[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 …

Laminin-111 improves muscle repair in a mouse model of merosin-deficient congenital muscular dystrophy

PM Van Ry, P Minogue, BL Hodges… - Human molecular …, 2014 - academic.oup.com
Merosin-deficient congenital muscular dystrophy type 1A (MDC1A) is a severe and fatal
muscle-wasting disease with no cure. MDC1A patients and the dyW−/− mouse model exhibit …

Skeletal muscle laminin and MDC1A: pathogenesis and treatment strategies

KI Gawlik, M Durbeej - Skeletal muscle, 2011 - Springer
Laminin-211 is a cell-adhesion molecule that is strongly expressed in the basement
membrane of skeletal muscle. By binding to the cell surface receptors dystroglycan and …

Current understanding and treatment of cardiac and skeletal muscle pathology in laminin-α2 chain-deficient congenital muscular dystrophy

Q Nguyen, KRQ Lim, T Yokota - The application of clinical genetics, 2019 - Taylor & Francis
Congenital muscular dystrophy (CMD) is a class of severe early-onset muscular dystrophies
affecting skeletal/cardiac muscles as well as the central nervous system (CNS). Laminin-α2 …

Transgenic overexpression of the α7 integrin reduces muscle pathology and improves viability in the dyW mouse model of merosin-deficient congenital muscular …

JA Doe, RD Wuebbles, ET Allred… - Journal of cell …, 2011 - journals.biologists.com
Merosin-deficient congenital muscular dystrophy 1A (MDC1A) is a devastating
neuromuscular disease that results in children being confined to a wheelchair, requiring …

Laminin-α2 chain-deficient congenital muscular dystrophy: pathophysiology and development of treatment

M Durbeej - Current topics in membranes, 2015 - Elsevier
Laminin-211 is a major constituent of the skeletal muscle basement membrane. It stabilizes
skeletal muscle and influences signal transduction events from the myomatrix to the muscle …

Transgenic overexpression of laminin α1 chain in laminin α2 chain–deficient mice rescues the disease throughout the lifespan

KI Gawlik, M Durbeej - Muscle & nerve, 2010 - Wiley Online Library
Several approaches to treat laminin α2 chain–deficient congenital muscular dystrophy
(MDC1A) in mouse models have been undertaken. Most have shown promising results in …

[HTML][HTML] Laminin and integrin in LAMA2-related congenital muscular dystrophy: from disease to therapeutics

P Barraza-Flores, CR Bates… - Frontiers in molecular …, 2020 - frontiersin.org
Laminin-α2-related congenital muscular dystrophy (LAMA2-CMD) is a devastating
neuromuscular disease caused by mutations in the LAMA2 gene. These mutations result in …

Laminin-111 protein therapy prevents muscle disease in the mdx mouse model for Duchenne muscular dystrophy

JE Rooney, PB Gurpur… - Proceedings of the …, 2009 - National Acad Sciences
Duchenne muscular dystrophy (DMD) is a devastating neuromuscular disease caused by
mutations in the gene encoding dystrophin. Loss of dystrophin results in reduced …

Laminin-deficient muscular dystrophy: Molecular pathogenesis and structural repair strategies

PD Yurchenco, KK McKee, JR Reinhard, MA Rüegg - Matrix Biology, 2018 - Elsevier
Laminins are large heterotrimers composed of the α, β and γ subunits with distinct tissue-
specific and developmentally regulated expression patterns. The laminin-α2 subunit …