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Chemical chaperone ameliorates pathological protein aggregation in plectin-deficient muscle
The ubiquitously expressed multifunctional cytolinker protein plectin is essential for muscle fiber integrity and myofiber cytoarchitecture. Patients suffering from plectinopathy-associated epidermolysis bullosa simplex with muscular dystrophy (EBS-MD) and mice lacking plectin in skeletal muscle dis...
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| Main Authors: | , , , , , , |
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| Formato: | Artigo |
| Idioma: | Inglês |
| Publicado em: |
American Society for Clinical Investigation
2014
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| Assuntos: | |
| Acesso em linha: | https://ncbi.nlm.nih.gov/pmc/articles/PMC3934181/ https://ncbi.nlm.nih.gov/pubmed/24487589 https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1172/JCI71919 |
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