CUGBP elav-like family member 1; CELF-1; 50 kD nuclear polyadenylated RNA-binding protein; Bruno-like protein 2; CUG triplet repeat RNA-binding protein 1; CUG-BP1; CUG-BP- & ETR-3-like factor 1; deadenylation factor CUG-BP; embryo deadenylation element-binding protein homolog; EDEN-BP homolog; RNA-binding protein BRUNOL-2 (CELF1, BRUNOL2, CUGBP, CUGBP1, NAB50)

Function: - RNA-binding protein - implicated in regulation of several post-transcriptional events - role in pre-mRNA alternative splicing, mRNA translation & stability - mediates exon inclusion &/or exclusion in pre-mRNA that are subject to tissue-specific & developmentally regulated alternative splicing - specifically activates exon 5 inclusion of cardiac isoforms of TNNT2 during heart remodeling at the juvenile to adult transition - acts as both an activator & repressor of a pair of coregulated exons: promotes inclusion of the smooth muscle exon but exclusion of the non-muscle exon in actinin pre-mRNAs - activates smooth muscle exon 5 inclusion by antagonizing the repressive effect of PTB - promotes exclusion of exon 11 of the INSR pre-mRNA - inhibits, together with HNRNPH1, insulin receptor pre-mRNA exon 11 inclusion in myoblast - increases translation & controls choice of translation initiation codon of CEBPB mRNA - increases mRNA translation of CEBPB in aging liver - increases translation of CDKN1A mRNA by antagonizing the repressive effect of CALR3 - mediates rapid cytoplasmic mRNA deadenylation - recruits the deadenylase PARN to the poly(A) tail of EDEN- containing mRNAs to promote their deadenylation - required for completion of spermatogenesis (putative) - binds to (CUG)n triplet repeats in the 3'-UTR of transcripts such as DMPK & to Bruno response elements (BREs) - binds to muscle-specific splicing enhancer (MSE) intronic sites flanking the alternative exon 5 of TNNT2 pre-mRNA - binds to AU-rich sequences (AREs or EDEN-like) localized in the 3'-UTR of JUN & FOS mRNAs. - binds to the IR RNA - binds to the 5'-region of CDKN1A & CEBPB mRNAs - binds with the 5'-region of CEBPB mRNA in aging liver - phosphorylated; phosphorylation status increases in senescent cells - component of an EIF2 complex at least composed of CELF1/CUGBP1, CALR, CALR3, EIF2S1, EIF2S2, HSP90B1 & HSPA5 - associates with polysomes (putative) - interacts with HNRNPH1 (RNA-dependent) - interacts with PARN Structure: - belongs to the CELF/BRUNOL family - contains 3 RRM domain (RNA recognition motif)s Compartment: nucleus. cytoplasm. Note: RNA-binding activity is detected in both nuclear & cytoplasmic compartments Alternative splicing: - named isoforms=4 - at least one isoform may be produced at very low levels due to a premature stop codon in the mRNA, leading to nonsense-mediated mRNA decay Expression: - ubiquitous Pathology: - up-regulated in myotonic dystrophy pathophysiology

General

CUG-BP- & ETR-3-like factor (CELF, Bruno-like protein, RNA-binding protein BRUNOL, CUG triplet repeat RNA-binding protein, CUG-BP, CUGBP, BRUNOL) phosphoprotein splicing factor; snRNP-associated protein

Properties

SIZE: entity length = 486 aa MW = 52 kD COMPARTMENT: cytoplasm cell nucleus MOTIF: acetylation site SITE: N-TERMINUS EFFECTOR-BOUND: acetyl RNP motif NAME: RNP motif SITE: 16-99 FOR-BINDING-OF: ribonucleic acid MOTIF: ribonucleoprotein-1 motif NAME: ribonucleoprotein-1 motif FOR-BINDING-OF: ribonucleic acid MOTIF: ribonucleoprotein-1 motif ribonucleoprotein-2 motif ribonucleoprotein-2 motif FOR-BINDING-OF: ribonucleic acid MOTIF: ribonucleoprotein-1 motif ribonucleoprotein-2 motif RNP motif NAME: RNP motif SITE: 108-188 FOR-BINDING-OF: ribonucleic acid MOTIF: ribonucleoprotein-1 motif NAME: ribonucleoprotein-1 motif FOR-BINDING-OF: ribonucleic acid MOTIF: ribonucleoprotein-1 motif ribonucleoprotein-2 motif ribonucleoprotein-2 motif FOR-BINDING-OF: ribonucleic acid MOTIF: ribonucleoprotein-1 motif ribonucleoprotein-2 motif serine-rich region {287-308} MOTIF: serine residue (SEVERAL) Ser phosphorylation site {S302} RNP motif NAME: RNP motif SITE: 401-479 FOR-BINDING-OF: ribonucleic acid MOTIF: ribonucleoprotein-1 motif NAME: ribonucleoprotein-1 motif FOR-BINDING-OF: ribonucleic acid MOTIF: ribonucleoprotein-1 motif ribonucleoprotein-2 motif ribonucleoprotein-2 motif FOR-BINDING-OF: ribonucleic acid MOTIF: ribonucleoprotein-1 motif ribonucleoprotein-2 motif

Database Correlations

OMIM 601074 UniProt Q92879 Pfam PF00076 Entrez Gene 10658 Kegg hsa:10658

References

UniProt :accession Q92879