Nature Communications (Jun 2017)
WNT10A mutation causes ectodermal dysplasia by impairing progenitor cell proliferation and KLF4-mediated differentiation
- Mingang Xu,
- Jeremy Horrell,
- Melinda Snitow,
- Jiawei Cui,
- Heather Gochnauer,
- Camille M. Syrett,
- Staci Kallish,
- John T. Seykora,
- Fei Liu,
- Dany Gaillard,
- Jonathan P. Katz,
- Klaus H. Kaestner,
- Brooke Levin,
- Corinne Mansfield,
- Jennifer E. Douglas,
- Beverly J. Cowart,
- Michael Tordoff,
- Fang Liu,
- Xuming Zhu,
- Linda A. Barlow,
- Adam I. Rubin,
- John A. McGrath,
- Edward E. Morrisey,
- Emily Y. Chu,
- Sarah E. Millar
Affiliations
- Mingang Xu
- Department of Dermatology, Perelman School of Medicine, University of Pennsylvania
- Jeremy Horrell
- Department of Dermatology, Perelman School of Medicine, University of Pennsylvania
- Melinda Snitow
- Department of Medicine, Perelman School of Medicine, University of Pennsylvania
- Jiawei Cui
- Department of Dermatology, Perelman School of Medicine, University of Pennsylvania
- Heather Gochnauer
- Department of Dermatology, Perelman School of Medicine, University of Pennsylvania
- Camille M. Syrett
- Department of Dermatology, Perelman School of Medicine, University of Pennsylvania
- Staci Kallish
- Department of Medicine, Perelman School of Medicine, University of Pennsylvania
- John T. Seykora
- Department of Dermatology, Perelman School of Medicine, University of Pennsylvania
- Fei Liu
- Molecular & Cellular Medicine Department, Texas A&M University Health Science Center
- Dany Gaillard
- Department of Cell and Developmental Biology, and Rocky Mountain Taste and Smell Center, University of Colorado School of Medicine
- Jonathan P. Katz
- Department of Medicine, Perelman School of Medicine, University of Pennsylvania
- Klaus H. Kaestner
- Department of Genetics, Perelman School of Medicine, University of Pennsylvania
- Brooke Levin
- William G. Rohrer Cancer Genetics Program, M.D. Anderson Cancer Center at Cooper
- Corinne Mansfield
- Monell Chemical Senses Center
- Jennifer E. Douglas
- Monell Chemical Senses Center
- Beverly J. Cowart
- Monell Chemical Senses Center
- Michael Tordoff
- Monell Chemical Senses Center
- Fang Liu
- Department of Dermatology, Perelman School of Medicine, University of Pennsylvania
- Xuming Zhu
- Department of Dermatology, Perelman School of Medicine, University of Pennsylvania
- Linda A. Barlow
- Department of Cell and Developmental Biology, and Rocky Mountain Taste and Smell Center, University of Colorado School of Medicine
- Adam I. Rubin
- Department of Dermatology, Perelman School of Medicine, University of Pennsylvania
- John A. McGrath
- Department of Medical and Molecular Genetics, St John’s Institute of Dermatology, King’s College London
- Edward E. Morrisey
- Department of Medicine, Perelman School of Medicine, University of Pennsylvania
- Emily Y. Chu
- Department of Dermatology, Perelman School of Medicine, University of Pennsylvania
- Sarah E. Millar
- Department of Dermatology, Perelman School of Medicine, University of Pennsylvania
- DOI
- https://doi.org/10.1038/ncomms15397
- Journal volume & issue
-
Vol. 8,
no. 1
pp. 1 – 21
Abstract
Human WNT10A mutations are associated with dental defects and adult onset ectodermal dysplasia. Xuet al. show that WNT10A-activated ß-catenin plays dual roles in adult epithelial progenitor proliferation and differentiation by complexing with KLF4 in differentiating, but not proliferating, cells.