Article
Visual deficits in a mouse model of Batten disease are the result of optic nerve degeneration and loss of dorsal lateral geniculate thalamic neurons.
Neurobiology of disease - 1 May 2006
Weimer Jill M, Custer Andrew W, Benedict Jared W, Alexander Noreen A, Kingsley Evan, Federoff Howard J, Cooper Jonathan D, Pearce David A
Abstract excerpt
Juvenile neuronal ceroid lipofuscinosis (JNCL) is an autosomal recessive disorder of childhood caused by mutations in CLN3. Although visual deterioration is typically the first clinical sign to manifest in affected children, loss of Cln3 in a mouse model of JNCL does not recapitulate this retinal deterioration. This suggests that either the loss of CLN3 does not directly affect retinal cell survival or that...
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