{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2024,7,9]],"date-time":"2024-07-09T01:30:15Z","timestamp":1720488615373},"reference-count":29,"publisher":"Wiley","issue":"13","license":[{"start":{"date-parts":[[2009,5,26]],"date-time":"2009-05-26T00:00:00Z","timestamp":1243296000000},"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":[[2009,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Niemann\u2010Pick C (NPC) disease is an autosomal recessive, lethal, neurodegenerative disorder caused by mutations in <jats:italic>NPC1<\/jats:italic>. By using the glial fibrillary acidic protein (GFAP) promoter, we demonstrated previously that astrocyte\u2010specific expression of <jats:italic>Npc1<\/jats:italic> decreased neuronal storage of cholesterol in <jats:italic>Npc1<\/jats:italic><jats:sup>\u2212\/\u2212<\/jats:sup> mice; reduced numbers of axonal spheroids; and produced less degeneration of neurons, reactive astrocytes, and loss of myelin tracts in the central nervous system. GFAP\u2010Npc1, <jats:italic>Npc1<\/jats:italic><jats:sup>\u2212\/\u2212<\/jats:sup> mice exhibited markedly enhanced survival, and death was not associated with the severe terminal weight loss observed in <jats:italic>Npc1<\/jats:italic><jats:sup>\u2212\/\u2212<\/jats:sup> mice. Intestinal transit is delayed in <jats:italic>Npc1<\/jats:italic><jats:sup>\u2212\/\u2212<\/jats:sup> mice but is normal in GFAP\u2010NPC1, <jats:italic>Npc1<\/jats:italic><jats:sup>\u2212\/\u2212<\/jats:sup> until late in the course of their disease. Because glia play an important role in the enteric nervous system, we studied morphology and cholesterol content of intestines from <jats:italic>Npc1<\/jats:italic><jats:sup>\u2212\/\u2212<\/jats:sup> mice and examined the effect of GFAP\u2010promoted restoration of Npc1 in enteric glia. Although the number of neurons was not altered, the total amount of cholesterol stored in the small intestine was decreased, as were the number of neurons with inclusions and the number of inclusions per neuron. We conclude that expression of Npc1 by enteric glial cells can ameliorate the enteric neuropathology, and we speculate that dysfunction of the enteric nervous system contributes to the retarded intestinal transit, weight loss, and demise of <jats:italic>Npc1<\/jats:italic><jats:sup>\u2212\/\u2212<\/jats:sup> mice. \u00a9 2009 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/jnr.22126","type":"journal-article","created":{"date-parts":[[2009,5,26]],"date-time":"2009-05-26T15:24:01Z","timestamp":1243351441000},"page":"2994-3001","source":"Crossref","is-referenced-by-count":14,"title":["Amelioration of enteric neuropathology in a mouse model of Niemann\u2010Pick C by Npc1 expression in enteric glia"],"prefix":"10.1002","volume":"87","author":[{"given":"Raj","family":"Kapur","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carolyn","family":"Donohue","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David","family":"Jelinek","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Robert P.","family":"Erickson","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2009,5,26]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1050-3862(96)00167-2"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0092-8674(00)81571-8"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1053\/j.gastro.2007.08.043"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.231474098"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1159\/000459289"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0006-8993(99)01678-9"},{"key":"e_1_2_6_8_1","first-page":"320","volume-title":"The second brain: a groundbreaking new understanding of nervous disorders of the stomach and intestine","author":"Gershon MD","year":"1998"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/glia.440040211"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00442993"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1023\/A:1010942521671"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1038\/325253a0"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.277.5323.232"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/11.24.3107"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1007\/s002210100870"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1097\/00001756-199503000-00017"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.96.4.1657"},{"key":"e_1_2_6_18_1","first-page":"3611","volume-title":"The metabolic and molecular bases of inherited disease","author":"Patterson MC","year":"2001"},{"key":"e_1_2_6_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0378-4347(00)81820-4"},{"key":"e_1_2_6_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jneumeth.2003.10.004"},{"key":"e_1_2_6_21_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1471-4159.2000.0751250.x"},{"key":"e_1_2_6_22_1","doi-asserted-by":"publisher","DOI":"10.1038\/labinvest.3700600"},{"key":"e_1_2_6_23_1","first-page":"150","article-title":"Lysosome lipd sotrage disorder in NCTR\u2010BALB\/c mice. 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