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Lamin A/C Assembly Defects in LMNA-Congenital Muscular Dystrophy Is Responsible for the Increased Severity of the Disease Compared with Emery–Dreifuss Muscular Dystrophy
LMNA encodes for Lamin A/C, type V intermediate filaments that polymerize under the inner nuclear membrane to form the nuclear lamina. A small fraction of Lamin A/C, less polymerized, is also found in the nucleoplasm. Lamin A/C functions include roles in nuclear resistance to mechanical stress and g...
Autores principales: | Bertrand, Anne T., Brull, Astrid, Azibani, Feriel, Benarroch, Louise, Chikhaoui, Khadija, Stewart, Colin L., Medalia, Ohad, Ben Yaou, Rabah, Bonne, Gisèle |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7226786/ https://www.ncbi.nlm.nih.gov/pubmed/32244403 http://dx.doi.org/10.3390/cells9040844 |
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