Gregory Grabowski to Glucosylceramidase
This is a "connection" page, showing publications Gregory Grabowski has written about Glucosylceramidase.
Connection Strength
7.721
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The LIMP-2/SCARB2 binding motif on acid ?-glucosidase: basic and applied implications for Gaucher disease and associated neurodegenerative diseases. J Biol Chem. 2014 Oct 24; 289(43):30063-74.
Score: 0.444
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Gaucher disease: transcriptome analyses using microarray or mRNA sequencing in a Gba1 mutant mouse model treated with velaglucerase alfa or imiglucerase. PLoS One. 2013; 8(10):e74912.
Score: 0.416
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Gaucher disease: chemotactic factors and immunological cell invasion in a mouse model. Mol Genet Metab. 2014 Feb; 111(2):163-71.
Score: 0.415
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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: 0.400
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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: 0.367
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Comparative therapeutic effects of velaglucerase alfa and imiglucerase in a Gaucher disease mouse model. PLoS One. 2010 May 20; 5(5):e10750.
Score: 0.330
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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: 0.321
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Specific saposin C deficiency: CNS impairment and acid beta-glucosidase effects in the mouse. Hum Mol Genet. 2010 Feb 15; 19(4):634-47.
Score: 0.320
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In vivo and ex vivo evaluation of L-type calcium channel blockers on acid beta-glucosidase in Gaucher disease mouse models. PLoS One. 2009 Oct 07; 4(10):e7320.
Score: 0.316
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Gaucher disease: resetting the clinical and scientific agenda. Am J Hematol. 2009 Apr; 84(4):205-7.
Score: 0.305
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Participation of asparagine 370 and glutamine 235 in the catalysis by acid beta-glucosidase: the enzyme deficient in Gaucher disease. Mol Genet Metab. 2009 May; 97(1):65-74.
Score: 0.302
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Neurological deficits and glycosphingolipid accumulation in saposin B deficient mice. Hum Mol Genet. 2008 Aug 01; 17(15):2345-56.
Score: 0.287
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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: 0.283
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Conditional expression of human acid beta-glucosidase improves the visceral phenotype in a Gaucher disease mouse model. J Lipid Res. 2006 Oct; 47(10):2161-70.
Score: 0.253
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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: 0.236
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Viable mouse models of acid beta-glucosidase deficiency: the defect in Gaucher disease. Am J Pathol. 2003 Nov; 163(5):2093-101.
Score: 0.209
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Saposin C is required for normal resistance of acid beta-glucosidase to proteolytic degradation. J Biol Chem. 2003 Aug 22; 278(34):31918-23.
Score: 0.204
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Enzyme therapy of gaucher disease: clinical and biochemical changes during production of and tolerization for neutralizing antibodies. Blood Cells Mol Dis. 2003 Jan-Feb; 30(1):90-6.
Score: 0.198
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Ex vivo localization of the mouse saposin C activation region for acid beta-glucosidase. Mol Genet Metab. 2002 Jul; 76(3):189-200.
Score: 0.191
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Substrate Reduction Therapy Reverses Mitochondrial, mTOR, and Autophagy Alterations in a Cell Model of Gaucher Disease. Cells. 2021 09 02; 10(9).
Score: 0.180
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Systemic enzyme delivery by blood-brain barrier-penetrating SapC-DOPS nanovesicles for treatment of neuronopathic Gaucher disease. EBioMedicine. 2020 May; 55:102735.
Score: 0.164
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Intravenous infusion of iPSC-derived neural precursor cells increases acid ?-glucosidase function in the brain and lessens the neuronopathic phenotype in a mouse model of Gaucher disease. Hum Mol Genet. 2019 10 15; 28(20):3406-3421.
Score: 0.158
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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: 0.152
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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: 0.141
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Complement drives glucosylceramide accumulation and tissue inflammation in Gaucher disease. Nature. 2017 03 02; 543(7643):108-112.
Score: 0.132
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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: 0.128
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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: 0.115
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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: 0.113
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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: 0.105
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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: 0.089
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Acid ?-glucosidase mutants linked to Gaucher disease, Parkinson disease, and Lewy body dementia alter a-synuclein processing. Ann Neurol. 2011 Jun; 69(6):940-53.
Score: 0.088
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Acid beta-glucosidase 1 counteracts p38delta-dependent induction of interleukin-6: possible role for ceramide as an anti-inflammatory lipid. J Biol Chem. 2009 May 08; 284(19):12979-88.
Score: 0.076
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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: 0.076
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Temporal gene expression profiling reveals CEBPD as a candidate regulator of brain disease in prosaposin deficient mice. BMC Neurosci. 2008 Aug 01; 9:76.
Score: 0.073
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PGRN deficiency exacerbates, whereas a brain penetrant PGRN derivative protects, GBA1 mutation-associated pathologies and diseases. Proc Natl Acad Sci U S A. 2023 01 03; 120(1):e2210442120.
Score: 0.049
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Activation of p38 Mitogen-Activated Protein Kinase in Gaucher's Disease. PLoS One. 2015; 10(8):e0136633.
Score: 0.030
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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: 0.020
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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: 0.019
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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: 0.017