Loss of peroxiredoxin-2 exacerbates eccentric contraction-induced force loss in dystrophin-deficient muscle

JT Olthoff, A Lindsay, R Abo-Zahrah… - Nature …, 2018 - nature.com
Force loss in skeletal muscle exposed to eccentric contraction is often attributed to injury. We
show that EDL muscles from dystrophin-deficient mdx mice recover 65% of lost force within …

[HTML][HTML] Utrophin up-regulation by artificial transcription factors induces muscle rescue and impacts the neuromuscular junction in mdx mice

C Pisani, G Strimpakos, F Gabanella… - … et Biophysica Acta (BBA …, 2018 - Elsevier
Up-regulation of the dystrophin-related gene utrophin represents a promising therapeutic
strategy for the treatment of Duchenne Muscular Dystrophy (DMD). In order to re-program …

[HTML][HTML] Improvement of dystrophic muscle fragility by short-term voluntary exercise through activation of calcineurin pathway in mdx mice

C Delacroix, J Hyzewicz, M Lemaitre, B Friguet… - The American Journal of …, 2018 - Elsevier
Dystrophin deficiency in mdx mice, a model for Duchenne muscular dystrophy, leads to
muscle weakness revealed by a reduced specific maximal force as well as fragility (ie …

[HTML][HTML] Mitochondrial protein S-nitrosation protects against ischemia reperfusion-induced denervation at neuromuscular junction in skeletal muscle

RJ Wilson, JC Drake, D Cui, BM Lewellen… - Free radical biology & …, 2018 - ncbi.nlm.nih.gov
Deterioration of neuromuscular junction (NMJ) integrity and function is causal to muscle
atrophy and frailty, ultimately hindering quality of life and increasing the risk of death. In …

Imaging analysis of the neuromuscular junction in dystrophic muscle

SJP Pratt, SR Iyer, SB Shah, RM Lovering - Duchenne Muscular Dystrophy …, 2018 - Springer
Duchenne muscular dystrophy (DMD), caused by the absence of the protein dystrophin, is
characterized as a neuromuscular disease in which muscle weakness, increased …

Neopterin/7,8‐dihydroneopterin is elevated in Duchenne muscular dystrophy patients and protects mdx skeletal muscle function

A Lindsay, A Schmiechen… - Experimental …, 2018 - Wiley Online Library
New Findings What is the central question of this study? We examined whether the
macrophage‐synthesized antioxidant 7, 8‐dihydroneopterin was elevated in Duchenne …

Selective vulnerability in neuronal populations in nmd/SMARD1 mice

E Villalón, M Shababi, R Kline… - Human Molecular …, 2018 - academic.oup.com
Spinal muscular atrophy with respiratory distress type 1 (SMARD1) is an autosomal
recessive motor neuron disease causing distal limb muscle atrophy that progresses …

Neuromuscular synapse degeneration without muscle function loss in the diaphragm of a murine model for Huntington's Disease

PAC Valadão, MPSM Gomes, BC Aragão… - Neurochemistry …, 2018 - Elsevier
Huntington's disease (HD) is an autosomal dominant neurodegenerative disease
characterized by chorea, incoordination and psychiatric and behavioral symptoms. The …

Optical recording of action potential initiation and propagation in mouse skeletal muscle fibers

Q Banks, SJP Pratt, SR Iyer, RM Lovering… - Biophysical journal, 2018 - cell.com
Skeletal muscle fibers have been used to examine a variety of cellular functions and
pathologies. Among other parameters, skeletal muscle action potential (AP) propagation has …

[HTML][HTML] Cellular therapy for chronic traumatic brachial plexus injury

A Sharma, H Sane, N Gokulchandran… - Advanced Biomedical …, 2018 - journals.lww.com
Cellular therapy is being actively pursued as a therapeutic modality in many of the
neurological diseases. A variety of stem cells from diverse sources have been studied in …