| 1 |
33152398 | Brain pathology and cerebellar purkinje cell loss in a mouse model of chronic neuronopathic Gaucher disease. | 2021 Feb |
2 |
| 2 |
33802460 | GCase and LIMP2 Abnormalities in the Liver of Niemann Pick Type C Mice. | 2021 Mar 3 |
1 |
| 3 |
34106956 | Decreased glucocerebrosidase activity and substrate accumulation of glycosphingolipids in a novel GBA1 D409V knock-in mouse model. | 2021 |
1 |
| 4 |
34458595 | Identification of genetic modifiers of murine hepatic β-glucocerebrosidase activity. | 2021 Dec |
2 |
| 5 |
34509608 | A novel glucosylceramide synthase inhibitor attenuates alpha synuclein pathology and lysosomal dysfunction in preclinical models of synucleinopathy. | 2021 Nov |
2 |
| 6 |
34622801 | Direct targeting of wild-type glucocerebrosidase by antipsychotic quetiapine improves pathogenic phenotypes in Parkinson's disease models. | 2021 Oct 8 |
1 |
| 7 |
34686711 | Preclinical pharmacology of glucosylceramide synthase inhibitor venglustat in a GBA-related synucleinopathy model. | 2021 Oct 22 |
2 |
| 8 |
32144204 | Glucocerebrosidases catalyze a transgalactosylation reaction that yields a newly-identified brain sterol metabolite, galactosylated cholesterol. | 2020 Apr 17 |
5 |
| 9 |
33189821 | Ceramide ratios are affected by cigarette smoke but not heat-not-burn or e-vapor aerosols across four independent mouse studies. | 2020 Dec 15 |
2 |
| 10 |
29900534 | Absence of infiltrating peripheral myeloid cells in the brains of mouse models of lysosomal storage disorders. | 2019 Mar |
1 |
| 11 |
30578288 | Validation of anti-glucocerebrosidase antibodies for western blot analysis on protein lysates of murine and human cells. | 2019 Jan 25 |
1 |
| 12 |
30600575 | In vivo inactivation of glycosidases by conduritol B epoxide and cyclophellitol as revealed by activity-based protein profiling. | 2019 Feb |
2 |
| 13 |
30689867 | GBA haploinsufficiency accelerates alpha-synuclein pathology with altered lipid metabolism in a prodromal model of Parkinson's disease. | 2019 Jun 1 |
1 |
| 14 |
31447678 | Ambroxol Hydrochloride Improves Motor Functions and Extends Survival in a Mouse Model of Familial Amyotrophic Lateral Sclerosis. | 2019 |
2 |
| 15 |
31660434 | A Localized Chimeric Hydrogel Therapy Combats Tumor Progression through Alteration of Sphingolipid Metabolism. | 2019 Oct 23 |
1 |
| 16 |
28225753 | Complement drives glucosylceramide accumulation and tissue inflammation in Gaucher disease. | 2017 Mar 2 |
2 |
| 17 |
28485919 | Stabilization of Glucocerebrosidase by Active Site Occupancy. | 2017 Jul 21 |
2 |
| 18 |
28686011 | Glucosylceramide and Glucosylsphingosine Quantitation by Liquid Chromatography-Tandem Mass Spectrometry to Enable In Vivo Preclinical Studies of Neuronopathic Gaucher Disease. | 2017 Aug 15 |
2 |
| 19 |
27482815 | A new glucocerebrosidase-deficient neuronal cell model provides a tool to probe pathophysiology and therapeutics for Gaucher disease. | 2016 Jul 1 |
1 |
| 20 |
27539321 | LRRK2 deficiency impacts ceramide metabolism in brain. | 2016 Sep 23 |
2 |
| 21 |
28933411 | Mitochondrial Dysfunction in Lysosomal Storage Disorders. | 2016 Oct 11 |
2 |
| 22 |
25996484 | High β-glucosidase (GBA) activity not attributable to GBA1 and GBA2 in live normal and enzyme-deficient fibroblasts may emphasise the role of additional GBAs. | 2015 Nov |
4 |
| 23 |
26094487 | Sustained Systemic Glucocerebrosidase Inhibition Induces Brain α-Synuclein Aggregation, Microglia and Complement C1q Activation in Mice. | 2015 Aug 20 |
2 |
| 24 |
26275242 | Reducing GBA2 Activity Ameliorates Neuropathology in Niemann-Pick Type C Mice. | 2015 |
4 |
| 25 |
26312487 | Activation of p38 Mitogen-Activated Protein Kinase in Gaucher's Disease. | 2015 |
3 |
| 26 |
