A murine model for juvenile NCL: gene targeting of mouse Cln3.
Mol Genet Metab
; 66(4): 309-13, 1999 Apr.
Article
in En
| MEDLINE
| ID: mdl-10191119
ABSTRACT
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.
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Collection:
01-internacional
Database:
MEDLINE
Main subject:
Cyclins
/
Saccharomyces cerevisiae Proteins
/
Disease Models, Animal
/
Neuronal Ceroid-Lipofuscinoses
Limits:
Animals
/
Humans
Language:
En
Journal:
Mol Genet Metab
Journal subject:
BIOLOGIA MOLECULAR
/
BIOQUIMICA
/
METABOLISMO
Year:
1999
Document type:
Article
Affiliation country:
United kingdom