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P50213

- IDH3A_HUMAN

UniProt

P50213 - IDH3A_HUMAN

Protein

Isocitrate dehydrogenase [NAD] subunit alpha, mitochondrial

Gene

IDH3A

Organism
Homo sapiens (Human)
Status
Reviewed - Annotation score: 5 out of 5- Experimental evidence at protein leveli
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    • History
      Entry version 147 (01 Oct 2014)
      Sequence version 1 (01 Oct 1996)
      Previous versions | rss
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    Functioni

    Catalytic activityi

    Isocitrate + NAD+ = 2-oxoglutarate + CO2 + NADH.

    Cofactori

    Binds 1 magnesium or manganese ion per subunit.By similarity

    Sites

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Binding sitei115 – 1151SubstrateBy similarity
    Binding sitei125 – 1251SubstrateBy similarity
    Binding sitei146 – 1461SubstrateBy similarity
    Sitei153 – 1531Critical for catalysisBy similarity
    Sitei200 – 2001Critical for catalysisBy similarity
    Metal bindingi233 – 2331Magnesium or manganeseBy similarity
    Binding sitei233 – 2331SubstrateBy similarity
    Metal bindingi257 – 2571Magnesium or manganeseBy similarity
    Metal bindingi261 – 2611Magnesium or manganeseBy similarity

    GO - Molecular functioni

    1. isocitrate dehydrogenase (NAD+) activity Source: UniProtKB-EC
    2. magnesium ion binding Source: InterPro
    3. NAD binding Source: InterPro

    GO - Biological processi

    1. carbohydrate metabolic process Source: ProtInc
    2. cellular metabolic process Source: Reactome
    3. small molecule metabolic process Source: Reactome
    4. tricarboxylic acid cycle Source: Reactome

    Keywords - Molecular functioni

    Oxidoreductase

    Keywords - Biological processi

    Tricarboxylic acid cycle

    Keywords - Ligandi

    Magnesium, Manganese, Metal-binding, NAD

    Enzyme and pathway databases

    BioCyciMetaCyc:ENSG00000166411-MONOMER.
    ReactomeiREACT_1785. Citric acid cycle (TCA cycle).
    SABIO-RKP50213.

    Names & Taxonomyi

    Protein namesi
    Recommended 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 namesi
    Name:IDH3A
    OrganismiHomo sapiens (Human)
    Taxonomic identifieri9606 [NCBI]
    Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
    ProteomesiUP000005640: Chromosome 15

    Organism-specific databases

    HGNCiHGNC:5384. IDH3A.

    Subcellular locationi

    GO - Cellular componenti

    1. mitochondrial matrix Source: Reactome
    2. mitochondrion Source: HPA
    3. nucleus Source: UniProt

    Keywords - Cellular componenti

    Mitochondrion

    Pathology & Biotechi

    Organism-specific databases

    PharmGKBiPA29632.

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Transit peptidei1 – 2727MitochondrionBy similarityAdd
    BLAST
    Chaini28 – 366339Isocitrate dehydrogenase [NAD] subunit alpha, mitochondrialPRO_0000014436Add
    BLAST

    Amino acid modifications

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Modified residuei77 – 771N6-succinyllysineBy similarity
    Modified residuei223 – 2231N6-acetyllysineBy similarity
    Modified residuei343 – 3431N6-acetyllysine; alternate1 Publication
    Modified residuei343 – 3431N6-succinyllysine; alternateBy similarity
    Modified residuei350 – 3501N6-succinyllysineBy similarity

    Keywords - PTMi

    Acetylation

    Proteomic databases

    MaxQBiP50213.
    PaxDbiP50213.
    PRIDEiP50213.

    2D gel databases

    OGPiP50213.
    REPRODUCTION-2DPAGEIPI00030702.

    PTM databases

    PhosphoSiteiP50213.

    Expressioni

    Gene expression databases

    ArrayExpressiP50213.
    BgeeiP50213.
    CleanExiHS_IDH3A.
    GenevestigatoriP50213.

    Organism-specific databases

    HPAiHPA041465.

