[HTML][HTML] Bortezomib partially improves laminin α2 chain–deficient muscular dystrophy

Z Körner, CC Fontes-Oliveira, J Holmberg… - The American journal of …, 2014 - Elsevier
Congenital muscular dystrophy, caused by mutations in LAMA2 (the gene encoding laminin
α2 chain), is a severe and incapacitating disease for which no therapy is yet available. We …

Laminin α1 reduces muscular dystrophy in dy2J mice

KI Gawlik, VM Harandi, RY Cheong, Å Petersén… - Matrix Biology, 2018 - Elsevier
Muscular dystrophies, including laminin α2 chain-deficient muscular dystrophy (LAMA2-
CMD), are associated with immense personal, social and economic burdens. Thus, effective …

Membrane glucocorticoid receptors are localised in the extracellular matrix and signal through the MAPK pathway in mammalian skeletal muscle fibres

S Boncompagni, L Arthurton, E Akujuru… - The Journal of …, 2015 - Wiley Online Library
Key points Many studies have previously suggested the existence of stress hormone
receptors on the cell membrane of many cell types, including skeletal muscle fibres; …

[HTML][HTML] Novel acellular scaffold made from decellularized schwann cell sheets for peripheral nerve regeneration

R Junka, X Yu - Regenerative engineering and translational medicine, 2015 - Springer
Extracellular matrix surrounding Schwann cells and neurons provides critical determinants
of cellular phenotype during development as well as essential cues in stimulating and …

[HTML][HTML] At the crossroads of clinical and preclinical research for muscular dystrophy—are we closer to effective treatment for patients?

KI Gawlik - International journal of molecular sciences, 2018 - mdpi.com
Among diseases affecting skeletal muscle, muscular dystrophy is one of the most
devastating and complex disorders. The term 'muscular dystrophy'refers to a heterogeneous …

[HTML][HTML] Antioxidants Reduce Muscular Dystrophy in the dy2J/dy2J Mouse Model of Laminin α2 Chain-Deficient Muscular Dystrophy

VM Harandi, B Moreira Soares Oliveira, V Allamand… - Antioxidants, 2020 - mdpi.com
Congenital muscular dystrophy with laminin α2 chain-deficiency (LAMA2-CMD) is a severe
neuromuscular disorder without a cure. Using transcriptome and proteome profiling as well …

IGF-1/GH axis enhances losartan treatment in Lama2-related muscular dystrophy

A Accorsi, A Kumar, Y Rhee, A Miller… - Human molecular …, 2016 - academic.oup.com
As the complexities of dystrophic pathology have been elucidated over the last few years, it
has become increasingly clear that primary monogenetic defects result in multiple …

[HTML][HTML] Prospective of extracellular matrix and drug correlations in disease management

V Ahmad - Asian Journal of Pharmaceutical Sciences, 2021 - Elsevier
The extracellular matrix (ECM) comprises of many structural molecules that constitute the
extracellular environment. ECM molecules are characterized by specific features like …

[PDF][PDF] ECM-related myopathies and muscular dystrophies: pros and cons of protein therapies

PM Van Ry, TM Fontelonga, P Barraza-Flores… - Compr …, 2017 - researchgate.net
Extracellular matrix (ECM) myopathies and muscular dystrophies are a group of genetic
diseases caused by mutations in genes encoding proteins that provide critical links between …

[HTML][HTML] Mesenchymal stromal cells for sphincter regeneration: role of laminin isoforms upon myogenic differentiation

T Seeger, M Hart, M Patarroyo, B Rolauffs, WK Aicher… - PLoS …, 2015 - journals.plos.org
Multipotent mesenchymal stromal cells (MSCs) are well known for their tri-lineage potential
and ability to differentiate in vitro into osteogenic, chondrogenic or adipogenic lineages. By …