Skeletal muscle in health and disease

J Morgan, T Partridge - Disease models & mechanisms, 2020 - journals.biologists.com
Skeletal muscle fibres are multinucleated cells that contain postmitotic nuclei (ie they are no
longer able to divide) and perform muscle contraction. They are formed by fusion of muscle …

[HTML][HTML] Tissue-engineered skeletal muscle models to study muscle function, plasticity, and disease

A Khodabukus - Frontiers in Physiology, 2021 - frontiersin.org
Skeletal muscle possesses remarkable plasticity that permits functional adaptations to a
wide range of signals such as motor input, exercise, and disease. Small animal models have …

[HTML][HTML] TGF-β–driven muscle degeneration and failed regeneration underlie disease onset in a DMD mouse model

DAG Mázala, JS Novak, MW Hogarth, M Nearing… - JCI insight, 2020 - ncbi.nlm.nih.gov
Duchenne muscular dystrophy (DMD) is a chronic muscle disease characterized by poor
myogenesis and replacement of muscle by extracellular matrix. Despite the shared genetic …

[HTML][HTML] The D2.mdx mouse as a preclinical model of the skeletal muscle pathology associated with Duchenne muscular dystrophy

DW Hammers, CC Hart, MK Matheny, LA Wright… - Scientific reports, 2020 - nature.com
Duchenne muscular dystrophy (DMD) is an X-linked, lethal muscle degenerative disease
caused by loss of dystrophin protein. DMD has no cure and few treatment options …

[HTML][HTML] Modulating fast skeletal muscle contraction protects skeletal muscle in animal models of Duchenne muscular dystrophy

AJ Russell, M DuVall, B Barthel, Y Qian… - The Journal of …, 2023 - Am Soc Clin Investig
Duchenne muscular dystrophy (DMD) is a lethal muscle disease caused by absence of the
protein dystrophin, which acts as a structural link between the basal lamina and contractile …

Advanced models of human skeletal muscle differentiation, development and disease: Three-dimensional cultures, organoids and beyond

S Jalal, S Dastidar, FS Tedesco - Current opinion in cell biology, 2021 - Elsevier
Advanced in vitro models of human skeletal muscle tissue are increasingly needed to model
complex developmental dynamics and disease mechanisms not recapitulated in animal …

Passive stiffness of fibrotic skeletal muscle in mdx mice relates to collagen architecture

SE Brashear, RP Wohlgemuth… - The Journal of …, 2021 - Wiley Online Library
Key points The amount of fibrotic material in dystrophic mouse muscles relates to contractile
function, but not passive function. Collagen fibres in skeletal muscle are associated with …

[HTML][HTML] Collagen cross-links scale with passive stiffness in dystrophic mouse muscles, but are not altered with administration of a lysyl oxidase inhibitor

SE Brashear, RP Wohlgemuth, LY Hu, EH Jbeily… - PLoS …, 2022 - journals.plos.org
In Duchenne muscular dystrophy (DMD), a lack of functional dystrophin leads to myofiber
instability and progressive muscle damage that results in fibrosis. While fibrosis is primarily …

[HTML][HTML] The multifaceted view of heart problem in Duchenne muscular dystrophy

U Florczyk-Soluch, K Polak, J Dulak - Cellular and Molecular Life Sciences, 2021 - Springer
Dystrophin is a large protein serving as local scaffolding repetitively bridging cytoskeleton
and the outside of striated muscle cell. As such dystrophin is a critical brick primarily in …

[HTML][HTML] Neuromuscular Development and Disease: Learning From in vitro and in vivo Models

Z Fralish, EM Lotz, T Chavez, A Khodabukus… - Frontiers in cell and …, 2021 - frontiersin.org
The neuromuscular junction (NMJ) is a specialized cholinergic synaptic interface between a
motor neuron and a skeletal muscle fiber that translates presynaptic electrical impulses into …