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. |