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12. Structural specializations of the sperm tail

Author

Leung, Miguel Ricardo, Zeng, Jianwei, Wang, Xiangli, Roelofs, Marc C., Huang, Wei, Zenezini Chiozzi, Riccardo, Hevler, Johannes F., Heck, Albert J.R., Dutcher, Susan K., Brown, Alan, Zhang, Rui, and Zeev-Ben-Mordehai, Tzviya

Published
2023
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23. Undocking of an extensive ciliary network induces proteostasis and cell fate switching resulting in severe primary ciliary dyskinesia.

Author

Brody, Steven L., Pan, Jiehong, Huang, Tao, Xu, Jian, Xu, Huihui, Koenitizer, Jeffrey R., Brennan, Steven K., Nanjundappa, Rashmi, Saba, Thomas G., Rumman, Nisreen, Berical, Andrew, Hawkins, Finn J., Wang, Xiangli, Zhang, Rui, Mahjoub, Moe R., Horani, Amjad, and Dutcher, Susan K.

Subjects
CILIARY motility disorders, HETERODIMERS, CYTOSKELETAL proteins, GENE expression, CELL physiology
Abstract

Primary ciliary dyskinesia is a rare monogenic syndrome that is associated with chronic respiratory disease, infertility, and laterality defects. Although more than 50 genes causative of primary ciliary dyskinesia have been identified, variants in the genes encoding coiled-coil domain-containing 39 (CCDC39) and CCDC40 in particular cause severe disease that is not explained by loss of ciliary motility alone. Here, we sought to understand the consequences of these variants on cellular functions beyond impaired motility. We used human cells with pathogenic variants in CCDC39 and CCDC40, Chlamydomonas reinhardtii genetics, cryo–electron microscopy, and proteomics to define perturbations in ciliary assembly and cilia stability, as well as multiple motility-independent pathways. Analysis of proteomics of cilia from patient cells identified that the absence of the axonemal CCDC39/CCDC40 heterodimer resulted in the loss of a network of more than 90 ciliary structural proteins, including 14 that were defined as ciliary address recognition proteins, which provide docking for the missing structures. The absence of the network impaired microtubule architecture, activated cell quality control pathways, switched multiciliated cell fate to mucus-producing cells and resulted in a defective periciliary barrier. In CCDC39 variant cells, these phenotypes were reversed through expression of a normal CCDC39 transgene. These findings indicate that the CCDC39/CCDC40 heterodimer functions as a scaffold to support the assembly of an extensive network of ciliary proteins, whose loss results in both motility-dependent and motility-independent phenotypes that may explain the severity of disease. Gene therapy might be a potential treatment option to be explored in future studies. Editor's summary: Primary ciliary dyskinesia (PCD) is a genetic disease characterized by chronic respiratory infections, and individuals with PCD caused by variants in CCDC39 (coiled-coil domain-containing 39) or CCDC40 exhibit more severe phenotypes than those with disease caused variants in other PCD-causing genes. Here, Brody and colleagues showed that the absence of the CCDC39/CCDC40 heterodimer results in loss of a large network of ciliary proteins that dock to the heterodimer through ciliary address recognition proteins, cell fate switching from multiciliated cells to mucus-producing cells, and loss of the periciliary barrier. These motility-dependent and motility-independent cellular phenotypes may account for the severe disease seen in patients and could be improved by expression of a normal CCDC39 transgene in CCDC39 variant cells, suggesting that gene therapy could be further explored for this disease. —Melissa L. Norton [ABSTRACT FROM AUTHOR]

Published
2025
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28. Quantitative Analysis of Uranium in Gas Sampling Filter by Laser-induced Breakdown Spectroscopy

Author

WANG Xiangli;WANG Yanling;GAO Zhixing;CHENG Yimei;SI Yu

Subjects
laser-induced breakdown spectroscopy, uranium, gas sampling filter, Nuclear engineering. Atomic power, TK9001-9401, Nuclear and particle physics. Atomic energy. Radioactivity, QC770-798
Abstract

For nuclear facility exhaust gas monitoring, it is necessary to measure and analyze the concentration of uranium in the gas sampling filter. In this paper, the reasearch of laser-induced breakdown spectroscopy (LIBS) quantitative analysis technology basing on calibration curve was carried out. By analyzing the filters containing uranium by using LIBS technology, the effective characteristic spectrum of uranium was selected after optimizing the delay time parameter, and quantitative calibration curves within a relatively lower concentration range were obtained. The verification research results show that, under the optimized experimental conditions, the bias of measurement is less than 20%, and the RSD is less than 10%.

Published
2022

37. Exploration of TCM Culture Communication Based on the New Media

Author

Wang, Xiangli, Du, Xueqin, Kacprzyk, Janusz, Series Editor, Pal, Nikhil R., Advisory Editor, Bello Perez, Rafael, Advisory Editor, Corchado, Emilio S., Advisory Editor, Hagras, Hani, Advisory Editor, Kóczy, László T., Advisory Editor, Kreinovich, Vladik, Advisory Editor, Lin, Chin-Teng, Advisory Editor, Lu, Jie, Advisory Editor, Melin, Patricia, Advisory Editor, Nedjah, Nadia, Advisory Editor, Nguyen, Ngoc Thanh, Advisory Editor, Wang, Jun, Advisory Editor, Atiquzzaman, Mohammed, editor, Yen, Neil, editor, and Xu, Zheng, editor

Published
2020
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43. Effect of Pore Size on Carbon Dioxide Adsorption and Specific Capacity as Supercapacitors Electrode by Activated Carbon Fibers Prepared from Natural Cotton Fibers

Author

Wang, Peiyu, primary, Liu, Hang, additional, Zhu, Wenting, additional, Zhang, Guoheng, additional, Chen, Wanjun, additional, Wang, Xiangli, additional, Chen, Qiong, additional, Deng, Xiaoyan, additional, Yang, Bao, additional, Limao, Cairang, additional, and Cairang, Zuoma, additional

Published
2024
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