Cargando…
Linker molecules between laminins and dystroglycan ameliorate laminin-α2–deficient muscular dystrophy at all disease stages
Mutations in laminin-α2 cause a severe congenital muscular dystrophy, called MDC1A. The two main receptors that interact with laminin-α2 are dystroglycan and α7β1 integrin. We have previously shown in mouse models for MDC1A that muscle-specific overexpression of a miniaturized form of agrin (mini-ag...
Autores principales: | Meinen, Sarina, Barzaghi, Patrizia, Lin, Shuo, Lochmüller, Hanns, Ruegg, Markus A. |
---|---|
Formato: | Texto |
Lenguaje: | English |
Publicado: |
The Rockefeller University Press
2007
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2064083/ https://www.ncbi.nlm.nih.gov/pubmed/17389231 http://dx.doi.org/10.1083/jcb.200611152 |
Ejemplares similares
-
Angiotensin II type 1 receptor antagonists alleviate muscle pathology in the mouse model for laminin-α2-deficient congenital muscular dystrophy (MDC1A)
por: Meinen, Sarina, et al.
Publicado: (2012) -
An atypical expression of core α-Dystroglycan and Laminin-α2 in skin fibroblasts of patients with congenital muscular dystrophies
por: Sabry, Sahar, et al.
Publicado: (2023) -
Improving Reproducibility of Phenotypic Assessments in the DyW Mouse Model of Laminin-α2 Related Congenital Muscular Dystrophy
por: Willmann, Raffaella, et al.
Publicado: (2017) -
Structural basis of laminin binding to the LARGE glycans on dystroglycan
por: Briggs, David C., et al.
Publicado: (2016) -
Dysregulation of matricellular proteins is an early signature of pathology in laminin-deficient muscular dystrophy
por: Mehuron, Thomas, et al.
Publicado: (2014)