Harvest of cell-only muscle fibers using thermally expandable hydrogels with adhesive patterns

YB Lee, S Kim, EM Kim, H Byun… - Tissue Engineering Part C …, 2023 - liebertpub.com
Muscle tissue engineering has been the focus of extensive research due to its potential for
numerous medical applications, including ex vivo actuator development and clinical …

Early skeletal muscle pathology and disease progress in the dy3K/dy3K mouse model of congenital muscular dystrophy with laminin α2 chain-deficiency

KI Gawlik, Z Körner, BM Oliveira, M Durbeej - Scientific Reports, 2019 - nature.com
Deficiency of laminin α2 chain leads to a severe form of congenital muscular dystrophy
(LAMA2-CMD), and dystrophic symptoms progress rapidly in early childhood. Currently …

Immobilization of Dystrophin and Laminin α2-Chain Deficient Zebrafish Larvae In Vivo Prevents the Development of Muscular Dystrophy

M Li, A Arner - PLoS One, 2015 - journals.plos.org
Muscular dystrophies are often caused by genetic alterations in the dystrophin-dystroglycan
complex or its extracellular ligands. These structures are associated with the cell membrane …

Increased polyamines as protective disease modifiers in congenital muscular dystrophy

DU Kemaladewi, JS Benjamin, E Hyatt… - Human molecular …, 2018 - academic.oup.com
Most Mendelian disorders, including neuromuscular disorders, display extensive clinical
heterogeneity that cannot be solely explained by primary genetic mutations. This phenotypic …

Clinical and molecular genetic analysis of a family with late-onset LAMA2-related muscular dystrophy

J Ding, D Zhao, R Du, Y Zhang, H Yang, J Liu… - Brain and …, 2016 - Elsevier
Purpose LAMA2-related muscular dystrophy (LAMA2 MD) is an autosomal recessive
inherited disease caused by LAMA2 gene mutation. The spectrum of the phenotype is …

Pax7, Pax3 and Mamstr genes are involved in skeletal muscle impaired regeneration of dy2J/dy2J mouse model of Lama2-CMD

N Yanay, M Elbaz, J Konikov-Rozenman… - Human molecular …, 2019 - academic.oup.com
Congenital muscular dystrophy type-1A (Lama2-CMD) and Duchenne muscular dystrophy
(DMD) result from deficiencies of laminin-α2 and dystrophin proteins, respectively. Although …

Effects of metformin on congenital muscular dystrophy type 1A disease progression in mice: a gender impact study

CC Fontes-Oliveira, B M. Soares Oliveira, Z Körner… - Scientific reports, 2018 - nature.com
Congenital muscular dystrophy with laminin α2 chain-deficiency (LAMA2-CMD) is a severe
muscle disorder with complex underlying pathogenesis. We have previously employed …

[HTML][HTML] Loss of Dystrophin and β-Sarcoglycan Significantly Exacerbates the Phenotype of Laminin α2 Chain–Deficient Animals

KI Gawlik, J Holmberg, M Durbeej - The American journal of pathology, 2014 - Elsevier
The adhesion molecule laminin α2 chain interacts with the dystrophin-glycoprotein complex,
contributes to normal muscle function, and protects skeletal muscles from damage …

Super-Resolution Imaging Reveals the Nanoscale Distributions of Dystroglycan and Integrin Itga7 in Zebrafish Muscle Fibers

K Shivanna, M Astumian, P Raut, VN Ngo, ST Hess… - Biomedicines, 2023 - mdpi.com
Cell signaling is determined partially by the localization and abundance of proteins.
Dystroglycan and integrin are both transmembrane receptors that connect the cytoskeleton …

Potent pro-inflammatory and pro-fibrotic molecules, osteopontin and galectin-3, are not major disease modulators of laminin α2 chain-deficient muscular dystrophy

KI Gawlik, J Holmberg, M Svensson, M Einerborg… - Scientific reports, 2017 - nature.com
A large number of human diseases are caused by chronic tissue injury with fibrosis
potentially leading to organ failure. There is a need for more effective anti-fibrotic therapies …