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 Cln3Δex7/8-knock-in mice, the latter mimicking the most frequent dis...

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Bibliographic Details
Main Authors: Attila D. Kovács, David A. Pearce
Format: Article
Language:English
Published: The Company of Biologists 2015-04-01
Series:Disease Models & Mechanisms
Subjects:
Online Access:http://dmm.biologists.org/content/8/4/351