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analyzer. Dystrophin restoration increased basal OCR, ATP production , and maximal respiratory capacity in MPCs. Spare respiratory capacity and mitochondrial coupling were improved as well. Four replicate ...
with neuromuscular transmission, muscle contractility, mitochondrial respiration, and carbohydrate metabolism in skeletal muscle. Neuronal NOS is constitutively expressed at the muscle fiber sarcolemma linked ...
therapies aiming to enable dystrophin expression in dystrophic muscle have emerged from preclinical work, with a few of them now entering the clinical arena. Some of these therapeutic strategies, like ...
in the tibialis anterior of the mdx mouse model of DMD. Notably, 3272 proteins were quantifiable and 525 identified as differentially expressed in mdx muscle (P < 0.01). Therapeutic restoration of dystrophin ...
Introduction Duchenne muscular dystrophy (DMD) is an X-linked, recessively inherited neuromuscular disorder caused by lack of dystrophin , the protein product of the DMD gene[1]. Multi-exon deletions ...
in cardiac, but not in skeletal muscle.3 In addition, a short splice variant of dystrophin (Dp71) expressed in cardiac muscle is specifically located in transverse (T)-tubular membranes, though its role ...
proteins (ACTN3, MYOZ, FLNC, MYH1). Heat shock proteins (HSPB1, HSPA9) and protein chaperones had decreased expression in RER. Decreased expression was found for mitochondrial proteins myoglobin (MB), 6 ...
studies in mdx mice to achieve robust dystrophin expression in skeletal muscle and in the heart (22,23). Results Marked cardiac functional changes and damage in mdx and Cmah−/−;mdx mice from 12 weeks of age ...
in kDNA-mutated chickens. Fluorochromes were used to label bone marrow CD3+, CD28+, and CD45+ precursors of the thymus-dependent CD8α+ and CD8β+ effector cells that expressed TCRγδ, vβ1 and vβ2 receptors ...
gene (CAPN3) that encodes a Ca2+-dependent cysteine protease predominantly expressed in the skeletal muscle. Underlying pathological mechanisms have not yet been fully elucidated. Mitochondrial ...
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