Critical role of intracellular RyR1 calcium release channels in skeletal muscle function and disease
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 …
(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 …
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) …
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 …
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 …
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
Background Dystrophin deficiency sensitizes skeletal muscle of mice to eccentric contraction
(ECC)-induced strength loss. ECC protocols distinguish dystrophin-deficient from healthy …
(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 …
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 …
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 …
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 …
affecting 1 in 3500 live male births. Although there is no cure for DMD, therapeutic strategies …