[HTML][HTML] Laminin-111 protein therapy reduces muscle pathology and improves viability of a mouse model of merosin-deficient congenital muscular dystrophy

JE Rooney, JR Knapp, BL Hodges… - The American journal of …, 2012 - Elsevier
Merosin-deficient congenital muscular dystrophy type 1A (MDC1A) is a lethal muscle-
wasting disease that is caused by mutations in the LAMA2 gene, resulting in the loss of …

Duchenne muscular dystrophy: an updated review of common available therapies

A Salmaninejad, SF Valilou, H Bayat… - International Journal …, 2018 - Taylor & Francis
Background and purpose: Duchenne muscular dystrophy (DMD) is a lethal progressive
pediatric muscle disorder and genetically inherited as an X-linked disease that caused by …

Collagen Q and anti-MuSK autoantibody competitively suppress agrin/LRP4/MuSK signaling

K Otsuka, M Ito, B Ohkawara, A Masuda, Y Kawakami… - Scientific reports, 2015 - nature.com
MuSK antibody-positive myasthenia gravis (MuSK-MG) accounts for 5 to 15% of
autoimmune MG. MuSK and LRP4 are coreceptors for agrin in the signaling pathway that …

Postdevelopmental knockout of Orai1 improves muscle pathology in a mouse model of Duchenne muscular dystrophy

M García-Castañeda, A Michelucci, N Zhao… - Journal of General …, 2022 - rupress.org
Duchenne muscular dystrophy (DMD), an X-linked disorder caused by loss-of-function
mutations in the dystrophin gene, is characterized by progressive muscle degeneration and …

Cell–matrix interactions in muscle disease

V Carmignac, M Durbeej - The Journal of pathology, 2012 - Wiley Online Library
The extracellular matrix (ECM) provides a solid scaffold and signals to cells through ECM
receptors. The cell–matrix interactions are crucial for normal biological processes and when …

Common therapeutic advances for Duchenne muscular dystrophy (DMD)

A Salmaninejad, Y Jafari Abarghan… - International Journal …, 2021 - Taylor & Francis
Background and purpose: Duchenne muscular dystrophy (DMD), a lethal X-linked recessive
muscle dystrophy, is resulted in by different mutations including mostly frame-shifting gross …

Skeletal muscle degenerative diseases and strategies for therapeutic muscle repair

M Tabebordbar, ET Wang… - Annual Review of …, 2013 - annualreviews.org
Skeletal muscle is a highly specialized, postmitotic tissue that must withstand chronic
mechanical and physiological stress throughout life to maintain proper contractile function …

Biglycan is an extracellular MuSK binding protein important for synapse stability

AR Amenta, HE Creely, MLT Mercado… - Journal of …, 2012 - Soc Neuroscience
The receptor tyrosine kinase MuSK is indispensable for nerve-muscle synapse formation
and maintenance. MuSK is necessary for prepatterning of the endplate zone anlage and as …

Current translational research and murine models for Duchenne muscular dystrophy

M Rodrigues, Y Echigoya, S Fukada… - Journal of …, 2016 - content.iospress.com
Duchenne muscular dystrophy (DMD) is an X-linked genetic disorder characterized by
progressive muscle degeneration. Mutations in the DMD gene result in the absence of …

Arthritis and Duchenne muscular dystrophy: The role of chondroitin sulfate and its associated proteoglycans in disease pathology and as a diagnostic marker

ML Maciej-Hulme, J Melrose… - American Journal of …, 2023 - journals.physiology.org
Chondroitin sulfate (CS) is a ubiquitous glycosaminoglycan covalently attached to the core
proteins of cell surface, extracellular, and intracellular proteoglycans. The multistep and …