Reviewed,
UniProtKB/Swiss-Prot P50213 (IDH3A_HUMAN)
Last modified
June 16, 2009.
Version 91.
History...
Clusters with 100%,
90%,
50% identity |
Documents (3) |
Third-party data |
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Names and origin
| Protein names | Recommended name: Isocitrate dehydrogenase [NAD] subunit alpha, mitochondrial EC=1.1.1.41 Alternative name(s): Isocitric dehydrogenase NAD(+)-specific ICDH | ||
| Gene names |
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| Organism | Homo sapiens (Human) | ||
| Taxonomic identifier | 9606 [NCBI] | ||
| Taxonomic lineage | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo |
Protein attributes
| Sequence length | 366 AA. |
| Sequence status | Complete. |
| Sequence processing | The displayed sequence is further processed into a mature form. |
| Protein existence | Evidence at protein level. |
General annotation (Comments)
| Catalytic activity | Isocitrate + NAD+ = 2-oxoglutarate + CO2 + NADH. |
| Cofactor | Binds 1 magnesium or manganese ion per subunit By similarity. |
| Subunit structure | Heterooligomer of subunits alpha, beta, and gamma in the apparent ratio of 2:1:1 By similarity. |
| Subcellular location | |
| Sequence similarities | Belongs to the isocitrate and isopropylmalate dehydrogenases family. |
Ontologies
| Keywords | |
|---|---|
| Biological process | Tricarboxylic acid cycle |
| Cellular component | Mitochondrion |
| Coding sequence diversity | Alternative splicing |
| Domain | Transit peptide |
| Ligand | Magnesium Manganese Metal-binding NAD |
| Molecular function | Oxidoreductase |
| Technical term | Direct protein sequencing |
| Gene Ontology (GO) | |
| Biological process | carbohydrate metabolic process Non-traceable author statement. Source: ProtInc oxidation reductionInferred from electronic annotation. Source: UniProtKB-KW tricarboxylic acid cycleInferred from electronic annotation. Source: UniProtKB-KW |
| Cellular component | mitochondrial matrix Inferred from Experiment. Source: Reactome |
| Molecular function | NAD or NADH binding Inferred from electronic annotation. Source: InterPro isocitrate dehydrogenase (NAD+) activityInferred from electronic annotation. Source: EC magnesium ion bindingInferred from electronic annotation. Source: UniProtKB-KW manganese ion bindingInferred from electronic annotation. Source: UniProtKB-KW |
| Complete GO annotation... | |
Alternative products
| This entry describes 2 isoforms produced by alternative splicing. [Align] [Select] | ||||||
| Isoform 1 (identifier: P50213-1) This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry. | ||||||
| Isoform 2 (identifier: P50213-2) The sequence of this isoform differs from the canonical sequence as follows: 1-78: Missing. | ||||||
| Note: No experimental confirmation available. |
Sequence annotation (Features)
| Feature key | Position(s) | Length | Description | Graphical view | Feature identifier | ||||
Molecule processing | |||||||||
|---|---|---|---|---|---|---|---|---|---|
| Transit peptide | 1 – 27 | 27 | Mitochondrion By similarity | ||||||
| Chain | 28 – 366 | 339 | Isocitrate dehydrogenase [NAD] subunit alpha, mitochondrial | PRO_0000014436 | |||||
Sites | |||||||||
| Metal binding | 233 | 1 | Magnesium or manganese By similarity | ||||||
| Metal binding | 257 | 1 | Magnesium or manganese By similarity | ||||||
| Metal binding | 261 | 1 | Magnesium or manganese By similarity | ||||||
| Binding site | 115 | 1 | Substrate By similarity | ||||||
| Binding site | 125 | 1 | Substrate By similarity | ||||||
| Binding site | 146 | 1 | Substrate By similarity | ||||||
| Binding site | 233 | 1 | Substrate By similarity | ||||||
| Site | 153 | 1 | Critical for catalysis By similarity | ||||||
| Site | 200 | 1 | Critical for catalysis By similarity | ||||||
Natural variations | |||||||||
| Alternative sequence | 1 – 78 | 78 | Missing in isoform 2. | VSP_014516 | |||||
Sequences
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References
