[HTML][HTML] Hyperphosphorylation and aggregation of Tau in laforin-deficient mice, an animal model for Lafora disease

R Puri, T Suzuki, K Yamakawa, S Ganesh - Journal of biological chemistry, 2009 - ASBMB
Lafora progressive myoclonous epilepsy (Lafora disease; LD) is caused by mutations in the
EPM2A gene encoding a dual specificity protein phosphatase named laforin. Our analyses
on the Epm2a gene knock-out mice, which developed most of the symptoms of LD, reveal
the presence of hyperphosphorylated Tau protein (Ser 396 and Ser 202) as neurofibrillary
tangles (NFTs) in the brain. Intriguingly, NFTs were also observed in the skeletal muscle
tissues of the knock-out mice. The hyperphosphorylation of Tau was associated with …