A murine model for juvenile NCL: Gene targeting of mouse Cln3

JNCL is a neurodegenerative disease of childhood caused by mutations in the CLN3 gene. A mouse model for JNCL was created by disrupting exons 1-6 of C ln3, resulting in a null allele, Cln3 null mice appear clinically normal at 5 months of age; however, like JNCL patients, they exhibit intracellular a ccumulation of autofluorescent material, A second approach will generate mi ce in which exons 7 and 8 of Cln3 are deleted, mimicking the common mutatio n in JNCL patients, (C) 1999 Academic Press.