{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,8]],"date-time":"2025-11-08T17:25:39Z","timestamp":1762622739177},"reference-count":35,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2001,7,1]],"date-time":"2001-07-01T00:00:00Z","timestamp":993945600000},"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 Inher Metab Disea"],"published-print":{"date-parts":[[2001,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>A feline model of Niemann\u2013Pick disease type C (NPC) was employed to evaluate the effect of dietary cholesterol restriction on progression of disease. Two NPC\u2010affected treated cats were fed a cholesterol\u2010restricted diet beginning at 8 weeks of age; the cats remained on the diet for 150 and 270 days respectively. The study goal was to lower the amount of low density lipoprotein (LDL) available to cells, hypothetically reducing subsequent lysosomal accumulation of unesterified cholesterol and other lipids. Neurological progression of disease was not altered and dietary cholesterol restriction did not significantly decrease storage in NPC\u2010affected treated cats. One NPC\u2010affected treated cat had decreased serum alkaline phosphatase activity (ALP) and decreased serum cholesterol concentration. Liver lipid concentrations of unesterified cholesterol, cholesterol ester and phospholipids in NPC\u2010affected treated cats were similar to those seen in NPC\u2010affected untreated cats. Ganglioside concentrations in the NPC\u2010affected treated cats and NPC\u2010affected untreated cats were similar. Histological findings in liver sections from NPC\u2010affected treated cats showed a diffuse uniform microvacuolar pattern within hepatocytes and Kupffer cells, in contrast to a heterogeneous macro\/microvacuolar pattern and prominent nodular fibrosis in NPC\u2010affected untreated cats. Similar differences in vacuolar patterns were seen in splenic macrophages. Although some hepatic parameters were modified, dietary cholesterol restriction did not appear to alter disease progression in NPC\u2010affected kittens.<\/jats:p>","DOI":"10.1023\/a:1010588112003","type":"journal-article","created":{"date-parts":[[2002,12,23]],"date-time":"2002-12-23T17:42:08Z","timestamp":1040665328000},"page":"427-436","source":"Crossref","is-referenced-by-count":34,"title":["Effects of dietary cholesterol restriction in a feline model of Niemann\u2013Pick type C disease"],"prefix":"10.1002","volume":"24","author":[{"given":"K. 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