Abstract:Background: Lafora disease (LD) is a fatal childhood onset dementia characterized by the extensive accumulation of glycogen aggregates the so-called Lafora Bodies (LBs) in several organs. The accumulation of LBs in the brain underlies the neurological phenotype of the disease. LBs are composed of abnormal glycogen and various associated proteins, including p62, an autophagy adaptor that participates in the aggregation and clearance of misfolded proteins.
Methods: To study the role of p62 in the formation of L… Show more
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