ArticlePloS one2010
Distinct roles for laminin globular domains in laminin alpha1 chain mediated rescue of murine laminin alpha2 chain deficiency.
Article in PloS one, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers.
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Who cites it
23 citing papers in PubMed, 40 citations in OpenAlex.
- Dual transgene amelioration of Lama2-null muscular dystrophy.Matrix biology : journal of the International Society for Matrix Biology · 2023Article
- A Family of Laminin α2 Chain-Deficient Mouse Mutants: Advancing the Research on LAMA2-CMD.Frontiers in molecular neuroscience · 2020Review
- Early skeletal muscle pathology and disease progress in the dyScientific reports · 2019Article
- Analysis of α-Dystroglycan/LG Domain Binding Modes: Investigating Protein Motifs That Regulate the Affinity of Isolated LG Domains.Frontiers in molecular biosciences · 2019Review
- Laminin: loss-of-function studies.Cellular and molecular life sciences : CMLS · 2017Review
- Laminin differentially regulates the stemness of type I and type II pericytes.Stem cell research & therapy · 2017Article
- Deletion of integrin α7 subunit does not aggravate the phenotype of laminin α2 chain-deficient mice.Scientific reports · 2015Article
- Quantitative proteomic analysis reveals metabolic alterations, calcium dysregulation, and increased expression of extracellular matrix proteins in laminin α2 chain-deficient muscle.Molecular & cellular proteomics : MCP · 2014Article
- Laminin-111 improves muscle repair in a mouse model of merosin-deficient congenital muscular dystrophy.Human molecular genetics · 2014Article
- Schwann cell myelination requires integration of laminin activities.Journal of cell science · 2012Article
- Integrins in renal development.Pediatric nephrology (Berlin, Germany) · 2012Review
- Laminin-111 protein therapy reduces muscle pathology and improves viability of a mouse model of merosin-deficient congenital muscular dystrophy.The American journal of pathology · 2012Article
- Chronic nerve compression alters Schwann cell myelin architecture in a murine model.Muscle & nerve · 2012Article
- Glia unglued: how signals from the extracellular matrix regulate the development of myelinating glia.Developmental neurobiology · 2011Review
- Non-redundant function of dystroglycan and β1 integrins in radial sorting of axons.Development (Cambridge, England) · 2011Article
- Muscle-specific expression of insulin-like growth factor 1 improves outcome in Lama2Dy-w mice, a model for congenital muscular dystrophy type 1A.Human molecular genetics · 2011Article
- Transgenic expression of Laminin α1 chain does not prevent muscle disease in the mdx mouse model for Duchenne muscular dystrophy.The American journal of pathology · 2011Article
- Skeletal muscle laminin and MDC1A: pathogenesis and treatment strategies.Skeletal muscle · 2011Article
- Basement membranes: cell scaffoldings and signaling platforms.Cold Spring Harbor perspectives in biology · 2011Review
- The dystrophin-glycoprotein complex in the prevention of muscle damage.Journal of biomedicine & biotechnology · 2011Review
Corrections and comments
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Authors and funding
5 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundLaminin alpha2 chain mutations cause congenital muscular dystrophy with dysmyelination neuropathy (MDC1A). Previously, we demonstrated that laminin alpha1 chain ameliorates the disease in mice. Dystroglycan and integrins are major laminin receptors. Unlike laminin alpha2 chain, alpha1 chain binds the receptors by separate domains; laminin globular (LG) domains 4 and LG1-3, respectively. Thus, the laminin alpha1 chain is an excellent tool to distinguish between the roles of dystroglycan and integrins in the neuromuscular system. METHODOLOGY/PRINCIPAL
findingsHere, we provide insights into the functions of laminin alpha1LG domains and the division of their roles in MDC1A pathogenesis and rescue. Overexpression of laminin alpha1 chain that lacks the dystroglycan binding LG4-5 domains in alpha2 chain deficient mice resulted in prolonged lifespan and improved health. Importantly, diaphragm and heart muscles were corrected, whereas limb muscles were dystrophic, indicating that different muscles have different requirements for LG4-5 domains. Furthermore, the regenerative capacity of the skeletal muscle did not depend on laminin alpha1LG4-5. However, this domain was crucial for preventing apoptosis in limb muscles, essential for myelination in peripheral nerve and important for basement membrane assembly. CONCLUSIONS/SIGNIFICANCE: These results show that laminin alpha1LG domains and consequently their receptors have disparate functions in the neuromuscular system. Understanding these interactions could contribute to design and optimization of future medical treatment for MDC1A patients.
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