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ResearchIn-Press PreviewCell biologyPulmonology
Open Access | 
10.1172/JCI193367
Loss of RPGR disrupts motile cilia and causes primary ciliary dyskinesia by affecting F-actin dynamics
Yang Wu,1 Erika Tavares,2 Binrun Liang,1 Wallace B Wee,3 Vito Mennella,4 Han-Chao Feng,1 Jiaying Cao,1 Pui Yee Wong,1 Jiayi Zheng,5 Mu He,5 Kirk AJ Stephenson,6 Liran Hanan Hochma,6 Janice Min Li,2 Nan-Peng Chen,7 Sharon D Dell,3 Elise Heon,2 and ZHEN LIU1
1Department of Life Science, The Hong Kong University of Science and Technology, Sai Kung, Hong Kong
2Genetics & Genome Biology Program, The Hospital for Sick Children, Toronto, Canada
3Child Health Evaluative Sciences, The Hospital for Sick Children, Toronto, Canada
4Department of Biochemistry, Queen Mary University of London, London, United Kingdom
5School of Biomedical Sciences, The University of Hong Kong, Pok Fu Lam, Hong Kong
6Department of Ophthalmology and Vision Sciences, The Hospital for Sick Children, Toronto, Canada
7Institute of Systems and Physical Biology, Shenzhen Bay Laboratory, Shenzhen, China
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1Department of Life Science, The Hong Kong University of Science and Technology, Sai Kung, Hong Kong
2Genetics & Genome Biology Program, The Hospital for Sick Children, Toronto, Canada
3Child Health Evaluative Sciences, The Hospital for Sick Children, Toronto, Canada
4Department of Biochemistry, Queen Mary University of London, London, United Kingdom
5School of Biomedical Sciences, The University of Hong Kong, Pok Fu Lam, Hong Kong
6Department of Ophthalmology and Vision Sciences, The Hospital for Sick Children, Toronto, Canada
7Institute of Systems and Physical Biology, Shenzhen Bay Laboratory, Shenzhen, China
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Tavares, E.
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1Department of Life Science, The Hong Kong University of Science and Technology, Sai Kung, Hong Kong
2Genetics & Genome Biology Program, The Hospital for Sick Children, Toronto, Canada
3Child Health Evaluative Sciences, The Hospital for Sick Children, Toronto, Canada
4Department of Biochemistry, Queen Mary University of London, London, United Kingdom
5School of Biomedical Sciences, The University of Hong Kong, Pok Fu Lam, Hong Kong
6Department of Ophthalmology and Vision Sciences, The Hospital for Sick Children, Toronto, Canada
7Institute of Systems and Physical Biology, Shenzhen Bay Laboratory, Shenzhen, China
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1Department of Life Science, The Hong Kong University of Science and Technology, Sai Kung, Hong Kong
2Genetics & Genome Biology Program, The Hospital for Sick Children, Toronto, Canada
3Child Health Evaluative Sciences, The Hospital for Sick Children, Toronto, Canada
4Department of Biochemistry, Queen Mary University of London, London, United Kingdom
5School of Biomedical Sciences, The University of Hong Kong, Pok Fu Lam, Hong Kong
6Department of Ophthalmology and Vision Sciences, The Hospital for Sick Children, Toronto, Canada
7Institute of Systems and Physical Biology, Shenzhen Bay Laboratory, Shenzhen, China
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1Department of Life Science, The Hong Kong University of Science and Technology, Sai Kung, Hong Kong
2Genetics & Genome Biology Program, The Hospital for Sick Children, Toronto, Canada
3Child Health Evaluative Sciences, The Hospital for Sick Children, Toronto, Canada
4Department of Biochemistry, Queen Mary University of London, London, United Kingdom
5School of Biomedical Sciences, The University of Hong Kong, Pok Fu Lam, Hong Kong
6Department of Ophthalmology and Vision Sciences, The Hospital for Sick Children, Toronto, Canada
7Institute of Systems and Physical Biology, Shenzhen Bay Laboratory, Shenzhen, China
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1Department of Life Science, The Hong Kong University of Science and Technology, Sai Kung, Hong Kong
2Genetics & Genome Biology Program, The Hospital for Sick Children, Toronto, Canada
