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FSHD region gene 1

FRG1, FSHD region gene 1, FSGI
This gene maps to a location 100 kb centromeric of the repeat units on chromosome 4q35 which are deleted in facioscapulohumeral muscular dystrophy (FSHD). It is evolutionarily conserved and has related sequences on multiple human chromosomes but DNA sequence analysis did not reveal any homology to known genes. In vivo studies demonstrate the encoded protein is localized to the nucleolus. [provided by RefSeq, Jul 2008] (from NCBI)
Top mentioned proteins: CAN, HAD, fibrillin-1, CIs, Actin
Papers using FRG1 antibodies
Analysis of cell cycle phases and progression in cultured mammalian cells.
Feany Mel B., In PLoS ONE, 2006
... FRG1 transgenic mice (H- ...
Transduction by adeno-associated virus vectors in the rabbit airway: efficiency, persistence, and readministration
Gabellini Davide et al., In Molecular Therapy, 1996
... FRG1 vector-injected n = 5, transgenic ...
Papers on FRG1
Cryptic 13q34 and 4q35.2 Deletions in an Italian Family.
Martorana et al., Parma, Italy. In Cytogenet Genome Res, Jan 2016
The terminal deletion in 4q35.2 contains other OMIM genes (FRG1, FRG2 and DBET); moreover, the 4q region is reported as a susceptibility locus for Crohn's disease, diagnosed in the proband's father.
Direct interplay between two candidate genes in FSHD muscular dystrophy.
Gabellini et al., Milano, Italy. In Hum Mol Genet, Apr 2015
Among the FSHD candidate genes are double homeobox 4 (DUX4), encoded by the most telomeric D4Z4 unit, and FSHD region gene 1 (FRG1).
SNF2 chromatin remodeler-family proteins FRG1 and -2 are required for RNA-directed DNA methylation.
Ausin et al., Fuzhou, China. In Proc Natl Acad Sci U S A, 2015
Here we report the discovery of two redundant proteins, SNF2-ring-helicase-like1 and -2 (FRG1 and -2) that are putative chromatin modifiers belonging to the SNF2 family of helicase-like proteins.
FHL1 reduces dystrophy in transgenic mice overexpressing FSHD muscular dystrophy region gene 1 (FRG1).
Mitchell et al., Australia. In Plos One, 2014
Several disease candidate genes have been proposed including DUX4 and FRG1.
Altered Tnnt3 characterizes selective weakness of fast fibers in mice overexpressing FSHD region gene 1 (FRG1).
Tupler et al., Reggio nell'Emilia, Italy. In Am J Physiol Regul Integr Comp Physiol, 2014
Mice overexpressing FRG1 (FSHD region gene 1), a candidate gene for this disease, develop a progressive myopathy with features of the human disorder.
DUX4 promotes transcription of FRG2 by directly activating its promoter in facioscapulohumeral muscular dystrophy.
Van der Maarel et al., Leiden, Netherlands. In Skelet Muscle, 2013
In some studies, genes centromeric to the D4Z4 repeat array have been reported to be over-expressed in FSHD, including FRG1 and FRG2, presumably due to decreased long-distance repression by the shorter array through a mechanism similar to position-effect variegation.
FSHD muscular dystrophy region gene 1 binds Suv4-20h1 histone methyltransferase and impairs myogenesis.
Gabellini et al., Milano, Italy. In J Mol Cell Biol, 2013
Among them, we focused on FSHD region gene 1 (FRG1) since its over-expression in mice, Xenopus laevis and Caenorhabditis elegans, leads to muscular dystrophy-like defects, suggesting that FRG1 plays a relevant role in muscle biology.
Super-telomeres in transformed human fibroblasts.
Mondello et al., Pavia, Italy. In Biochim Biophys Acta, 2013
Super-telomeres neither influenced subtelomeric region global methylation nor the expression of the subtelomeric gene FRG1, attesting the lack of a clear-cut relationship between telomere length, subtelomeric DNA methylation and expression in human cells.
Overexpression of facioscapulohumeral muscular dystrophy region gene 1 causes primary defects in myogenic stem cells.
