Article
Retinal pathology and function in a Cln3 knockout mouse model of juvenile Neuronal Ceroid Lipofuscinosis (batten disease).
Molecular and cellular neurosciences - 1 Apr 2002
Seigel Gail M, Lotery Andrew, Kummer Ari, Bernard David J, Greene Nicholas D E, Turmaine Mark, Derksen Todd, Nussbaum Robert L, Davidson Beverly, Wagner Janet, Mitchison Hannah M
Abstract excerpt
Batten disease or JNCL, is the juvenile form of Neuronal Ceroid Lipofuscinosis (NCL) an autosomal recessive neurodegenerative disorder. Since retinal degeneration is an early consequence of Batten disease, we examined the eyes of Cln3 knockout mice (1-20 months of age), along with heterozygotes and appropriate controls, to determine whether or not the Cln3 defect would lead to characteristic retinal degeneration...
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