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P97376

- FRG1_MOUSE

UniProt

P97376 - FRG1_MOUSE

Protein

Protein FRG1

Gene

Frg1

Organism
Mus musculus (Mouse)
Status
Reviewed - Annotation score: 4 out of 5- Experimental evidence at protein leveli
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    • History
      Entry version 113 (01 Oct 2014)
      Sequence version 2 (26 Sep 2001)
      Previous versions | rss
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    Functioni

    Binds to mRNA in a sequence-independent manner. May play a role in regulation of pre-mRNA splicing or in the assembly of rRNA into ribosomal subunits. May be involved in mRNA transport. May be involved in epigenetic regulation of muscle differentiation through regulation of activity of the histone-lysine N-methyltransferase SUV420H1.1 Publication

    GO - Biological processi

    1. mRNA processing Source: UniProtKB-KW
    2. RNA splicing Source: UniProtKB-KW
    3. rRNA processing Source: UniProtKB-KW

    Keywords - Biological processi

    mRNA processing, mRNA splicing, Myogenesis, Ribosome biogenesis, rRNA processing

    Keywords - Ligandi

    Actin-binding, RNA-binding

    Names & Taxonomyi

    Protein namesi
    Recommended name:
    Protein FRG1
    Alternative name(s):
    FSHD region gene 1 protein
    Gene namesi
    Name:Frg1
    OrganismiMus musculus (Mouse)
    Taxonomic identifieri10090 [NCBI]
    Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
    ProteomesiUP000000589: Chromosome 8

    Organism-specific databases

    MGIiMGI:893597. Frg1.

    Subcellular locationi

    NucleusCajal body By similarity. Nucleusnucleolus By similarity. Cytoplasm. CytoplasmmyofibrilsarcomereZ line
    Note: Localization changes during myogenesis from mainly cytoplasmic in undifferentiated myoblasts, to strongly nucleolar in early myotubes and back to cytoplasmic 5 days post-differentiation. Localized at the Z-line in the sarcomere of matured myotubes 8 days post-differentiation.

    GO - Cellular componenti

    1. Cajal body Source: UniProtKB-SubCell
    2. catalytic step 2 spliceosome Source: Ensembl
    3. nuclear speck Source: UniProtKB-SubCell

    Keywords - Cellular componenti

    Cytoplasm, Nucleus, Spliceosome

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Chaini1 – 258258Protein FRG1PRO_0000220768Add
    BLAST

    Proteomic databases

    MaxQBiP97376.
    PaxDbiP97376.
    PRIDEiP97376.

    PTM databases

    PhosphoSiteiP97376.

    Expressioni

    Gene expression databases

    ArrayExpressiP97376.
    BgeeiP97376.
    CleanExiMM_FRG1.
    GenevestigatoriP97376.

    Interactioni

    Subunit structurei

    Homodimer and homotetramer in solution. Identified in the spliceosome C complex. Interacts (via N-terminus) with KPNA2 and NXF1/TAP. Interacts with F-actin with a stoichiometry of 2:1 By similarity. Interacts with SUV420H1 (via C-terminus).By similarity1 Publication

    Protein-protein interaction databases

    BioGridi199743. 2 interactions.
    IntActiP97376. 1 interaction.

    Structurei

    Secondary structure

    1
    258
    Legend: HelixTurnBeta strand
    Show more details
    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Beta strandi47 – 493
    Helixi53 – 553
    Beta strandi58 – 636
    Beta strandi65 – 673
    Beta strandi69 – 724
    Beta strandi78 – 803
    Beta strandi85 – 884
    Turni93 – 953
    Beta strandi97 – 1015
    Beta strandi103 – 1053
    Beta strandi107 – 1115
    Beta strandi116 – 1194
    Beta strandi121 – 1277
    Turni134 – 1363
    Beta strandi137 – 1415
    Beta strandi148 – 1514
    Beta strandi156 – 1594
    Beta strandi161 – 1633
    Beta strandi165 – 1673
    Turni174 – 1763
    Beta strandi179 – 1824

    3D structure databases

    Select the link destinations:
    PDBe
    RCSB PDB
    PDBj
    Links Updated
    EntryMethodResolution (Å)ChainPositionsPDBsum
    2YUGNMR-A41-188[»]
    ProteinModelPortaliP97376.
    SMRiP97376. Positions 41-188.
    ModBaseiSearch...
    MobiDBiSearch...

