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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
Alternative polyadenylation regulates CELF1/CUGBP1 target transcripts following T cell activation.
New
Bohjanen et al., Minneapolis, United States. In Gene, 15 Nov 2014
We previously showed that GU-rich elements (GREs) found in the 3' untranslated regions of select transcripts mediate rapid mRNA decay by recruiting the protein CELF1/CUGBP1.
LDB3 splicing abnormalities are specific to skeletal muscles of patients with myotonic dystrophy type 1 and alter its PKC binding affinity.
New
Ohno et al., Nagoya, Japan. In Neurobiol Dis, 30 Sep 2014
Myotonic dystrophy type 1 (DM1) is caused by transcription of CUG repeat RNA, which causes sequestration of muscleblind-like 1 (MBNL1) and upregulation of CUG triplet repeat RNA-binding protein (CUG-BP1).
Knockdown of CUG-binding protein 1 induces apoptosis of human laryngeal cancer cells.
New
Wang et al., Beijing, China. In Cell Biol Int, Aug 2014
UNLABELLED: To investigate the role of CUG-binding protein 1 (CUGBP1) in human laryngeal cancer, we employed lentivirus-mediated short hairpin RNA (shRNA) to knockdown CUGBP1 expression in Hep-2 cells.
RNA Bind-n-Seq: quantitative assessment of the sequence and structural binding specificity of RNA binding proteins.
New
Burge et al., Cambridge, United States. In Mol Cell, Jul 2014
Here, we describe RNA Bind-n-Seq (RBNS), a method that comprehensively characterizes sequence and structural specificity of RNA binding proteins (RBPs), and its application to the developmental alternative splicing factors RBFOX2, CELF1/CUGBP1, and MBNL1.
Small molecule kinase inhibitors alleviate different molecular features of myotonic dystrophy type 1.
New
Krzyzosiak et al., Poznań, Poland. In Rna Biol, Jun 2014
These foci sequester splicing factors of the MBNL family and trigger upregulation of the CUGBP family of proteins resulting in the mis-splicing of their target transcripts.
Molecular mechanisms of muscle atrophy in myotonic dystrophies.
Review
New
Timchenko, Houston, United States. In Int J Biochem Cell Biol, Oct 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
New
Timchenko et al., Milano, Italy. In Histol Histopathol, Sep 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
New
Ladd, Cleveland, United States. In Mol Cell Neurosci, Sep 2013
These include the six members of the CUG-BP, Elav-like family (CELF).
GSK3β is a new therapeutic target for myotonic dystrophy type 1.
Timchenko et al., Houston, United States. In Rare Dis, 2012
One such protein is CUG RNA-binding protein (CUGBP1).
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 role of CUGBP1 in age-dependent changes of liver functions.
Review
Timchenko et al., Houston, United States. In Ageing Res Rev, 2012
This review is focused on the role of a conserved, multi-functional RNA-binding protein, CUGBP1, in the development of aging phenotype in the liver.
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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