Thursday, June 6, 2013
Publication and contact
Modeling skeletal muscle
deficits in Huntington's disease (HD)
Mouse muscle fibers could
be useful for modeling HD-associated musculoskeletal deficits and could aid
the development of new therapies to treat the disease. In a mouse model for
HD, ex vivo skeletal muscle fibers were hyperexcitable because of
dysfunction in muscle chloride channel 1 (Clcn1)
and in potassium channels. In the affected muscle fibers, Clcn1 and potassium
channel mRNA levels were both lower than those in wild-type
muscle fibers, and the Clcn1 mRNA appeared aberrantly spliced. Next
steps include assessing the development of the observed muscle fiber deficits
in longitudinal studies, assessing other mouse models and determining whether
such deficits are present in muscle fibers from patients with HD.
Published online June 6, 2013
Patent and licensing status
Waters, C.W. et al.
Proc. Natl. Acad. Sci. USA; published online May 13, 2013;
Contact: Andrew A. Voss, California State Polytechnic
University, Pomona, Calif.
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