Journal article
Defective neurite elongation and branching in Nibp/Trappc9 deficient zebrafish and mice
International journal of biological sciences, v 19(10), pp 3226-3248
01 Jan 2023
Featured in Collection : UN Sustainable Development Goals @ Drexel
Abstract
Loss of function in transport protein particles (TRAPP) links a new set of emerging genetic disorders called “TRAPPopathies”. One such disorder is NIBP syndrome, characterized by microcephaly and intellectual disability, and caused by mutations of
NIBP/TRAPPC9
, a crucial and unique member of TRAPPII. To investigate the neural cellular/molecular mechanisms underlying microcephaly, we developed Nibp/Trappc9-deficient animal models using different techniques, including morpholino knockdown and CRISPR/Cas mutation in zebrafish and Cre/LoxP-mediated gene targeting in mice. Nibp/Trappc9 deficiency impaired the stability of the TRAPPII complex at actin filaments and microtubules of neurites and growth cones. This deficiency also impaired elongation and branching of neuronal dendrites and axons, without significant effects on neurite initiation or neural cell number/types in embryonic and adult brains. The positive correlation of TRAPPII stability and neurite elongation/branching suggests a potential role for TRAPPII in regulating neurite morphology. These results provide novel genetic/molecular evidence to define patients with a type of non-syndromic autosomal recessive intellectual disability and highlight the importance of developing therapeutic approaches targeting the TRAPPII complex to cure TRAPPopathies.
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Details
- Title
- Defective neurite elongation and branching in Nibp/Trappc9 deficient zebrafish and mice
- Creators
- Min Hu - Laboratory of Stem Cell Biology, State Key Laboratory of Biotherapy, West China Hospital, West China Medical School, Sichuan University, Chengdu 610041, China. Center for Metabolic Disease Research, Department of Pathalogy and Laboratory Medicine, Temple University Lewis Katz School of Medicine, Philadelphia, PA, USA. Center for Stem Cell Research and Application, Institute of Blood Transfusion, Chinese Academy of Medical Sciences & Peking Union Medical College (CAMS & PUMC), Chengdu 610052, China. Department of Biology, Drexel University, Philadelphia, PA, USA. Division of Digestive Diseases, Department of Medicine, Emory University School of Medicine, Atlanta, Georgia, USA. Department of Clinical Laboratory, Xi'an NO. 3 Hospital, Xi'an, Shaanxi, 710018, ChinaBrittany Bodnar - Laboratory of Stem Cell Biology, State Key Laboratory of Biotherapy, West China Hospital, West China Medical School, Sichuan University, Chengdu 610041, China. Center for Metabolic Disease Research, Department of Pathalogy and Laboratory Medicine, Temple University Lewis Katz School of Medicine, Philadelphia, PA, USA. Center for Stem Cell Research and Application, Institute of Blood Transfusion, Chinese Academy of Medical Sciences & Peking Union Medical College (CAMS & PUMC), Chengdu 610052, China. Department of Biology, Drexel University, Philadelphia, PA, USA. Division of Digestive Diseases, Department of Medicine, Emory University School of Medicine, Atlanta, Georgia, USA. Department of Clinical Laboratory, Xi'an NO. 3 Hospital, Xi'an, Shaanxi, 710018, ChinaYonggang Zhang - Drexel UniversityFangxin Xie - Laboratory of Stem Cell Biology, State Key Laboratory of Biotherapy, West China Hospital, West China Medical School, Sichuan University, Chengdu 610041, China. Center for Metabolic Disease Research, Department of Pathalogy and Laboratory Medicine, Temple University Lewis Katz School of Medicine, Philadelphia, PA, USA. Center for Stem Cell Research and Application, Institute of Blood Transfusion, Chinese Academy of Medical Sciences & Peking Union Medical College (CAMS & PUMC), Chengdu 610052, China. Department of Biology, Drexel University, Philadelphia, PA, USA. Division of Digestive Diseases, Department of Medicine, Emory University School of Medicine, Atlanta, Georgia, USA. Department of Clinical Laboratory, Xi'an NO. 3 Hospital, Xi'an, Shaanxi, 710018, ChinaFang Li - Laboratory of Stem Cell Biology, State Key Laboratory of Biotherapy, West China Hospital, West China Medical School, Sichuan University, Chengdu 610041, China. Center for Metabolic Disease Research, Department of Pathalogy and Laboratory Medicine, Temple University Lewis Katz School of Medicine, Philadelphia, PA, USA. Center for Stem Cell Research and Application, Institute of Blood Transfusion, Chinese Academy of Medical Sciences & Peking Union Medical College (CAMS & PUMC), Chengdu 610052, China. Department of Biology, Drexel University, Philadelphia, PA, USA. Division of Digestive Diseases, Department