Therapy of Pelizaeus-Merzbacher disease in mice by feeding a cholesterol-enriched diet (Q57986020)

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object named as

Klaus-Armin Nave

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Torben Ruhwedel

object named as

Torben Ruhwedel

4

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Gesine Saher

object named as

Gesine Saher

1

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author name string

Fabian Rudolphi

2

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Kristina Corthals

3

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Karl-Friedrich Schmidt

5

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Siegrid Löwel

6

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Payam Dibaj

7

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Benoit Barrette

https://scigraph.springernature.com/pub.10.1038/nm.2833

8

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language of work or name

English

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publication date

17 June 2012

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published in

Nature Medicine

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volume

18

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page(s)

1130-1135

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issue

7

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exact match

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cites work

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Assembly of myelin by association of proteolipid protein with cholesterol- and galactosylceramide-rich membrane domains

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High cholesterol level is essential for myelin membrane growth

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Premature arrest of myelin formation in transgenic mice with increased proteolipid protein gene dosage

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Distinct phenotypes associated with increasing dosage of the PLP gene: implications for CMT1A due to PMP22 gene duplication

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Late-onset neurodegeneration in mice with increased dosage of the proteolipid protein gene

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Demyelination and axonal preservation in a transgenic mouse model of Pelizaeus-Merzbacher disease

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PLP/DM20 expression and turnover in a transgenic mouse model of Pelizaeus-Merzbacher disease

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Membrane traffic in myelinating oligodendrocytes

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Overexpression of the myelin proteolipid protein leads to accumulation of cholesterol and proteolipid protein in endosomes/lysosomes: implications for Pelizaeus-Merzbacher disease

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Brain cholesterol: long secret life behind a barrier.

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Quantitation of two pathways for cholesterol excretion from the brain in normal mice and mice with neurodegeneration

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Reversal of defective lysosomal transport in NPC disease ameliorates liver dysfunction and neurodegeneration in the npc1-/- mouse

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Cholesterol regulates the endoplasmic reticulum exit of the major membrane protein P0 required for peripheral myelin compaction.

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Elevated plasma membrane cholesterol content alters macrophage signaling and function.

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Lysosomal storage causes cellular dysfunction in mucolipidosis II skin fibroblasts

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Deficiency of niemann-pick type C-1 protein impairs release of human immunodeficiency virus type 1 and results in Gag accumulation in late endosomal/lysosomal compartments

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Role of the oligodendroglial cytoskeleton in differentiation and myelination

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12 December 2020

Neuropathology and genetics of Pelizaeus-Merzbacher disease

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The SREBP pathway: regulation of cholesterol metabolism by proteolysis of a membrane-bound transcription factor

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12 December 2020

Pre-synaptic defects of NPC1-deficient hippocampal neurons are not directly related to plasma membrane cholesterol

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12 December 2020

Monoclonal antibody O10 defines a conformationally sensitive cell-surface epitope of proteolipid protein (PLP): evidence that PLP misfolding underlies dysmyelination in mutant mice.

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12 December 2020

PLP overexpression perturbs myelin protein composition and myelination in a mouse model of Pelizaeus-Merzbacher disease

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12 December 2020