ArticleJournal of inherited metabolic disease2001
Effects of dietary cholesterol restriction in a feline model of Niemann-Pick type C disease.
Article in Journal of inherited metabolic disease, 2001. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers.
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Who cites it
20 citing papers in PubMed, 37 citations in OpenAlex.
- Delineation of metabolic responses of Npc1Steroids · 2020Article
- Understanding and Treating Niemann-Pick Type C Disease: Models Matter.International journal of molecular sciences · 2020Review
- Dietary plant stanol ester supplementation reduces peripheral symptoms in a mouse model of Niemann-Pick type C1 disease.Journal of lipid research · 2020Article
- Niemann-Pick C1-deficient mice lacking sterol O-acyltransferase 2 have less hepatic cholesterol entrapment and improved liver function.American journal of physiology. Gastrointestinal and liver physiology · 2018Article
- Mitochondrial GSH replenishment as a potential therapeutic approach for Niemann Pick type C disease.Redox biology · 2017Article
- Cyclodextrins and Iatrogenic Hearing Loss: New Drugs with Significant Risk.Frontiers in cellular neuroscience · 2017Review
- Diagnostic workup and management of patients with suspected Niemann-Pick type C disease.Therapeutic advances in neurological disorders · 2016Review
- Intracisternal cyclodextrin prevents cerebellar dysfunction and Purkinje cell death in feline Niemann-Pick type C1 disease.Science translational medicine · 2015Article
- Aging in the canine and feline brain.The Veterinary clinics of North America. Small animal practice · 2014Review
- The potential of histone deacetylase inhibitors in Niemann - Pick type C disease.The FEBS journal · 2013Review
- Treatment of Niemann--pick type C disease by histone deacetylase inhibitors.Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics · 2013Review
- Electrodiagnostic testing and histopathologic changes confirm peripheral nervous system myelin abnormalities in the feline model of niemann-pick disease type C.Journal of neuropathology and experimental neurology · 2013Article
- Microarray expression analysis and identification of serum biomarkers for Niemann-Pick disease, type C1.Human molecular genetics · 2012Article
- Therapeutic potential of cyclodextrins in the treatment of Niemann-Pick type C disease.Clinical lipidology · 2012Article
- Miglustat improves purkinje cell survival and alters microglial phenotype in feline Niemann-Pick disease type C.Journal of neuropathology and experimental neurology · 2012Article
- 2-hydroxypropyl-beta-cyclodextrin raises hearing threshold in normal cats and in cats with Niemann-Pick type C disease.Pediatric research · 2010Article
- Niemann-Pick type C pathogenesis and treatment: from statins to sugars.Clinical lipidology · 2010Article
- Clinical, electrophysiological, and serum biochemical measures of progressive neurological and hepatic dysfunction in feline Niemann-Pick type C disease.Pediatric research · 2008Article
- Niemann-Pick C1 functions in regulating lysosomal amine content.The Journal of biological chemistry · 2008Article
- In vivo antisense oligonucleotide reduction of NPC1 expression as a novel mouse model for Niemann Pick type C- associated liver disease.Hepatology (Baltimore, Md.) · 2008Article
Corrections and comments
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Authors and funding
12 authors at 2 institutions in 1 country.
Funding
Abstract
A feline model of Niemann-Pick disease type C (NPC) was employed to evaluate the effect of dietary cholesterol restriction on progression of disease. Two NPC-affected treated cats were fed a cholesterol-restricted 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-affected treated cats. One NPC-affected 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-affected treated cats were similar to those seen in NPC-affected untreated cats. Ganglioside concentrations in the NPC-affected treated cats and NPC-affected untreated cats were similar. Histological findings in liver sections from NPC-affected 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-affected 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-affected kittens.
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