To this end, we isolated microglia from Cln3Δex7/8 homozygous knock-in mice (hereafter referred as Cln3KI/KI), which express the most common loss-of-function allelic variant of the lysosomal gene Cln3 found in patients with juvenile-forms of neuronal ceroid lipofuscinosis (NCL) (Cotman et al., 2002). This evidence concerns the gene CLN3 and infantile neuronal ceroid lipofuscinosis.