A histometric analysis of the alterations at the motor end plate in dystrophic Bar Harbor mice has been performed. In both forms of mouse dystrophy, simplification and focal atrophy of the junctional folds and retraction of the nerve terminal represented the significant changes. The postsynaptic alterations were similar to those described in Duchenne dystrophy. In constrast, the presence of presynaptic alterations in these mice indicated the presence of both a neural and a muscular abnormality. The wedge-shaped focal lesions in the muscle of dystrophic mice were demonstrated by both in vitro and in vivo HRP tracer techniques. These focal degenerative changes in muscle were similar to those described in Duchenne dystrophy. Employing the technique of extracellular space tracing, a comparison was made of the appearance of the transverse tubular system in the various types of dystrophic muscle fibers.
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Muscle Nerve
January 2025
Department of Anatomy, Federal University of Alfenas (UNIFAL-MG), Alfenas, Brazil.
Introduction/aims: Duchenne muscular dystrophy (DMD) is caused by pathogenic variants in the DMD gene, making muscle fibers susceptible to contraction-induced membrane damage. Given the potential beneficial action of cannabidiol (CBD), we evaluated the in vitro effect of full-spectrum CBD oil on the viability of dystrophic muscle fibers and the in vivo effect on myopathy of the mdx mouse, a DMD model.
Methods: In vitro, dystrophic cells from the mdx mouse were treated with full-spectrum CBD oil and assessed with cell viability and cytotoxic analyses.
Front Vet Sci
January 2025
Department of Veterinary Integrative Biosciences, Texas A&M University, College Station, TX, United States.
Duchenne muscular dystrophy (DMD) is an X-linked muscle disease with weakness, loss of ambulation, and premature death. DMD patients have reduced bone health, including decreased femur length (FL), density, and fractures. The mouse model has paradoxically greater FL, density, and strength, positively correlating with muscle mass.
View Article and Find Full Text PDFERJ Open Res
January 2025
Faculty of Health and Life Sciences, Northumbria University Newcastle, Newcastle upon Tyne, UK.
Background: In response to exercise-based pulmonary rehabilitation (PR), the type of muscle fibre remodelling differs between COPD patients with peripheral muscle wasting (atrophic patients with COPD) and those without wasting (nonatrophic patients with COPD). Extracellular matrix (ECM) proteins are major constituents of the cell micro-environment steering cell behaviour and regeneration. We investigated whether the composition of ECM in atrophic compared to nonatrophic patients with COPD differs in response to PR.
View Article and Find Full Text PDFbioRxiv
January 2025
Institute of Muscle Biology and Cachexia, University of Houston College of Pharmacy, Houston, TX, USA.
Skeletal muscle regeneration in adults is predominantly driven by satellite cells. Loss of satellite cell pool and function leads to skeletal muscle wasting in many conditions and disease states. Here, we demonstrate that the levels of fibroblast growth factor-inducible 14 (Fn14) are increased in satellite cells after muscle injury.
View Article and Find Full Text PDFInt J Mol Sci
December 2024
Clinic of Nuclear Medicine Central University Emergency Military Hospital "Dr Carol Davila", 10825 Bucharest, Romania.
Amyloidosis is a rare pathology characterized by protein deposits in various organs and tissues. Cardiac amyloidosis (CA) can be caused by various protein deposits, but transthyretin amyloidosis (ATTR) and immunoglobulin light chain (AL) are the most frequent pathologies. Protein misfolding can be induced by several factors such as oxidative stress, genetic mutations, aging, chronic inflammation, and neoplastic disorders.
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