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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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| Autors principals: | , , , , , , , |
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| Format: | Artigo |
| Idioma: | Inglês |
| Publicat: |
2012
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| Matèries: | |
| Accés en línia: | 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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