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Proteasome inhibitors reduce thrombospondin-1 release in human dysferlin-deficient myotubes

BACKGROUND: Dysferlinopathies are a group of muscle disorders causing muscle weakness and absence or low levels of dysferlin, a type-II transmembrane protein and the causative gene of these dystrophies. Dysferlin is implicated in vesicle fusion, trafficking, and membrane repair. Muscle biopsy of pat...

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Dades bibliogràfiques
Publicat a:BMC Musculoskelet Disord
Autors principals: Fernández-Simón, Esther, Lleixà, Cinta, Suarez-Calvet, Xavier, Diaz-Manera, Jordi, Illa, Isabel, Gallardo, Eduard, de Luna, Noemí
Format: Artigo
Idioma:Inglês
Publicat: BioMed Central 2020
Matèries:
Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC7697384/
https://ncbi.nlm.nih.gov/pubmed/33246442
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1186/s12891-020-03756-7
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