3Child Health Evaluative Sciences, The Hospital for Sick Children, Toronto, Canada
4Department of Biochemistry, Queen Mary University of London, London, United Kingdom
5School of Biomedical Sciences, The University of Hong Kong, Pok Fu Lam, Hong Kong
6Department of Ophthalmology and Vision Sciences, The Hospital for Sick Children, Toronto, Canada
7Institute of Systems and Physical Biology, Shenzhen Bay Laboratory, Shenzhen, China
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1Department of Life Science, The Hong Kong University of Science and Technology, Sai Kung, Hong Kong
2Genetics & Genome Biology Program, The Hospital for Sick Children, Toronto, Canada
3Child Health Evaluative Sciences, The Hospital for Sick Children, Toronto, Canada
4Department of Biochemistry, Queen Mary University of London, London, United Kingdom
5School of Biomedical Sciences, The University of Hong Kong, Pok Fu Lam, Hong Kong
6Department of Ophthalmology and Vision Sciences, The Hospital for Sick Children, Toronto, Canada
7Institute of Systems and Physical Biology, Shenzhen Bay Laboratory, Shenzhen, China
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1Department of Life Science, The Hong Kong University of Science and Technology, Sai Kung, Hong Kong
2Genetics & Genome Biology Program, The Hospital for Sick Children, Toronto, Canada
3Child Health Evaluative Sciences, The Hospital for Sick Children, Toronto, Canada
4Department of Biochemistry, Queen Mary University of London, London, United Kingdom
5School of Biomedical Sciences, The University of Hong Kong, Pok Fu Lam, Hong Kong
6Department of Ophthalmology and Vision Sciences, The Hospital for Sick Children, Toronto, Canada
7Institute of Systems and Physical Biology, Shenzhen Bay Laboratory, Shenzhen, China
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1Department of Life Science, The Hong Kong University of Science and Technology, Sai Kung, Hong Kong
2Genetics & Genome Biology Program, The Hospital for Sick Children, Toronto, Canada
3Child Health Evaluative Sciences, The Hospital for Sick Children, Toronto, Canada
4Department of Biochemistry, Queen Mary University of London, London, United Kingdom
5School of Biomedical Sciences, The University of Hong Kong, Pok Fu Lam, Hong Kong
6Department of Ophthalmology and Vision Sciences, The Hospital for Sick Children, Toronto, Canada
7Institute of Systems and Physical Biology, Shenzhen Bay Laboratory, Shenzhen, China
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1Department of Life Science, The Hong Kong University of Science and Technology, Sai Kung, Hong Kong
2Genetics & Genome Biology Program, The Hospital for Sick Children, Toronto, Canada
3Child Health Evaluative Sciences, The Hospital for Sick Children, Toronto, Canada
4Department of Biochemistry, Queen Mary University of London, London, United Kingdom
5School of Biomedical Sciences, The University of Hong Kong, Pok Fu Lam, Hong Kong
6Department of Ophthalmology and Vision Sciences, The Hospital for Sick Children, Toronto, Canada
7Institute of Systems and Physical Biology, Shenzhen Bay Laboratory, Shenzhen, China
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1Department of Life Science, The Hong Kong University of Science and Technology, Sai Kung, Hong Kong
2Genetics & Genome Biology Program, The Hospital for Sick Children, Toronto, Canada
3Child Health Evaluative Sciences, The Hospital for Sick Children, Toronto, Canada
4Department of Biochemistry, Queen Mary University of London, London, United Kingdom
5School of Biomedical Sciences, The University of Hong Kong, Pok Fu Lam, Hong Kong
6Department of Ophthalmology and Vision Sciences, The Hospital for Sick Children, Toronto, Canada
7Institute of Systems and Physical Biology, Shenzhen Bay Laboratory, Shenzhen, China
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1Department of Life Science, The Hong Kong University of Science and Technology, Sai Kung, Hong Kong
2Genetics & Genome Biology Program, The Hospital for Sick Children, Toronto, Canada
3Child Health Evaluative Sciences, The Hospital for Sick Children, Toronto, Canada
