Accumulation of the Inner Nuclear Envelope Protein Sun1 is Pathogenic in Progeric and Dystrophic Laminopathies

Human LMNA gene mutations result in laminopathies that include Emery-Dreifuss Muscular Dystrophy (AD-EDMD) and Hutchinson-Gilford Progeria, the premature aging syndrome (HGPS). The Lmna null (Lmna(−/−)) and progeroid LmnaΔ9 mutant mice are models for AD-EDMD and HGPS respectively. Both animals devel...

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Detalhes bibliográficos
Main Authors: Chen, Chia-Yen, Chi, Ya-Hui, Mutalif, Rafidah Abdul, Starost, Matthew F., Myers, Timothy G., Anderson, Stasia A., Stewart, Colin L., Jeang, Kuan-Teh
Formato: Artigo
Idioma:Inglês
Publicado em: 2012
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Article
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC3340584/
https://ncbi.nlm.nih.gov/pubmed/22541428
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.cell.2012.01.059
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