Sites and temporal changes of gangliosides GM1/GM2 storage in the Niemann-Pick disease type C mouse brain. (Q48770929)

15 February 2018

title

Sites and temporal changes of gangliosides GM1/GM2 storage in the Niemann-Pick disease type C mouse brain. (English)

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15 February 2018

Niemann-Pick disease, type C1

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Taniguchi M

1

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15 February 2018

Shinoda Y

2

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15 February 2018

Ninomiya H

3

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15 February 2018

Vanier MT

4

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15 February 2018

Ohno K

5

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15 February 2018

publication date

1 October 2001

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15 February 2018

published in

Brain and Development

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15 February 2018

volume

23

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15 February 2018

issue

6

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15 February 2018

page(s)

414-421

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Niemann-Pick type C: a disorder of cellular cholesterol trafficking

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Transmembrane molecular pump activity of Niemann-Pick C1 protein

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

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Increased levels of GM2 ganglioside in fibroblasts from a patient with juvenile Niemann-Pick disease type C.

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Ganglioside GM1 binds to the Trk protein and regulates receptor function

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

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GM2 ganglioside and pyramidal neuron dendritogenesis

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Cerebellar degeneration in the Niemann-Pick type C mouse.

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Recent advances in elucidating Niemann-Pick C disease

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Immunocytochemical analysis of gangliosides in rat primary cerebellar cultures using specific monoclonal antibodies.

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Immunostaining of ganglioside GD1b, GD3 and GM1 in rat cerebellum: cellular layer and cell type specific associations

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An improved method for culturing cerebellar Purkinje cells with differentiated dendrites under a mixed monolayer setting

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

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Alleviation of neuronal ganglioside storage does not improve the clinical course of the Niemann-Pick C disease mouse

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Embryonic Striatal Neurons from Niemann-Pick Type C Mice Exhibit Defects in Cholesterol Metabolism and Neurotrophin Responsiveness

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Growth of ectopic dendrites on cortical pyramidal neurons in neuronal storage diseases correlates with abnormal accumulation of GM2 ganglioside.

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

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10.1016/S0387-7604(01)00252-2

Identifiers

DOI

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15 February 2018

PubMed ID

11578853

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15 February 2018