A Drug Inhibits the Mitochondrial Protease Inducing Calmitine Deficiency in Skeletal Muscle of Patients with Duchenne's Muscular Dystrophy and dy/dy Dystrophic Mice

1997 ◽  
Vol 232 (2) ◽  
pp. 559-561
Author(s):  
Brigitte Lucas-Heron
Keyword(s):  
Skeletal Muscle ◽  
Muscular Dystrophy ◽  
Duchenne's Muscular Dystrophy ◽  
Dystrophic Mice

Concentrations of K and Na in Skeletal Muscle of Mice With a Hereditary Myopathy (Dystrophia Muscularis)

1958 ◽  
Vol 193 (3) ◽  
pp. 530-533 ◽  
Author(s):  
Nome Baker ◽  
William H. Blahd ◽  
P. Hart
Keyword(s):  
Skeletal Muscle ◽  
Vitamin E ◽  
Muscular Dystrophy ◽  
Extracellular Space ◽  
Partial Replacement ◽  
Vitamin E Deficiency ◽  
Normal Value ◽  
Hereditary Myopathy ◽  
The Mean ◽  
Dystrophic Mice

Exchangeable body potassium (Ke), muscle potassium and muscle sodium concentrations have been measured in hereditarily dystrophic mice and in their normal littermates. Both Ke and K39/gm muscle were depressed in the dystrophics approximately 20% below the mean normal value; however, the concentration of Na23/gm muscle was higher by 50%, on the average, in the dystrophic tissue. The data suggest a partial replacement of intracellular by extracellular space in the dystrophic mice. Thus, this form of hereditary muscular dystrophy is qualitatively similar, with regard to K and Na concentrations, to human muscular dystrophy, vitamin E deficiency in rabbits, nutritional dystrophy in calves, and denervation in puppies.

Biochemical changes in progressive muscular dystrophy. XIII. Nucleic acids, proteins, and total nucleotides in the thymus and spleen of dystrophic mice

1985 ◽  
Vol 63 (5) ◽  
pp. 325-332 ◽  
Author(s):  
Uma Srivastava ◽  
Mikael Sebag ◽  
Manohar Thakur
Keyword(s):  
Skeletal Muscle ◽  
Amino Acid ◽  
Muscular Dystrophy ◽  
Free Amino Acid ◽  
Free Amino ◽  
Specific Activity ◽  
Cellular Growth ◽  
Free Amino Acid Pool ◽  
Acid Pool ◽  
Dystrophic Mice

Assessments were made of the thymus and spleen weights and the total nucleotide, nucleic acid, and protein content as well as the incorporation of [14C]leucine into protein and of [3H]orotate into RNA, in the thymus, spleen, liver, brain, kidney, lungs, heart, pancreas, and skeletal muscle of normal (+/+) and dystrophic (dy/dy) 129 ReJ mice aged 40, 60, or 90 days. The weights of the thymus and spleen were lower at all stages of dystrophy. Total nucleotide and RNA levels per thymus were reduced at 90 days, while total DNA content was decreased at 60 and 90 days. Protein concentrations per thymus were diminished at each stage of the disease. The specific activity of the free amino acid pool and total free nucleotide pool did not show any significant variations in the thymus at any phase of dystrophy. Incorporation of [14C]leucine into protein and of [3H]orotate into RNA was considerably lower in the thymus at each stage of the disease. Total nucleotide content per spleen was decreased at 40 days, with no change at 60 days and followed by an increase at 90 days in the dystrophic mice. DNA, RNA, and protein levels were all reduced in the spleen at each stage of the disease. The specific activity of the free amino acid pool and total free nucleotide pool, as well as the incorporation of [14C]leucine into protein and of [3H]orotate into RNA, showed similar changes in the spleen as noted in the thymus at each phase of dystrophy. These observations indicate that significant alterations in cellular growth occur not only in skeletal muscle and other nonlymphoid organs, but also in the lymphoid organs of dystrophic mice. Such changes in the cellular growth of lymphoid organs could be responsible for an impairment of immunologic responses reflecting thymic atrophy in murine muscular dystrophy.

scholarly journals Reversal of cardiac and skeletal manifestations of Duchenne muscular dystrophy by cardiosphere-derived cells and their exosomes in mdx dystrophic mice and in human Duchenne cardiomyocytes

