{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,5]],"date-time":"2026-05-05T03:19:16Z","timestamp":1777951156125,"version":"3.51.4"},"reference-count":37,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2011,10,19]],"date-time":"2011-10-19T00:00:00Z","timestamp":1318982400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Neuroscience Research"],"published-print":{"date-parts":[[2012,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Mutations in the <jats:italic>CLN6<\/jats:italic> gene cause a variant form of late infantile neuronal ceroid lipofuscinosis, a relentless neurodegenerative disease that is inherited as an autosomal recessive trait in humans and in the naturally occurring <jats:italic>nclf<\/jats:italic> mouse strain. The CLN6 protein is localized in the endoplasmic reticulum, but it has an unknown function. To develop a molecular understanding of neurodegeneration induced by mutations in CLN6, we examined the spatial and temporal distribution of <jats:italic>Cln6<\/jats:italic> mRNA expression in murine brain. By using Northern blot and tissue qPCR array techniques, a single <jats:italic>Cln6<\/jats:italic> transcript was detected throughout the adult brain, with greatest expression in the cerebellum and hypothalamus. Real\u2010time qPCR showed 2.4\u20134\u2010fold increases in <jats:italic>Cln6<\/jats:italic> mRNA levels in the cortex and cerebellum during the first 28 days of life, with less prominent enhancement of expression in the hippocampus. In situ hybridization analyses demonstrated <jats:italic>Cln6<\/jats:italic> expression in brainstem, dentate gyrus, and hippocampal neurons of newborn P0 mice. From P14 onward, <jats:italic>Cln6<\/jats:italic> expression is widely distributed throughout the brain and is most prominent in cells of cortical layers II\u2013VI, the Purkinje cell layer, dentate gyrus, and hippocampal CA1 region of adult mice. In different regions of the brain in P0 and P28 <jats:italic>nclf<\/jats:italic> mice, the <jats:italic>Cln6<\/jats:italic> mRNA abundance was reduced by 30\u201340% compared with control mice. These findings implicate Cln6 in the survival and maturation of specific neuronal populations during development and make it possible to compare regional Cln6 expression with the distribution of subsequent pathology. \u00a9 2011 Wiley Periodicals, Inc.<\/jats:p>","DOI":"10.1002\/jnr.22773","type":"journal-article","created":{"date-parts":[[2011,10,19]],"date-time":"2011-10-19T12:07:41Z","timestamp":1319026061000},"page":"568-574","source":"Crossref","is-referenced-by-count":11,"title":["High expression of disease\u2010related <i>Cln6<\/i> in the cerebral cortex, purkinje cells, dentate gyrus, and hippocampal ca1 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