Results 1 to 10 of about 21,278 (246)
Dysferlin and the Regulation of Ca2+ Release in Skeletal Muscle [PDF]
Dysferlin is a large transmembrane protein that is mutated or absent in Limb Girdle Muscular Dystrophy Type R2 (LGMD R2). Although it may have several functions in healthy skeletal muscle, most research on dysferlin has addressed its roles in repair of ...
Robert J. Bloch +2 more
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Deficient Sarcolemma Repair in ALS: A Novel Mechanism with Therapeutic Potential [PDF]
The plasma membrane (sarcolemma) of skeletal muscle myofibers is susceptible to injury caused by physical and chemical stresses during normal daily movement and/or under disease conditions. These acute plasma membrane disruptions are normally compensated
Ang Li +6 more
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From function to structure: how myofibrillogenesis influences the transverse–axial tubular system development and its peculiarities [PDF]
The transverse–axial tubular system (TATS) is the extension of sarcolemma growing to the cell interior, providing sufficient calcium signaling to induce calcium release from sarcoplasmic reticulum cisternae and stimulate the contraction of neighboring ...
Zuzana Sevcikova Tomaskova +1 more
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Abnormal Calcium Handling in Duchenne Muscular Dystrophy: Mechanisms and Potential Therapies
Duchenne muscular dystrophy (DMD) is an X-linked muscle-wasting disease caused by the loss of dystrophin. DMD is associated with muscle degeneration, necrosis, inflammation, fatty replacement, and fibrosis, resulting in muscle weakness, respiratory and ...
Satvik Mareedu +4 more
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The systematic bioanalytical characterization of the protein product of the DMD gene, which is defective in the pediatric disorder Duchenne muscular dystrophy, led to the discovery of the membrane cytoskeletal protein dystrophin.
Paul Dowling +6 more
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Duchenne muscular dystrophy (DMD) is a common X-linked degenerative muscle disorder that involves mutations in the DMD gene that frequently reduce the expression of the dystrophin protein, compromising the structural integrity of the sarcolemmal membrane
Brian J. Paleo +9 more
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The intricate regulation of the compartmental Ca2+ concentrations in cardiomyocytes is critical for electrophysiology, excitation-contraction coupling, and other signaling pathways.
Molly E. Streiff +3 more
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Biomechanical Properties of the Sarcolemma and Costameres of Skeletal Muscle Lacking Desmin
Intermediate filaments (IFs), composed primarily by desmin and keratins, link the myofibrils to each other, to intracellular organelles, and to the sarcolemma.
Karla P. Garcia-Pelagio, Robert J. Bloch
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Electron Microscopy of the Sarcolemma [PDF]
STRIATED muscle fibres are surrounded by a delicate membrane or sheath, the sarcolemma. It has frequently been stated1,2,3 that this sheath is composed of a network of collagenous or reticular fibrils, arranged like a knitted sock. One of us (R. B.) in an investigation to be described elsewhere has failed to find any evidence of fibrillar structure ...
W M, JONES, R, BARER
openaire +2 more sources
Trafficking of Annexins during Membrane Repair in Human Skeletal Muscle Cells
Defects in membrane repair contribute to the development of muscular dystrophies, such as Miyoshi muscular dystrophy 1, limb girdle muscular dystrophy (LGMD), type R2 or R12.
Coralie Croissant +4 more
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