医学分子生物学杂志2025，Vol.22Issue(6)：531-538,8.DOI:10.3870/j.issn.1672-8009.2025.06.001
ClC-7在肺泡Ⅱ型细胞板层小体生物发生过程中的作用
Role of ClC-7 in Biogenesis of Lamellar Body in Alveolar Type Ⅱ Cells
摘要
Abstract
Objective To explore the role of ClC-7 in lamellar body(LB)biogenesis and to investigate the possible pathogenesis of some ClC-7 mutants from patients.Methods siRNA knock-down was employed to decrease the expression of ClC-7 in A549 cells,followed by observing LB morphological changes using laser confocal microscopy and transmission electron microscopy.Wild-type and mutant ClC-7 plasmids were constructed,and the co-localization of ClC-7 with LB and oth-er organelles was observed using immunofluorescence method.Results ClC-7 was localized to LBs and lysosomes.The size of LBs in the ClC-7 knockdown cells was enlarged.Mutations of p.G203D,p.G215R,p.G292E,p.R403Q,and p.L766P,led to mislocalization of ClC-7 to the endoplasmic reticulum.Mutation of p.P470L lead to partial retention of ClC-7 on early endosomes but mostly localizes on LBs.Conclusion ClC-7 in A549 cells is localized to both LBs and lyso-somes.Knockdown of ClC-7 in cultured cells leads to enlarged LBs.Most of the mutated ClC-7 can-not be correctly localized on lamellar bodies and lysosomes.关键词
ClC-7/板层小体/溶酶体相关细胞器/肺泡Ⅱ型细胞/间质性肺病Key words
ClC-7/lamellar body/lysosome-related organelles/alveolar type Ⅱ cell/inter-stitial lung disease分类
医药卫生引用本文复制引用
周子旋,郝振华,李巍..ClC-7在肺泡Ⅱ型细胞板层小体生物发生过程中的作用[J].医学分子生物学杂志,2025,22(6):531-538,8.基金项目
国家自然科学基金(No.32293204,No.32271157) This work was supported by grants from the National Natural Science Foundation of China(No.32293204,No.32271157) （No.32293204,No.32271157）
