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Protein

Protein FRG1

Gene

Frg1

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: -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 KMT5B.1 Publication

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 KMT5B, 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

GO - Molecular functioni

GO - Biological processi

Keywordsi

Molecular functionActin-binding, RNA-binding
Biological processmRNA processing, mRNA splicing, Myogenesis, Ribosome biogenesis, rRNA processing

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 lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 8

Organism-specific databases

MGIiMGI:893597 Frg1

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Cytoplasm, Nucleus, Spliceosome

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00002207681 – 258Protein FRG1Add BLAST258

Proteomic databases

EPDiP97376
PaxDbiP97376
PeptideAtlasiP97376
PRIDEiP97376

PTM databases

iPTMnetiP97376
PhosphoSitePlusiP97376

Expressioni

Gene expression databases

BgeeiENSMUSG00000031590
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 KMT5B (via C-terminus).By similarity1 Publication

GO - Molecular functioni

Protein-protein interaction databases

BioGridi199743, 2 interactors
IntActiP97376, 2 interactors
STRINGi10090.ENSMUSP00000033999

Structurei

Secondary structure

1258
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Beta strandi47 – 49Combined sources3
Helixi53 – 55Combined sources3
Beta strandi58 – 63Combined sources6
Beta strandi65 – 67Combined sources3
Beta strandi69 – 72Combined sources4
Beta strandi78 – 80Combined sources3
Beta strandi85 – 88Combined sources4
Turni93 – 95Combined sources3
Beta strandi97 – 101Combined sources5
Beta strandi103 – 105Combined sources3
Beta strandi107 – 111Combined sources5
Beta strandi116 – 119Combined sources4
Beta strandi121 – 127Combined sources7
Turni134 – 136Combined sources3
Beta strandi137 – 141Combined sources5
Beta strandi148 – 151Combined sources4
Beta strandi156 – 159Combined sources4
Beta strandi161 – 163Combined sources3
Beta strandi165 – 167Combined sources3
Turni174 – 176Combined sources3
Beta strandi179 – 182Combined sources4

3D structure databases

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

Miscellaneous databases

EvolutionaryTraceiP97376

Family & Domainsi

Motif

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Motifi22 – 32Nuclear localization signalSequence analysisAdd BLAST11
Motifi235 – 251Bipartite nuclear localization signalSequence analysisAdd BLAST17

Compositional bias

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Compositional biasi8 – 32Lys-richAdd BLAST25

Sequence similaritiesi

Belongs to the FRG1 family.Curated

Phylogenomic databases

eggNOGiKOG3962 Eukaryota
ENOG411009G LUCA
GeneTreeiENSGT00390000004552
HOGENOMiHOG000007130
HOVERGENiHBG018564
InParanoidiP97376
KOiK13122
OMAiEQWEPIF
OrthoDBiEOG091G0M4K
PhylomeDBiP97376
TreeFamiTF314108

Family and domain databases

InterProiView protein in InterPro
IPR008999 Actin-crosslinking
IPR010414 FRG1
PANTHERiPTHR12928 PTHR12928, 1 hit
PfamiView protein in Pfam
PF06229 FRG1, 1 hit
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)DescriptionActionsGraphical viewLength
Sequence conflicti140P → Q in AAB39540 (PubMed:9714712).Curated1

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
XP_017168059.1, XM_017312570.1
UniGeneiMm.217312

Genome annotation databases

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

Similar proteinsi

Entry informationi

Entry nameiFRG1_MOUSE
AccessioniPrimary (citable) accession number: P97376
Secondary accession number(s): Q99M42
Entry historyiIntegrated into UniProtKB/Swiss-Prot: September 26, 2001
Last sequence update: September 26, 2001
Last modified: March 28, 2018
This is version 138 of the entry and version 2 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Reference proteome
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Main funding by: National Institutes of Health