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P50213 (IDH3A_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Last modified April 16, 2014. Version 142. Feed History...

Clusters with 100%, 90%, 50% identity | Documents (3) | Third-party data text xml rdf/xml gff fasta
to top of pageNames·Attributes·General annotation·Ontologies·Alt products·Sequence annotation·Sequences·References·Cross-refs·Entry info·DocumentsCustomize order

Names and origin

Protein namesRecommended name:
Isocitrate dehydrogenase [NAD] subunit alpha, mitochondrial

EC=1.1.1.41
Alternative name(s):
Isocitric dehydrogenase subunit alpha
NAD(+)-specific ICDH subunit alpha
Gene names
Name:IDH3A
OrganismHomo sapiens (Human) [Reference proteome]
Taxonomic identifier9606 [NCBI]
Taxonomic lineageEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo

Protein attributes

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

Mitochondrion.

Sequence similarities

Belongs to the isocitrate and isopropylmalate dehydrogenases family.

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 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

Amino acid modifications

Modified residue771N6-succinyllysine By similarity
Modified residue2231N6-acetyllysine By similarity
Modified residue3431N6-acetyllysine; alternate Ref.6
Modified residue3431N6-succinyllysine; alternate By similarity
Modified residue3501N6-succinyllysine By similarity

Natural variations

Alternative sequence1 – 7878Missing in isoform 2.
VSP_014516

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 

« Hide

Isoform 2 [UniParc].

Checksum: BF8327BD4AA7D3EC
Show »

FASTA28831,381

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] [Europe PMC] [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., Bloecker H., Heubner D., Hoerlein A., Michel G., Wedler H., Koehrer K., Ottenwaelder B., Poustka A., Wiemann S., Schupp I.
BMC Genomics 8:399-399(2007) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 2).
Tissue: Brain cortex.
[3]Mural R.J., Istrail S., Sutton G.G., Florea L., Halpern A.L., Mobarry C.M., Lippert R., Walenz B., Shatkay H., Dew I., Miller J.R., Flanigan M.J., Edwards N.J., Bolanos R., Fasulo D., Halldorsson B.V., Hannenhalli S., Turner R. expand/collapse author list , Yooseph S., Lu F., Nusskern D.R., Shue B.C., Zheng X.H., Zhong F., Delcher A.L., Huson D.H., Kravitz S.A., Mouchard L., Reinert K., Remington K.A., Clark A.G., Waterman M.S., Eichler E.E., Adams M.D., Hunkapiller M.W., Myers E.W., Venter J.C.
Submitted (SEP-2005) to the EMBL/GenBank/DDBJ databases
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
[4]"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] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 1).
Tissue: Lymph.
[5]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, IDENTIFICATION BY MASS SPECTROMETRY.
Tissue: Brain, Cajal-Retzius cell and Fetal brain cortex.
[6]"Lysine acetylation targets protein complexes and co-regulates major cellular functions."
Choudhary C., Kumar C., Gnad F., Nielsen M.L., Rehman M., Walther T.C., Olsen J.V., Mann M.
Science 325:834-840(2009) [PubMed] [Europe PMC] [Abstract]
Cited for: ACETYLATION [LARGE SCALE ANALYSIS] AT LYS-343, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
[7]"Initial characterization of the human central proteome."
Burkard T.R., Planyavsky M., Kaupe I., Breitwieser F.P., Buerckstuemmer T., Bennett K.L., Superti-Furga G., Colinge J.
BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract]
Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
+Additional computationally mapped references.

Cross-references

Sequence databases

EMBL
GenBank
DDBJ
U07681 mRNA. Translation: AAA85639.1.
AL442090 mRNA. Translation: CAC09449.1.
CH471136 Genomic DNA. Translation: EAW99181.1.
CH471136 Genomic DNA. Translation: EAW99182.1.
BC021967 mRNA. Translation: AAH21967.1.
PIRS55282.
RefSeqNP_005521.1. NM_005530.2.
UniGeneHs.591110.

3D structure databases

ProteinModelPortalP50213.
SMRP50213. Positions 4-362.
ModBaseSearch...
MobiDBSearch...

Protein-protein interaction databases

BioGrid109645. 16 interactions.
IntActP50213. 9 interactions.
MINTMINT-1148360.
STRING9606.ENSP00000299518.

Chemistry

DrugBankDB00157. NADH.

PTM databases

PhosphoSiteP50213.

Polymorphism databases

DMDM1708399.

2D gel databases

OGPP50213.
REPRODUCTION-2DPAGEIPI00030702.

Proteomic databases

PaxDbP50213.
PRIDEP50213.

Protocols and materials databases

DNASU3419.
StructuralBiologyKnowledgebaseSearch...

Genome annotation databases

EnsemblENST00000299518; ENSP00000299518; ENSG00000166411. [P50213-1]
GeneID3419.
KEGGhsa:3419.
UCSCuc002bdd.3. human. [P50213-1]

Organism-specific databases

CTD3419.
GeneCardsGC15P078423.
HGNCHGNC:5384. IDH3A.
HPAHPA041465.
MIM601149. gene.
neXtProtNX_P50213.
PharmGKBPA29632.
GenAtlasSearch...

Phylogenomic databases

eggNOGCOG0473.
HOGENOMHOG000021113.
HOVERGENHBG052080.
InParanoidP50213.
KOK00030.
OMAEGMYSGL.
OrthoDBEOG75B85R.
PhylomeDBP50213.
TreeFamTF105692.

Enzyme and pathway databases

BioCycMetaCyc:ENSG00000166411-MONOMER.
ReactomeREACT_111217. Metabolism.
SABIO-RKP50213.

Gene expression databases

ArrayExpressP50213.
BgeeP50213.
CleanExHS_IDH3A.
GenevestigatorP50213.

Family and domain databases

Gene3D3.40.718.10. 1 hit.
InterProIPR019818. IsoCit/isopropylmalate_DH_CS.
IPR001804. Isocitrate/isopropylmalate_DH.
IPR004434. Isocitrate_DH_NAD.
IPR024084. IsoPropMal-DH-like_dom.
[Graphical view]
PANTHERPTHR11835. PTHR11835. 1 hit.
PfamPF00180. Iso_dh. 1 hit.
[Graphical view]
TIGRFAMsTIGR00175. mito_nad_idh. 1 hit.
PROSITEPS00470. IDH_IMDH. 1 hit.
[Graphical view]
ProtoNetSearch...

Other

GeneWikiIDH3A.
GenomeRNAi3419.
NextBio13478.
PROP50213.
SOURCESearch...

Entry information

Entry nameIDH3A_HUMAN
AccessionPrimary (citable) accession number: P50213
Secondary accession number(s): D3DW83, Q9H3X0
Entry history
Integrated into UniProtKB/Swiss-Prot: October 1, 1996
Last sequence update: October 1, 1996
Last modified: April 16, 2014
This is version 142 of the entry and version 1 of the sequence. [Complete history]
Entry statusReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Relevant documents

SIMILARITY comments

Index of protein domains and families

MIM cross-references

Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot

Human chromosome 15

Human chromosome 15: entries, gene names and cross-references to MIM