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Publication : A murine model for juvenile NCL: gene targeting of mouse Cln3.

First Author  Greene ND Year  1999
Journal  Mol Genet Metab Volume  66
Issue  4 Pages  309-13
PubMed ID  10191119 Mgi Jnum  J:55232
Mgi Id  MGI:1337525 Doi  10.1006/mgme.1999.2828
Citation  Greene ND, et al. (1999) A murine model for juvenile NCL: gene targeting of mouse Cln3. Mol Genet Metab 66(4):309-13
abstractText  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 Cln3, resulting in a null allele. Cln3 null mice appear clinically normal at 5 months of age; however, like JNCL patients, they exhibit intracellular accumulation of autofluorescent material. A second approach will generate mice in which exons 7 and 8 of Cln3 are deleted, mimicking the common mutation in JNCL patients. Copyright 1999 Academic Press.
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