    Interactioni

    Subunit structurei

    Heterooligomer of subunits alpha, beta, and gamma in the apparent ratio of 2:1:1.By similarity

    Protein-protein interaction databases

    BioGridi109645. 17 interactions.
    IntActiP50213. 9 interactions.
    MINTiMINT-1148360.
    STRINGi9606.ENSP00000299518.

    Structurei

    3D structure databases

    ProteinModelPortaliP50213.
    SMRiP50213. Positions 4-362.
    ModBaseiSearch...
    MobiDBiSearch...

    Family & Domainsi

    Sequence similaritiesi

    Keywords - Domaini

    Transit peptide

    Phylogenomic databases

    eggNOGiCOG0473.
    HOGENOMiHOG000021113.
    HOVERGENiHBG052080.
    InParanoidiP50213.
    KOiK00030.
    OMAiTPIAWEP.
    OrthoDBiEOG75B85R.
    PhylomeDBiP50213.
    TreeFamiTF105692.

    Family and domain databases

    Gene3Di3.40.718.10. 1 hit.
    InterProiIPR019818. IsoCit/isopropylmalate_DH_CS.
    IPR001804. Isocitrate/isopropylmalate_DH.
    IPR004434. Isocitrate_DH_NAD.
    IPR024084. IsoPropMal-DH-like_dom.
    [Graphical view]
    PANTHERiPTHR11835. PTHR11835. 1 hit.
    PfamiPF00180. Iso_dh. 1 hit.
    [Graphical view]
    TIGRFAMsiTIGR00175. mito_nad_idh. 1 hit.
    PROSITEiPS00470. IDH_IMDH. 1 hit.
    [Graphical view]

    Sequences (2)i

    Sequence statusi: Complete.

    Sequence processingi: The displayed sequence is further processed into a mature form.

    This entry describes 2 isoformsi produced by alternative splicing. Align

    Isoform 1 (identifier: P50213-1) [UniParc]FASTAAdd to Basket

    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.

    « Hide

    MAGPAWISKV SRLLGAFHNP KQVTRGFTGG VQTVTLIPGD GIGPEISAAV    50
    MKIFDAAKAP IQWEERNVTA IQGPGGKWMI PSEAKESMDK NKMGLKGPLK 100
    TPIAAGHPSM NLLLRKTFDL YANVRPCVSI EGYKTPYTDV NIVTIRENTE 150
    GEYSGIEHVI VDGVVQSIKL ITEGASKRIA EFAFEYARNN HRSNVTAVHK 200
    ANIMRMSDGL FLQKCREVAE SCKDIKFNEM YLDTVCLNMV QDPSQFDVLV 250
    MPNLYGDILS DLCAGLIGGL GVTPSGNIGA NGVAIFESVH GTAPDIAGKD 300
    MANPTALLLS AVMMLRHMGL FDHAARIEAA CFATIKDGKS LTKDLGGNAK 350
    CSDFTEEICR RVKDLD 366
    Length:366
    Mass (Da):39,592
    Last modified:October 1, 1996 - v1
    Checksum:i695F6A34F97430CF
    GO
    Isoform 2 (identifier: P50213-2) [UniParc]FASTAAdd to Basket

    The sequence of this isoform differs from the canonical sequence as follows:
         1-78: Missing.

    Note: No experimental confirmation available.

    Show »
    Length:288
    Mass (Da):31,381
    Checksum:iBF8327BD4AA7D3EC
    GO

    Alternative sequence

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Alternative sequencei1 – 7878Missing in isoform 2. 1 PublicationVSP_014516Add
    BLAST

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    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.
    CCDSiCCDS10297.1. [P50213-1]
    PIRiS55282.
    RefSeqiNP_005521.1. NM_005530.2. [P50213-1]
    UniGeneiHs.591110.

    Genome annotation databases

    EnsembliENST00000299518; ENSP00000299518; ENSG00000166411. [P50213-1]
    GeneIDi3419.
    KEGGihsa:3419.
    UCSCiuc002bdd.3. human. [P50213-1]

    Polymorphism databases

    DMDMi1708399.

