Critical role of intracellular RyR1 calcium release channels in skeletal muscle function and disease

EO Hernández-Ochoa, SJP Pratt, RM Lovering… - Frontiers in …, 2016 - frontiersin.org
The skeletal muscle Ca2+ release channel, also known as ryanodine receptor type 1
(RyR1), is the largest ion channel protein known and is crucial for effective skeletal muscle …

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 …

Therapeutic approaches to preserve the musculature in Duchenne muscular dystrophy: The importance of the secondary therapies

G Angelini, G Mura, G Messina - Experimental Cell Research, 2022 - Elsevier
Muscular dystrophies (MDs) are heterogeneous diseases, characterized by primary wasting
of skeletal muscle, which in severe cases, such as Duchenne Muscular Dystrophy (DMD) …

Endoplasmic reticulum maintains ion homeostasis required for plasma membrane repair

G Chandra, SC Sreetama, DAG Mázala… - Journal of Cell …, 2021 - rupress.org
Of the many crucial functions of the ER, homeostasis of physiological calcium increase is
critical for signaling. Plasma membrane (PM) injury causes a pathological calcium influx …

[HTML][HTML] Mineralocorticoid receptor antagonists and glucocorticoids differentially affect skeletal muscle inflammation and pathology in muscular dystrophy

ZM Howard, CK Gomatam, CP Rabolli, J Lowe… - JCI insight, 2022 - ncbi.nlm.nih.gov
Mineralocorticoid receptor antagonists (MRAs) slow cardiomyopathy in patients with
Duchenne muscular dystrophy (DMD) and improve skeletal muscle pathology and function …

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 …

Profiling of chromatin accessibility in pigs across multiple tissues and developmental stages

J Bai, Y Lin, J Zhang, Z Chen, Y Wang, M Li… - International Journal of …, 2023 - mdpi.com
The study of chromatin accessibility across tissues and developmental stages is essential for
elucidating the transcriptional regulation of various phenotypes and biological processes …

Calcium Mechanisms in Limb-Girdle Muscular Dystrophy with CAPN3 Mutations

J Lasa-Elgarresta, L Mosqueira-Martín… - International journal of …, 2019 - mdpi.com
Limb-girdle muscular dystrophy recessive 1 (LGMDR1), previously known as LGMD2A, is a
rare disease caused by mutations in the CAPN3 gene. It is characterized by progressive …

Multi-omics analysis of sarcospan overexpression in mdx skeletal muscle reveals compensatory remodeling of cytoskeleton-matrix interactions that promote …

JL McCourt, KM Stearns-Reider, H Mamsa, P Kannan… - Skeletal muscle, 2023 - Springer
Background The dystrophin-glycoprotein complex (DGC) is a critical adhesion complex of
the muscle cell membrane, providing a mechanical link between the extracellular matrix …

Sarcolipin deletion in mdx mice impairs calcineurin signalling and worsens dystrophic pathology

VA Fajardo, PJ Chambers, ES Juracic… - Human Molecular …, 2018 - academic.oup.com
Duchenne muscular dystrophy (DMD) is the most severe form of muscular dystrophy
affecting 1 in 3500 live male births. Although there is no cure for DMD, therapeutic strategies …