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您的检索式：作者名="Binyi Yang"
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| 1 | Dynein axonemal heavy chain 10 deficiency causes primary ciliary dyskinesia in humans and mice显示文摘Primary ciliary dyskinesia(PCD)is a congenital,motile ciliopathy with pleiotropic symptoms.Although nearly 50 causative genes have been identified,they only account for approximately 70%of definitive PCD cases.Dynein axonemal heavy chain 10(DNAH10)encodes a subunit of the inner arm dynein heavy chain in motile cilia and sperm flagella.Based on the common axoneme structure of motile cilia and sperm flagella,DNAH10 variants are likely to cause PCD.Using exome sequencing,we identified a novel DNAH10 homozygous variant(c.589C>T,p.R197W)in a patient with PCD from a consanguineous family.The patient manifested sinusitis,bronchiectasis,situs inversus,and asthenoteratozoospermia.Immunostaining analysis showed the absence of DNAH10 and DNALI1 in the respiratory cilia,and transmission electron microscopy revealed strikingly disordered axoneme 9+2 architecture and inner dynein arm defects in the respiratory cilia and sperm flagella.Subsequently,animal models of Dnah10-knockin mice harboring missense variants and Dnah10-knockout mice recapitulated the phenotypes of PCD,including chronic respiratory infection,male infertility,and hydrocephalus.To the best of our knowledge,this study is the first to report DNAH10 deficiency related to PCD in human and mouse models,which suggests that DNAH10 recessive mutation is causative of PCD. | Rongchun Wang Danhui Yang Chaofeng Tu Cheng Lei Shuizi Ding Ting Guo Lin Wang Ying Liu Chenyang Lu Binyi Yang Shi Ouyang Ke Gong Zhiping Tan Yun Deng Yueqiu Tan Jie Qing Hong Luo | 2023 | Frontiers of Medicine2023，17，5： | 0 |
| 2 | Effect of Heat Treatment on the Wear Properties of Selective Laser Melted Ti–6Al–4V Alloy Under Different Loads显示文摘As is well known,titanium alloy precipitates when subjected to aging treatment,which poses great influence on its mechanical properties.Thus,solution and solution-aging treatments were conducted in this work,and the wear performance was investigated under diff erent normal loads.The results showed that acicularα′martensite in the original selective laser melted Ti–6 Al–4 V was decomposed intoα+βphases after the solution treatment,and then Ti;Al nanoparticles clusters were further formed in theα-matrix after the solution-aging treatment.The coefficient of friction decreased straightly as the normal load was increased from 20 to 100 N,and the wear mechanisms were characterized by abrasion,adhesive and oxidative wears.The wear rate of solution and solution-aging treated samples increased significantly comparing to the as-built counterpart,but the increment of the solution-aging treated sample was smaller than that of the solution-only treated sample,because of the Ti;Al precipitation in the solution-aging treated sample.These particles provided ball-like effect,reduced material losses and stabilized the wear process.All these factors contributed to the improvement in wear properties of the parts made by selective laser melting. | Lu Yao Yeqin He Ziqiang Wang Binyi Peng Guoping Li Yang Liu | 2022 | Acta Metallurgica Sinica(English Letters)2022，35，3： | 0 |
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