Dysregulation of calcium handling in duchenne muscular dystrophy-associated dilated cardiomyopathy: mechanisms and experimental therapeutic strategies

ML Law, H Cohen, AA Martin, ABB Angulski… - Journal of clinical …, 2020 - mdpi.com
Duchenne muscular dystrophy (DMD) is an X-linked recessive disease resulting in the loss
of dystrophin, a key cytoskeletal protein in the dystrophin-glycoprotein complex. Dystrophin …

Restoration of Sarcoplasmic Reticulum Ca2+ ATPase (SERCA) Activity Prevents Age-Related Muscle Atrophy and Weakness in Mice

R Qaisar, G Pharaoh, S Bhaskaran, H Xu… - International journal of …, 2020 - mdpi.com
Sarcopenia has a significant negative impact on healthspan in the elderly and effective
pharmacologic interventions remain elusive. We have previously demonstrated that …

Single SERCA2a therapy ameliorated dilated cardiomyopathy for 18 months in a mouse model of Duchenne muscular dystrophy

NB Wasala, Y Yue, W Lostal, LP Wasala, N Niranjan… - Molecular therapy, 2020 - cell.com
Loss of dystrophin leads to Duchenne muscular dystrophy (DMD). A pathogenic feature of
DMD is the significant elevation of cytosolic calcium. Supraphysiological calcium triggers …

Mechanical factors tune the sensitivity of mdx muscle to eccentric strength loss and its protection by antioxidant and calcium modulators

A Lindsay, CW Baumann, RT Rebbeck, SL Yuen… - Skeletal muscle, 2020 - Springer
Background Dystrophin deficiency sensitizes skeletal muscle of mice to eccentric contraction
(ECC)-induced strength loss. ECC protocols distinguish dystrophin-deficient from healthy …

Measurement of skeletal muscle fiber contractility with high-speed traction microscopy

M Rausch, D Böhringer, M Steinmann, DW Schubert… - Biophysical journal, 2020 - cell.com
We describe a technique for simultaneous quantification of the contractile forces and
cytosolic calcium dynamics of muscle fibers embedded in three-dimensional biopolymer …

Influence of the MuSK-system on muscle pathology in the mdx mouse model of Duchenne Muscular Dystrophy

B Beqaj - 2020 - ses.library.usyd.edu.au
Duchenne Muscular Dystrophy (DMD) is a severe muscle wasting disorder caused by a
mutation in the dystrophin gene. The dystrophin-deficient mdx mouse is the most common …