A. Bigot

Identification of bazedoxifene for the treatment of LGMD R2 by high throughput screening.

This study identifies bazedoxifene as a potential treatment for LGMD R2 through high-throughput screening of drugs targeting dysferlin aggregation.

Disease exacerbation by fibroblast inclusion in Duchenne Muscular Dystrophy MYOrganoids reveals limitations of microdystrophin therapeutic efficacy

This paper develops a human DMD model using MYOrganoids to study fibroblast inclusion effects and evaluates microdystrophin therapy efficacy, finding limited success in reducing fibrosis and improving membrane stability.

SETDB1 modulates the TGFbeta response in Duchenne muscular dystrophy myotubes

This study shows that SETDB1 enhances TGFβ-driven fibrosis in DMD myotubes by modulating TGFβ-target gene expression and secretome effects on myoblast differentiation.

Dystrophin is a mechanical tension modulator

This study shows that dystrophin modulates baseline homeostatic tension in skeletal muscle, linking increased tissue tension to DMD pathogenesis.

Caveolae and Bin1 form ring-shaped platforms for T-tubule initiation

This study shows that caveolae and Bin1 form ring-shaped structures that initiate T-tubule formation in muscle cells.

Bioengineering a Miniaturized In Vitro 3D Myotube Contraction Monitoring Chip For Modelization of Muscular Dystrophies

This paper develops a miniaturized 3D myotube contraction monitoring chip to model muscular dystrophies, showing contractile dysfunction in L-CMD mutant myotubes with reduced starting material requirements.

The SMYD3 methyltransferase promotes myogenesis by activating the myogenin regulatory network

This study shows SMYD3 promotes myogenesis by activating the myogenin regulatory network through transcriptomic analysis in murine myoblasts.

CHC22 clathrin mediates traffic from early secretory compartments for human GLUT4 pathway biogenesis

The paper identifies CHC22 clathrin as mediating ERGIC-derived membrane traffic essential for GLUT4 sequestration in humans, distinct from mice lacking CHC22.

A gold standard method for human cell quantification after xenotransplantation

The paper describes a combined immunofluorescence and real-time quantitative PCR method to accurately quantify and visualize human cell engraftment in immunodeficient mice.

A three-dimensional culture model of innervated human skeletal muscle enables studies of the adult neuromuscular junction and disease modeling

This paper introduces a 3D co-culture model of human skeletal muscle and motor neurons to study adult neuromuscular junction development and disease, showing increased receptor upregulation and disease modeling capability.

Mechanosensitive clathrin platforms anchor desmin intermediate filaments in skeletal muscle

This study shows that clathrin plaques and dynamin 2 anchor desmin intermediate filaments and regulate YAP signaling in skeletal muscle in response to mechanical cues.