    Miscellaneous databases

    EvolutionaryTraceiP97376.

    Family & Domainsi

    Motif

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Motifi22 – 3211Nuclear localization signalSequence AnalysisAdd
    BLAST
    Motifi235 – 25117Bipartite nuclear localization signalSequence AnalysisAdd
    BLAST

    Compositional bias

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Compositional biasi8 – 3225Lys-richAdd
    BLAST

    Sequence similaritiesi

    Belongs to the FRG1 family.Curated

    Phylogenomic databases

    eggNOGiNOG289849.
    HOGENOMiHOG000007130.
    HOVERGENiHBG018564.
    InParanoidiP97376.
    KOiK13122.
    OMAiNSCFISY.
    OrthoDBiEOG7NKKMG.
    PhylomeDBiP97376.
    TreeFamiTF314108.

    Family and domain databases

    InterProiIPR008999. Actin_cross-linking.
    IPR010414. FRG1.
    [Graphical view]
    PANTHERiPTHR12928. PTHR12928. 1 hit.
    PfamiPF06229. FRG1. 1 hit.
    [Graphical view]
    SUPFAMiSSF50405. SSF50405. 1 hit.

    Sequencei

    Sequence statusi: Complete.

    P97376-1 [UniParc]FASTAAdd to Basket

    « Hide

    MAEYSYVKST KLVLKGTKAK SKKKKSKDKK RKREEDEETQ LDIVGIWWTV    50
    SNFGEISGTI AIEMDKGAYI HALDNGLFTL GAPHREVDEG PSPPEQFTAV 100
    KLSDSRIALK SGYGKYLGIN SDGLVVGRSD AIGPREQWEP VFQDGKMALL 150
    ASNSCFIRCN EAGDIEAKNK TAGEEEMIKI RSCAERETKK KDDIPEEDKG 200
    SVKQCEINYV KKFQSFQDHK LKISKEDSKI LKKARKDGFL HETLLDRRAK 250
    LKADRYCK 258
    Length:258
    Mass (Da):29,127
    Last modified:September 26, 2001 - v2
    Checksum:i1D0C07CF83897ACE
    GO

    Experimental Info

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Sequence conflicti140 – 1401P → Q in AAB39540. (PubMed:9714712)Curated

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    U62105 mRNA. Translation: AAB39540.1.
    AK079229 mRNA. Translation: BAC37582.1.
    BC002027 mRNA. Translation: AAH02027.1.
    CCDSiCCDS22263.1.
    RefSeqiNP_038550.2. NM_013522.3.
    UniGeneiMm.217312.

    Genome annotation databases

    EnsembliENSMUST00000033999; ENSMUSP00000033999; ENSMUSG00000031590.
    GeneIDi14300.
    KEGGimmu:14300.
    UCSCiuc012gcs.1. mouse.

    Cross-referencesi

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    U62105 mRNA. Translation: AAB39540.1 .
    AK079229 mRNA. Translation: BAC37582.1 .
    BC002027 mRNA. Translation: AAH02027.1 .
    CCDSi CCDS22263.1.
    RefSeqi NP_038550.2. NM_013522.3.
    UniGenei Mm.217312.

    3D structure databases

    Select the link destinations:
    PDBe
    RCSB PDB
    PDBj
    Links Updated
    Entry Method Resolution (Å) Chain Positions PDBsum
    2YUG NMR - A 41-188 [» ]
    ProteinModelPortali P97376.
    SMRi P97376. Positions 41-188.
    ModBasei Search...
    MobiDBi Search...

    Protein-protein interaction databases

    BioGridi 199743. 2 interactions.
    IntActi P97376. 1 interaction.

    PTM databases

    PhosphoSitei P97376.

    Proteomic databases

    MaxQBi P97376.
    PaxDbi P97376.
    PRIDEi P97376.

