Abstract | BACKGROUND: METHODS: Sections of skeletal muscle from LmnaH222P/H222P mice were stained with hematoxylin and eosin and histological analysis performed using light microscopy. ERK1/2 activity was assessed in mouse tissue and cultured cells by immunoblotting and real-time polymerase chain reaction to measure expression of downstream target genes. LmnaH222P/H222P mice were treated with selumetinib, which blocks mitogen-activated protein kinase/ extracellular signal-regulated kinase kinase 1/2 that activates ERK1/2, from 16 to 20 weeks of age to assess the effects of treatment on muscle histology, ERK1/2 activity and limb grip strength. RESULTS: CONCLUSIONS: Our results show that ERK1/2 plays a role in the development of skeletal muscle pathology in LmnaH222/H222P mice. They further provide the first evidence that a small molecule drug may be beneficial for skeletal muscle in autosomal Emery-Dreifuss muscular dystrophy.
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Authors | Antoine Muchir, Young Jin Kim, Sarah A Reilly, Wei Wu, Jason C Choi, Howard J Worman |
Journal | Skeletal muscle
(Skelet Muscle)
Vol. 3
Issue 1
Pg. 17
(Jul 01 2013)
ISSN: 2044-5040 [Print] England |
PMID | 23815988
(Publication Type: Journal Article)
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