Gregory Grabowski to Animals
This is a "connection" page, showing publications Gregory Grabowski has written about Animals.
Connection Strength
0.992
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Complement drives glucosylceramide accumulation and tissue inflammation in Gaucher disease. Nature. 2017 03 02; 543(7643):108-112.
Score: 0.041
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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: 0.035
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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: 0.034
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Multiple pathogenic proteins implicated in neuronopathic Gaucher disease mice. Hum Mol Genet. 2014 Aug 01; 23(15):3943-57.
Score: 0.034
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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: 0.033
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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: 0.032
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Altered autophagy in the mice with a deficiency of saposin A and saposin B. Autophagy. 2013 Jul; 9(7):1115-6.
Score: 0.032
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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: 0.031
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Tissue-specific effects of saposin A and saposin B on glycosphingolipid degradation in mutant mice. Hum Mol Genet. 2013 Jun 15; 22(12):2435-50.
Score: 0.031
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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: 0.029
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Isofagomine in vivo effects in a neuronopathic Gaucher disease mouse. PLoS One. 2011 Apr 20; 6(4):e19037.
Score: 0.027
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Global gene expression profile progression in Gaucher disease mouse models. BMC Genomics. 2011 Jan 11; 12:20.
Score: 0.027
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Impaired autophagosomes and lysosomes in neuronopathic Gaucher disease. Autophagy. 2010 Jul; 6(5):648-9.
Score: 0.026
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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: 0.026
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Multi-system disorders of glycosphingolipid and ganglioside metabolism. J Lipid Res. 2010 Jul; 51(7):1643-75.
Score: 0.025
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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: 0.025
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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: 0.025
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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: 0.025
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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: 0.023
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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: 0.022
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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: 0.022
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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: 0.021
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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: 0.020
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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: 0.018
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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: 0.016
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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: 0.016
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Analyses of temporal regulatory elements of the prosaposin gene in transgenic mice. Biochem J. 2003 Mar 01; 370(Pt 2):557-66.
Score: 0.016
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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: 0.015
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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: 0.015
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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: 0.014
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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: 0.013
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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: 0.012
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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: 0.012
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LAL (Lysosomal Acid Lipase) Promotes Reverse Cholesterol Transport In Vitro and In Vivo. Arterioscler Thromb Vasc Biol. 2018 05; 38(5):1191-1201.
Score: 0.011
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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: 0.011
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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: 0.011
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Tissue Localization of Glycosphingolipid Accumulation in a Gaucher Disease Mouse Brain by LC-ESI-MS/MS and High-Resolution MALDI Imaging Mass Spectrometry. SLAS Discov. 2017 12; 22(10):1218-1228.
Score: 0.011
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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: 0.010
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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: 0.010
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Association Between Progranulin and Gaucher Disease. EBioMedicine. 2016 Sep; 11:127-137.
Score: 0.010
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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: 0.010
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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: 0.009
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Activation of p38 Mitogen-Activated Protein Kinase in Gaucher's Disease. PLoS One. 2015; 10(8):e0136633.
Score: 0.009
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Role of saposin C and D in auditory and vestibular function. Laryngoscope. 2016 Feb; 126(2):452-9.
Score: 0.009
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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: 0.009
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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: 0.009
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An in vivo model of human small intestine using pluripotent stem cells. Nat Med. 2014 Nov; 20(11):1310-4.
Score: 0.009
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Discrete mechanisms of mTOR and cell cycle regulation by AMPK agonists independent of AMPK. Proc Natl Acad Sci U S A. 2014 Jan 28; 111(4):E435-44.
Score: 0.008
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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: 0.008
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Saposins utilize two strategies for lipid transfer and CD1 antigen presentation. Proc Natl Acad Sci U S A. 2012 Mar 20; 109(12):4357-64.
Score: 0.007
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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: 0.007
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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: 0.007
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The role of UDP-Glc:glycoprotein glucosyltransferase 1 in the maturation of an obligate substrate prosaposin. J Cell Biol. 2010 May 31; 189(5):829-41.
Score: 0.006
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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: 0.006
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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: 0.005
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Saposin C: neuronal effect and CNS delivery by liposomes. Ann N Y Acad Sci. 2005 Aug; 1053:237-46.
Score: 0.005