553|IDH3G
     1  MALKVATVAG SAAKAVLGPA LLCRPWEVLG AHEVPSRNIF SEQTIPPSAK YGGRHTVTMI
    61  PGDGIGPELM LHVKSVFRHA CVPVDFEEVH VSSNADEEDI RNAIMAIRRN RVALKGNIET
   121  NHNLPPSHKS RNNILRTSLD LYANVIHCKS LPGVVTRHKD IDILIVRENT EGEYSSLEHE
   181  SVAGVVESLK IITKAKSLRI AEYAFKLAQE SGRKKVTAVH KANIMKLGDG LFLQCCREVA
   241  ARYPQITFEN MIVDNTTMQL VSRPQQFDVM VMPNLYGNIV NNVCAGLVGG PGLVAGANYG
   301  HVYAVFETAT RNTGKSIANK NIANPTATLL ASCMMLDHLK LHSYATSIRK AVLASMDNEN
   361  MHTPDIGGQG TTSEAIQDVI RHIRVINGRA VEA

LocalizationUniProt · AlphaFold · HPA

Whether an antibody against IDH3G can act on the native protein depends on physical access: surface and secreted proteins are reachable by circulating antibodies, intracellular proteins usually are not.

Antibody reachability

Intracellular

Secreted

No

Transmembrane segments

0

Mean surface accessibility (rSASA)

0.28

Highest tissue expression

105 nTPM

Expression across tissuesHPA

DiseaseUniProt · ClinVar · IEDB · PubMed

Four sources answering four different questions about IDH3G.

Disease | AllUniProt

Conditions IDH3G is implicated in, by any mechanism.

• Retinitis pigmentosa 99 (RP99) MIM:301148

Disease | GeneticClinVar

2 pathogenic / likely-pathogenic of 106 ClinVar records.

Conditions with pathogenic or likely-pathogenic variants.

• Retinitis pigmentosa 99

Disease | ImmuneIEDB

Conditions an epitope on IDH3G was assayed in.

• viral infectious disease T cell

Genetic constraint and essentialitygnomAD · DepMap

Does the body need this protein intact? Low LOEUF or a strong DepMap dependency means loss or blockade of the protein is likely to be felt.

gnomAD LOEUF (loss-of-function intolerance)

0.71

gnomAD pLI

0.09

gnomAD missense Z

2.19

DepMap mean gene effect

-0.06

DepMap dependency class

selective

Cancer expressionTCGA

Across TCGA tumor cohorts, this protein is over-expressed in roughly 7% of surveyed tumor types (aggregate summary; per-cohort expression, alteration, and survival load in the interactive view).

OntologyGO

Biological processes

• carbohydrate metabolic process
• isocitrate metabolic process
• tricarboxylic acid cycle

Molecular functions

• ATP binding
• isocitrate dehydrogenase (NAD+) activity
• magnesium ion binding
• NAD binding

Cellular components

• isocitrate dehydrogenase complex (NAD+)
• mitochondrial matrix
• mitochondrion
• nucleolus
• photoreceptor inner segment

Protein domainsUniProt · Pfam · InterPro

• Isocitrate dehydrogenase NAD-dependent
• Isocitrate/isopropylmalate dehydrogenase, conserved site
• Isopropylmalate dehydrogenase-like domain
• Isocitrate/isopropylmalate dehydrogenase

KeywordsUniProt

• ATP-binding
• Magnesium
• Manganese
• Metal-binding
• Mitochondrion
• Nucleotide-binding
• Retinitis pigmentosa
• Transit peptide
• Tricarboxylic acid cycle

InteractionsUniProt · HPA

Protein binding partners of IDH3G in the human serome: UniProt-annotated complex subunits plus reported interactors. Each links to its own Seroatlas record.

• IDH3A