Cryo-EM structure and oligomerization of the human planar cell polarity core protein Vangl1

Abstract Vangl is a planar cell polarity (PCP) core protein essential for aligned cell orientation along the epithelial plane perpendicular to the apical-basal direction, which is important for tissue morphogenesis, development and collective cell behavior. Mutations in Vangl are associated with dev...

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Main Authors: Fan Zhang, Shaobai Li, Hao Wu, Shanshuang Chen
Format: Article
Language:English
Published: Nature Portfolio 2025-01-01
Series:Nature Communications
Online Access:https://doi.org/10.1038/s41467-024-55397-2
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author Fan Zhang
Shaobai Li
Hao Wu
Shanshuang Chen
author_facet Fan Zhang
Shaobai Li
Hao Wu
Shanshuang Chen
author_sort Fan Zhang
collection DOAJ
description Abstract Vangl is a planar cell polarity (PCP) core protein essential for aligned cell orientation along the epithelial plane perpendicular to the apical-basal direction, which is important for tissue morphogenesis, development and collective cell behavior. Mutations in Vangl are associated with developmental defects, including neural tube defects (NTDs), according to human cohort studies of sporadic and familial cases. The complex mechanisms underlying Vangl-mediated PCP signaling or Vangl-associated human congenital diseases have been hampered by the lack of molecular characterizations of Vangl. Here, we show biochemical and structural evidence that human Vangl1 oligomerizes as dimers of trimers, and that the dimerization of trimers promotes binding to the PCP effector Prickle1 (Pk1) in vitro. Mapping of human disease-associated point mutations suggests potential pathological mechanisms and paves the way for future studies on the importance of lipid binding, central vestibule and oligomerization of Vangl, thereby providing insights into the molecular mechanisms of the PCP signaling pathway.
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spelling doaj-art-e42bfd4445844fab80b71f9010dd8a7d2025-08-20T01:48:02ZengNature PortfolioNature Communications2041-17232025-01-0116111110.1038/s41467-024-55397-2Cryo-EM structure and oligomerization of the human planar cell polarity core protein Vangl1Fan Zhang0Shaobai Li1Hao Wu2Shanshuang Chen3Shanghai Institute of Precision Medicine, Ninth People’s Hospital, Shanghai Jiao Tong University School of MedicineShanghai Institute of Precision Medicine, Ninth People’s Hospital, Shanghai Jiao Tong University School of MedicineDepartment of Otolaryngology-Head and Neck Surgery, Shanghai Ninth People’s HospitalShanghai Institute of Precision Medicine, Ninth People’s Hospital, Shanghai Jiao Tong University School of MedicineAbstract Vangl is a planar cell polarity (PCP) core protein essential for aligned cell orientation along the epithelial plane perpendicular to the apical-basal direction, which is important for tissue morphogenesis, development and collective cell behavior. Mutations in Vangl are associated with developmental defects, including neural tube defects (NTDs), according to human cohort studies of sporadic and familial cases. The complex mechanisms underlying Vangl-mediated PCP signaling or Vangl-associated human congenital diseases have been hampered by the lack of molecular characterizations of Vangl. Here, we show biochemical and structural evidence that human Vangl1 oligomerizes as dimers of trimers, and that the dimerization of trimers promotes binding to the PCP effector Prickle1 (Pk1) in vitro. Mapping of human disease-associated point mutations suggests potential pathological mechanisms and paves the way for future studies on the importance of lipid binding, central vestibule and oligomerization of Vangl, thereby providing insights into the molecular mechanisms of the PCP signaling pathway.https://doi.org/10.1038/s41467-024-55397-2
spellingShingle Fan Zhang
Shaobai Li
Hao Wu
Shanshuang Chen
Cryo-EM structure and oligomerization of the human planar cell polarity core protein Vangl1
Nature Communications
title Cryo-EM structure and oligomerization of the human planar cell polarity core protein Vangl1
title_full Cryo-EM structure and oligomerization of the human planar cell polarity core protein Vangl1
title_fullStr Cryo-EM structure and oligomerization of the human planar cell polarity core protein Vangl1
title_full_unstemmed Cryo-EM structure and oligomerization of the human planar cell polarity core protein Vangl1
title_short Cryo-EM structure and oligomerization of the human planar cell polarity core protein Vangl1
title_sort cryo em structure and oligomerization of the human planar cell polarity core protein vangl1
url https://doi.org/10.1038/s41467-024-55397-2
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AT haowu cryoemstructureandoligomerizationofthehumanplanarcellpolaritycoreproteinvangl1
AT shanshuangchen cryoemstructureandoligomerizationofthehumanplanarcellpolaritycoreproteinvangl1