{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,3]],"date-time":"2026-03-03T03:11:21Z","timestamp":1772507481554,"version":"3.50.1"},"reference-count":34,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2009,2,23]],"date-time":"2009-02-23T00:00:00Z","timestamp":1235347200000},"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":[[2009,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Mutations in <jats:italic>CLN6<\/jats:italic> cause variant late\u2010onset neuronal ceroid lipofuscinosis (vLINCL), a childhood neurodegenerative disorder resulting from aberrant neuronal cell loss and pathological accumulation of lysosomal autofluorescent storage material in the central nervous system. The direct function of the endoplasmic reticulum\u2013resident protein CLN6 and how dysfunction of this protein results in vLINCL are unknown. We report that CLN6 interacts with collapsin response mediator protein\u20102 (CRMP\u20102). To further understand the significance and possible contribution to vLINCL of the CLN6\u2013CRMP\u20102 interaction, we utilized the <jats:italic>nclf<\/jats:italic> mouse, which harbors mutations in <jats:italic>CLN6<\/jats:italic>. Significantly, CRMP\u20102 protein level was found to be reduced in the <jats:italic>nclf<\/jats:italic> mouse brain, particularly in the thalamus. Because CRMP\u20102 functions in growth cone collapse and is an effector protein downstream of Sema3A signaling, this pathway was examined via a dorsal root ganglion (DRG) repulsion assay. However, there were no defects in the repulsion of DRGs derived from <jats:italic>nclf<\/jats:italic> mice, indicating that the loss of CLN6 does not affect Sema3A signaling. CRMP\u20102 has also been implicated in controlling axon number and outgrowth, as observed in cultured hippocampal neurons. Therefore, we explored the formation and maturation of hippocampal neurons derived from <jats:italic>nclf<\/jats:italic> mice in a glial coculture system. The maturation of these neurons was reduced; by day in vitro (DIV) 8, more than 50% of <jats:italic>nclf<\/jats:italic>\u2010derived hippocampal neurons had died. Additionally, beginning around DIV4, <jats:italic>nclf<\/jats:italic> neurons were less mature than their WT counterparts, presumably because of an inability to form mature synaptic connections. We concluded that alterations in neurite maturation resulting from a loss of CLN6\u2013CRMP\u20102 interaction may contribute to neuronal dysfunction and pathology in vLINCL. \u00a9 2009 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/jnr.22032","type":"journal-article","created":{"date-parts":[[2009,2,23]],"date-time":"2009-02-23T18:19:20Z","timestamp":1235413160000},"page":"2157-2166","source":"Crossref","is-referenced-by-count":34,"title":["Protein product of <i>CLN6<\/i> gene responsible for variant late\u2010onset infantile neuronal ceroid lipofuscinosis interacts with CRMP\u20102"],"prefix":"10.1002","volume":"87","author":[{"given":"Jared W.","family":"Benedict","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Amanda L.","family":"Getty","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Thomas M.","family":"Wishart","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Thomas H.","family":"Gillingwater","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David A.","family":"Pearce","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2009,2,23]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/neu.10269"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1128\/MCB.25.22.9973-9984.2005"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1468-1331.2007.01692.x"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00234-007-0220-6"},{"key":"e_1_2_6_6_1","volume-title":"Rat Hippocampal Neurons in Low\u2010Density Culture","author":"Banker G","year":"1991"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-8628(19980526)77:4<289::AID-AJMG8>3.0.CO;2-I"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.35.9.717"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.3184-04.2004"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.C400412200"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1751-0813.2002.tb10847.x"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.08-04-01454.1988"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1038\/ncb825"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1086\/338190"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M400643200"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1080\/09687860600967317"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1042\/BJ20030598"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.1038\/90476"},{"key":"e_1_2_6_19_1","doi-asserted-by":"publisher","DOI":"10.1006\/mgme.1999.2821"},{"key":"e_1_2_6_20_1","doi-asserted-by":"publisher","DOI":"10.1128\/MCB.25.22.9920-9935.2005"},{"key":"e_1_2_6_21_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1471-4159.2005.03063.x"},{"key":"e_1_2_6_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/0022-510X(78)90087-4"},{"key":"e_1_2_6_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.yexcr.2004.04.042"},{"key":"e_1_2_6_24_1","unstructured":"MoleSEea.2008. 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