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Toll-like receptor mediated lysozyme expression in Niemann-pick disease, type C1
Molecular Genetics and Metabolism ( IF 3.7 ) Pub Date : 2020-10-18 , DOI: 10.1016/j.ymgme.2020.10.009
Antony Cougnoux 1 , Julia C Yerger 1 , Mason Fellmeth 1 , Jenny Serra-Vinardell 2 , Christopher A Wassif 1 , Niamh X Cawley 1 , Forbes D Porter 1
Affiliation  

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.



中文翻译:

Toll 样受体介导的 Niemann-pick 病 C1 型溶菌酶表达

Niemann-Pick C1 型 (NPC1) 是一种罕见的神经退行性疾病。在 NPC1 小鼠小脑中,抗菌酶溶菌酶 ( Lyz2 ) 在多种细胞类型中显着增加。由于其在毒性原纤维沉积中的可能作用,我们证实了Lyz2在不同对照和 NPC1 细胞类型(包括人 NPC1 成纤维细胞)的培养物中过表达。Lyz2表达由 Toll 样受体诱导,可能对脂质储存作出反应,但在 NPC 疾病病理学中不发挥功能作用。

更新日期:2020-12-09
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