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ZC4H2 stabilizes RNF220 to pattern ventral spinal cord through modulating Shh/Gli signaling

摘要ZC4H2 encodes a C4H2 type zinc-finger nuclear factor,the mutation of which has been associated with disorders with various clinical phenotypes in human,including developmental delay,intellectual disability and dystonia.ZC4H2 has been suggested to regulate spinal cord patterning in zebrafish as a co-factor for RNF220,an ubiquitin E3 ligase involved in Gli signaling.Here we showed that ZC4H2 and RNF220 knockout animals phenocopy each other in spinal patterning in both mouse and zebrafish,with mispatterned progenitor and neuronal domains in the ventral spinal cord.We showed evidence that ZC4H2 is required for the stability of RNF220 and also proper Gli ubiquitination and signaling in vivo.Our data provides new insights into the possible etiology of the neurodevelopmental impairments observed in ZC4H2-associated syndromes.

作者单位 State Key Laboratory of Genetic Resources and Evolution, Kunming Institute of Zoology, Chinese Academy of Sciences, Kunming 650223, China ^[1] State Key Laboratory of Medical Neurobiology and MOE Frontiers Center for Brain Science, Institute of Brain Sciences, Fudan University, Shanghai 200032, China ^[2] Affiliated Hospital of Guangdong Medical University, Zhanjiang 524001, China ^[3] State Key Laboratory of Genetic Resources and Evolution, Kunming Institute of Zoology, Chinese Academy of Sciences, Kunming 650223, China;Kunming College of Life Science, University of Chinese Academy of Sciences, Kunming 650223, China ^[4] Key Laboratory of Arrhythmias, Ministry of Education of China, East Hospital, and Department of Anatomy and Neurobiology, Collaborative Innovation Center for Brain Science, Tongji University School of Medicine, Shanghai 200092, China ^[5] State Key Laboratory of Genetic Resources and Evolution, Kunming Institute of Zoology, Chinese Academy of Sciences, Kunming 650223, China;Department of Clinical Laboratory, The Affiliated Hospital of KMUST, Medical Faculty, Kunming University of Science and Technology, Kunming 650032, China ^[6] State Key Laboratory of Phytochemistry and Plant Resources in West China, Kunming Institute of Botany, Chinese Academy of Sciences, Kunming 650203, China ^[7] State Key Laboratory of Medical Neurobiology and MOE Frontiers Center for Brain Science, Institute of Brain Sciences, Fudan University, Shanghai 200032, China;Key Laboratory of Arrhythmias, Ministry of Education of China, East Hospital, and Department of Anatomy and Neurobiology, Collaborative Innovation Center for Brain Science, Tongji University School of Medicine, Shanghai 200092, China;Department of Laboratory Animal Science, Fundan University, Shanghai 200032, China ^[8] State Key Laboratory of Genetic Resources and Evolution, Kunming Institute of Zoology, Chinese Academy of Sciences, Kunming 650223, China;Center for Excellence in Animal Evolution and Genetics, Chinese Academy of Sciences, Kunming 650223, China ^[9]

关键词 ZC4H2 RNF220 spinal cord patterning Gli signaling

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DOI 10.1093/jmcb/mjz087

发布时间 2020-07-27（万方平台首次上网日期，不代表论文的发表时间）

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