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Image Search Results
Journal: Neurobiology of disease
Article Title: N-butyldeoxynojirimycin delays motor deficits, cerebellar microgliosis, and Purkinje cell loss in a mouse model of mucolipidosis type IV.
doi: 10.1016/j.nbd.2017.06.003
Figure Lengend Snippet: Fig. 4. Cerebellar lipid analysis via mass spectrometry reveals alterations in the Mcoln1−/−lipid profile with improvements to specific lipid classes upon miglustat treatment. A) Schematic of GSL synthesis pathway and abbreviations used. Purported point of action of miglustat denoted in red. B–D) Total levels detected for each lipid listed in cerebellum, including those enriched in white matter (B), complex gangliosides (C), and ceramide & globo-series glycolipids (D). E–I) Major (ie, most prevalent) isoforms detected are plotted for each lipid (complete isoform analysis plotted in Supp. Fig. 3–5.), including ceramides (E, ratio of ceramides C18:0 to C24:1 are plotted in F), CMH (G), sulfatides (H), and GM1 (I). n = 4 per group. Differences were detected by two-way ANOVA for genotype in total CMH, total CDH, total sulfatides, total GM2, C24:1 CMH, and C24:1 sulfatide; for treatment in the ratio of C18:0/C24:1 ceramide; and for both genotype and treatment in total GM1 and C18:0 GM1. Where appropriate, post hoc pairwise comparisons were made with significant differences detected between WT saline - Mcoln1−/−saline, Mcoln1−/−saline - Mcoln1−/−miglustat, WT saline - Mcoln1−/−miglustat, and WT saline - WT miglustat denoted. *p b 0.05, **p b 0.01, ***p b 0.001, ****p b 0.0001.
Article Snippet: To quantitate, the following internal standards were added to the samples: C17:0 ceramide (Avanti Polar Lipids), C15:0 GalCer for CMH (Matreya), C16:0-D3 LacCer for CDH (Matreya), C17:0 Gb3Cer for Gb3Cer and Gb4Cer (Matreya), C17:0 sphingomyelin (Avanti Polar Lipids),
Techniques: Mass Spectrometry, Saline
Journal: Neurobiology of disease
Article Title: N-butyldeoxynojirimycin delays motor deficits, cerebellar microgliosis, and Purkinje cell loss in a mouse model of mucolipidosis type IV.
doi: 10.1016/j.nbd.2017.06.003
Figure Lengend Snippet: Fig. 5. Cerebral lipid analysis in Mcoln1−/−mice reveals changes to the Mcoln1−/−lipid profile not corrected with miglustat. A–C) Total levels of lipids enriched in cerebral white matter, complex gangliosides, and ceramide & globo-series glycolipids are plotted as in Fig. 4. D–H) Major isoforms detected are plotted for each lipid as in Fig. 4 (complete isoform analysis plotted in Supp. Figs. 3–5), including ceramides (D, ratio of ceramides C18:0 to C24:1 are plotted in E), CMH (F), sulfatides (G), and GM1 (H). n = 4 per group. Differences were detected by two- way ANOVA for genotype in total CMH, total CDH, total sulfatides, total GM2, total GM3, C18:0 Cer, C24:1 Cer, C24:1 CMH, and C24:1 sulfatide; for treatment in total GM1, total Gb3Cer, total Gb4Cer, the ratio of C18:0/C24:1 ceramide, and C18:0 GM1. Pairwise comparisons were made when appropriate, with significant differences between WT saline - Mcoln1−/−saline, Mcoln1−/−saline - Mcoln1−/−miglustat, WT saline - Mcoln1−/−miglustat, and WT saline - WT miglustat denoted. *p b 0.05, **p b 0.01, ***p b 0.001, ****p b 0.0001.
Article Snippet: To quantitate, the following internal standards were added to the samples: C17:0 ceramide (Avanti Polar Lipids), C15:0 GalCer for CMH (Matreya), C16:0-D3 LacCer for CDH (Matreya), C17:0 Gb3Cer for Gb3Cer and Gb4Cer (Matreya), C17:0 sphingomyelin (Avanti Polar Lipids),
Techniques: Saline