Thursday, June 6, 2013
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Mouse model for
autosomal-dominant leukodystrophy (ADLD)
A mouse model for
adult-onset ADLD could be useful for identifying myelin regeneration
therapies to treat ADLD and multiple sclerosis (MS). ADLD is a rare genetic
disease caused by duplication of lamin
and is often misdiagnosed as chronic progressive MS. Mice engineered to
overexpress Lmnb1 in oligodendrocytes showed increased myelin
abnormalities, demyelination and motor deficits compared with wild-type mice.
Next steps include testing the effect of myelin regeneration therapies in the
Published online June 6, 2013
status not applicable
Heng, M.Y. et al. J.
Clin. Invest.; published online May 15, 2013;
Contact: Ying-Hui Fu, University of California, San
Contact: Louis J. Ptáček,
same affiliation as above
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