Journal
MOLECULAR GENETICS AND METABOLISM
Volume 131, Issue 3, Pages 364-366Publisher
ACADEMIC PRESS INC ELSEVIER SCIENCE
DOI: 10.1016/j.ymgme.2020.10.009
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Funding
- Eunice Kennedy Shriver National Institute of Child Health and Human Development
- National Human Genome Institute
- Ara Parseghian Medical Research Fund at the University of Notre Dame
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Niemann-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. Lyz2 expression is induced by Toll-like receptors potentially in response to lipid storage but does not play a functional role in NPC disease pathology.
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