Analysis of Wnt7B and BMP4 expression patterns in congenital pulmonary airway malformation
Analysis of Wnt7B and BMP4 expression patterns in congenital pulmonary airway malformation
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先天性肺气道畸形中Wnt7B和BMP4表达模式分析
DOI：
10.1002/ppul.24651
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发表时间：
2020-01-21
影响因子：
3.1
通讯作者：
Jia，Huimin
中科院分区：
文献类型：
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作者：
Zhu，Hao;Liu，Dan;Jia，Huimin
研究背景先天性肺气道畸形（congenitalpulmonaryairwaymalformation，CPAM）是一种以终末细支气管异常过度生长为特征的罕见疾病.本研究的目的是描述无翅型MMTV整合位点家族7 B（Wnt 7 B）和骨形态发生蛋白4（BMP 4）在人CPAM病变中的表达模式，并探讨Wnt 7 B和BMP 4在CPAM发病机制中的可能作用。采用免疫组化（IHC）、定量真实的时间聚合酶链反应（qRT-PCR）和Western blotting检测Wnt 7 B和BMP 4的表达。Wnt 7 B和BMP 4免疫阳性细胞在CPAM病变中的表达高于癌旁正常肺组织。qRT-PCR和Western blotting显示，Wnt 7 B和BMP 4 mRNA和蛋白在CPAM病变中的表达显著高于邻近正常肺组织（P<0.05）。结论Wnt 7 B和BMP 4的表达增加可能与肺动脉高压的发病机制及肺发育异常有关。Wnt 7 B和BMP 4的上调可能在表征CPAM的支气管肺泡结构的发育中发挥重要作用。
BackgroundCongenital pulmonary airway malformation (CPAM) is a rare disorder characterized by aberrant overgrowth of terminal bronchioles. The objective of this study was to describe wingless‐type MMTV integration site family 7B (Wnt7B) and bone morphogenetic protein 4 (BMP4) expression patterns in human CPAM lesions and to explore the possible roles of Wnt7B and BMP4 in the pathogenesis of CPAM.MethodsFifteen tissue samples from patients with CPAM were obtained from the Pathology Department of Shengjing Hospital of China Medical University. Samples representing CPAM lesions and adjacent normal lung tissues were collected and Wnt7B and BMP4 expression was detected through immunohistochemical (IHC) staining, quantitative real‐time polymerase chain reaction (qRT‐PCR), and Western blotting.ResultsIHC revealed that Wnt7B immunopositive cells were only detected in epithelial cells, whereas BMP4 immunopositive cells were detected in epithelial and mesenchymal cells. Expression of Wnt7B and BMP4 immunopositive cells was higher in CPAM lesions than that in adjacent normal lung tissue. qRT‐PCR and Western blotting showed that Wnt7B and BMP4 mRNA and protein expression were significantly higher in CPAM lesions than in adjacent normal lung tissue (P< .05). Overall, the level of BMP4 was higher than that of Wnt7B.ConclusionsIncreased expression of Wnt7B and BMP4 appear to be related to the pathogenesis of CPAM and abnormal pulmonary development. Upregulation of Wnt7B and BMP4 could play an important role in the development of the bronchial‐alveolar structures that characterize CPAM.