Abstract
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.
Published by Elsevier Inc.
Publication types
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Research Support, N.I.H., Intramural
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Research Support, Non-U.S. Gov't
MeSH terms
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Animals
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Astrocytes / metabolism
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Fibroblasts
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Gene Expression / genetics
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Humans
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Intracellular Signaling Peptides and Proteins / genetics*
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Mice
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Mice, Knockout
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Microglia / metabolism
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Muramidase / genetics*
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Niemann-Pick C1 Protein
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Niemann-Pick Disease, Type C / genetics*
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Niemann-Pick Disease, Type C / pathology
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Toll-Like Receptors / genetics*
Substances
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Intracellular Signaling Peptides and Proteins
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NPC1 protein, human
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Niemann-Pick C1 Protein
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Toll-Like Receptors
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Muramidase