A symptomatic Fabry disease mouse model generated by inducing globotriaosylceramide synthesis (Q34159448)

31 July 2017

A symptomatic Fabry disease mouse model generated by inducing globotriaosylceramide synthesis (English)

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31 July 2017

1 reference

Atsumi Taguchi

1

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31 July 2017

Hiroki Maruyama

2

1 reference

31 July 2017

Masaaki Nameta

3

1 reference

31 July 2017

Tadashi Yamamoto

4

1 reference

31 July 2017

Junichiro Matsuda

5

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31 July 2017

Ashok B Kulkarni

6

1 reference

31 July 2017

Hidekatsu Yoshioka

7

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31 July 2017

Satoshi Ishii

8

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31 July 2017

language of work or name

English

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publication date

1 December 2013

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31 July 2017

11

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inferred from page(s)

published in

Biochemical Journal

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31 July 2017

456

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31 July 2017

373-383

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31 July 2017

3

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Screening of male dialysis patients for fabry disease by plasma globotriaosylsphingosine

31 July 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4160053

Pharmacological chaperone therapy for Fabry disease

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4160053

IRS2 increases mitochondrial dysfunction and oxidative stress in a mouse model of Huntington disease

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4160053

Iminosugar-based inhibitors of glucosylceramide synthase increase brain glycosphingolipids and survival in a mouse model of Sandhoff disease

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4160053

Substrate reduction augments the efficacy of enzyme therapy in a mouse model of Fabry disease

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4160053

Increased globotriaosylceramide levels in a transgenic mouse expressing human alpha1,4-galactosyltransferase and a mouse model for treating Fabry disease

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4160053

Kidney dendritic cell activation is required for progression of renal disease in a mouse model of glomerular injury

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4160053

Neurological deficits and glycosphingolipid accumulation in saposin B deficient mice

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4160053

Elevated globotriaosylsphingosine is a hallmark of Fabry disease.

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4160053

Effective cell and gene therapy in a murine model of Gaucher disease

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4160053

Lysosomal storage diseases: natural history and ethical and economic aspects

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4160053

Fabry disease and the heart: an overview of the natural history and the effect of enzyme replacement therapy.

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4160053

Alpha-galactosidase A deficiency accelerates atherosclerosis in mice with apolipoprotein E deficiency

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4160053

Fabry disease defined: baseline clinical manifestations of 366 patients in the Fabry Outcome Survey

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4160053

Natural history and treatment of renal involvement in Fabry disease.

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4160053

Safety and efficacy of recombinant human alpha-galactosidase A replacement therapy in Fabry's disease

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4160053

Enzyme replacement therapy in Fabry disease: a randomized controlled trial

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4160053

Tumor necrosis factor alpha increases human cerebral endothelial cell Gb3 and sensitivity to Shiga toxin

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4160053

Fabry disease: preclinical studies demonstrate the effectiveness of alpha-galactosidase A replacement in enzyme-deficient mice

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4160053

Reduction of globotriaosylceramide in Fabry disease mice by substrate deprivation

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4160053

Long-term enzyme correction and lipid reduction in multiple organs of primary and secondary transplanted Fabry mice receiving transduced bone marrow cells

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4160053

Megalin knockout mice as an animal model of low molecular weight proteinuria.

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4160053

Aging accentuates and bone marrow transplantation ameliorates metabolic defects in Fabry disease mice

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4160053

alpha-Galactosidase A deficient mice: a model of Fabry disease

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4160053

Treatment of Gaucher disease with an enzyme inhibitor

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4160053

Females with Fabry disease frequently have major organ involvement: lessons from the Fabry Registry.

5 July 2018

1 reference

https://api.crossref.org/works/10.1042%2FBJ20130825

Enzyme therapy for Fabry disease: neutralizing antibodies toward agalsidase alpha and beta

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBJ20130825

Transgenic mouse expressing human mutant alpha-galactosidase A in an endogenous enzyme deficient background: a biochemical animal model for studying active-site specific chaperone therapy for Fabry disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBJ20130825

Preclinical efficacy and safety of 1-deoxygalactonojirimycin in mice for Fabry disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBJ20130825

LC-MS/MS analysis of plasma lyso-Gb3 in Fabry disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBJ20130825

Plasma globotriaosylsphingosine: diagnostic value and relation to clinical manifestations of Fabry disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBJ20130825

Long-term therapy with agalsidase alfa for Fabry disease: safety and effects on renal function in a home infusion setting

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBJ20130825

Molecular basis for up-regulation by inflammatory cytokines of Shiga toxin 1 cytotoxicity and globotriaosylceramide expression

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBJ20130825

Novel oral treatment of Gaucher's disease with N-butyldeoxynojirimycin (OGT 918) to decrease substrate biosynthesis.

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBJ20130825

Fabry disease in mice is associated with age-dependent susceptibility to vascular thrombosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBJ20130825

An in vitro model of Fabry disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBJ20130825

Renal proximal tubular dysfunction is a major determinant of urinary connective tissue growth factor excretion

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBJ20130825

Enzymatic Defect in Fabry's Disease

21 January 2018

1 reference

12 December 2020

Neurological manifestations of knockout mice with beta-galactosidase deficiency

1 reference

12 December 2020

Accelerated transport and maturation of lysosomal α–galactosidase A in Fabry lymphoblasts by an enzyme inhibitor

1 reference

12 December 2020

Renal biopsy findings in children and adolescents with Fabry disease and minimal albuminuria

1 reference

12 December 2020

Early Detection of Fabry Cardiomyopathy by Tissue Doppler Imaging

1 reference

12 December 2020

Identifiers

DOI

10.1042/BJ20130825

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31 July 2017

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31 July 2017

1 reference