scholarly journals Tweak regulates astrogliosis, microgliosis and skeletal muscle atrophy in a mouse model of amyotrophic lateral sclerosis

2015 ◽  
Vol 24 (12) ◽  
pp. 3440-3456 ◽  
Author(s):  
M. Bowerman ◽  
C. Salsac ◽  
E. Coque ◽  
E. Eiselt ◽  
R. G. Deschaumes ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Amyotrophic Lateral Sclerosis ◽  
Mouse Model ◽  
Muscle Atrophy ◽  
Skeletal Muscle Atrophy ◽  
Lateral Sclerosis

scholarly journals Protocatechuic Acid Extends Survival, Improves Motor Function, Diminishes Gliosis, and Sustains Neuromuscular Junctions in the hSOD1G93A Mouse Model of Amyotrophic Lateral Sclerosis

Nutrients ◽  
2020 ◽  
Vol 12 (6) ◽  
pp. 1824 ◽  
Author(s):  
Lilia A. Koza ◽  
Aimee N. Winter ◽  
Jessica Holsopple ◽  
Angela N. Baybayon-Grandgeorge ◽  
Claudia Pena ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Amyotrophic Lateral Sclerosis ◽  
Mouse Model ◽  
Muscle Atrophy ◽  
Protocatechuic Acid ◽  
Neuromuscular Junctions ◽  
Disease Onset ◽  
Skeletal Muscle Atrophy ◽  
Therapeutic Effects ◽  
Lateral Sclerosis

Amyotrophic lateral sclerosis (ALS) is a devastating disorder characterized by motor neuron apoptosis and subsequent skeletal muscle atrophy caused by oxidative and nitrosative stress, mitochondrial dysfunction, and neuroinflammation. Anthocyanins are polyphenolic compounds found in berries that possess neuroprotective and anti-inflammatory properties. Protocatechuic acid (PCA) is a phenolic acid metabolite of the parent anthocyanin, kuromanin, found in blackberries and bilberries. We explored the therapeutic effects of PCA in a transgenic mouse model of ALS that expresses mutant human Cu, Zn-superoxide dismutase 1 with a glycine to alanine substitution at position 93. These mice display skeletal muscle atrophy, hindlimb weakness, and weight loss. Disease onset occurs at approximately 90 days old and end stage is reached at approximately 120 days old. Daily treatment with PCA (100 mg/kg) by oral gavage beginning at disease onset significantly extended survival (121 days old in untreated vs. 133 days old in PCA-treated) and preserved skeletal muscle strength and endurance as assessed by grip strength testing and rotarod performance. Furthermore, PCA reduced astrogliosis and microgliosis in spinal cord, protected spinal motor neurons from apoptosis, and maintained neuromuscular junction integrity in transgenic mice. PCA lengthens survival, lessens the severity of pathological symptoms, and slows disease progression in this mouse model of ALS. Given its significant preclinical therapeutic effects, PCA should be further investigated as a treatment option for patients with ALS.

scholarly journals Histone deacetylase 4 protects from denervation and skeletal muscle atrophy in a murine model of amyotrophic lateral sclerosis

EBioMedicine ◽  
2019 ◽  
Vol 40 ◽  
pp. 717-732 ◽  
Author(s):  
Eva Pigna ◽  
Elena Simonazzi ◽  
Krizia Sanna ◽  
Krzysztof Marian Bernadzki ◽  
Tomek Proszynski ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Amyotrophic Lateral Sclerosis ◽  
Histone Deacetylase ◽  
Muscle Atrophy ◽  
Murine Model ◽  
Skeletal Muscle Atrophy ◽  
Histone Deacetylase 4 ◽  
Lateral Sclerosis

scholarly journals Perturbations in intracellular Ca2+ handling in skeletal muscle in the G93A*SOD1 mouse model of amyotrophic lateral sclerosis

2014 ◽  
Vol 307 (11) ◽  
pp. C1031-C1038 ◽  
Author(s):  
Eva R. Chin ◽  
Dapeng Chen ◽  
Kostyantyn D. Bobyk ◽  
Davi A. G. Mázala
Keyword(s):  
Skeletal Muscle ◽  
Amyotrophic Lateral Sclerosis ◽  
Mouse Model ◽  
Disease Progression ◽  
Skeletal Muscle Atrophy ◽  
Fura 2 ◽  
Intracellular Ca ◽  
G93a Sod1 Mouse ◽  
G93a Sod1 ◽  
Lateral Sclerosis

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease characterized by skeletal muscle atrophy and weakness, ultimately leading to respiratory failure. The purpose of this study was to assess changes in skeletal muscle excitation-contraction (E-C) coupling and intracellular Ca2+ handling during disease progression in the G93A*SOD1 ALS transgenic (ALS Tg) mouse model. To assess E-C coupling, single muscle fibers were electrically stimulated (10–150 Hz), and intracellular free Ca2+ concentration was assessed using fura-2. There were no differences in peak fura-2 ratio at any stimulation frequency at 70 days (early presymptomatic). However, at 90 days (late presymptomatic) and 120–140 days (symptomatic), fura-2 ratio was increased at 10 Hz in ALS Tg compared with wild-type (WT) fibers (0.670 ± 0.02 vs. 0.585 ± 0.02 for 120–140 days; P < 0.05). There was also a significant increase in resting fura-2 ratio at 90 days (0.351 ± 0.008 vs. 0.390 ± 0.009 in WT vs. ALS Tg; P < 0.05) and 120–140 days (0.374 ± 0.001 vs. 0.415 ± 0.003 in WT vs. ALS Tg; P < 0.05). These increases in intracellular Ca2+ in ALS Tg muscle were associated with reductions in the sarcoplasmic/endoplasmic reticulum Ca2+ ATPase proteins SERCA1 (to 54% and 19% of WT) and SERCA2 (to 56% and 11% of WT) and parvalbumin (to 80 and 62% of WT) in gastrocnemius muscle at 90 and 120–140 days, respectively. There was no change in dihydropyridine receptor/l-type Ca2+ channel at any age. Overall, these data demonstrate minimal changes in electrically evoked Ca2+ transients but elevations in intracellular Ca2+ attributable to decreased Ca2+-clearance proteins. These data suggest that elevations in cellular Ca2+ could contribute to muscle weakness during disease progression in ALS mice.

Human skeletal muscle atrophy in amyotrophic lateral sclerosis reveals a reduction in Akt and an increase in atrogin‐1

2006 ◽  
Vol 20 (3) ◽  
pp. 583-585 ◽  
Author(s):  
Bertrand Léger ◽  
Lodovica Vergani ◽  
Gianni Sorarù ◽  
Peter Hespel ◽  
Wim Derave ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Amyotrophic Lateral Sclerosis ◽  
Muscle Atrophy ◽  
Human Skeletal Muscle ◽  
Skeletal Muscle Atrophy ◽  
Lateral Sclerosis

scholarly journals Effects of Nandrolone in the Counteraction of Skeletal Muscle Atrophy in a Mouse Model of Muscle Disuse: Molecular Biology and Functional Evaluation

PLoS ONE ◽  
2015 ◽  
Vol 10 (6) ◽  
pp. e0129686 ◽  
Author(s):  
Giulia Maria Camerino ◽  
Jean-François Desaphy ◽  
Michela De Bellis ◽  
Roberta Francesca Capogrosso ◽  
Anna Cozzoli ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Molecular Biology ◽  
Mouse Model ◽  
Muscle Atrophy ◽  
Skeletal Muscle Atrophy ◽  
Functional Evaluation ◽  
Muscle Disuse
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