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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...

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Detalhes bibliográficos
Publicado no:Cells
Main Authors: Bertrand, Anne T., Brull, Astrid, Azibani, Feriel, Benarroch, Louise, Chikhaoui, Khadija, Stewart, Colin L., Medalia, Ohad, Ben Yaou, Rabah, Bonne, Gisèle
Formato: Artigo
Idioma:Inglês
Publicado em: MDPI 2020
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Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC7226786/
https://ncbi.nlm.nih.gov/pubmed/32244403
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.3390/cells9040844
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