(Q48740625)

15 February 2018

title

The natural history of cerebellar degeneration of Niemann-Pick C mice monitored in vitro. (English)

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15 February 2018

Niemann–Pick disease, type C

main subject

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Nils Marschalek

1

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15 February 2018

Frank Albert

series ordinal

2

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15 February 2018

Volker Meske

series ordinal

3

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15 February 2018

Thomas Georg Ohm

series ordinal

4

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15 February 2018

publication date

1 December 2014

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15 February 2018

Neuropathology and Applied Neurobiology

published in

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15 February 2018

volume

40

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15 February 2018

issue

7

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15 February 2018

page(s)

933-945

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15 February 2018

Niemann-Pick disease type C

cites work

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Recommendations for the diagnosis and management of Niemann-Pick disease type C: an update

21 January 2018

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Loss of Niemann-Pick C1 or C2 protein results in similar biochemical changes suggesting that these proteins function in a common lysosomal pathway

21 January 2018

1 reference

Neurons in Niemann-Pick disease type C accumulate gangliosides as well as unesterified cholesterol and undergo dendritic and axonal alterations

21 January 2018

1 reference

Structure of N-Terminal Domain of NPC1 Reveals Distinct Subdomains for Binding and Transfer of Cholesterol

21 January 2018

1 reference

Identification of surface residues on Niemann-Pick C2 essential for hydrophobic handoff of cholesterol to NPC1 in lysosomes

21 January 2018

1 reference

Cerebellar degeneration in the Niemann-Pick type C mouse

21 January 2018

1 reference

Temporal and cell-specific deletion establishes that neuronal Npc1 deficiency is sufficient to mediate neurodegeneration

21 January 2018

1 reference

The synaptophysin/synaptobrevin interaction critically depends on the cholesterol content.

21 January 2018

1 reference

Spatial and temporal distribution of intracellular free cholesterol in brains of a Niemann-Pick type C mouse model showing hyperphosphorylated tau protein. Implications for Alzheimer's disease

21 January 2018

1 reference

Pathological cholesterol metabolism fails to modify electrophysiological properties of afflicted neurones in Niemann-Pick disease type C.

21 January 2018

1 reference

Lentivector-mediated rescue from cerebellar ataxia in a mouse model of spinocerebellar ataxia.

21 January 2018

1 reference

Mutant mice as a model for cerebellar ataxia

21 January 2018

1 reference

Ataxia with loss of Purkinje cells in a mouse model for Refsum disease

21 January 2018

1 reference

A protein-protein interaction network for human inherited ataxias and disorders of Purkinje cell degeneration

21 January 2018

1 reference

A simple method for organotypic cultures of nervous tissue

21 January 2018

1 reference

Cellular and molecular control of dendritic growth and development of cerebellar Purkinje cells

21 January 2018

1 reference

Protein kinase C activity modulates dendritic differentiation of rat Purkinje cells in cerebellar slice cultures.

21 January 2018

1 reference

Differentiation of Purkinje cells in cerebellar slice cultures: an immunocytochemical and Golgi EM study.

21 January 2018

1 reference

A versatile prion replication assay in organotypic brain slices

21 January 2018

1 reference

Patterned Purkinje cell degeneration in mouse models of Niemann-Pick type C disease.

21 January 2018

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Weekly cyclodextrin administration normalizes cholesterol metabolism in nearly every organ of the Niemann-Pick type C1 mouse and markedly prolongs life.

21 January 2018

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Activation mobilizes the cholesterol in the late endosomes-lysosomes of Niemann Pick type C cells

21 January 2018

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NPC1 and NPC2 Regulate Cellular Cholesterol Homeostasis through Generation of Low Density Lipoprotein Cholesterol-derived Oxysterols

21 January 2018

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Altered lipid content inhibits autophagic vesicular fusion

21 January 2018

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Inhibitory effect of dietary lipids on chaperone-mediated autophagy

21 January 2018

1 reference

Regulation of lipid stores and metabolism by lipophagy

21 January 2018

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The autophagic defect in Niemann-Pick disease type C neurons differs from somatic cells and reduces neuronal viability.

21 January 2018

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Cell-autonomous death of cerebellar purkinje neurons with autophagy in Niemann-Pick type C disease

21 January 2018

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Organotypic rat cerebellar slice culture as a model to analyze the molecular pharmacology of GABAA receptors

21 January 2018

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Synaptic organization of the mouse cerebellar cortex in organotypic slice cultures

21 January 2018

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Altered dendritic development of cerebellar Purkinje cells in slice cultures from protein kinase Cgamma-deficient mice

21 January 2018

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Unesterified cholesterol accumulation in late endosomes/lysosomes causes neurodegeneration and is prevented by driving cholesterol export from this compartment

21 January 2018

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Autophagy regulates lipid metabolism

21 January 2018

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Suppression of basal autophagy in neural cells causes neurodegenerative disease in mice

21 January 2018

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Loss of autophagy in the central nervous system causes neurodegeneration in mice

21 January 2018

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Disruption and therapeutic rescue of autophagy in a human neuronal model of Niemann Pick type C1

21 January 2018

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The prion organotypic slice culture assay—POSCA

21 January 2018

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21 January 2018

Identifiers

DOI

10.1111/NAN.12154

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15 February 2018

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15 February 2018