Publications by authors named "Julia C Yerger"
Niemann-Pick disease, type C1 is a progressive, lethal, neurodegenerative disorder due to endolysosomal storage of unesterified cholesterol. Cerebellar ataxia, as a result of progressive loss of cerebellar Purkinje neurons, is a major symptom of Nieman-Pick disease, type C1. Comparing single cell RNAseq data from control (Npc1) and mutant (Npc1) mice, we observed significantly decreased expression of Slc1a3 in Npc1 astrocytes.
View Article and Find Full Text PDFNiemann-Pick type C1 (NPC1) is a rare neurodegenerative disease. In NPC1 mouse cerebella, the antibacterial enzyme, lysozyme (Lyz2), is significantly increased in multiple cell types. Due to its possible role in toxic fibril deposition, we confirmed Lyz2 overexpression in culture in different control and NPC1 cell types including human NPC1 fibroblasts.
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- * Researchers analyzed single-cell transcriptome data from mutant mice at different ages to understand cerebellar neuropathology, finding increased activation of microglia and expression of innate immunity-related genes in symptomatic mice.
- * The study suggests that microglial activation occurs before observable neuronal dysfunction, raising questions about the potential neurotoxic effects of immune response genes in non-immune cells and offering avenues for future therapeutic research.