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P51553

- IDH3G_HUMAN

UniProt

P51553 - IDH3G_HUMAN

Protein

Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial

Gene

IDH3G

Organism
Homo sapiens (Human)
Status
Reviewed - Annotation score: 5 out of 5- Experimental evidence at protein leveli
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    • History
      Entry version 155 (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

    Enzyme regulationi

    Activated by increasing ADP/ATP ratios and by Ca2+.By similarity

    Sites

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Binding sitei136 – 1361SubstrateBy similarity
    Binding sitei167 – 1671SubstrateBy similarity
    Sitei174 – 1741Critical for catalysisBy similarity
    Sitei221 – 2211Critical for catalysisBy similarity
    Metal bindingi254 – 2541Magnesium or manganeseBy similarity
    Binding sitei254 – 2541SubstrateBy similarity

    Regions

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Nucleotide bindingi56 – 8429NADSequence AnalysisAdd
    BLAST
    Nucleotide bindingi309 – 3168ATPSequence Analysis

    GO - Molecular functioni

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

    GO - Biological processi

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

    Keywords - Molecular functioni

    Oxidoreductase

    Keywords - Biological processi

    Tricarboxylic acid cycle

    Keywords - Ligandi

    ATP-binding, Magnesium, Manganese, Metal-binding, NAD, Nucleotide-binding

    Enzyme and pathway databases

    BioCyciMetaCyc:ENSG00000067829-MONOMER.
    ReactomeiREACT_118595. Mitochondrial protein import.
    REACT_1785. Citric acid cycle (TCA cycle).
    SABIO-RKP51553.

    Names & Taxonomyi

    Protein namesi
    Recommended name:
    Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial (EC:1.1.1.41)
    Alternative name(s):
    Isocitric dehydrogenase subunit gamma
    NAD(+)-specific ICDH subunit gamma
    Gene namesi
    Name:IDH3G
    OrganismiHomo sapiens (Human)
    Taxonomic identifieri9606 [NCBI]
    Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
    ProteomesiUP000005640: Chromosome X

    Organism-specific databases

    HGNCiHGNC:5386. IDH3G.

    Subcellular locationi

    Mitochondrion 1 Publication

    GO - Cellular componenti

    1. mitochondrial matrix Source: Reactome
    2. mitochondrion Source: UniProtKB
    3. nucleolus Source: HPA
    4. nucleus Source: HPA

    Keywords - Cellular componenti

    Mitochondrion

    Pathology & Biotechi

    Organism-specific databases

    PharmGKBiPA29634.

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Transit peptidei1 – 3939MitochondrionBy similarityAdd
    BLAST
    Chaini40 – 393354Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrialPRO_0000014449Add
    BLAST

    Amino acid modifications

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Modified residuei206 – 2061N6-acetyllysineBy similarity
    Modified residuei226 – 2261N6-succinyllysineBy similarity

    Keywords - PTMi

    Acetylation

    Proteomic databases

    MaxQBiP51553.
    PaxDbiP51553.
    PRIDEiP51553.

    PTM databases

    PhosphoSiteiP51553.

    Expressioni

    Gene expression databases

    ArrayExpressiP51553.
    BgeeiP51553.
    CleanExiHS_IDH3G.
    GenevestigatoriP51553.

    Organism-specific databases

    HPAiHPA000425.
    HPA002017.

    Interactioni

    Subunit structurei

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

    Protein-protein interaction databases

    BioGridi109647. 15 interactions.
    IntActiP51553. 2 interactions.
    MINTiMINT-3018829.
    STRINGi9606.ENSP00000217901.

    Structurei

    3D structure databases

    ProteinModelPortaliP51553.
    SMRiP51553. Positions 50-386.
    ModBaseiSearch...
    MobiDBiSearch...

    Family & Domainsi

    Sequence similaritiesi

    Keywords - Domaini

    Transit peptide

    Phylogenomic databases

    eggNOGiCOG0473.
    HOGENOMiHOG000021113.
    HOVERGENiHBG052080.
    InParanoidiP51553.
    KOiK00030.
    OMAiNETRLHT.
    OrthoDBiEOG75B85R.
    PhylomeDBiP51553.
    TreeFamiTF315033.

