Muscular Dystrophy, Emery-Dreifuss

Publication Title: 

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 Lmna?9 mutant mice are models for AD-EDMD and HGPS, respectively. Both animals develop severe tissue pathologies with abbreviated life spans. Like HGPS cells, Lmna(-/-) and Lmna?9 fibroblasts have typically misshapen nuclei.

Chen, Chia-Yen
Chi, Ya-Hui
Mutalif, Rafidah Abdul
Starost, Matthew F.
Myers, Timothy G.
Anderson, Stasia A.
Stewart, Colin L.
Jeang, Kuan-Teh
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