    Keywords - Coding sequence diversityi

    Alternative splicing

    Cross-referencesi

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    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 .
    CCDSi CCDS10297.1. [P50213-1 ]
    PIRi S55282.
    RefSeqi NP_005521.1. NM_005530.2. [P50213-1 ]
    UniGenei Hs.591110.

    3D structure databases

    ProteinModelPortali P50213.
    SMRi P50213. Positions 4-362.
    ModBasei Search...
    MobiDBi Search...

    Protein-protein interaction databases

    BioGridi 109645. 17 interactions.
    IntActi P50213. 9 interactions.
    MINTi MINT-1148360.
    STRINGi 9606.ENSP00000299518.

    Chemistry

    DrugBanki DB00157. NADH.

    PTM databases

    PhosphoSitei P50213.

    Polymorphism databases

    DMDMi 1708399.

    2D gel databases

    OGPi P50213.
    REPRODUCTION-2DPAGE IPI00030702.

    Proteomic databases

    MaxQBi P50213.
    PaxDbi P50213.
    PRIDEi P50213.

    Protocols and materials databases

    DNASUi 3419.
    Structural Biology Knowledgebase Search...

    Genome annotation databases

    Ensembli ENST00000299518 ; ENSP00000299518 ; ENSG00000166411 . [P50213-1 ]
    GeneIDi 3419.
    KEGGi hsa:3419.
    UCSCi uc002bdd.3. human. [P50213-1 ]

    Organism-specific databases

    CTDi 3419.
    GeneCardsi GC15P078423.
    HGNCi HGNC:5384. IDH3A.
    HPAi HPA041465.
    MIMi 601149. gene.
    neXtProti NX_P50213.
    PharmGKBi PA29632.
    GenAtlasi Search...

    Phylogenomic databases

    eggNOGi COG0473.
    HOGENOMi HOG000021113.
    HOVERGENi HBG052080.
    InParanoidi P50213.
    KOi K00030.
    OMAi TPIAWEP.
    OrthoDBi EOG75B85R.
    PhylomeDBi P50213.
    TreeFami TF105692.

    Enzyme and pathway databases

    BioCyci MetaCyc:ENSG00000166411-MONOMER.
    Reactomei REACT_1785. Citric acid cycle (TCA cycle).
    SABIO-RK P50213.

    Miscellaneous databases

    GeneWikii IDH3A.
    GenomeRNAii 3419.
    NextBioi 13478.
    PROi P50213.
    SOURCEi Search...

    Gene expression databases

    ArrayExpressi P50213.
    Bgeei P50213.
    CleanExi HS_IDH3A.
    Genevestigatori P50213.

    Family and domain databases

    Gene3Di 3.40.718.10. 1 hit.
    InterProi IPR019818. IsoCit/isopropylmalate_DH_CS.
    IPR001804. Isocitrate/isopropylmalate_DH.
    IPR004434. Isocitrate_DH_NAD.
    IPR024084. IsoPropMal-DH-like_dom.
    [Graphical view ]
    PANTHERi PTHR11835. PTHR11835. 1 hit.
    Pfami PF00180. Iso_dh. 1 hit.
    [Graphical view ]
    TIGRFAMsi TIGR00175. mito_nad_idh. 1 hit.
    PROSITEi PS00470. IDH_IMDH. 1 hit.
    [Graphical view ]
    ProtoNeti Search...

    Publicationsi

    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. Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 2).
      Tissue: Brain cortex.
    3. 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. Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].

    Entry informationi

    Entry nameiIDH3A_HUMAN
    AccessioniPrimary (citable) accession number: P50213
    Secondary accession number(s): D3DW83, Q9H3X0
    Entry historyi
    Integrated into UniProtKB/Swiss-Prot: October 1, 1996
    Last sequence update: October 1, 1996
    Last modified: October 1, 2014
    This is version 147 of the entry and version 1 of the sequence. [Complete history]
    Entry statusiReviewed (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.

    Miscellaneousi

    Keywords - Technical termi

    Complete proteome, Direct protein sequencing, Reference proteome

    Documents

    1. Human chromosome 15
      Human chromosome 15: entries, gene names and cross-references to MIM
    2. MIM cross-references
      Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
    3. SIMILARITY comments
      Index of protein domains and families

    External Data

    Dasty 3