    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: P51553-1) [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

    MALKVATVAG SAAKAVLGPA LLCRPWEVLG AHEVPSRNIF SEQTIPPSAK    50
    YGGRHTVTMI PGDGIGPELM LHVKSVFRHA CVPVDFEEVH VSSNADEEDI 100
    RNAIMAIRRN RVALKGNIET NHNLPPSHKS RNNILRTSLD LYANVIHCKS 150
    LPGVVTRHKD IDILIVRENT EGEYSSLEHE SVAGVVESLK IITKAKSLRI 200
    AEYAFKLAQE SGRKKVTAVH KANIMKLGDG LFLQCCREVA ARYPQITFEN 250
    MIVDNTTMQL VSRPQQFDVM VMPNLYGNIV NNVCAGLVGG PGLVAGANYG 300
    HVYAVFETAT RNTGKSIANK NIANPTATLL ASCMMLDHLK LHSYATSIRK 350
    AVLASMDNEN MHTPDIGGQG TTSEAIQDVI RHIRVINGRA VEA 393
    Length:393
    Mass (Da):42,794
    Last modified:October 1, 1996 - v1
    Checksum:iA0870F2B7D228A37
    GO
    Isoform 2 (identifier: P51553-2) [UniParc]FASTAAdd to Basket

    The sequence of this isoform differs from the canonical sequence as follows:
         361-393: MHTPDIGGQGTTSEAIQDVIRHIRVINGRAVEA → VRFPSHPTLLPRPVSPCSLL

    Note: Gene prediction based on EST data.

    Show »
    Length:380
    Mass (Da):41,468
    Checksum:i2D5987CC70725245
    GO

    Experimental Info

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Sequence conflicti267 – 2671F → L in AAH01902. (PubMed:15489334)Curated

    Alternative sequence

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Alternative sequencei361 – 39333MHTPD…RAVEA → VRFPSHPTLLPRPVSPCSLL in isoform 2. CuratedVSP_046771Add
    BLAST

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    Z68907 mRNA. Translation: CAA93143.1.
    Z68129 Genomic DNA. Translation: CAA92214.1.
    U52111 Genomic DNA. No translation available.
    U40272 mRNA. Translation: AAD09357.1.
    BC000933 mRNA. Translation: AAH00933.1.
    BC001902 mRNA. Translation: AAH01902.1.
    CCDSiCCDS14730.1. [P51553-1]
    CCDS44019.1. [P51553-2]
    RefSeqiNP_004126.1. NM_004135.3. [P51553-1]
    NP_777358.1. NM_174869.2. [P51553-2]
    UniGeneiHs.410197.

    Genome annotation databases

    EnsembliENST00000217901; ENSP00000217901; ENSG00000067829. [P51553-1]
    ENST00000370092; ENSP00000359110; ENSG00000067829. [P51553-2]
    GeneIDi3421.
    KEGGihsa:3421.
    UCSCiuc004fip.4. human. [P51553-1]

    Polymorphism databases

    DMDMi1708404.

    Keywords - Coding sequence diversityi

    Alternative splicing

    Cross-referencesi

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    Z68907 mRNA. Translation: CAA93143.1 .
    Z68129 Genomic DNA. Translation: CAA92214.1 .
    U52111 Genomic DNA. No translation available.
    U40272 mRNA. Translation: AAD09357.1 .
    BC000933 mRNA. Translation: AAH00933.1 .
    BC001902 mRNA. Translation: AAH01902.1 .
    CCDSi CCDS14730.1. [P51553-1 ]
    CCDS44019.1. [P51553-2 ]
    RefSeqi NP_004126.1. NM_004135.3. [P51553-1 ]
    NP_777358.1. NM_174869.2. [P51553-2 ]
    UniGenei Hs.410197.

    3D structure databases

    ProteinModelPortali P51553.
    SMRi P51553. Positions 50-386.
    ModBasei Search...
    MobiDBi Search...

    Protein-protein interaction databases

    BioGridi 109647. 15 interactions.
    IntActi P51553. 2 interactions.
    MINTi MINT-3018829.
    STRINGi 9606.ENSP00000217901.

    Chemistry

    DrugBanki DB00157. NADH.

    PTM databases

    PhosphoSitei P51553.

    Polymorphism databases

    DMDMi 1708404.

