Gregory Grabowski to Disease Models, Animal
This is a "connection" page, showing publications Gregory Grabowski has written about Disease Models, Animal.
Connection Strength
1.815
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Isofagomine in vivo effects in a neuronopathic Gaucher disease mouse. PLoS One. 2011 Apr 20; 6(4):e19037.
Score: 0.217
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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: 0.204
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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: 0.156
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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: 0.146
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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: 0.129
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Complement drives glucosylceramide accumulation and tissue inflammation in Gaucher disease. Nature. 2017 03 02; 543(7643):108-112.
Score: 0.081
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Reversal of advanced disease in lysosomal acid lipase deficient mice: a model for lysosomal acid lipase deficiency disease. Mol Genet Metab. 2014 Jul; 112(3):229-41.
Score: 0.067
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Multiple pathogenic proteins implicated in neuronopathic Gaucher disease mice. Hum Mol Genet. 2014 Aug 01; 23(15):3943-57.
Score: 0.066
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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: 0.064
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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: 0.062
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Global gene expression profile progression in Gaucher disease mouse models. BMC Genomics. 2011 Jan 11; 12:20.
Score: 0.053
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Impaired autophagosomes and lysosomes in neuronopathic Gaucher disease. Autophagy. 2010 Jul; 6(5):648-9.
Score: 0.051
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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: 0.050
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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: 0.049
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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: 0.049
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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: 0.045
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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: 0.044
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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: 0.025
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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: 0.024
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Chitinase-3-like Protein 1: A Progranulin Downstream Molecule and Potential Biomarker for Gaucher Disease. EBioMedicine. 2018 Feb; 28:251-260.
Score: 0.022
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Modulating ryanodine receptors with dantrolene attenuates neuronopathic phenotype in Gaucher disease mice. Hum Mol Genet. 2016 12 01; 25(23):5126-5141.
Score: 0.020
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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: 0.020
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Association Between Progranulin and Gaucher Disease. EBioMedicine. 2016 Sep; 11:127-137.
Score: 0.020
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CNS-accessible Inhibitor of Glucosylceramide Synthase for Substrate Reduction Therapy of Neuronopathic Gaucher Disease. Mol Ther. 2016 06; 24(6):1019-1029.
Score: 0.019
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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: 0.018
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Activation of p38 Mitogen-Activated Protein Kinase in Gaucher's Disease. PLoS One. 2015; 10(8):e0136633.
Score: 0.018
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Role of saposin C and D in auditory and vestibular function. Laryngoscope. 2016 Feb; 126(2):452-9.
Score: 0.018
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Spiral ganglion degeneration and hearing loss as a consequence of satellite cell death in saposin B-deficient mice. J Neurosci. 2015 Feb 18; 35(7):3263-75.
Score: 0.018
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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: 0.018
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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: 0.014
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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: 0.014
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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: 0.013