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Finding the most appropriate mouse model of juvenile CLN3 (Batten) disease for therapeutic studies: the importance of genetic background and gender

Mutations in the CLN3 gene cause a fatal neurodegenerative disorder: juvenile CLN3 disease, also known as juvenile Batten disease. The two most commonly utilized mouse models of juvenile CLN3 disease are Cln3-knockout (Cln3(−/−)) and Cln3(Δex7/8)-knock-in mice, the latter mimicking the most frequent...

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Detalles Bibliográficos
Autores principales: Kovács, Attila D., Pearce, David A.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Company of Biologists Limited 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4381334/
https://www.ncbi.nlm.nih.gov/pubmed/26035843
http://dx.doi.org/10.1242/dmm.018804