    Protocols and materials databases

    Structural Biology Knowledgebase Search...

    Genome annotation databases

    Ensembli ENSMUST00000033999 ; ENSMUSP00000033999 ; ENSMUSG00000031590 .
    GeneIDi 14300.
    KEGGi mmu:14300.
    UCSCi uc012gcs.1. mouse.

    Organism-specific databases

    CTDi 2483.
    MGIi MGI:893597. Frg1.

    Phylogenomic databases

    eggNOGi NOG289849.
    HOGENOMi HOG000007130.
    HOVERGENi HBG018564.
    InParanoidi P97376.
    KOi K13122.
    OMAi NSCFISY.
    OrthoDBi EOG7NKKMG.
    PhylomeDBi P97376.
    TreeFami TF314108.

    Miscellaneous databases

    EvolutionaryTracei P97376.
    NextBioi 285717.
    PROi P97376.
    SOURCEi Search...

    Gene expression databases

    ArrayExpressi P97376.
    Bgeei P97376.
    CleanExi MM_FRG1.
    Genevestigatori P97376.

    Family and domain databases

    InterProi IPR008999. Actin_cross-linking.
    IPR010414. FRG1.
    [Graphical view ]
    PANTHERi PTHR12928. PTHR12928. 1 hit.
    Pfami PF06229. FRG1. 1 hit.
    [Graphical view ]
    SUPFAMi SSF50405. SSF50405. 1 hit.
    ProtoNeti Search...

    Publicationsi

    1. "FRG1, a gene in the FSH muscular dystrophy region on human chromosome 4q35, is highly conserved in vertebrates and invertebrates."
      Grewal P.K., Todd L.C., van der Maarel S., Frants R.R., Hewitt J.E.
      Gene 216:13-19(1998) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [MRNA].
      Strain: NIH Swiss.
    2. "The transcriptional landscape of the mammalian genome."
      Carninci P., Kasukawa T., Katayama S., Gough J., Frith M.C., Maeda N., Oyama R., Ravasi T., Lenhard B., Wells C., Kodzius R., Shimokawa K., Bajic V.B., Brenner S.E., Batalov S., Forrest A.R., Zavolan M., Davis M.J.
      , Wilming L.G., Aidinis V., Allen J.E., Ambesi-Impiombato A., Apweiler R., Aturaliya R.N., Bailey T.L., Bansal M., Baxter L., Beisel K.W., Bersano T., Bono H., Chalk A.M., Chiu K.P., Choudhary V., Christoffels A., Clutterbuck D.R., Crowe M.L., Dalla E., Dalrymple B.P., de Bono B., Della Gatta G., di Bernardo D., Down T., Engstrom P., Fagiolini M., Faulkner G., Fletcher C.F., Fukushima T., Furuno M., Futaki S., Gariboldi M., Georgii-Hemming P., Gingeras T.R., Gojobori T., Green R.E., Gustincich S., Harbers M., Hayashi Y., Hensch T.K., Hirokawa N., Hill D., Huminiecki L., Iacono M., Ikeo K., Iwama A., Ishikawa T., Jakt M., Kanapin A., Katoh M., Kawasawa Y., Kelso J., Kitamura H., Kitano H., Kollias G., Krishnan S.P., Kruger A., Kummerfeld S.K., Kurochkin I.V., Lareau L.F., Lazarevic D., Lipovich L., Liu J., Liuni S., McWilliam S., Madan Babu M., Madera M., Marchionni L., Matsuda H., Matsuzawa S., Miki H., Mignone F., Miyake S., Morris K., Mottagui-Tabar S., Mulder N., Nakano N., Nakauchi H., Ng P., Nilsson R., Nishiguchi S., Nishikawa S., Nori F., Ohara O., Okazaki Y., Orlando V., Pang K.C., Pavan W.J., Pavesi G., Pesole G., Petrovsky N., Piazza S., Reed J., Reid J.F., Ring B.Z., Ringwald M., Rost B., Ruan Y., Salzberg S.L., Sandelin A., Schneider C., Schoenbach C., Sekiguchi K., Semple C.A., Seno S., Sessa L., Sheng Y., Shibata Y., Shimada H., Shimada K., Silva D., Sinclair B., Sperling S., Stupka E., Sugiura K., Sultana R., Takenaka Y., Taki K., Tammoja K., Tan S.L., Tang S., Taylor M.S., Tegner J., Teichmann S.A., Ueda H.R., van Nimwegen E., Verardo R., Wei C.L., Yagi K., Yamanishi H., Zabarovsky E., Zhu S., Zimmer A., Hide W., Bult C., Grimmond S.M., Teasdale R.D., Liu E.T., Brusic V., Quackenbush J., Wahlestedt C., Mattick J.S., Hume D.A., Kai C., Sasaki D., Tomaru Y., Fukuda S., Kanamori-Katayama M., Suzuki M., Aoki J., Arakawa T., Iida J., Imamura K., Itoh M., Kato T., Kawaji H., Kawagashira N., Kawashima T., Kojima M., Kondo S., Konno H., Nakano K., Ninomiya N., Nishio T., Okada M., Plessy C., Shibata K., Shiraki T., Suzuki S., Tagami M., Waki K., Watahiki A., Okamura-Oho Y., Suzuki H., Kawai J., Hayashizaki Y.
      Science 309:1559-1563(2005) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
      Strain: C57BL/6J.
      Tissue: Urinary bladder.
    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] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
      Tissue: Mammary tumor.
    4. Cited for: OVEREXPRESSION, SUBCELLULAR LOCATION.
    5. "Facioscapulohumeral muscular dystrophy (FSHD) region gene 1 (FRG1) is a dynamic nuclear and sarcomeric protein."
      Hanel M.L., Sun C.Y., Jones T.I., Long S.W., Zanotti S., Milner D., Jones P.L.
      Differentiation 81:107-118(2011) [PubMed] [Europe PMC] [Abstract]
      Cited for: SUBCELLULAR LOCATION.
    6. "FSHD muscular dystrophy region gene 1 binds Suv4-20h1 histone methyltransferase and impairs myogenesis."
      Neguembor M.V., Xynos A., Onorati M.C., Caccia R., Bortolanza S., Godio C., Pistoni M., Corona D.F., Schotta G., Gabellini D.
      J. Mol. Cell Biol. 5:294-307(2013) [PubMed] [Europe PMC] [Abstract]
      Cited for: FUNCTION, OVEREXPRESSION, INTERACTION WITH SUV420H1.
    7. "Solution structure of mouse Frg1 protein."
      RIKEN structural genomics initiative (RSGI)
      Submitted (APR-2008) to the PDB data bank
      Cited for: STRUCTURE BY NMR OF 41-188.

