Gregory Grabowski to Glucosylceramides
This is a "connection" page, showing publications Gregory Grabowski has written about Glucosylceramides.
Connection Strength
3.199
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Complement drives glucosylceramide accumulation and tissue inflammation in Gaucher disease. Nature. 2017 03 02; 543(7643):108-112.
Score: 0.569
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Substrate compositional variation with tissue/region and Gba1 mutations in mouse models--implications for Gaucher disease. PLoS One. 2013; 8(3):e57560.
Score: 0.432
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Ex vivo and in vivo effects of isofagomine on acid ?-glucosidase variants and substrate levels in Gaucher disease. J Biol Chem. 2012 Feb 03; 287(6):4275-87.
Score: 0.397
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Neuronopathic Gaucher disease in the mouse: viable combined selective saposin C deficiency and mutant glucocerebrosidase (V394L) mice with glucosylsphingosine and glucosylceramide accumulation and progressive neurological deficits. Hum Mol Genet. 2010 Mar 15; 19(6):1088-97.
Score: 0.347
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Combined saposin C and D deficiencies in mice lead to a neuronopathic phenotype, glucosylceramide and alpha-hydroxy ceramide accumulation, and altered prosaposin trafficking. Hum Mol Genet. 2007 Apr 15; 16(8):957-71.
Score: 0.285
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Properties of neurons derived from induced pluripotent stem cells of Gaucher disease type 2 patient fibroblasts: potential role in neuropathology. PLoS One. 2015; 10(3):e0118771.
Score: 0.125
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Ubiquitous transgene expression of the glucosylceramide-synthesizing enzyme accelerates glucosylceramide accumulation and storage cells in a Gaucher disease mouse model. PLoS One. 2014; 9(12):e116023.
Score: 0.123
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CNS, lung, and lymph node involvement in Gaucher disease type 3 after 11 years of therapy: clinical, histopathologic, and biochemical findings. Mol Genet Metab. 2015 Feb; 114(2):233-241.
Score: 0.120
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Endogenous ?-glucocerebrosidase activity in Abca12?/?epidermis elevates ceramide levels after topical lipid application but does not restore barrier function. J Lipid Res. 2014 Mar; 55(3):493-503.
Score: 0.114
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Improved management of lysosomal glucosylceramide levels in a mouse model of type 1 Gaucher disease using enzyme and substrate reduction therapy. J Inherit Metab Dis. 2010 Jun; 33(3):281-9.
Score: 0.088
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Gaucher disease: resetting the clinical and scientific agenda. Am J Hematol. 2009 Apr; 84(4):205-7.
Score: 0.082
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Involvement of acid beta-glucosidase 1 in the salvage pathway of ceramide formation. J Biol Chem. 2009 May 08; 284(19):12972-8.
Score: 0.082
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Dependence of reversibility and progression of mouse neuronopathic Gaucher disease on acid beta-glucosidase residual activity levels. Mol Genet Metab. 2008 Jun; 94(2):190-203.
Score: 0.077
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A specific and potent inhibitor of glucosylceramide synthase for substrate inhibition therapy of Gaucher disease. Mol Genet Metab. 2007 Jul; 91(3):259-67.
Score: 0.072
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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. J Lipid Res. 2005 Oct; 46(10):2102-13.
Score: 0.064
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Combination of acid ?-glucosidase mutation and Saposin C deficiency in mice reveals Gba1 mutation dependent and tissue-specific disease phenotype. Sci Rep. 2019 04 03; 9(1):5571.
Score: 0.041
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An unexpected player in Gaucher disease: The multiple roles of complement in disease development. Semin Immunol. 2018 06; 37:30-42.
Score: 0.038
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Progression of Behavioral and CNS Deficits in a Viable Murine Model of Chronic Neuronopathic Gaucher Disease. PLoS One. 2016; 11(9):e0162367.
Score: 0.034
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Neuronopathic Gaucher disease: dysregulated mRNAs and miRNAs in brain pathogenesis and effects of pharmacologic chaperone treatment in a mouse model. Hum Mol Genet. 2015 Dec 15; 24(24):7031-48.
Score: 0.032
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Activation of p38 Mitogen-Activated Protein Kinase in Gaucher's Disease. PLoS One. 2015; 10(8):e0136633.
Score: 0.032
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Gaucher disease glucocerebrosidase and a-synuclein form a bidirectional pathogenic loop in synucleinopathies. Cell. 2011 Jul 08; 146(1):37-52.
Score: 0.024
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Characterization of Gaucher disease bone marrow mesenchymal stromal cells reveals an altered inflammatory secretome. Blood. 2009 Oct 08; 114(15):3181-90.
Score: 0.021