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CUGBP, Elav-like family member 1

CUGBP1, CUG-BP
Members of the CELF/BRUNOL protein family contain two N-terminal RNA recognition motif (RRM) domains, one C-terminal RRM domain, and a divergent segment of 160-230 aa between the second and third RRM domains. Members of this protein family regulate pre-mRNA alternative splicing and may also be involved in mRNA editing, and translation. This gene may play a role in myotonic dystrophy type 1 (DM1) via interactions with the dystrophia myotonica-protein kinase (DMPK) gene. Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Jul 2008] (from NCBI)
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Top mentioned proteins: DM2, EXP, 4E-BP1, CUGBP2, V1a
Papers on CUGBP1
Destabilization of microRNAs in human cells by 3' deadenylation mediated by PARN and CUGBP1.
New
Suzuki et al., Tokyo, Japan. In Nucleic Acids Res, 30 Jul 2015
We also demonstrate that CUG-binding protein 1 (CUGBP1) specifically interacts with miR-122 and other UG-rich miRNAs, and promotes their destabilization.
p300 regulates liver functions by controlling p53 and C/EBP family proteins through multiple signaling pathways.
New
Timchenko et al., Cincinnati, United States. In Mol Cell Biol, 22 Jul 2015
Under-laying mechanisms of resistance to liver injury and increased proliferation in dnp300 mice include ubiquitin proteasome-mediated degradation of C/EBPα and translational repression of p53 protein by CUGBP1-eIF2 repressor complex.
Effect of denervation on the regulation of mitochondrial transcription factor A expression in skeletal muscle.
New
Hood et al., York, United Kingdom. In Am J Physiol Cell Physiol, 10 Jul 2015
Protein expression of RNA-binding proteins which promote mRNA degradation (KSRP and CUGBP1) was elevated at 3 and 7 days of denervation.
Antagonistic regulation of mRNA expression and splicing by CELF and MBNL proteins.
New
Burge et al., Cambridge, United States. In Genome Res, 30 Jun 2015
RNA binding proteins of the conserved CUGBP1, Elav-like factor (CELF) family contribute to heart and skeletal muscle development and are implicated in myotonic dystrophy (DM).
Competition between RNA-binding proteins CELF1 and HuR modulates MYC translation and intestinal epithelium renewal.
New
Wang et al., Baltimore, United States. In Mol Biol Cell, 15 Jun 2015
The RNA-binding protein (RBP) ELAV-like family member 1 (CELF1), also referred to as CUG-binding protein 1 (CUGBP1), regulates the stability and translation of target mRNAs and is implicated in many aspects of cellular physiology.
Exercise, skeletal muscle and inflammation: ARE-binding proteins as key regulators in inflammatory and adaptive networks.
Review
New
Munz et al., Tübingen, Germany. In Exerc Immunol Rev, Dec 2014
The best-characterized ARE-binding proteins are HuR, CUGBP1, KSRP, AUF1, and the three ZFP36 proteins, especially TTP/ZFP36.
Gene Expression Analyses during Spontaneous Reversal of Cardiomyopathy in Mice with Repressed Nuclear CUG-BP, Elav-Like Family (CELF) Activity in Heart Muscle.
New
Ladd et al., Cleveland, United States. In Plos One, Dec 2014
CUG-BP, Elav-like family (CELF) proteins regulate cell type- and developmental stage-specific alternative splicing in the heart.
Molecular mechanisms of muscle atrophy in myotonic dystrophies.
Review
Timchenko, Houston, United States. In Int J Biochem Cell Biol, 2013
These expansions cause DM pathologies through accumulation of mutant RNAs that alter RNA metabolism in patients' tissues by targeting RNA-binding proteins such as CUG-binding protein 1 (CUGBP1) and Muscle blind-like protein 1 (MBNL1).
Dysfunction of protein homeostasis in myotonic dystrophies.
Review
Timchenko et al., Milano, Italy. In Histol Histopathol, 2013
The pathogenic role of CUG and CCUG repeats in the mis-regulation of alternative splicing, mediated by RNA-binding proteins CUGBP1 and MBNL1, has been discussed in a number of excellent reviews.
