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Reviewed, UniProtKB/Swiss-Prot P50213 (IDH3A_HUMAN)

Last modified June 16, 2009. Version 91. Feed History...

Clusters with 100%, 90%, 50% identity | Documents (3) | Third-party data | Customize display text xml rdf/xml gff fasta
Names and origin · Protein attributes · General annotation (Comments) · Ontologies · Alternative products · Sequence annotation (Features) · Sequences · References · Cross-references · Entry information · Relevant documents

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Names and origin

Protein namesRecommended name:
    Isocitrate dehydrogenase [NAD] subunit alpha, mitochondrial
    EC=1.1.1.41
Alternative name(s):
    Isocitric dehydrogenase
    NAD(+)-specific ICDH
Gene names
Name: IDH3A
OrganismHomo sapiens (Human)
Taxonomic identifier9606 [NCBI]
Taxonomic lineageEukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo

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Protein attributes

Sequence length366 AA.
Sequence statusComplete.
Sequence processingThe displayed sequence is further processed into a mature form.
Protein existenceEvidence at protein level.

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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

Mitochondrion.

Sequence similarities

Belongs to the isocitrate and isopropylmalate dehydrogenases family.

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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.

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Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Transit peptide1 – 2727Mitochondrion By similarity
Chain28 – 366339Isocitrate dehydrogenase [NAD] subunit alpha, mitochondrial
PRO_0000014436

Sites

Metal binding2331Magnesium or manganese By similarity
Metal binding2571Magnesium or manganese By similarity
Metal binding2611Magnesium or manganese By similarity
Binding site1151Substrate By similarity
Binding site1251Substrate By similarity
Binding site1461Substrate By similarity
Binding site2331Substrate By similarity
Site1531Critical for catalysis By similarity
Site2001Critical for catalysis By similarity

Natural variations

Alternative sequence1 – 7878Missing in isoform 2.
VSP_014516

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Sequences

Sequence LengthMass (Da)Tools
Isoform 1 [UniParc].

Last modified October 1, 1996. Version 1.
Checksum: 695F6A34F97430CF

FASTA36639,592
        10         20         30         40         50         60 
MAGPAWISKV SRLLGAFHNP KQVTRGFTGG VQTVTLIPGD GIGPEISAAV MKIFDAAKAP 

        70         80         90        100        110        120 
IQWEERNVTA IQGPGGKWMI PSEAKESMDK NKMGLKGPLK TPIAAGHPSM NLLLRKTFDL 

       130        140        150        160        170        180 
YANVRPCVSI EGYKTPYTDV NIVTIRENTE GEYSGIEHVI VDGVVQSIKL ITEGASKRIA 

       190        200        210        220        230        240 
EFAFEYARNN HRSNVTAVHK ANIMRMSDGL FLQKCREVAE SCKDIKFNEM YLDTVCLNMV 

       250        260        270        280        290        300 
QDPSQFDVLV MPNLYGDILS DLCAGLIGGL GVTPSGNIGA NGVAIFESVH GTAPDIAGKD 

       310        320        330        340        350        360 
MANPTALLLS AVMMLRHMGL FDHAARIEAA CFATIKDGKS LTKDLGGNAK CSDFTEEICR 


RVKDLD

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Isoform 2.

Checksum: BF8327BD4AA7D3EC
Show »

FASTA28831,381

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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.

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Cross-references

Sequence databases

U07681 mRNA. Translation: AAA85639.1.
AL442090 mRNA. Translation: CAC09449.1.
BC021967 mRNA. Translation: AAH21967.1.
IPIIPI00030702.
IPI00792971.
PIRS55282.
RefSeqNP_005521.1.
UniGeneHs.591110

3D structure databases

HSSPHSSP built from PDB template 1ISO based on UniProtKB P08200.
ModBaseSearch...

Protein-protein interaction databases

IntActP50213. 6 interactions.

PTM databases

PhosphoSiteP50213.

2-D gel databases

OGPP50213.
REPRODUCTION-2DPAGEIPI00030702.

Proteomic databases

PRIDEP50213.

Genome annotation databases

EnsemblENSG00000166411. Homo sapiens. [Contig view]
GeneID3419.
KEGGhsa:3419.
NMPDRfig|9606.3.peg.11057.

Organism-specific databases

GeneCardsGC15P076228.
HGNCHGNC:5384. IDH3A.
MIM601149. gene.
PharmGKBPA29632.
GenAtlasSearch...

Phylogenomic databases

HOGENOMP50213.
HOVERGENP50213.
OMAP50213. GGNSKCS.

Enzyme and pathway databases

BRENDA1.1.1.41. 247.

Gene expression databases

ArrayExpressP50213.
BgeeP50213.
CleanExHS_IDH3A.
GermOnlineENSG00000166411. Homo sapiens.

Family and domain databases

InterProIPR019818. IsoCit/isopropylmalate_DH_CS.
IPR001804. Isocitrate/isopropylmalate_DH.
IPR004434. Isocitrate_DH_NAD_mit.
[Graphical view]
Gene3DG3DSA:3.40.718.10. IDH_IMDH. 1 hit.
PANTHERPTHR11835. IDH_IMDH_dimeric. 1 hit.
PfamPF00180. Iso_dh. 1 hit.
[Graphical view]
TIGRFAMsTIGR00175. mito_nad_idh. 1 hit.
PROSITEPS00470. IDH_IMDH. 1 hit.
[Graphical view]
ProtoNetSearch...

Other Resources

DrugBankDB00157. NADH.
NextBio13478.
SOURCESearch...

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Entry information

Entry nameIDH3A_HUMAN
AccessionPrimary (citable) accession number: P50213
Secondary accession number(s): Q9H3X0
Entry history
Integrated into UniProtKB/Swiss-Prot: October 1, 1996
Last sequence update: October 1, 1996
Last modified: June 16, 2009
This is version 91 of the entry and version 1 of the sequence. [Complete history]
Entry statusReviewed (UniProtKB/Swiss-Prot)
Annotation projectHPI (Human Proteome Initiative)

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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

Names and origin · Protein attributes · General annotation (Comments) · Ontologies · Alternative products · Sequence annotation (Features) · Sequences · References · Cross-references · Entry information · Relevant documents