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 …

Abnormal calcium handling in Duchenne muscular dystrophy: mechanisms and potential therapies

S Mareedu, ED Million, D Duan, GJ Babu - Frontiers in physiology, 2021 - frontiersin.org
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 …

Important considerations for protein analyses using antibody based techniques: down‐sizing Western blotting up‐sizes outcomes

RM Murphy, GD Lamb - The Journal of physiology, 2013 - Wiley Online Library
Western blotting has been used for protein analyses in a wide range of tissue samples for>
30 years. Fundamental to Western blotting success are a number of important …

Constitutive activation of the calcium sensor STIM1 causes tubular-aggregate myopathy

J Böhm, F Chevessier, AM De Paula, C Koch… - The American Journal of …, 2013 - cell.com
Tubular aggregates are regular arrays of membrane tubules accumulating in muscle with
age. They are found as secondary features in several muscle disorders, including alcohol …

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
Duchenne muscular dystrophy (DMD) is caused by the absence of the dystrophin protein
and a properly functioning dystrophin-associated protein complex (DAPC) in muscle cells …

Orai1-dependent calcium entry promotes skeletal muscle growth and limits fatigue

L Wei-LaPierre, EM Carrell, S Boncompagni… - Nature …, 2013 - nature.com
Abstract Store-operated Ca2+ entry (SOCE) in skeletal muscle involves signalling between
stromal-interacting molecule 1 (STIM1) in the sarcoplasmic reticulum (SR) and Ca2+ …

Dysregulation of calcium homeostasis in muscular dystrophies

A Vallejo-Illarramendi, I Toral-Ojeda… - Expert reviews in …, 2014 - cambridge.org
Muscular dystrophies are a group of diseases characterised by the primary wasting of
skeletal muscle, which compromises patient mobility and in the most severe cases originate …

Altered Ca2+ Handling and Oxidative Stress Underlie Mitochondrial Damage and Skeletal Muscle Dysfunction in Aging and Disease

A Michelucci, C Liang, F Protasi, RT Dirksen - Metabolites, 2021 - mdpi.com
Skeletal muscle contraction relies on both high-fidelity calcium (Ca2+) signals and robust
capacity for adenosine triphosphate (ATP) generation. Ca2+ release units (CRUs) are highly …

Role of STIM1/ORAI1-mediated store-operated Ca2+ entry in skeletal muscle physiology and disease

A Michelucci, M García-Castañeda, S Boncompagni… - Cell calcium, 2018 - Elsevier
Abstract Store-operated Ca 2+ entry (SOCE) is a Ca 2+ entry mechanism activated by
depletion of intracellular Ca 2+ stores. In skeletal muscle, SOCE is mediated by an …

Genetic evidence in the mouse solidifies the calcium hypothesis of myofiber death in muscular dystrophy

AR Burr, JD Molkentin - Cell Death & Differentiation, 2015 - nature.com
Muscular dystrophy (MD) refers to a clinically and genetically heterogeneous group of
degenerative muscle disorders characterized by progressive muscle wasting and often …