Skeletal muscle fibrosis: an overview

MAA Mahdy - Cell and tissue research, 2019 - Springer
Extracellular matrix (ECM) is an essential component of skeletal muscle. It provides a
framework structure that holds myofibers and blood capillaries and nerves supplying the …

Humanizing the mdx mouse model of DMD: the long and the short of it

N Yucel, AC Chang, JW Day, N Rosenthal… - NPJ Regenerative …, 2018 - nature.com
Duchenne muscular dystrophy (DMD) is a common fatal heritable myopathy, with
cardiorespiratory failure occurring by the third decade of life. There is no specific treatment …

[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 …

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 …

Early myopathy in Duchenne muscular dystrophy is associated with elevated mitochondrial H2O2 emission during impaired oxidative phosphorylation

MC Hughes, SV Ramos, PC Turnbull… - Journal of cachexia …, 2019 - Wiley Online Library
Background Muscle wasting and weakness in Duchenne muscular dystrophy (DMD) causes
severe locomotor limitations and early death due in part to respiratory muscle failure. Given …

[HTML][HTML] Natural disease history of the D2-mdx mouse model for Duchenne muscular dystrophy

M van Putten, K Putker, M Overzier… - The FASEB …, 2019 - ncbi.nlm.nih.gov
Abstract The C57BL/10ScSn-Dmd mdx/J (BL10-mdx) mouse has been the most commonly
used model for Duchenne muscular dystrophy (DMD) for decades. Their muscle dysfunction …

Ribozyme-activated mRNA trans-ligation enables large gene delivery to treat muscular dystrophies

SR Lindley, KCV Subbaiah, F Priyanka, P Poosala… - Science, 2024 - science.org
Ribozymes are small catalytic RNA sequences capable of nucleotide-specific self-cleavage
found widespread in nature. Ribozyme cleavage generates distinct 2′, 3′-phosphate and …

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 …

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 …

2, 4 Dinitrophenol as medicine

JG Geisler - Cells, 2019 - mdpi.com
In the sanctity of pure drug discovery, objective reasoning can become clouded when
pursuing ideas that appear unorthodox, but are spot on physiologically. To put this into …