Brain pathology in Niemann Pick disease type A: insights from the acid sphingomyelinase knockout mice (Q34685652)

3 August 2017

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title

Brain pathology in Niemann Pick disease type A: insights from the acid sphingomyelinase knockout mice (English)

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3 August 2017

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4

Edward H Schuchman

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3 August 2017

author name string

Maria Dolores Ledesma

series ordinal

1

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3 August 2017

Alessandro Prinetti

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2

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3 August 2017

Sandro Sonnino

series ordinal

3

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3 August 2017

language of work or name

English

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publication date

7 January 2011

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Journal of Neurochemistry

3 August 2017

published in

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3 August 2017

volume

116

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3 August 2017

issue

5

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3 August 2017

page(s)

779-788

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Lipid rafts as a membrane-organizing principle

3 August 2017

cites work

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7 July 2018

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Acid sphingomyelinase-deficient mice mimic the neurovisceral form of human lysosomal storage disease (Niemann-Pick disease)

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7 July 2018

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21 January 2018

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21 January 2018

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Alterations of myelin-specific proteins and sphingolipids characterize the brains of acid sphingomyelinase-deficient mice, an animal model of Niemann-Pick disease type A.

21 January 2018

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21 January 2018

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21 January 2018

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Proper axonal distribution of PrP(C) depends on cholesterol-sphingomyelin-enriched membrane domains and is developmentally regulated in hippocampal neurons

21 January 2018

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Defective calcium homeostasis in the cerebellum in a mouse model of Niemann-Pick A disease

21 January 2018

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Intracerebral transplantation of mesenchymal stem cells into acid sphingomyelinase-deficient mice delays the onset of neurological abnormalities and extends their life span.

21 January 2018

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Treatment with miglustat reverses the lipid-trafficking defect in Niemann-Pick disease type C.

21 January 2018

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Neuropathology of the acid sphingomyelinase knockout mouse model of Niemann-Pick A disease including structure-function studies associated with cerebellar Purkinje cell degeneration.

21 January 2018

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21 January 2018

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21 January 2018

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ENZYMIC HYDROLYSIS AND SYNTHESIS OF CERAMIDES

21 January 2018

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26 October 2018

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Membrane lipids, selectively diminished in Alzheimer brains, suggest synapse loss as a primary event in early-onset form (type I) and demyelination in late-onset form (type II).

26 October 2018

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26 October 2018

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26 October 2018

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21 January 2018

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12 December 2020

Secretory sphingomyelinase, a product of the acid sphingomyelinase gene, can hydrolyze atherogenic lipoproteins at neutral pH. Implications for atherosclerotic lesion development

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12 December 2020

Infusion of recombinant human acid sphingomyelinase into niemann-pick disease mice leads to visceral, but not neurological, correction of the pathophysiology

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12 December 2020

Lysosomal localization of sphingomyelinase in rat liver

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Partial enzyme deficiencies: residual activities and the development of neurological disorders

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12 December 2020

Peripheral neuropathy in type A Niemann-Pick disease. A morphological study

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12 December 2020

Different molecular mechanisms leading to white matter hypomyelination in infantile onset lysosomal disorders

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12 December 2020

10.1111/J.1471-4159.2010.07034.X

Identifiers

DOI

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3 August 2017

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3 August 2017

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