2017 ◽  
Author(s):  
Mark A. Aminzadeh ◽  
Russell G. Rogers ◽  
Kenneth Gouin ◽  
Mario Fournier ◽  
Rachel E. Tobin ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Mouse Model ◽  
Cardiac Function ◽  
Premature Death ◽  
Next Generation ◽  
Skeletal Myopathy ◽  
Genetic Deficiency ◽  
Dystrophic Mice

Genetic deficiency of dystrophin leads to disability and premature death in Duchenne muscular dystrophy, affecting the heart as well as skeletal muscle. Here we report that cardiosphere-derived cells (CDCs), which are being tested clinically for the treatment of Duchenne cardiomyopathy, improve cardiac and skeletal myopathy in the mdx mouse model of DMD and in human Duchenne cardiomyocytes. Injection of CDCs into the hearts of mdx mice augments cardiac function, ambulatory capacity and survival. Exosomes secreted by human CDCs reproduce the benefits of CDCs in mdx mice and in human Duchenne cardiomyocytes. The findings further motivate the testing of CDCs in Duchenne patients, while identifying exosomes as next-generation therapeutic candidates.

Electrolyte and lipid composition of skeletal and cardiac muscle in mice with hereditary muscular dystrophy

1959 ◽  
Vol 197 (2) ◽  
pp. 487-490 ◽  
Author(s):  
Ho Lee Young ◽  
Wei Young ◽  
Isidore S. Edelman
Keyword(s):  
Skeletal Muscle ◽  
Muscular Dystrophy ◽  
Cardiac Muscle ◽  
Dry Weight ◽  
Phospholipid Content ◽  
Infrared Analysis ◽  
Total Lipids ◽  
Nonesterified Fatty Acids ◽  
Lack Of Information ◽  
Dystrophic Mice

Skeletal and cardiac muscle from strain 129 mice with hereditary muscular dystrophy and from their normal littermates were analyzed for sodium, potassium, total lipids and the separate lipid components. Dystrophic skeletal muscle contained 12–23% less potassium and 68–107% more sodium than the normal anatomical counterparts when referred to fat-free dry weight. Cardiac muscle from dystrophic mice did not differ from the normal in potassium content and had only 20% more total lipid. Fat content was substantially greater in dystrophic skeletal muscle than in normal muscle by the following percentages: 92% for total lipids, 168% for triglycerides, 140% for total cholesterol and 87% for nonesterified fatty acids assayed by infrared analysis. The phospholipid content of dystrophic and normal skeletal muscle, however, was approximately the same. The lack of information concerning interstitial connective tissue content of these tissues bars any precise interpretation of the electrolyte or lipid changes in this genetic myopathy.

scholarly journals Skeletal-muscle sarcolemma from normal and dystrophic mice. Isolation, characterization and lipid composition

1977 ◽  
Vol 168 (2) ◽  
pp. 229-237 ◽  
Author(s):  
T A de Kretser ◽  
B G Livett
Keyword(s):  
Skeletal Muscle ◽  
Muscular Dystrophy ◽  
Neutral Lipid ◽  
Lipid Composition ◽  
Free Cholesterol ◽  
Total Lipid Content ◽  
Dystrophic Muscle ◽  
Mouse Muscle ◽  
Membrane Function ◽  
Dystrophic Mice

1. Mouse skeletal-muscle sarcolemma was isolated, and the preparations obtained from normal mouse muscle and from muscle of mice with hereditary muscular dystrophy were characterized with respect to appearance under the optical and electron microscopes, distribution of marker enzymes, histochemical properties and biochemical composition. 2. The sarcolemmal membranes from normal and dystrophic muscle were subjected to detailed lipied analysis. Total lipid content was shown to increase in sarcolemma from dystrophic mice as a result of a large increase in neutral lipid and a smaller increase in total phospholipids. Further analysis of the neutral-lipid fraction showed that total acylglycerols increased 6-fold, non-esterified fatty acid 4-fold and cholesterol esters 2-fold, whereas the amount of free cholesterol remained unchanged in sarcolemma from dystrophic muscle. Significant increases were found in lysophosphatidylcholine, phosphatidylcholine and phosphatidylethanolamine in dystrophic-muscle sarcolemma; however, the relative composition of the phospholipid fraction remained essentially the same as in the normal case. 3. The overall result of alterations in lipid composition of the sarcolemma in mouse muscular dystrophy was an increase in neutral lipid compared with total phospholipid, and a 4-fold decrease in the relative amount of free cholesterol in the membrane. The possible impact of these changes on membrane function is discussed.

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