Gabellini et al., Milano, Italy. In J Cell Sci, 2013
Overexpression of facioscapulohumeral muscular dystrophy region gene 1 (FRG1) in mice, frogs and worms leads to muscular and vascular abnormalities.
Rbfox1 downregulation and altered calpain 3 splicing by FRG1 in a mouse model of Facioscapulohumeral muscular dystrophy (FSHD).
Gabellini et al., Milano, Italy. In Plos Genet, 2012
Over-expression of FSHD region gene 1 (FRG1) in mice, frogs, and worms perturbs muscle development and causes FSHD-like phenotypes.
Effects of creatine and exercise on skeletal muscle of FRG1-transgenic mice.
Tarnopolsky et al., Hamilton, Canada. In Can J Neurol Sci, 2012
BACKGROUND: The FRG1-transgenic mouse displays muscle dysfunction and atrophy reminiscent of fascioscapulohumeral muscular dystrophy (FSHD) and could provide a model to determine potential therapeutic interventions.
Bending amplitude - a new quantitative assay of C. elegans locomotion: identification of phenotypes for mutants in genes encoding muscle focal adhesion components.
Benian et al., Atlanta, United States. In Methods, 2012
Loss of function mutants for eight proteins had been reported to have normal locomotion (ZYX-1 (zyxin), ALP-1 (Enigma), DIM-1, SCPL-1), or locomotion that was not previously investigated (FRG-1 (FRG1), KIN-32 (focal adhesion kinase), LIM-8), or had only slightly decreased locomotion (PFN-3 (profilin)).
Facioscapulohumeral muscular dystrophy region gene 1 is a dynamic RNA-associated and actin-bundling protein.
Jones et al., Urbana, United States. In J Mol Biol, 2011
These data provide the first biochemical activities (actin binding and RNA binding) for human FRG1 and the characterization of the endogenous human FRG1, together indicating that FRG1 is involved in multiple aspects of RNA biogenesis.
Facioscapulohumeral muscular dystrophy (FSHD) region gene 1 (FRG1) is a dynamic nuclear and sarcomeric protein.
Jones et al., Urbana, United States. In Differentiation, 2011
in muscle FRG1 is a developmentally regulated sarcomeric protein suggesting FRG1 may perform a muscle-specific function
Decreased proliferation kinetics of mouse myoblasts overexpressing FRG1.
Kennedy et al., Seattle, United States. In Plos One, 2010
depressed myoblast proliferation may contribute to the pathology of mice overexpressing FRG1 and may play a part in facioscapulohumeral muscular dystrophy
Expression profiling of FSHD-1 and FSHD-2 cells during myogenic differentiation evidences common and distinctive gene dysregulation patterns.
Meneveri et al., Milano, Italy. In Plos One, 2010
new insights into the gene deregulation characterizing both FSHD-1 and FSHD-2, in which miRNAs may play a role
Remodeling of the chromatin structure of the facioscapulohumeral muscular dystrophy (FSHD) locus and upregulation of FSHD-related gene 1 (FRG1) expression during human myogenic differentiation.
Ginelli et al., Milano, Italy. In Bmc Biol, 2008
study compared chromatin structure & tridimensional interaction of the D4Z4 array and FRG1 gene promoter, and FRG1 expression, in control and FSHD cells
Facioscapulohumeral muscular dystrophy in mice overexpressing FRG1.
Tupler et al., Worcester, United States. In Nature, 2006
FRG1 transgenic mice develop a muscular dystrophy with features characteristic of the human disease; by contrast, FRG2 and ANT1 transgenic mice seem normal
[Progress in researches on the molecular genetics of facioscapulohumeral muscular dystrophy].
Zhang et al., Guangzhou, China. In Zhonghua Yi Xue Yi Chuan Xue Za Zhi, 2001
FRG1 (FSHD region gene 1) from human chromosome 4q35 is identified as a candidate gene for FSHD.
Molecular genetics of facioscapulohumeral muscular dystrophy (FSHD).
Upadhyaya et al., Cardiff, United Kingdom. In Neuromuscul Disord, 1997
Thus so far, only one gene, FRG1 (FSHD region gene 1) has been identified from the FSHD candidate region on 4q35.
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