IDH3A Gene Summary [Human]
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]
Details
| Type | Processed Transcript |
| Official Symbol | IDH3A |
| Official Name | isocitrate dehydrogenase (NAD(+)) 3 catalytic subunit alpha [Source:HGNC Symbol;Acc:HGNC:5384] |
| Ensembl ID | ENSG00000166411 |
| Bio databases IDs | |
| Aliases | isocitrate dehydrogenase (NAD(+)) 3 catalytic subunit alpha, H-IDH alpha, isocitric dehydrogenase, isocitrate dehydrogenase [NAD] subunit alpha, mitochondrial, NAD+-specific ICDH, NAD(H)-specific isocitrate dehydrogenase alpha subunit, isocitrate dehydrogenase (NAD+) alpha chain |
| Synonyms | 1110003P10Rik, 1500012E04Rik, isocitrate dehydrogenase 3 (NAD+) alpha, isocitrate dehydrogenase 3 (NAD+) α, isocitrate dehydrogenase (NAD(+)) 3 catalytic subunit alpha, isocitrate dehydrogenase (NAD(+)) 3 catalytic subunit α, Isocitrate/isopropylmalate Dehydrogenase, RP90 |
| Species | Human, Homo sapiens |
| Orthologies |
Protein Domains
A protein domain is a distinct structural or functional region within a protein that can evolve, function, and exist independently of the rest of the protein chain. These domains in human IDH3A often fold into stable, three-dimensional structures and are associated with specific biological functions, such as binding to DNA, other proteins, or small molecules.
- Isocitrate/isopropylmalate dehydrogenase
- tartrate dehydrogenase
- isocitrate dehydrogenase (NAD)
- enzyme
- protein binding
- magnesium ion binding
Top Findings
The most significant associations for this gene, including commonly observed domains, pathway involvement, and functional highlights based on current data.
disease
- neoplasia
- retinitis pigmentosa type 90
- Huntington disease
- psoriasis
- breast cancer
- Alzheimer disease
- retinitis pigmentosa
- retinal dystrophy
- osteoporosis
regulates
- HIF1A
- alpha-hydroxyglutarate
- 2-oxoglutaric acid
role in cell
- accumulation in
- glycolysis in
Subcellular Expression
Locations within the cell where the protein is known or predicted to be active, providing insight into its function and cellular context.
- Cytoplasm
- myelin enriched fraction
- Mitochondria
- cytosol
- mitochondrial matrix
Gene Ontology Annotations
Describes the biological processes, cellular components, and molecular functions associated with the human IDH3A gene, providing context for its role in the cell.
Biological Process
Functions and activities the gene product is involved in
- carbohydrate metabolic process
- isocitrate metabolic process
- tricarboxylic acid cycle
Cellular Component
Where in the cell the gene product is active
- nucleus
- mitochondrial matrix
- mitochondrion
Molecular Function
What the gene product does at the molecular level
- magnesium ion binding
- isocitrate dehydrogenase (NAD+) activity
- protein binding
- NAD binding