Ion channels of the sarcolemma and intracellular organelles in Duchenne muscular dystrophy: a role in the dysregulation of ion homeostasis and a possible target for …

MV Dubinin, KN Belosludtsev - International Journal of Molecular …, 2023 - mdpi.com
… cardiac and skeletal muscle function in dystrophin-deficient … The loss of dystrophin and DAPC
integrity in DMD makes … by the dysfunctional contractile apparatus of skeletal muscles [115]…

Mechanisms of skeletal muscle repair and regeneration in health and disease

DE Michele - The FEBS Journal, 2022 - Wiley Online Library
… to mechanical muscle contraction. Fully differentiated … from injuries or loss of the capacity
to repair muscle after injury is … muscular dystrophies that show loss of sarcolemma integrity, …

Mimicking sarcolemmal damage in vitro: a contractile 3D model of skeletal muscle for drug testing in Duchenne muscular dystrophy

A Tejedera-Villafranca, M Montolio… - …, 2023 - iopscience.iop.org
contractile regimes induced the loss of myotube integrity, … of dystrophin levels due to limited
uptake by skeletal muscle … of long twitch contraction cycles on sarcolemmal integrity in the …

The dystrophin node as integrator of cytoskeletal organization, lateral force transmission, fiber stability and cellular signaling in skeletal muscle

P Dowling, S Gargan, S Murphy, M Zweyer, H Sabir… - Proteomes, 2021 - mdpi.com
… at the muscle periphery, as well as providing sarcolemmal stabilization and contractile force
… in (i) the provision of sarcolemmal membrane integrity via a stabilizing linkage between the …

Reduced sarcolemmal membrane repair exacerbates striated muscle pathology in a mouse model of Duchenne Muscular Dystrophy

BJ Paleo, KE McElhanon, HR Bulgart, KK Banford… - Cells, 2022 - mdpi.com
… the dystrophin protein, compromising the structural integrity … decrease in muscle ex vivo
contractile function as compared … dysferlin function to compensate for the loss of dystrophin in the …

Molecular and cellular basis of genetically inherited skeletal muscle disorders

JJ Dowling, CC Weihl, MJ Spencer - Nature Reviews Molecular Cell …, 2021 - nature.com
… to the myofibres or function in regulating sarcolemmal integrity, … In skeletal muscle, dystrophin
is enriched at costameres, … the contractile unit of the myofibre — connect to the sarcolemma

The role of the dystrophin glycoprotein complex in muscle cell mechanotransduction

DGS Wilson, A Tinker, T Iskratsch - Communications Biology, 2022 - nature.com
… a critical role in maintaining the integrity of the sarcolemma. … Dystrophin further acts to
mitigate contraction-induced injury … Improvement in skeletal muscle function enabled higher …

Skeletal muscle: A review of molecular structure and function, in health and disease

K Mukund, S Subramaniam - Wiley Interdisciplinary Reviews …, 2020 - Wiley Online Library
… most basic units that drive skeletal muscle contraction, namely (a) … a loss in sarcolemmal
integrity making muscle vulnerable to stresses leading to various types of muscular dystrophies

Biomechanical properties of the sarcolemma and Costameres of skeletal muscle lacking Desmin

KP Garcia-Pelagio, RJ Bloch - Frontiers in Physiology, 2021 - frontiersin.org
… that the sarcolemma and its costameric links to nearby contractile … values determined for
muscles lacking dystrophin or synemin … the peripheral myofibrils and the sarcolemma were lost. …

Mechanics of dystrophin deficient skeletal muscles in very young mice and effects of age

MA Lopez, S Bontiff, M Adeyeye… - … of Physiology-Cell …, 2021 - journals.physiology.org
… from the sarcolemma and renders muscle prone to contraction-… We have shown that loss of
dystrophin causes aberrant … study and took responsibility for the integrity of the data and the …