Proteomics

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Multiomics analysis of a DNAH5-mutated PCD organoid model revealed the key role of the TGF-β/BMP and Notch pathways in epithelial differentiation and the immune response in DNAH5-mutated patients

ABSTRACT: Dynein axonemal heavy chain 5 (DNAH5) is the most mutated gene in primary ciliary dyskinesia (PCD), leading to abnormal cilia ultrastructure and function. Few studies have revealed the genetic characteristics and pathogenetic mechanisms of PCD caused by DNAH5 mutation. Here, we established a child PCD airway organoid directly from the bronchoscopic biopsy of a patient with DNAH5 mutation. We found abnormal ciliary function and a decreased immune response caused by DNAH5 mutation through proteomic analyses.

INSTRUMENT(S): Q Exactive

ORGANISM(S): Homo Sapiens (human)

TISSUE(S): Cell Culture

SUBMITTER: Wenhao Yang  

LAB HEAD: Hanmin Liu

PROVIDER: PXD038024 | Pride | 2023-03-11

REPOSITORIES: Pride

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Multiomics Analysis of a <i>DNAH5</i>-Mutated PCD Organoid Model Revealed the Key Role of the TGF-β/BMP and Notch Pathways in Epithelial Differentiation and the Immune Response in <i>DNAH5</i>-Mutated Patients.

Yang Wenhao W   Chen Lina L   Guo Juncen J   Shi Fang F   Yang Qingxin Q   Xie Liang L   Lu Danli D   Li Yingna Y   Luo Jiaxin J   Wang Li L   Qiu Li L   Chen Ting T   Li Yan Y   Zhang Rui R   Chen Lu L   Xu Wenming W   Liu Hanmin H  

Cells 20221212 24

Dynein axonemal heavy chain 5 (DNAH5) is the most mutated gene in primary ciliary dyskinesia (PCD), leading to abnormal cilia ultrastructure and function. Few studies have revealed the genetic characteristics and pathogenetic mechanisms of PCD caused by DNAH5 mutation. Here, we established a child PCD airway organoid directly from the bronchoscopic biopsy of a patient with the DNAH5 mutation. The motile cilia in the organoid were observed and could be stably maintained for an extended time. We f  ...[more]

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