The pathogenesis of Niemann-Pick type C disease: a role for autophagy? (Q24650767)

Expert Reviews in Molecular Medicine

Europe PubMed Central

title

The pathogenesis of Niemann-Pick type C disease: a role for autophagy? (English)

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1 reference

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Chris D Pacheco

1

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Andrew P Lieberman

series ordinal

2

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language of work or name

English

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publication date

2008

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published in

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volume

10

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page(s)

e26

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cites work

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28 September 2017

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28 September 2017

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28 September 2017

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Paired helical filament tau (PHFtau) in Niemann-Pick type C disease is similar to PHFtau in Alzheimer's disease

28 September 2017

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28 September 2017

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28 September 2017

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The intracellular transport of low density lipoprotein-derived cholesterol is defective in Niemann-Pick type C fibroblasts

28 September 2017

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Clinical Aspects of Niemann-Pick Type C Disease in the Adult

28 September 2017

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28 September 2017

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Genotype-phenotype relationship of Niemann-Pick disease type C: a possible correlation between clinical onset and levels of NPC1 protein in isolated skin fibroblasts

30 May 2018

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Murine model of Niemann-Pick C disease: mutation in a cholesterol homeostasis gene.

30 May 2018

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30 May 2018

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The effect of cholesterol-lowering agents on hepatic and plasma cholesterol in Niemann-Pick disease type C.

30 May 2018

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30 May 2018

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A clinical staging classification for type C Niemann-Pick disease.

30 May 2018

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Novel oral treatment of Gaucher's disease with N-butyldeoxynojirimycin (OGT 918) to decrease substrate biosynthesis.

30 May 2018

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Cholesterol accumulation in NPC1-deficient neurons is ganglioside dependent

21 January 2018

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Neurodegeneration in heterozygous Niemann-Pick type C1 (NPC1) mouse: implication of heterozygous NPC1 mutations being a risk for tauopathy

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Cerebellar degeneration in the Niemann-Pick type C mouse.

21 January 2018

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Perturbed myelination process of premyelinating oligodendrocyte in Niemann-Pick type C mouse

21 January 2018

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Cholesterol is sequestered in the brains of mice with Niemann-Pick type C disease but turnover is increased.

21 January 2018

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Secretion of sterols and the NPC2 protein from primary astrocytes

21 January 2018

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Type C Niemann-Pick disease in a boxer dog.

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A Drosophila model of the Niemann-Pick type C lysosome storage disease: dnpc1a is required for molting and sterol homeostasis

21 January 2018

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A model for niemann-pick type C disease in the nematode Caenorhabditis elegans

21 January 2018

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Niemann-Pick disease type C (a cellular cholesterol lipidosis) treated by bone marrow transplantation

21 January 2018

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The transport of low density lipoprotein-derived cholesterol to the plasma membrane is defective in NPC1 cells

21 January 2018

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27-hydroxycholesterol is an endogenous ligand for liver X receptor in cholesterol-loaded cells

21 January 2018

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CNS synaptogenesis promoted by glia-derived cholesterol

21 January 2018

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Ontogenesis and regulation of cholesterol metabolism in the central nervous system of the mouse

21 January 2018

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Niemann-Pick disease type C in adults

21 January 2018

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Enhanced survival in Sandhoff disease mice receiving a combination of substrate deprivation therapy and bone marrow transplantation.

21 January 2018

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Liver X receptor activation enhances cholesterol loss from the brain, decreases neuroinflammation, and increases survival of the NPC1 mouse.

21 January 2018

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Cholesterol overload promotes morphogenesis of a Niemann-Pick C (NPC)-like compartment independent of inhibition of NPC1 or HE1/NPC2 function

21 January 2018

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Niemann-Pick type C disease involves disrupted neurosteroidogenesis and responds to allopregnanolone

21 January 2018

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Critical role for glycosphingolipids in Niemann-Pick disease type C.

21 January 2018

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Trafficking defects in endogenously synthesized cholesterol in fibroblasts, macrophages, hepatocytes, and glial cells from Niemann-Pick type C1 mice

21 January 2018

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Trafficking of cholesterol from cell bodies to distal axons in Niemann Pick C1-deficient neurons

21 January 2018

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Cholesterol accumulates in cell bodies, but is decreased in distal axons, of Niemann-Pick C1-deficient neurons

21 January 2018

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Mutations in the leucine zipper motif and sterol-sensing domain inactivate the Niemann-Pick C1 glycoprotein

21 January 2018

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12 December 2020

Neurodegeneration in the Niemann-Pick C mouse: glial involvement

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12 December 2020

Pyramidal neurons with ectopic dendrites in storage diseases exhibit increased GM2 ganglioside immunoreactivity

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12 December 2020

Niemann-Pick disease group C: clinical variability and diagnosis based on defective cholesterol esterification. A collaborative study on 70 patients

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12 December 2020

Correction of sphingomyelinase deficiency in Niemann-Pick type C fibroblasts by removal of lipoprotein fraction from culture media

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12 December 2020

The cholesterol storage disorder of the mutant BALB/c mouse. A primary genetic lesion closely linked to defective esterification of exogenously derived cholesterol and its relationship to human type C Niemann-Pick disease

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12 December 2020

The role of autophagy-lysosome pathway in neurodegeneration associated with Parkinson's disease

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7 January 2021