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CELF1 / CUGBP1

CUGBP, Elav-like family member 1

RNA-binding protein implicated in the regulation of several post-transcriptional events. Involved in pre-mRNA alternative splicing, mRNA translation and stability. Mediates exon inclusion and/or exclusion in pre-mRNA that are subject to tissue-specific and 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 and repressor of a pair of coregulated exons: promotes inclusion of the smooth muscle (SM) exon but exclusion of the non-muscle (NM) exon in actinin pre-mRNAs. Activates SM 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 (IR) pre-mRNA exon 11 inclusion in myoblast. Increases translation and controls the choice of translation initiation codon of CEBPB mRNA. Increases mRNA translation of CEBPB in aging liver (By similarity). 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 (By similarity). Binds to (CUG)n triplet repeats in the 3'-UTR of transcripts such as DMPK and 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 and FOS mRNAs. Binds to the IR RNA. Binds to the 5'-region of CDKN1A and CEBPB mRNAs. Binds with the 5'-region of CEBPB mRNA in aging liver.

Gene Name: CUGBP, Elav-like family member 1
Synonyms: CELF1, Bruno-like protein 2, BRUNOL2, Bruno-like 2, CELF-1, CUG RNA-binding protein, CUGBP1, CUG-BP, EDEN-BP, HNab50, NAB50, NAPOR, RNA-binding protein BRUNOL-2, CUG-BP1, CUGBP, Deadenylation factor CUG-BP, EDEN-BP homolog
Target Sequences: NM_006560 NP_006551.1 Q92879

Publications (10)

1
A family of human RNA-binding proteins related to the Drosophila Bruno translational regulator. Good PJ, Chen Q, Warner SJ, Herring DC. The Journal of biological chemistry. 2000 275:28583-92. [PubMed:10893231]
2
Ribonuclear inclusions in skeletal muscle in myotonic dystrophy types 1 and 2. Mankodi A, Teng-Umnuay P, Krym M, Henderson D, Swanson M, Thornton CA. Annals of neurology. 2003 54:760-8. [PubMed:14681885]
3
Nuclear RNA foci in the heart in myotonic dystrophy. Mankodi A, Lin X, Blaxall BC, Swanson MS, Thornton CA. Circulation research. 2005 97:1152-5. (IHC) [PubMed:16254211]
4
Failure of MBNL1-dependent post-natal splicing transitions in myotonic dystrophy. Lin X, Miller JW, Mankodi A, Kanadia RN, Yuan Y, Moxley RT, Swanson MS, Thornton CA. Human molecular genetics. 2006 15:2087-97. (ICC, WB; Mouse, Human) [PubMed:16717059]
5
Inactivation of CUG-BP1/CELF1 causes growth, viability, and spermatogenesis defects in mice. Kress C, Gautier-Courteille C, Osborne HB, Babinet C, Paillard L. Molecular and cellular biology. 2007 27:1146-57. [PubMed:17130239] [PMC:PMC1800704]
6
Expanded CTG repeats within the DMPK 3' UTR causes severe skeletal muscle wasting in an inducible mouse model for myotonic dystrophy. Orengo JP, Chambon P, Metzger D, Mosier DR, Snipes GJ, Cooper TA. Proceedings of the National Academy of Sciences of the United States of America. 2008 105:2646-51. [PubMed:18272483] [PMC:PMC2268190]
7
Ectopic expression of CGG containing mRNA is neurotoxic in mammals. Hashem V, Galloway JN, Mori M, Willemsen R, Oostra BA, Paylor R, Nelson DL. Human molecular genetics. 2009 18:2443-51. [PubMed:19377084] [PMC:PMC2694692]
8
CUGBP1 overexpression in mouse skeletal muscle reproduces features of myotonic dystrophy type 1. Ward AJ, Rimer M, Killian JM, Dowling JJ, Cooper TA. Human molecular genetics. 2010 19:3614-22. [PubMed:20603324] [PMC:PMC2928132]
9
Bcl-x pre-mRNA splicing regulates brain injury after neonatal hypoxia-ischemia. Xiao Q, Ford AL, Xu J, Yan P, Lee KY, Gonzales E, West T, Holtzman DM, Lee JM. The Journal of neuroscience : the official journal of the Society for Neuroscience. 2012 32:13587-96. (ICC, IHC, WB, GS; Mouse, Rat) [PubMed:23015448] [PMC:PMC3482490]
10
The FMRP-MOV10 complex: a translational regulatory switch modulated by G-Quadruplexes. Phillip J Kenny, Miri Kim, Geena Skariah, Joshua Nielsen, Monica C Lannom, Stephanie Ceman. Nucleic acids research. 2019 Jan;48:862-878. [Full Text Article] [PubMed:31740951] [PMC:PMC7145700] Related Antibodies: LS-C408897.
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The data on this page has been compiled from LifeSpan internal sources, the National Center for Biotechnology Information (NCBI), and The Universal Protein Resource (UniProt).