IDH3A

Protein identityUniProt · HPA

UniProt accession

P50213

Gene

IDH3A

Ensembl

ENSG00000166411

Chromosome

15

Canonical length

366 aa

Protein class

Citric acid cycle related proteins, Disease related genes, Enzymes, FDA approved drug targets, Human disease related genes, Metabolic proteins, Plasma proteins, Predicted intracellular proteins

Subcellular location

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 alpha subunit of one isozyme of NAD(+)-dependent isocitrate dehydrogenase. [provided by RefSeq, Jul 2008]

Canonical amino-acid sequenceUniProt

366 residues, UniProt reviewed canonical sequence.

>P50213|IDH3A
     1  MAGPAWISKV SRLLGAFHNP KQVTRGFTGG VQTVTLIPGD GIGPEISAAV MKIFDAAKAP
    61  IQWEERNVTA IQGPGGKWMI PSEAKESMDK NKMGLKGPLK TPIAAGHPSM NLLLRKTFDL
   121  YANVRPCVSI EGYKTPYTDV NIVTIRENTE GEYSGIEHVI VDGVVQSIKL ITEGASKRIA
   181  EFAFEYARNN HRSNVTAVHK ANIMRMSDGL FLQKCREVAE SCKDIKFNEM YLDTVCLNMV
   241  QDPSQFDVLV MPNLYGDILS DLCAGLIGGL GVTPSGNIGA NGVAIFESVH GTAPDIAGKD
   301  MANPTALLLS AVMMLRHMGL FDHAARIEAA CFATIKDGKS LTKDLGGNAK CSDFTEEICR
   361  RVKDLD

LocalizationUniProt · AlphaFold · HPA

Whether an antibody against IDH3A 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

93 nTPM

Expression across tissuesHPA

DiseaseUniProt · ClinVar · IEDB · PubMed

Four sources answering four different questions about IDH3A.

Disease | AllUniProt

Conditions IDH3A is implicated in, by any mechanism.

• Retinitis pigmentosa 90 (RP90) MIM:619007

Disease | GeneticClinVar

21 pathogenic / likely-pathogenic of 312 ClinVar records.

Conditions with pathogenic or likely-pathogenic variants.

• Retinitis pigmentosa 90
• Ovarian serous cystadenocarcinoma
• Retinal dystrophy

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)

0.55

gnomAD pLI

0.16

gnomAD missense Z

2.59

DepMap mean gene effect

-0.47

DepMap dependency class

selective

Cancer expressionTCGA

Across TCGA tumor cohorts, this protein is over-expressed in roughly 5% 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

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

Cellular components

• isocitrate dehydrogenase complex (NAD+)
• mitochondrial matrix
• mitochondrion
• nucleus

Protein domainsUniProt · Pfam · InterPro

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

KeywordsUniProt

• Acetylation
• Magnesium
• Manganese
• Metal-binding
• Mitochondrion
• NAD
• Oxidoreductase
• Phosphoprotein
• Retinitis pigmentosa
• Transit peptide
• Tricarboxylic acid cycle

InteractionsUniProt · HPA

Protein binding partners of IDH3A in the human serome: UniProt-annotated complex subunits plus reported interactors. Each links to its own Seroatlas record.

• IDH3G