Aditi Phadke

TL;DR: Overall, this manuscript provides a well-tested and standardized open field activity SOP for preclinical trials in animal models of neuromuscular diseases and provides recommendations for optimal study design when usingOpen field activity in a preclinical trial.

TL;DR: Successful improvement of dystrophy independent of hormonal, growth, or immunosuppressive effects is demonstrated, indicating VBP15 merits clinical investigation for DMD and would benefit other chronic inflammatory diseases.

TL;DR: The best-known consequence of lack of dystrophin that is common to DMD and the mdx mouse is the conspicuous necrosis and regeneration of muscle fibres, and protocols for measuring this are presented, in terms both of loss of muscle nuclei previously labelled with BrdU and of the intensity of myonuclear labelling withBrdU administered during the regeneration period.

TL;DR: Results show that perturbed Lap2α/pRb and Smad2/3 signaling are important regulatory pathways mediating defective muscle growth in Lmna(-/-) mice, and that inhibition of either pathway alone or in combination can ameliorate this deleterious phenotype.

TL;DR: It is shown that patients with idiopathic inflammatory myopathies (IIMs) develop autoimmunity to FHL1, which is a muscle-specific protein, and it is determined that FHL 1 is a target of the cytotoxic protease granzyme B, indicating that the generation of F HL1 fragments may initiate FHL2 autoim immunity.