| « Hide 'large scale' references | |
| [1] | "Characterization of a cDNA clone for human NAD(+)-specific isocitrate dehydrogenase alpha-subunit and structural comparison with its isoenzymes from different species." Kim Y.O., Oh I.U., Park H.S., Jeng J., Song B.J., Huh T.L. Biochem. J. 308:63-68(1995) [PubMed: 7755589] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1). Tissue: Heart. |
| [2] | "The full-ORF clone resource of the German cDNA consortium." Bechtel S., Rosenfelder H., Duda A., Schmidt C.P., Ernst U., Wellenreuther R., Mehrle A., Schuster C., Bahr A., Blocker H., Heubner D., Hoerlein A., Michel G., Wedler H., Kohrer K., Ottenwalder B., Poustka A., Wiemann S., Schupp I. BMC Genomics 8:399-399(2007) [PubMed: 17974005] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 2). Tissue: Brain cortex. |
| [3] | "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)." The MGC Project Team Genome Res. 14:2121-2127(2004) [PubMed: 15489334] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 1). Tissue: Lymph. |
| [4] | Lubec G., Vishwanath V., Chen W.-Q., Sun Y. Submitted (DEC-2008) to UniProtKB Cited for: PROTEIN SEQUENCE OF 135-146; 179-188 AND 300-316, MASS SPECTROMETRY. Tissue: Brain, Cajal-Retzius cell and Fetal brain cortex. |
| [5] | Colinge J., Superti-Furga G., Bennett K.L. Submitted (OCT-2008) to UniProtKB Cited for: IDENTIFICATION [LARGE SCALE ANALYSIS], MASS SPECTROMETRY. |
| + | Additional computationally mapped references. |
Cross-references
Sequence databases | |
|---|---|
| U07681 mRNA. Translation: AAA85639.1. AL442090 mRNA. Translation: CAC09449.1. BC021967 mRNA. Translation: AAH21967.1. | |
| IPI | IPI00030702. IPI00792971. |
| PIR | S55282. |
| RefSeq | NP_005521.1. |
| UniGene | Hs.591110 |
3D structure databases | |
| HSSP | HSSP built from PDB template 1ISO based on UniProtKB P08200. |
| ModBase | Search... |
Protein-protein interaction databases | |
| IntAct | P50213. 6 interactions. |
PTM databases | |
| PhosphoSite | P50213. |
2-D gel databases | |
| OGP | P50213. |
| REPRODUCTION-2DPAGE | IPI00030702. |
Proteomic databases | |
| PRIDE | P50213. |
Genome annotation databases | |
| Ensembl | ENSG00000166411. Homo sapiens. [Contig view] |
| GeneID | 3419. |
| KEGG | hsa:3419. |
| NMPDR | fig|9606.3.peg.11057. |
Organism-specific databases | |
| GeneCards | GC15P076228. |
| HGNC | HGNC:5384. IDH3A. |
| MIM | 601149. gene. |
| PharmGKB | PA29632. |
| GenAtlas | Search... |
Phylogenomic databases | |
| HOGENOM | P50213. |
| HOVERGEN | P50213. |
| OMA | P50213. GGNSKCS. |
Enzyme and pathway databases | |
| BRENDA | 1.1.1.41. 247. |
Gene expression databases | |
| ArrayExpress | P50213. |
| Bgee | P50213. |
| CleanEx | HS_IDH3A. |
| GermOnline | ENSG00000166411. Homo sapiens. |
Family and domain databases | |
| InterPro | IPR019818. IsoCit/isopropylmalate_DH_CS. IPR001804. Isocitrate/isopropylmalate_DH. IPR004434. Isocitrate_DH_NAD_mit. [Graphical view] |
| Gene3D | G3DSA:3.40.718.10. IDH_IMDH. 1 hit. |
| PANTHER | PTHR11835. IDH_IMDH_dimeric. 1 hit. |
| Pfam | PF00180. Iso_dh. 1 hit. [Graphical view] |
| TIGRFAMs | TIGR00175. mito_nad_idh. 1 hit. |
| PROSITE | PS00470. IDH_IMDH. 1 hit. [Graphical view] |
| ProtoNet | Search... |
Other Resources | |
| DrugBank | DB00157. NADH. |
| NextBio | 13478. |
| SOURCE | Search... |
Entry information
| Entry name | IDH3A_HUMAN | ||||||||
| Accession | Primary (citable) accession number: P50213 Secondary accession number(s): Q9H3X0 | ||||||||
| Entry history |
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| Entry status | Reviewed (UniProtKB/Swiss-Prot) | ||||||||
| Annotation project | HPI (Human Proteome Initiative) | ||||||||
Relevant documents
| Human chromosome 15 Human chromosome 15: entries, gene names and cross-references to MIM |
| MIM cross-references Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot |
| SIMILARITY comments Index of protein domains and families |

Clusters with


