Journal
NEUROBIOLOGY OF DISEASE
Volume 22, Issue 2, Pages 284-293Publisher
ACADEMIC PRESS INC ELSEVIER SCIENCE
DOI: 10.1016/j.nbd.2005.11.008
Keywords
juvenile neuronal ceroid lipofuscinosis; CLN3; lateral geniculate nucleus; thalamus; Axonal transport
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Funding
- NIMH NIH HHS [T32 MH065181] Funding Source: Medline
- NINDS NIH HHS [R01 NS044310, NS41930, NS40580, R21 NS041930, NS44310, R21 NS040580] Funding Source: Medline
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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 nuclei involved in visual processing are affected prior to retinal degeneration. Having previously demonstrated that Cln3(-/-) mice have decreased optic nerve axonal density, we now demonstrate a decrease in nerve conduction. Examination of retino-recipient regions revealed a decreased number of neurons within the dorsal lateral geniculate nucleus (LGNd). We demonstrate decreased transport of amino acids from the retina to the LGN, suggesting an impediment in communication between the retina and projection nuclei. This study defines a novel path of degeneration within the LGNd, providing a mechanism for causation of JNCL visual deficits. (c) 2005 Elsevier Inc. All rights reserved.
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