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)
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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
Pathology
- up-regulated in myotonic dystrophy pathophysiology