Article
Characterization of New Transgenic Mouse Models for Two Charcot-Marie-Tooth-Causing HspB1 Mutations using the Rosa26 Locus.
Journal of neuromuscular diseases - 27 May 2016
Bouhy Delphine, Geuens Thomas, De Winter Vicky, Almeida-Souza Leonardo, Katona Istvan, Weis Joachim, Hochepied Tino, Goossens Steven, Haigh Jody J, Janssens Sophie, Timmerman Vincent
Abstract excerpt
BACKGROUND: Charcot-Marie-Tooth (CMT) and associated neuropathies, the most common inherited diseases of the peripheral nervous system, remain so far incurable. Three existing murine models of Charcot-Marie-Tooth type 2F (CMT2F) and/or distal hereditary motor neuropathy type IIb (dHMNIIb), caused by mutations in the small heat shock protein B1 gene (HSPB1/HSP27), partially recapitulate the hallmarks of peripheral...
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