Toll 样受体介导尼曼-匹克病 C1 型溶菌酶表达。
Toll-like receptor mediated lysozyme expression in Niemann-pick disease, type C1.
机构信息
Division of Translational Medicine, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD, USA.
Human Biochemical Genetics Section, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD, USA.
出版信息
Mol Genet Metab. 2020 Nov;131(3):364-366. doi: 10.1016/j.ymgme.2020.10.009. Epub 2020 Oct 18.
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.
摘要
尼曼-匹克病 C1 型（NPC1）是一种罕见的神经退行性疾病。在 NPC1 小鼠小脑，多种细胞类型中溶菌酶（Lyz2）显著增加。由于其在毒性纤维沉积中的可能作用，我们在不同对照和 NPC1 细胞类型（包括人 NPC1 成纤维细胞）的培养中证实了 Lyz2 的过表达。Lyz2 的表达受 Toll 样受体诱导，可能是对脂质储存的反应，但在 NPC 疾病病理学中不起功能作用。
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