    Proteomic databases

    MaxQBi P51553.
    PaxDbi P51553.
    PRIDEi P51553.

    Protocols and materials databases

    DNASUi 3421.
    Structural Biology Knowledgebase Search...

    Genome annotation databases

    Ensembli ENST00000217901 ; ENSP00000217901 ; ENSG00000067829 . [P51553-1 ]
    ENST00000370092 ; ENSP00000359110 ; ENSG00000067829 . [P51553-2 ]
    GeneIDi 3421.
    KEGGi hsa:3421.
    UCSCi uc004fip.4. human. [P51553-1 ]

    Organism-specific databases

    CTDi 3421.
    GeneCardsi GC0XM153051.
    HGNCi HGNC:5386. IDH3G.
    HPAi HPA000425.
    HPA002017.
    MIMi 300089. gene.
    neXtProti NX_P51553.
    PharmGKBi PA29634.
    GenAtlasi Search...

    Phylogenomic databases

    eggNOGi COG0473.
    HOGENOMi HOG000021113.
    HOVERGENi HBG052080.
    InParanoidi P51553.
    KOi K00030.
    OMAi NETRLHT.
    OrthoDBi EOG75B85R.
    PhylomeDBi P51553.
    TreeFami TF315033.

    Enzyme and pathway databases

    BioCyci MetaCyc:ENSG00000067829-MONOMER.
    Reactomei REACT_118595. Mitochondrial protein import.
    REACT_1785. Citric acid cycle (TCA cycle).
    SABIO-RK P51553.

    Miscellaneous databases

    GeneWikii IDH3G.
    GenomeRNAii 3421.
    NextBioi 13490.
    PROi P51553.
    SOURCEi Search...

    Gene expression databases

    ArrayExpressi P51553.
    Bgeei P51553.
    CleanExi HS_IDH3G.
    Genevestigatori P51553.

