globotriaosylceramide

galactosidase, alpha ; Mus musculus







35 Article(s)
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1 34803097 The 30-year Natural History of Non-classic Fabry Disease with an R112H Mutation. 2021 Nov 20 2
2 32127409 Accumulation of Globotriaosylceramide in Podocytes in Fabry Nephropathy Is Associated with Progressive Podocyte Loss. 2020 Apr 1
3 32868283 Assessing the role of glycosphingolipids in the phenotype severity of Fabry disease mouse model. 2020 Nov 1
4 30013462 Altered Gene Expression in Prefrontal Cortex of a Fabry Disease Mouse Model. 2018 4
5 30054149 Non-clinical evaluation of JR-051 as a biosimilar to agalsidase beta for the treatment of Fabry disease. 2018 Sep 2
6 30328411 Characterization of small fiber pathology in a mouse model of Fabry disease. 2018 Oct 17 3
7 27756537 Differences in cleavage of globotriaosylceramide and its derivatives accumulated in organs of young Fabry mice following enzyme replacement therapy. 2017 Jan - Feb 2
8 28161408 Anti-BlyS antibody reduces the immune reaction against enzyme and enhances the efficacy of enzyme replacement therapy in Fabry disease model mice. 2017 May 1
9 28601129 Fabry disease: diagnostic methods in nephrology practice. 2017 Supplement 1 2
10 28649509 Fabry disease: Four case reports of meningioma and a review of the literature on other malignancies. 2017 Jun 2
11 28662189 Affective and cognitive behavior in the alpha-galactosidase A deficient mouse model of Fabry disease. 2017 2
12 28756410 Reduction of podocyte globotriaosylceramide content in adult male patients with Fabry disease with amenable GLA mutations following 6 months of migalastat treatment. 2017 Nov 1
13 27145802 Comprehensive and differential long-term characterization of the alpha-galactosidase A deficient mouse model of Fabry disease focusing on the sensory system and pain development. 2016 1
14 27531673 Increased expression of Trpv1 in peripheral terminals mediates thermal nociception in Fabry disease mouse model. 2016 1
15 24232002 Endothelial nitric oxide synthase uncoupling and microvascular dysfunction in the mesentery of mice deficient in α-galactosidase A. 2014 Jan 4
16 25337704 Pain related channels are differentially expressed in neuronal and non-neuronal cells of glabrous skin of fabry knockout male mice. 2014 1
17 22425450 Enzyme replacement therapy partially prevents invariant Natural Killer T cell deficiency in the Fabry disease mouse model. 2012 May 2
18 23007467 Possible role of transforming growth factor-β1 and vascular endothelial growth factor in Fabry disease nephropathy. 2012 Dec 4
19 23766930 Glycosphingolipid Mediated Caveolin-1 Oligomerization. 2012 Feb 18 1
20 20846825 Promoter-specific lentivectors for long-term, cardiac-directed therapy of Fabry disease. 2011 Jan 4
21 20961863 Increased globotriaosylceramide levels in a transgenic mouse expressing human alpha1,4-galactosyltransferase and a mouse model for treating Fabry disease. 2011 Feb 3
22 21047542 Enhanced endothelial delivery and biochemical effects of α-galactosidase by ICAM-1-targeted nanocarriers for Fabry disease. 2011 Feb 10 4
23 21328014 Kidney histologic alterations in α-Galactosidase-deficient mice. 2011 Apr 1
24 21477968 Myocardial alterations in the murine model of fabry disease can be reversed by enzyme replacement therapy. 2011 May-Jun 1
25 19639234 Expression of genes and their responses to enzyme replacement therapy in a Fabry disease mouse model. 2009 Sep 2
26 18219591 Naked plasmid DNA-based alpha-galactosidase A gene transfer partially reduces systemic accumulation of globotriaosylceramide in Fabry mice. 2008 Feb 5
27 18565198 Vascular dysfunction in the alpha-galactosidase A-knockout mouse is an endothelial cell-, plasma membrane-based defect. 2008 Oct 2
28 17535804 Caveolin-associated accumulation of globotriaosylceramide in the vascular endothelium of alpha-galactosidase A null mice. 2007 Jul 20 1
29 17697536 Enzyme replacement therapy in patients with Fabry's disease. 2007 Jul-Aug 2
30 14716680 Partial correction of the alpha-galactosidase A deficiency and reduction of glycolipid storage in Fabry mice using synthetic vectors. 2004 Jan 1
31 12538729 Fabry disease in mice is associated with age-dependent susceptibility to vascular thrombosis. 2003 Feb 1
32 12624185 Long-term correction of globotriaosylceramide storage in Fabry mice by recombinant adeno-associated virus-mediated gene transfer. 2003 Mar 18 4
33 12027559 Adenovirus-transduced lung as a portal for delivering alpha-galactosidase A into systemic circulation for Fabry disease. 2002 Jun 1
34 10840053 Long-term enzyme correction and lipid reduction in multiple organs of primary and secondary transplanted Fabry mice receiving transduced bone marrow cells. 2000 Jun 20 4
35 10841515 Reduction of globotriaosylceramide in Fabry disease mice by substrate deprivation. 2000 Jun 2