Pyroptotic cell death in the R6/2 mouse model of Huntington’s disease: new insight on the inflammasome
Emanuela Paldino, Vincenza D’Angelo, Giuseppe Sancesario, Francesca R. Fusco
Abstract
Mechanisms of tissue damage in Huntington's disease involve excitotoxicity, mitochondrial damage, and neuroinflammation, including microglia activation. In the present study, we investigate the role of pyroptosis process in the striatal neurons of the R6/2 mouse model of Huntington's disease. Transgenic mice were sacrificed at 4 and 13 weeks of age. After sacrifice, histological and immunohistochemical studies were performed. We found that NLRP3 and Caspase-1 were intensely expressed in 13-week-old R6/2 mice. Moreover, NLRP3 expression levels were higher in striatal spiny projection neurons and in parvalbumin interneurons, which are prone to degenerate in HD.
Topics & Concepts
InflammasomeHuntington's diseaseProgrammed cell deathDiseaseCell biologyBiologyApoptosisNeuroscienceMedicineImmunologyGeneticsPathologyInflammationInflammasome and immune disordersMitochondrial Function and PathologyHeme Oxygenase-1 and Carbon Monoxide