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Dysferlin and the Regulation of Ca2+ Release in Skeletal Muscle [PDF]

open access: yesCells
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
doaj   +2 more sources

Deficient Sarcolemma Repair in ALS: A Novel Mechanism with Therapeutic Potential [PDF]

open access: yesCells, 2022
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
doaj   +2 more sources

From function to structure: how myofibrillogenesis influences the transverse–axial tubular system development and its peculiarities [PDF]

open access: yesFrontiers in Physiology
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
doaj   +2 more sources

Abnormal Calcium Handling in Duchenne Muscular Dystrophy: Mechanisms and Potential Therapies

open access: yesFrontiers in Physiology, 2021
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
doaj   +1 more source

The Dystrophin Node as Integrator of Cytoskeletal Organization, Lateral Force Transmission, Fiber Stability and Cellular Signaling in Skeletal Muscle

open access: yesProteomes, 2021
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
doaj   +1 more source

Reduced Sarcolemmal Membrane Repair Exacerbates Striated Muscle Pathology in a Mouse Model of Duchenne Muscular Dystrophy

open access: yesCells, 2022
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
doaj   +1 more source

Effects of Sarcolemmal Background Ca2+ Entry and Sarcoplasmic Ca2+ Leak Currents on Electrophysiology and Ca2+ Transients in Human Ventricular Cardiomyocytes: A Computational Comparison

open access: yesFrontiers in Physiology, 2022
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
doaj   +1 more source

Biomechanical Properties of the Sarcolemma and Costameres of Skeletal Muscle Lacking Desmin

open access: yesFrontiers in Physiology, 2021
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
doaj   +1 more source

Electron Microscopy of the Sarcolemma [PDF]

open access: yesNature, 1948
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

open access: yesMembranes, 2022
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
doaj   +1 more source

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