of Medicine, Emory University School of Medicine, Atlanta, Georgia, USA. Department of Clinical Laboratory, Xi'an NO. 3 Hospital, Xi'an, Shaanxi, 710018, ChinaSiying Li - Laboratory of Stem Cell Biology, State Key Laboratory of Biotherapy, West China Hospital, West China Medical School, Sichuan University, Chengdu 610041, China. Center for Metabolic Disease Research, Department of Pathalogy and Laboratory Medicine, Temple University Lewis Katz School of Medicine, Philadelphia, PA, USA. Center for Stem Cell Research and Application, Institute of Blood Transfusion, Chinese Academy of Medical Sciences & Peking Union Medical College (CAMS & PUMC), Chengdu 610052, China. Department of Biology, Drexel University, Philadelphia, PA, USA. Division of Digestive Diseases, Department of Medicine, Emory University School of Medicine, Atlanta, Georgia, USA. Department of Clinical Laboratory, Xi'an NO. 3 Hospital, Xi'an, Shaanxi, 710018, ChinaJin Zhao - Laboratory of Stem Cell Biology, State Key Laboratory of Biotherapy, West China Hospital, West China Medical School, Sichuan University, Chengdu 610041, China. Center for Metabolic Disease Research, Department of Pathalogy and Laboratory Medicine, Temple University Lewis Katz School of Medicine, Philadelphia, PA, USA. Center for Stem Cell Research and Application, Institute of Blood Transfusion, Chinese Academy of Medical Sciences & Peking Union Medical College (CAMS & PUMC), Chengdu 610052, China. Department of Biology, Drexel University, Philadelphia, PA, USA. Division of Digestive Diseases, Department of Medicine, Emory University School of Medicine, Atlanta, Georgia, USA. Department of Clinical Laboratory, Xi'an NO. 3 Hospital, Xi'an, Shaanxi, 710018, ChinaRuotong Zhao - Laboratory of Stem Cell Biology, State Key Laboratory of Biotherapy, West China Hospital, West China Medical School, Sichuan University, Chengdu 610041, China. Center for Metabolic Disease Research, Department of Pathalogy and Laboratory Medicine, Temple University Lewis Katz School of Medicine, Philadelphia, PA, USA. Center for Stem Cell Research and Application, Institute of Blood Transfusion, Chinese Academy of Medical Sciences & Peking Union Medical College (CAMS & PUMC), Chengdu 610052, China. Department of Biology, Drexel University, Philadelphia, PA, USA. Division of Digestive Diseases, Department of Medicine, Emory University School of Medicine, Atlanta, Georgia, USA. Department of Clinical Laboratory, Xi'an NO. 3 Hospital, Xi'an, Shaanxi, 710018, ChinaNaveen Gedupoori - Laboratory of Stem Cell Biology, State Key Laboratory of Biotherapy, West China Hospital, West China Medical School, Sichuan University, Chengdu 610041, China. Center for Metabolic Disease Research, Department of Pathalogy and Laboratory Medicine, Temple University Lewis Katz School of Medicine, Philadelphia, PA, USA. Center for Stem Cell Research and Application, Institute of Blood Transfusion, Chinese Academy of Medical Sciences & Peking Union Medical College (CAMS & PUMC), Chengdu 610052, China. Department of Biology, Drexel University, Philadelphia, PA, USA. Division of Digestive Diseases, Department of Medicine, Emory University School of Medicine, Atlanta, Georgia, USA. Department of Clinical Laboratory, Xi'an NO. 3 Hospital, Xi'an, Shaanxi, 710018, ChinaYifan Mo - Laboratory of Stem Cell Biology, State Key Laboratory of Biotherapy, West China Hospital, West China Medical School, Sichuan University, Chengdu 610041, China. Center for Metabolic Disease Research, Department of Pathalogy and Laboratory Medicine, Temple University Lewis Katz School of Medicine, Philadelphia, PA, USA. Center for Stem Cell Research and Application, Institute of Blood Transfusion, Chinese Academy of Medical Sciences & Peking Union Medical College (CAMS & PUMC), Chengdu 610052, China. Department of Biology, Drexel University, Philadelphia, PA, USA. Division of Digestive Diseases, Department of Medicine, Emory University School of Medicine, Atlanta, Georgia, USA. Department of Clinical Laboratory, Xi'an NO. 3 Hospital, Xi'an, Shaanxi, 710018, ChinaLanyi Lin - Laboratory of Stem Cell Biology, State Key Laboratory of Biotherapy, West China Hospital, West China Medical School, Sichuan University, Chengdu 610041, China. Center for Metabolic Disease Research, Department of Pathalogy and Laboratory Medicine, Temple University Lewis Katz School of Medicine, Philadelphia, PA, USA. Center for Stem Cell Research and Application, Institute of Blood Transfusion, Chinese Academy of Medical Sciences & Peking Union Medical College (CAMS & PUMC), Chengdu 610052, China. Department of Biology, Drexel University, Philadelphia, PA, USA. Division of Digestive Diseases, Department of Medicine, Emory University School of