Article
Targeted disruption of the Cln3 gene provides a mouse model for Batten disease. The Batten Mouse Model Consortium [corrected].
Neurobiology of disease - 1 Oct 1999
Mitchison H M, Bernard D J, Greene N D, Cooper J D, Junaid M A, Pullarkat R K, de Vos N, Breuning M H, Owens J W, Mobley W C, Gardiner R M, Lake B D, Taschner P E, Nussbaum R L
Abstract excerpt
Batten disease, a degenerative neurological disorder with juvenile onset, is the most common form of the neuronal ceroid lipofuscinoses. Mutations in the CLN3 gene cause Batten disease. To facilitate studies of Batten disease pathogenesis and treatment, a murine model was created by targeted disruption of the Cln3 gene. Mice homozygous for the disrupted Cln3 allele had a neuronal storage disorder resembling that...
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