    Entry informationi

    Entry nameiFRG1_MOUSE
    AccessioniPrimary (citable) accession number: P97376
    Secondary accession number(s): Q99M42
    Entry historyi
    Integrated into UniProtKB/Swiss-Prot: September 26, 2001
    Last sequence update: September 26, 2001
    Last modified: October 1, 2014
    This is version 113 of the entry and version 2 of the sequence. [Complete history]
    Entry statusiReviewed (UniProtKB/Swiss-Prot)
    Annotation programChordata Protein Annotation Program

    Miscellaneousi

    Miscellaneous

    Overexpression of Frg1 leads to development of facioscapulohumeral muscular dystrophy (FSHD1)-like symptoms such as kyphosis, progressive muscle dystrophy and skeletal muscle atrophy. It also causes aberrant pre-mRNA splicing of Tnnt3 and Mtmr1, affects the localization and activity of Suv420h1, and leads to increased levels of Eid3, resulting in inhibited muscle differentiation. These results suggest that human FSHD1 results from inappropriate overexpression of FRG1 which leads to abnormal alternative splicing of specific pre-mRNAs (PubMed:16341202, PubMed:23720823).2 Publications

    Keywords - Technical termi

    3D-structure, Complete proteome, Reference proteome

    Documents

    1. MGD cross-references
      Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
    2. PDB cross-references
      Index of Protein Data Bank (PDB) cross-references
    3. SIMILARITY comments
      Index of protein domains and families

    External Data

    Dasty 3