ArticleJournal of inherited metabolic disease2000
Pharmacological and genetic modifications of somatic cholesterol do not substantially alter the course of CNS disease in Niemann-Pick C mice.
Article in Journal of inherited metabolic disease, 2000. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 21 papers.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
21 citing papers in PubMed, 82 citations in OpenAlex.
- Glycosphingolipids are linked to elevated neurotransmission and neurodegeneration in a Drosophila model of Niemann Pick type C.Disease models & mechanisms · 2023Article
- Treatment trials in Niemann-Pick type C disease.Metabolic brain disease · 2021Review
- Neurodegeneration in Niemann-Pick Type C Disease: An Updated Review on Pharmacological and Non-Pharmacological Approaches to Counteract Brain and Cognitive Impairment.International journal of molecular sciences · 2021Review
- Haploinsufficiency of tau decreases survival of the mouse model of Niemann-Pick disease type C1 but does not alter tau phosphorylation.Journal of applied genetics · 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
- Pre-clinical Mouse Models of Neurodegenerative Lysosomal Storage Diseases.Frontiers in molecular biosciences · 2020Review
- Co-treatment with probucol does not improve lung pathology in hydroxypropyl-β-cyclodextrin-treated Npc1Journal of applied genetics · 2019Article
- Niemann-Pick type C disease: The atypical sphingolipidosis.Advances in biological regulation · 2018Review
- The potential of histone deacetylase inhibitors in Niemann - Pick type C disease.The FEBS journal · 2013Review
- Therapeutic potential of cyclodextrins in the treatment of Niemann-Pick type C disease.Clinical lipidology · 2012Article
- Pulmonary function and pathology in hydroxypropyl-beta-cyclodextin-treated and untreated Npc1⁻/⁻ mice.Molecular genetics and metabolism · 2011Article
- Niemann-Pick type C pathogenesis and treatment: from statins to sugars.Clinical lipidology · 2010Article
- Treatment of a child diagnosed with Niemann-Pick disease type C with miglustat: a case report in Brazil.Journal of inherited metabolic disease · 2008Article
- Niemann-Pick C1 functions in regulating lysosomal amine content.The Journal of biological chemistry · 2008Article
- The natural history of Niemann-Pick disease type C in the UK.Journal of inherited metabolic disease · 2007Article
- Cyclin-dependent kinase inhibitors attenuate protein hyperphosphorylation, cytoskeletal lesion formation, and motor defects in Niemann-Pick Type C mice.The American journal of pathology · 2004Article
- Niemann-Pick disease type C in adults.Journal of inherited metabolic disease · 2002Article
- Deregulation of cdk5, hyperphosphorylation, and cytoskeletal pathology in the Niemann-Pick type C murine model.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2002Article
- Effects of dietary cholesterol restriction in a feline model of Niemann-Pick type C disease.Journal of inherited metabolic disease · 2001Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
5 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Niemann-Pick type C (NPC) is a neurodegenerative disorder with somatically altered cholesterol metabolism. The NPC1 gene has recently been cloned and shown to have sequences shared with known sterol-sensing proteins. We have used a mouse model with a disrupted Npc1 gene to study two cholesterol-lowering drugs (nifedipine and probucol) and the effects of introducing a null mutation in the low-density lipoprotein receptor (LDLR). Although these treatments significantly ameliorated liver cholesterol storage, little effect on the onset of neurological symptoms was found.
Indexed as
Identifiers
What OpenQuestion holds
Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.