Compromised astrocyte function and survival negatively impact neurons in infantile neuronal ceroid lipofuscinosis.

ABSTRACT: The neuronal ceroid lipofuscinoses (NCLs) are the most common cause of childhood dementia and are invariably fatal. Early localized glial activation occurs in these disorders, and accurately predicts where neuronal loss is most pronounced. Recent evidence suggests that glial dysfunction may contribute to neuron loss, and we have now explored this possibility in infantile NCL (INCL, CLN1 disease). We grew primary cultures of astrocytes, microglia, and neurons derived from Ppt1 deficient mice (Ppt1^-/-) and assessed their properties compared to wildtype (WT) cultures, before co-culturing them in different combinations (astrocytes with microglia, astrocytes or microglia with neurons, all three cell types together). These studies revealed that both Ppt1^-/- astrocytes and m

SUBMITTER: Lange J 

PROVIDER: S-EPMC6081811 | biostudies-literature | 2018 Aug

REPOSITORIES: biostudies-literature