An astrocytic cellular model of Lafora disease to study polyglucosan accumulation and inflammation.

ABSTRACT: Lafora disease (LD) is a devastating form of progressive myoclonus epilepsy characterized by the accumulation of insoluble forms of glycogen [polyglucosan bodies (PGBs)] in the brain and peripheral tissues. It has been proposed that the accumulation of PGBs is pathogenic. Several mouse models of LD have been generated to study the relationship between PGBs and the pathophysiology of LD. However, the use of LD mice is difficult and time consuming; thus, more amenable cellular systems would be desirable. We recently described a cellular model based on the culture of primary postnatal astrocytes from LD mice that are able to accumulate small PGBs. In this study, we extended this astrocytic model by maturing the astrocytes for longer times. These more mature astrocyte cultures accumulated larg

SUBMITTER: Moreno-Estelles M 

PROVIDER: S-EPMC12919960 | biostudies-literature | 2026 Jan

REPOSITORIES: biostudies-literature