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Protein

Protein FRG1

Gene

Frg1

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: Annotation score: 4 out of 5-Experimental evidence at protein leveli

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 - Molecular functioni

GO - Biological processi

Complete GO annotation...

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 Componenti: 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

Complete GO annotation...

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

BgeeiP97376.
CleanExiMM_FRG1.
ExpressionAtlasiP97376. baseline and differential.
GenevisibleiP97376. MM.

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. 2 interactions.
STRINGi10090.ENSMUSP00000033999.

Structurei

Secondary structure

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

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
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.
GeneTreeiENSGT00390000004552.
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

        10         20         30         40         50
MAEYSYVKST KLVLKGTKAK SKKKKSKDKK RKREEDEETQ LDIVGIWWTV
60 70 80 90 100
SNFGEISGTI AIEMDKGAYI HALDNGLFTL GAPHREVDEG PSPPEQFTAV
110 120 130 140 150
KLSDSRIALK SGYGKYLGIN SDGLVVGRSD AIGPREQWEP VFQDGKMALL
160 170 180 190 200
ASNSCFIRCN EAGDIEAKNK TAGEEEMIKI RSCAERETKK KDDIPEEDKG
210 220 230 240 250
SVKQCEINYV KKFQSFQDHK LKISKEDSKI LKKARKDGFL HETLLDRRAK

LKADRYCK
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:
EMBLi
GenBanki
DDBJi
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:
EMBLi
GenBanki
DDBJi
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.

3D structure databases

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

Protein-protein interaction databases

BioGridi199743. 2 interactions.
IntActiP97376. 2 interactions.
STRINGi10090.ENSMUSP00000033999.

PTM databases

PhosphoSiteiP97376.

Proteomic databases

MaxQBiP97376.
PaxDbiP97376.
PRIDEiP97376.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

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

Organism-specific databases

CTDi2483.
MGIiMGI:893597. Frg1.

Phylogenomic databases

eggNOGiNOG289849.
GeneTreeiENSGT00390000004552.
HOGENOMiHOG000007130.
HOVERGENiHBG018564.
InParanoidiP97376.
KOiK13122.
OMAiNSCFISY.
OrthoDBiEOG7NKKMG.
PhylomeDBiP97376.
TreeFamiTF314108.

Miscellaneous databases

EvolutionaryTraceiP97376.
NextBioi285717.
PROiP97376.
SOURCEiSearch...

Gene expression databases

BgeeiP97376.
CleanExiMM_FRG1.
ExpressionAtlasiP97376. baseline and differential.
GenevisibleiP97376. MM.

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.
ProtoNetiSearch...

Publicationsi

« Hide 'large scale' publications
  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: July 22, 2015
This is version 119 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

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into Uniref entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.