CELF and PTB proteins modulate the inclusion of the β-tropomyosin exon 6B during myogenic differentiation
Related Experiment Video
Updated: Jun 8, 2026

Modeling Myotonic Dystrophy 1 in C2C12 Myoblast Cells
Published on: July 29, 2016
Alain Sureau1, Jérôme Saulière, Alain Expert-Bezançon
1Centre de Génétique Moléculaire, FRE 3144, CNRS, Gif sur Yvette, France, Université Paris XI, Orsay, France.
Abstract:
Exon 6B from the chicken β-tropomyosin pre-mRNA is alternatively spliced during myogenic differentiation. Exon 6B is excluded in mRNA from myoblasts and included in mRNA from myotubes. We investigated the regulation of exon 6B inclusion ex vivo in a quail myogenic cell line, which behaves as myoblasts in undifferentiated state and as myotubes after differentiation. We show that the β-tropomyosin exon 6B is a novel target of CUG-BP and ETR-3-like factor (CELF). Overexpression of CELF proteins in myoblasts activates splicing of exon 6B. Using a dominant-negative form of CELF4, we demonstrate that CELF proteins are involved in switching splicing from exon 6A towards exon 6B inclusion during myogenic differentiation. We also found that polypyrimidine tract binding protein (PTB) is required for splicing repression of exon 6B in myoblasts. CELF and PTB proteins exhibit antagonistic properties toward inclusion of exon 6B during myogenic differentiation. Our results suggest that a change in the protein level of CUGBP1 and PTB proteins, associated with a distinct pattern of PTB during the transition from myoblasts to myotubes is one of the parameters involved in regulating splicing of exon 6B during myogenesis.
Insights
CUG-BP and ETR-3-like factor (CELF) proteins promote exon 6B inclusion in beta-tropomyosin mRNA during muscle cell differentiation. Polypyrimidine tract binding protein (PTB) represses this splicing event, indicating an antagonistic regulatory mechanism.
Area of Science:
- Molecular Biology
- Cell Biology
- RNA Splicing
Background:
- Alternative splicing of beta-tropomyosin pre-mRNA is crucial for myogenic differentiation.
- Exon 6B inclusion is observed in myotubes but excluded in myoblasts.
Purpose of the Study:
- To investigate the regulatory mechanisms governing the alternative splicing of exon 6B in beta-tropomyosin.
- To identify the roles of specific RNA-binding proteins in this process during myogenesis.
Main Methods:
- Ex vivo studies using a quail myogenic cell line.
- Overexpression of CUG-BP and ETR-3-like factor (CELF) proteins.
- Utilizing a dominant-negative form of CELF4.
- Investigating the role of polypyrimidine tract binding protein (PTB).
Main Results:
- Beta-tropomyosin exon 6B is a novel target of CELF proteins.
- CELF protein overexpression activates exon 6B splicing.
- CELF proteins mediate the switch towards exon 6B inclusion during differentiation.
- PTB is required for exon 6B splicing repression in myoblasts.
- CELF and PTB proteins act antagonistically on exon 6B inclusion.
Conclusions:
- Changes in CUGBP1 and PTB protein levels regulate exon 6B splicing during myogenesis.
- Distinct PTB expression patterns contribute to the regulation of exon 6B splicing transition.
Related Concept Videos
TGF - β Signaling Pathway
Master Transcription Regulators
General Transcription Factors
Formation of Muscle Fibers from Myoblasts
Muscle progenitor cells (MPCs) are formed from the myotomes. MPCs express genes that encode the transcription factors Pax3 and Pax7. Along with Pax 3/7, other transcription factors...
Transcription Factors
Cell Specific Gene Expression

