The role of the NLRP3 inflammasome in a CLN2 mouse model of neuroinflammation

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08 Nov 2026 0 comments

Neuronal ceroid lipofuscinoses, or Batten disease, comprises a group of inherited pediatric neurodegenerative disorders, and is characterized by the lysosomal accumulation of the fluorescent storage material lipofuscin. The human classical late-infantile form of the disorder (CLN2) is caused by mutations in the CLN2 / Tripeptidyl peptidase 1 ( TPP1 ) gene. Comparable to human CLN2, a knockout of the Tpp1 gene ( Tpp1 -/- ) in the mouse leads to progressive vision loss, ataxia, motor atrophy,...