Medicine, Atlanta, Georgia, USA. Department of Clinical Laboratory, Xi'an NO. 3 Hospital, Xi'an, Shaanxi, 710018, ChinaXue Li - Laboratory of Stem Cell Biology, State Key Laboratory of Biotherapy, West China Hospital, West China Medical School, Sichuan University, Chengdu 610041, China. Center for Metabolic Disease Research, Department of Pathalogy and Laboratory Medicine, Temple University Lewis Katz School of Medicine, Philadelphia, PA, USA. Center for Stem Cell Research and Application, Institute of Blood Transfusion, Chinese Academy of Medical Sciences & Peking Union Medical College (CAMS & PUMC), Chengdu 610052, China. Department of Biology, Drexel University, Philadelphia, PA, USA. Division of Digestive Diseases, Department of Medicine, Emory University School of Medicine, Atlanta, Georgia, USA. Department of Clinical Laboratory, Xi'an NO. 3 Hospital, Xi'an, Shaanxi, 710018, ChinaWentong Meng - Drexel UniversityXiaofeng Yang - Laboratory of Stem Cell Biology, State Key Laboratory of Biotherapy, West China Hospital, West China Medical School, Sichuan University, Chengdu 610041, China. Center for Metabolic Disease Research, Department of Pathalogy and Laboratory Medicine, Temple University Lewis Katz School of Medicine, Philadelphia, PA, USA. Center for Stem Cell Research and Application, Institute of Blood Transfusion, Chinese Academy of Medical Sciences & Peking Union Medical College (CAMS & PUMC), Chengdu 610052, China. Department of Biology, Drexel University, Philadelphia, PA, USA. Division of Digestive Diseases, Department of Medicine, Emory University School of Medicine, Atlanta, Georgia, USA. Department of Clinical Laboratory, Xi'an NO. 3 Hospital, Xi'an, Shaanxi, 710018, ChinaHong Wang - Laboratory of Stem Cell Biology, State Key Laboratory of Biotherapy, West China Hospital, West China Medical School, Sichuan University, Chengdu 610041, China. Center for Metabolic Disease Research, Department of Pathalogy and Laboratory Medicine, Temple University Lewis Katz School of Medicine, Philadelphia, PA, USA. Center for Stem Cell Research and Application, Institute of Blood Transfusion, Chinese Academy of Medical Sciences & Peking Union Medical College (CAMS & PUMC), Chengdu 610052, China. Department of Biology, Drexel University, Philadelphia, PA, USA. Division of Digestive Diseases, Department of Medicine, Emory University School of Medicine, Atlanta, Georgia, USA. Department of Clinical Laboratory, Xi'an NO. 3 Hospital, Xi'an, Shaanxi, 710018, ChinaMary F. Barbe - Laboratory of Stem Cell Biology, State Key Laboratory of Biotherapy, West China Hospital, West China Medical School, Sichuan University, Chengdu 610041, China. Center for Metabolic Disease Research, Department of Pathalogy and Laboratory Medicine, Temple University Lewis Katz School of Medicine, Philadelphia, PA, USA. Center for Stem Cell Research and Application, Institute of Blood Transfusion, Chinese Academy of Medical Sciences & Peking Union Medical College (CAMS & PUMC), Chengdu 610052, China. Department of Biology, Drexel University, Philadelphia, PA, USA. Division of Digestive Diseases, Department of Medicine, Emory University School of Medicine, Atlanta, Georgia, USA. Department of Clinical Laboratory, Xi'an NO. 3 Hospital, Xi'an, Shaanxi, 710018, ChinaShanthi Srinivasan - Drexel UniversityJohn R. Bethea - Drexel UniversityXianming Mo - Drexel UniversityHong Xu - Drexel UniversityWenhui Hu - Laboratory of Stem Cell Biology, State Key Laboratory of Biotherapy, West China Hospital, West China Medical School, Sichuan University, Chengdu 610041, China. Center for Metabolic Disease Research, Department of Pathalogy and Laboratory Medicine, Temple University Lewis Katz School of Medicine, Philadelphia, PA, USA. Center for Stem Cell Research and Application, Institute of Blood Transfusion, Chinese Academy of Medical Sciences & Peking Union Medical College (CAMS & PUMC), Chengdu 610052, China. Department of Biology, Drexel University, Philadelphia, PA, USA. Division of Digestive Diseases, Department of Medicine, Emory University School of Medicine, Atlanta, Georgia, USA. Department of Clinical Laboratory, Xi'an NO. 3 Hospital, Xi'an, Shaanxi, 710018, China
- Publication Details
- International journal of biological sciences, v 19(10), pp 3226-3248
- Publisher
- Ivyspring International Publisher
- Resource Type
- Journal article
- Language
- English
- Academic Unit
- Biology; College of Arts and Sciences
- Web of Science ID
- WOS:001032733300017
- Scopus ID
- 2-s2.0-85164260389
- Other Identifier
- 991020669939004721
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- Collaboration types
- Domestic collaboration
- International collaboration
- Web of Science research areas
- Biochemistry & Molecular Biology
- Biology