Article
Ontogeny of Lafora bodies and neurocytoskeleton changes in Laforin-deficient mice.
Experimental neurology - 1 Jul 2012
Machado-Salas Jesús, Avila-Costa María Rosa, Guevara Patricia, Guevara Jorge, Durón Reyna M, Bai Dongsheng, Tanaka Miyabi, Yamakawa Kazuhiro, Delgado-Escueta Antonio V
Abstract excerpt
Lafora disease (LD) is an autosomal recessive, always fatal progressive myoclonus epilepsy with rapid cognitive and neurologic deterioration. One of the pathological hallmarks of LD is the presence of cytoplasmic PAS+polyglucosan inclusions called Lafora bodies (LBs). Current clinical and neuropathological views consider LBs to be the cause of neurological derangement of patients. A systematic study of the...
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