(Q48464562)

11 February 2018

title

Conditional expression of human acid beta-glucosidase improves the visceral phenotype in a Gaucher disease mouse model (English)

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11 February 2018

author name string

Ying Sun

1

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11 February 2018

Brian Quinn

2

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11 February 2018

You-Hai Xu

3

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11 February 2018

Tatyana Leonova

4

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11 February 2018

David P Witte

5

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11 February 2018

Gregory A Grabowski

6

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11 February 2018

publication date

21 July 2006

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11 February 2018

Journal of Lipid Research

published in

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11 February 2018

volume

47

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11 February 2018

issue

10

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11 February 2018

page(s)

2161-2170

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11 February 2018

Gaucher disease: Perspectives on a prototype lysosomal disease

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21 January 2018

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Biosynthesis and molecular cloning of sulfated glycoprotein 1 secreted by rat Sertoli cells: sequence similarity with the 70-kilodalton precursor to sulfatide/GM1 activator

21 January 2018

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Saposins (sap) A and C activate the degradation of galactosylceramide in living cells

21 January 2018

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Targeted disruption of the mouse sphingolipid activator protein gene: a complex phenotype, including severe leukodystrophy and wide-spread storage of multiple sphingolipids.

21 January 2018

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A novel mutation in the coding region of the prosaposin gene leads to a complete deficiency of prosaposin and saposins, and is associated with a complex sphingolipidosis dominated by lactosylceramide accumulation

21 January 2018

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Additional biochemical findings in a patient and fetal sibling with a genetic defect in the sphingolipid activator protein (SAP) precursor, prosaposin. Evidence for a deficiency in SAP-1 and for a normal lysosomal neuraminidase

21 January 2018

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Saposin C is required for normal resistance of acid beta-glucosidase to proteolytic degradation

21 January 2018

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Gaucher disease mouse models: point mutations at the acid beta-glucosidase locus combined with low-level prosaposin expression lead to disease variants.

21 January 2018

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Viable mouse models of acid beta-glucosidase deficiency: the defect in Gaucher disease

21 January 2018

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Tight control of gene expression in mammalian cells by tetracycline-responsive promoters

21 January 2018

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Doxycycline-mediated quantitative and tissue-specific control of gene expression in transgenic mice

21 January 2018

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Tetracycline-regulated gene expression in the brain

21 January 2018

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Conditional tetracycline-regulated expression of TGF-β1 in liver of transgenic mice leads to reversible intermediary fibrosis

21 January 2018

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Prosaposin: threshold rescue and analysis of the "neuritogenic" region in transgenic mice

21 January 2018

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The role of mannosylated enzyme and the mannose receptor in enzyme replacement therapy

21 January 2018

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AAV8-mediated expression of glucocerebrosidase ameliorates the storage pathology in the visceral organs of a mouse model of Gaucher disease.

21 January 2018

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10.1194/JLR.M600239-JLR200

21 January 2018

Identifiers

DOI

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11 February 2018

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11 February 2018