26420838 | Neuronopathic Gaucher disease: dysregulated mRNAs and miRNAs in brain pathogenesis and effects of pharmacologic chaperone treatment in a mouse model. | 2015 Dec 15 |
1 |
| 27 |
24064337 | Neuronal accumulation of glucosylceramide in a mouse model of neuronopathic Gaucher disease leads to neurodegeneration. | 2014 Feb 15 |
3 |
| 28 |
25551612 | Ubiquitous transgene expression of the glucosylceramide-synthesizing enzyme accelerates glucosylceramide accumulation and storage cells in a Gaucher disease mouse model. | 2014 |
5 |
| 29 |
23377801 | The association between lysosomal protein glucocerebrosidase and Parkinson's disease. | 2013 Jan |
1 |
| 30 |
23520473 | Substrate compositional variation with tissue/region and Gba1 mutations in mouse models--implications for Gaucher disease. | 2013 |
5 |
| 31 |
23880767 | β-Glucosidase 2 (GBA2) activity and imino sugar pharmacology. | 2013 Sep 6 |
2 |
| 32 |
24070122 | Functional and genetic characterization of the non-lysosomal glucosylceramidase 2 as a modifier for Gaucher disease. | 2013 Sep 26 |
1 |
| 33 |
22566609 | Contribution of brain inflammation to neuronal cell death in neuronopathic forms of Gaucher's disease. | 2012 Jun |
1 |
| 34 |
22595426 | Immunological cell type characterization and Th1-Th17 cytokine production in a mouse model of Gaucher disease. | 2012 Jul |
1 |
| 35 |
22659419 | Beta-glucosidase 1 (GBA1) is a second bile acid β-glucosidase in addition to β-glucosidase 2 (GBA2). Study in β-glucosidase deficient mice and humans. | 2012 Jun 29 |
2 |
| 36 |
21257328 | Accumulation and distribution of α-synuclein and ubiquitin in the CNS of Gaucher disease mouse models. | 2011 Apr |
1 |
| 37 |
20015957 | Specific saposin C deficiency: CNS impairment and acid beta-glucosidase effects in the mouse. | 2010 Feb 15 |
2 |
| 38 |
20148966 | The pharmacological chaperone isofagomine increases the activity of the Gaucher disease L444P mutant form of beta-glucosidase. | 2010 Apr |
1 |
| 39 |
20962279 | Glucocerebrosidase gene-deficient mouse recapitulates Gaucher disease displaying cellular and molecular dysregulation beyond the macrophage. | 2010 Nov 9 |
2 |
| 40 |
16528760 | AAV8-mediated expression of glucocerebrosidase ameliorates the storage pathology in the visceral organs of a mouse model of Gaucher disease. | 2006 Jun |
1 |
| 41 |
17080196 | Mutation of beta-glucosidase 2 causes glycolipid storage disease and impaired male fertility. | 2006 Nov |
1 |
| 42 |
15912415 | An accumulation of glucosylceramide in the stratum corneum due to attenuated activity of beta-glucocerebrosidase is associated with the early phase of UVB-induced alteration in cutaneous barrier function. | 2005 Jul |
1 |
| 43 |
12813057 | Saposin C is required for normal resistance of acid beta-glucosidase to proteolytic degradation. | 2003 Aug 22 |
1 |
| 44 |
12126932 | Ex vivo localization of the mouse saposin C activation region for acid beta-glucosidase. | 2002 Jul |
3 |
| 45 |
11749048 | Temporal and spatial expression of murine acid beta-glucosidase mRNA. | 2001 Dec |
4 |
| 46 |
10037475 | Differential effects of glycolipid biosynthesis inhibitors on ceramide-induced cell death in neuroblastoma cells. | 1999 Mar |
2 |
| 47 |
10196186 | Sphingolipid activator proteins are required for epidermal permeability barrier formation. | 1999 Apr 16 |
1 |
| 48 |
10214939 | Accumulation of protein-bound epidermal glucosylceramides in beta-glucocerebrosidase deficient type 2 Gaucher mice. | 1999 Mar 26 |
1 |
| 49 |
7769132 | Glucosylceramides stimulate murine epidermal hyperproliferation. | 1995 Jun |
2 |
| 50 |
8163674 | Consequences of beta-glucocerebrosidase deficiency in epidermis. Ultrastructure and permeability barrier alterations in Gaucher disease. | 1994 Apr |
1 |