Absence of Dystrophin Disrupts Skeletal Muscle Signaling: Roles of Ca2+, Reactive Oxygen Species, and Nitric Oxide in the Development of Muscular Dystrophy

DG Allen, NP Whitehead… - Physiological …, 2016 - journals.physiology.org
Dystrophin is a long rod-shaped protein that connects the subsarcolemmal cytoskeleton to a
complex of proteins in the surface membrane (dystrophin protein complex, DPC), with further …

Calcium sparks

H Cheng, WJ Lederer - Physiological reviews, 2008 - journals.physiology.org
The calcium ion (Ca2+) is the simplest and most versatile intracellular messenger known.
The discovery of Ca2+ sparks and a related family of elementary Ca2+ signaling events has …

An injury-responsive Rac-to-Rho GTPase switch drives activation of muscle stem cells through rapid cytoskeletal remodeling

AP Kann, M Hung, W Wang, J Nguyen, PM Gilbert… - Cell Stem Cell, 2022 - cell.com
Many tissues harbor quiescent stem cells that are activated upon injury, subsequently
proliferating and differentiating to repair tissue damage. Mechanisms by which stem cells …

CRISPR-mediated genome editing restores dystrophin expression and function in mdx mice

L Xu, KH Park, L Zhao, J Xu, M El Refaey, Y Gao… - Molecular Therapy, 2016 - cell.com
Duchenne muscular dystrophy (DMD) is a degenerative muscle disease caused by genetic
mutations that lead to the disruption of dystrophin in muscle fibers. There is no curative …

Hypernitrosylated ryanodine receptor calcium release channels are leaky in dystrophic muscle

AM Bellinger, S Reiken, C Carlson, M Mongillo, X Liu… - Nature medicine, 2009 - nature.com
Duchenne muscular dystrophy is characterized by progressive muscle weakness and early
death resulting from dystrophin deficiency. Loss of dystrophin results in disruption of a large …

Microtubules underlie dysfunction in duchenne muscular dystrophy

RJ Khairallah, G Shi, F Sbrana, BL Prosser… - Science …, 2012 - science.org
Duchenne muscular dystrophy (DMD) is a fatal X-linked degenerative muscle disease
caused by the absence of the microtubule-associated protein dystrophin, which results in a …

Calcium-induced calcium release in skeletal muscle

M Endo - Physiological reviews, 2009 - journals.physiology.org
Calcium-induced calcium release (CICR) was first discovered in skeletal muscle. CICR is
defined as Ca2+ release by the action of Ca2+ alone without the simultaneous action of …

Calcium signaling in skeletal muscle development, maintenance and regeneration

MK Tu, JB Levin, AM Hamilton, LN Borodinsky - Cell calcium, 2016 - Elsevier
Skeletal muscle-specific stem cells are pivotal for tissue development and regeneration.
Muscle plasticity, inherent in these processes, is also essential for daily life activities. Great …

Remodeling of ryanodine receptor complex causes “leaky” channels: a molecular mechanism for decreased exercise capacity

AM Bellinger, S Reiken, M Dura… - Proceedings of the …, 2008 - National Acad Sciences
During exercise, defects in calcium (Ca2+) release have been proposed to impair muscle
function. Here, we show that during exercise in mice and humans, the major Ca2+ release …

Modulation of the ryanodine receptor and intracellular calcium

R Zalk, SE Lehnart, AR Marks - Annu. Rev. Biochem., 2007 - annualreviews.org
Abstract Ryanodine receptors (RyRs)/Ca2+ release channels, on the endoplasmic and
sarcoplasmic reticulum of most cell types, are required for intracellular Ca2+ release …