    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. "Genomic organization of two novel genes on human Xq28: compact head to head arrangement of IDH gamma and TRAP delta is conserved in rat and mouse."
      Brenner V., Nyakatura G., Rosenthal A., Platzer M.
      Genomics 44:8-14(1997) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA / MRNA] (ISOFORM 1).
      Tissue: Brain.
    2. "Identification and functional characterization of a novel, tissue-specific NAD+-dependent isocitrate dehydrogenase beta subunit isoform."
      Kim Y.-O., Koh H.-J., Kim S.-H., Jo S.-H., Huh J.-W., Jeong K.-S., Lee I.J., Song B.J., Huh T.-L.
      J. Biol. Chem. 274:36866-36875(1999) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1).
      Tissue: Heart.
    3. "The DNA sequence of the human X chromosome."
      Ross M.T., Grafham D.V., Coffey A.J., Scherer S., McLay K., Muzny D., Platzer M., Howell G.R., Burrows C., Bird C.P., Frankish A., Lovell F.L., Howe K.L., Ashurst J.L., Fulton R.S., Sudbrak R., Wen G., Jones M.C.
      , Hurles M.E., Andrews T.D., Scott C.E., Searle S., Ramser J., Whittaker A., Deadman R., Carter N.P., Hunt S.E., Chen R., Cree A., Gunaratne P., Havlak P., Hodgson A., Metzker M.L., Richards S., Scott G., Steffen D., Sodergren E., Wheeler D.A., Worley K.C., Ainscough R., Ambrose K.D., Ansari-Lari M.A., Aradhya S., Ashwell R.I., Babbage A.K., Bagguley C.L., Ballabio A., Banerjee R., Barker G.E., Barlow K.F., Barrett I.P., Bates K.N., Beare D.M., Beasley H., Beasley O., Beck A., Bethel G., Blechschmidt K., Brady N., Bray-Allen S., Bridgeman A.M., Brown A.J., Brown M.J., Bonnin D., Bruford E.A., Buhay C., Burch P., Burford D., Burgess J., Burrill W., Burton J., Bye J.M., Carder C., Carrel L., Chako J., Chapman J.C., Chavez D., Chen E., Chen G., Chen Y., Chen Z., Chinault C., Ciccodicola A., Clark S.Y., Clarke G., Clee C.M., Clegg S., Clerc-Blankenburg K., Clifford K., Cobley V., Cole C.G., Conquer J.S., Corby N., Connor R.E., David R., Davies J., Davis C., Davis J., Delgado O., Deshazo D., Dhami P., Ding Y., Dinh H., Dodsworth S., Draper H., Dugan-Rocha S., Dunham A., Dunn M., Durbin K.J., Dutta I., Eades T., Ellwood M., Emery-Cohen A., Errington H., Evans K.L., Faulkner L., Francis F., Frankland J., Fraser A.E., Galgoczy P., Gilbert J., Gill R., Gloeckner G., Gregory S.G., Gribble S., Griffiths C., Grocock R., Gu Y., Gwilliam R., Hamilton C., Hart E.A., Hawes A., Heath P.D., Heitmann K., Hennig S., Hernandez J., Hinzmann B., Ho S., Hoffs M., Howden P.J., Huckle E.J., Hume J., Hunt P.J., Hunt A.R., Isherwood J., Jacob L., Johnson D., Jones S., de Jong P.J., Joseph S.S., Keenan S., Kelly S., Kershaw J.K., Khan Z., Kioschis P., Klages S., Knights A.J., Kosiura A., Kovar-Smith C., Laird G.K., Langford C., Lawlor S., Leversha M., Lewis L., Liu W., Lloyd C., Lloyd D.M., Loulseged H., Loveland J.E., Lovell J.D., Lozado R., Lu J., Lyne R., Ma J., Maheshwari M., Matthews L.H., McDowall J., McLaren S., McMurray A., Meidl P., Meitinger T., Milne S., Miner G., Mistry S.L., Morgan M., Morris S., Mueller I., Mullikin J.C., Nguyen N., Nordsiek G., Nyakatura G., O'dell C.N., Okwuonu G., Palmer S., Pandian R., Parker D., Parrish J., Pasternak S., Patel D., Pearce A.V., Pearson D.M., Pelan S.E., Perez L., Porter K.M., Ramsey Y., Reichwald K., Rhodes S., Ridler K.A., Schlessinger D., Schueler M.G., Sehra H.K., Shaw-Smith C., Shen H., Sheridan E.M., Shownkeen R., Skuce C.D., Smith M.L., Sotheran E.C., Steingruber H.E., Steward C.A., Storey R., Swann R.M., Swarbreck D., Tabor P.E., Taudien S., Taylor T., Teague B., Thomas K., Thorpe A., Timms K., Tracey A., Trevanion S., Tromans A.C., d'Urso M., Verduzco D., Villasana D., Waldron L., Wall M., Wang Q., Warren J., Warry G.L., Wei X., West A., Whitehead S.L., Whiteley M.N., Wilkinson J.E., Willey D.L., Williams G., Williams L., Williamson A., Williamson H., Wilming L., Woodmansey R.L., Wray P.W., Yen J., Zhang J., Zhou J., Zoghbi H., Zorilla S., Buck D., Reinhardt R., Poustka A., Rosenthal A., Lehrach H., Meindl A., Minx P.J., Hillier L.W., Willard H.F., Wilson R.K., Waterston R.H., Rice C.M., Vaudin M., Coulson A., Nelson D.L., Weinstock G., Sulston J.E., Durbin R.M., Hubbard T., Gibbs R.A., Beck S., Rogers J., Bentley D.R.
      Nature 434:325-337(2005) [PubMed] [Europe PMC] [Abstract]
      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: Placenta and Skin.
    5. "Systematic subcellular localization of novel proteins identified by large-scale cDNA sequencing."
      Simpson J.C., Wellenreuther R., Poustka A., Pepperkok R., Wiemann S.
      EMBO Rep. 1:287-292(2000) [PubMed] [Europe PMC] [Abstract]
      Cited for: SUBCELLULAR LOCATION.
    6. Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].

    Entry informationi

    Entry nameiIDH3G_HUMAN
    AccessioniPrimary (citable) accession number: P51553
    Secondary accession number(s): E9PDD5, Q9BUU5
    Entry historyi
    Integrated into UniProtKB/Swiss-Prot: October 1, 1996
    Last sequence update: October 1, 1996
    Last modified: October 1, 2014
    This is version 155 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, Reference proteome

    Documents

    1. Human chromosome X
      Human chromosome X: 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