4Department of Biochemistry, Queen Mary University of London, London, United Kingdom
5School of Biomedical Sciences, The University of Hong Kong, Pok Fu Lam, Hong Kong
6Department of Ophthalmology and Vision Sciences, The Hospital for Sick Children, Toronto, Canada
7Institute of Systems and Physical Biology, Shenzhen Bay Laboratory, Shenzhen, China
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1Department of Life Science, The Hong Kong University of Science and Technology, Sai Kung, Hong Kong
2Genetics & Genome Biology Program, The Hospital for Sick Children, Toronto, Canada
3Child Health Evaluative Sciences, The Hospital for Sick Children, Toronto, Canada
4Department of Biochemistry, Queen Mary University of London, London, United Kingdom
5School of Biomedical Sciences, The University of Hong Kong, Pok Fu Lam, Hong Kong
6Department of Ophthalmology and Vision Sciences, The Hospital for Sick Children, Toronto, Canada
7Institute of Systems and Physical Biology, Shenzhen Bay Laboratory, Shenzhen, China
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1Department of Life Science, The Hong Kong University of Science and Technology, Sai Kung, Hong Kong
2Genetics & Genome Biology Program, The Hospital for Sick Children, Toronto, Canada
3Child Health Evaluative Sciences, The Hospital for Sick Children, Toronto, Canada
4Department of Biochemistry, Queen Mary University of London, London, United Kingdom
5School of Biomedical Sciences, The University of Hong Kong, Pok Fu Lam, Hong Kong
6Department of Ophthalmology and Vision Sciences, The Hospital for Sick Children, Toronto, Canada
7Institute of Systems and Physical Biology, Shenzhen Bay Laboratory, Shenzhen, China
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1Department of Life Science, The Hong Kong University of Science and Technology, Sai Kung, Hong Kong
2Genetics & Genome Biology Program, The Hospital for Sick Children, Toronto, Canada
3Child Health Evaluative Sciences, The Hospital for Sick Children, Toronto, Canada
4Department of Biochemistry, Queen Mary University of London, London, United Kingdom
5School of Biomedical Sciences, The University of Hong Kong, Pok Fu Lam, Hong Kong
6Department of Ophthalmology and Vision Sciences, The Hospital for Sick Children, Toronto, Canada
7Institute of Systems and Physical Biology, Shenzhen Bay Laboratory, Shenzhen, China
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1Department of Life Science, The Hong Kong University of Science and Technology, Sai Kung, Hong Kong
2Genetics & Genome Biology Program, The Hospital for Sick Children, Toronto, Canada
3Child Health Evaluative Sciences, The Hospital for Sick Children, Toronto, Canada
4Department of Biochemistry, Queen Mary University of London, London, United Kingdom
5School of Biomedical Sciences, The University of Hong Kong, Pok Fu Lam, Hong Kong
6Department of Ophthalmology and Vision Sciences, The Hospital for Sick Children, Toronto, Canada
7Institute of Systems and Physical Biology, Shenzhen Bay Laboratory, Shenzhen, China
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Heon, E.
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1Department of Life Science, The Hong Kong University of Science and Technology, Sai Kung, Hong Kong
2Genetics & Genome Biology Program, The Hospital for Sick Children, Toronto, Canada
3Child Health Evaluative Sciences, The Hospital for Sick Children, Toronto, Canada
4Department of Biochemistry, Queen Mary University of London, London, United Kingdom
5School of Biomedical Sciences, The University of Hong Kong, Pok Fu Lam, Hong Kong
6Department of Ophthalmology and Vision Sciences, The Hospital for Sick Children, Toronto, Canada
7Institute of Systems and Physical Biology, Shenzhen Bay Laboratory, Shenzhen, China
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Published March 31, 2026 - More info
Copyright © 2026, Wu et al. This work is licensed under the Creative Commons Attribution 4.0 International License. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/.
Copyright © 2026 American Society for Clinical Investigation
ISSN: 0021-9738 (print), 1558-8238 (online)