CUG-BP, Elav-like family (CELF)-mediated alternative splicing regulation in the brain during health and disease.
Review
Ladd, Cleveland, United States. In Mol Cell Neurosci, 2013
These include the six members of the CUG-BP, Elav-like family (CELF).
Celf1 regulation of dmrt2a is required for somite symmetry and left-right patterning during zebrafish development.
GeneRIF
Bessho et al., Nara, Japan. In Development, 2012
Celf1-dependent fine-tuning of dmrt2a expression is essential for generating bilateral symmetry of somites and left-right asymmetric patterning during zebrafish development.
Myotonic dystrophy: is a narrow focus obscuring the rest of the field?
Review
Mahadevan, Charlottesville, United States. In Curr Opin Neurol, 2012
The role of other RNA-binding proteins beyond MBNL1 and CUGBP1, such as Staufen 1 and DDX5, are being identified and studied with respect to their role in myotonic dystrophy.
The RNA-binding protein CUG-BP1 increases survivin expression in oesophageal cancer cells through enhanced mRNA stability.
GeneRIF
Battafarano et al., Baltimore, United States. In Biochem J, 2012
CUG-BP1 is overexpressed in oesophageal cancer cell lines and human oesophageal cancer specimens. CUG-BP1 associates with the 3'-untranslated region of survivin mRNA.
Regulation of CUG-binding protein 1 (CUGBP1) binding to target transcripts upon T cell activation.
GeneRIF
Bohjanen et al., Minneapolis, United States. In J Biol Chem, 2012
CUGBP1 binding to certain GRE-containing target transcripts decreased following T cell activation through activation-dependent phosphorylation of CUGBP1.
miR-503 represses CUG-binding protein 1 translation by recruiting CUGBP1 mRNA to processing bodies.
GeneRIF
Wang et al., Baltimore, United States. In Mol Biol Cell, 2012
These findings identify miR-503 as both a novel regulator of CUGBP1 expression and a modulator of intestinal epithelial homoeostasis
Regulation of cyclin-dependent kinase 4 translation through CUG-binding protein 1 and microRNA-222 by polyamines.
GeneRIF
Wang et al., Baltimore, United States. In Mol Biol Cell, 2011
polyamine-regulated CUG-binding protein 1 and miR-222 modulate cyclin-dependent kinase 4 (CDK4) translation at least in part by altering the recruitment of CDK4 mRNA to processing bodies
Expression of 24,426 human alternative splicing events and predicted cis regulation in 48 tissues and cell lines.
Impact
Johnson et al., Seattle, United States. In Nat Genet, 2008
An unbiased, systematic screen of 21,760 4-mer to 7-mer words for cis-regulatory motifs identified 143 RNA 'words' enriched near regulated cassette exons, including six clusters of motifs represented by UCUCU, UGCAUG, UGCU, UGUGU, UUUU and AGGG, which map to trans-acting regulators PTB, Fox, Muscleblind, CELF/CUG-BP, TIA-1 and hnRNP F/H, respectively.
Reversible model of RNA toxicity and cardiac conduction defects in myotonic dystrophy.
Impact
Phillips et al., Charlottesville, United States. In Nat Genet, 2006
However, we observed increased levels of CUG-binding protein (CUG-BP1) in skeletal muscle, as seen in individuals with DM1.
Aberrant regulation of insulin receptor alternative splicing is associated with insulin resistance in myotonic dystrophy.
Impact
Cooper et al., Houston, United States. In Nat Genet, 2001
Steady-state levels of CUG-BP, a regulator of pre-mRNA splicing proposed to mediate some aspects of DM1 pathogenesis, are increased in DM1 skeletal muscle; overexpression of CUG-BP in normal cells induces a switch to IR-A.
Disruption of splicing regulated by a CUG-binding protein in myotonic dystrophy.
Impact
Cooper et al., Houston, United States. In Science, 1998
Data presented here indicate that the conserved heterogeneous nuclear ribonucleoprotein, CUG-binding protein (CUG-BP), may mediate the trans-dominant effect of the RNA.
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