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STUDIES ON PATHOGENESIS OF WAARDENBURG SYNDROME TYPE Ⅱ AND TIETZ SYNDROME RESULTING FROM MITF GENE MUTATIONS

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Journal of Otology 2013年第2期8卷 97-103页

作者： ZHANG hua li Jiada LUO Hunjin CHEN Hongsheng MEI lingyun HE Chufeng JIANG Lu feng Yong department of Otolaryngology First Affiliated HospitalXinjiang Medical University State Key Laboratory of Medical Genetics Central South University department of Otolaryngology Xiangya Hospital Central South University

Microphthalmia-associated transcription factor (MITF) controls melanocyte survival and differentiation through directly regulating the expression of the tyrosinase (TYR) and tyrosinase-related proteins 1 and 2 (TYRP1 and TYRP2) genes. MITF mutations have been reported to result in an abnormal melanocyte devel-opment and lead to Waardenburg syndrome type 2 (WS2), characterized by variable degrees of sensorineu-ral hearing loss and patchy regional distribution of hypopigmentation. Recently, MITF was also indicated as a causative gene for a more severe syndrome, the Tietz Syndrome (TS), characterized by generalized hy-popigmentation and complete hearing loss. However, few functional studies have been performed to com-pare the diseases-causing mutations. Here, we analyzed the in vitro activity of two recent identified WS2-as-sociated mutation (p.R217I and p.T192fsX18) and one TS-associated mutation p.N210K. The R217I MITF retained partial activity, normal DNA-binding ability and nuclear distribution, whereas the T192fsX18 MITF failed to activate TYR promoter due to loss of DNA-binding activity, and aberrant subcellular localization. The aberrant subcellular localization of T192fsX18 MITF may be caused by deletion of a putative nuclear localization signal (NLS) at aa 213-218 (ERRRRF). Indeed, MITF with deletion of the NLS fragment failed to translocate into the nucleus and activated the TYR promoter. Tagging this NLS to GFP promoted the green fluorescence protein (GFP) translocated into the nucleus. The surprising finding of our study is that a TS-as-sociated MITF mutation, N210K, showed comparable in vitro activity as WT. Thus, the possible involve-ment of MITF in TS and its underlying mechanisms still need further investigation.

关键词： Waardenburg syndrome Teitz syndrome MITF mutation haploinsufficiency

Dating recent sediments from the subaqueous Yangtze Delta and adjacent continental shelf,china

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Journal of Palaeogeography 2014年第2期3卷 207-218页

作者： Zhang-hua Wang Yong-Hong Dong Jing Chen Xiao-feng li Juan Cao Zhi-Yong Deng State Key Laboratory of Estuarine and Coastal Research East China Normal University department of geography East China Normal University Shanghai Radiation Environmental Supervision Station

In this study we analyzed sediment lithology,fallout of 210Pb and 137Cs,and spheroidal carbonaceous particles(SCPs) for two short cores,YZE and CX38,obtained by gravity corer from the Yangtze River mouth offshore and adjacent continental shelf,to compare geochronological methods on the recent sediments of this *** and grain size changes in YZE suggested the re-discharging of the North Channel of the Yangtze River mouth by flood events during 1949-1954 and associated accretion in the offshore *** event was validated by a remarkable zone of declination in both 137Cs and 210Pb activities and the absolute ages derived from the 137Cs and *** contrast,210Pb results show obvious disturbance of grain size by sediment mixing and cannot be interpreted above 100 *** CX38,absolute ages for the early-and mid-1950s were derived by the 137Cs and the SCP profile respectively,which occurred in a reasonable *** excess 210Pb distribution shows exponentially decreasing activities with depth,and the mean sedimentation rate agrees roughly with the one inferred from the SCP *** suggest that the limitation of the 210Pb method needs consideration while the SCP profile has the potential to provide a useful and independent dating method for recent Yangtze offshore and adjacent shelf sediments.

关键词： geochronology lithology change 137Cs and 210Pb SCP profile Yangtze offshore

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用微量量热法测定石油微生物的最佳生长酸度

烟台师范学院学报（自然科学版） 2000年第2期16卷 132-135页

作者：李风华张洪林曲阜师范大学化学系山东曲阜273165

在瑞典产 2 2 77热活性检测仪上用停流法在 50℃下测定了石油微生物菌种 , ,P在不同酸度培养基中的生长代谢热谱 ,按限制性条件下微生物生长代谢模型进行数学处理 ,求出三种细菌在不同酸度下的生长速率常数 ( μ) ,拟合 μ- p H非线性... 详细信息

在瑞典产 2 2 77热活性检测仪上用停流法在 50℃下测定了石油微生物菌种 , ,P在不同酸度培养基中的生长代谢热谱 ,按限制性条件下微生物生长代谢模型进行数学处理 ,求出三种细菌在不同酸度下的生长速率常数 ( μ) ,拟合 μ- p H非线性方程 ,确定各菌种最佳生长酸度的 p H值分别为 8.89,8.2 7,8.82 。

关键词：微量量热法石油微生物最佳生长酸度测定

The inhibition of dopamine on the colorectal motility is predominantly mediated by D1 and β3 receptors in normal and stress rats

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The inhibition of dopamine on the colorectal motility is pre...

非小细胞肺癌组织NEK2、EPHA5表达与临床病理特征、EGFR突变和预后的关系

中国生理学会第23届全国会员代表大会暨生理学学术大会

作者： hua GUO,Yun li,Jing-Dong XU,Xiao-Hui ZHANG,li-Sheng li,Yue ZHANG,li-Fei ZHENG,Xin-liang MI, Xiao-Yan feng,Zhang-feng DOU,Tuo JI,Xiao-feng li,Jin-Xia ZHU~* department of Physiology,School of Basic Medical Sciences,Capital Medical university,Beijing 100069,china

Colorectum manifests different in structure,function and accessibility to the diseases,such as inflammation, ulcer,even ***(DA) has been reported to involve in the regulation of colonic ***,few studies have clearly addressed the innervation and spontaneous motiliy regulation mechanism of DA on distal colon (DC).The present study demonstrated that the immune histofluorescence of D,D receptors,β,β andβ adrenoceptors were all found in the smooth muscle layer in the rat ***,D,β andβ were stronger than D andβ.DA(≥3.0×10mol/L)inhibited DC spontaneous motility[motility index(358±50) mg×1 min,frequency (1.06±0.08) Hz]in dose dependent manner(EC,1.0×10 mol/L).This DA-induced inhibition of colonic motility was blocked by pretreatment withβ adrenoceptor antagonist cyanopindolol(7.5×10 mol/L) mainly,D receptor antagonist SCH23390(1.0×10 mol/L) andβ adrenoceptor antagonist atenolol(1.0×10 mol/L) separately,otherwise D receptor antagonist sulpiride(1.0×10 mol/L) orβ adrenoceptor antagonist ICI-118551(1.0×10 mol/L) had no the blockage ***,pretreatments of D and D receptor antagonists made the spontaneous motility stronger *** more,in acute cold-restraint stress(CRS) conditions,the stronger spontaneous motility of DC could also be markedly inhibited by DA(≥3.0×10 mol/L).Then the result of western blot showed that expressions of only D,β and mainlyβ receptors,not D orβ receptors enhanced in the layer of CRS rats’ DC smooth *** conclusion,DA modulates DC motility in normal and stress rats mainly with D andβ *** alterations of DA-inhibited colonic motility and the increased expressions of D,β andβ receptors in rat DC smooth muscle under CRS condition indicates that DA and it’s receptor system play an important role in the regulation of physiologic and pathophysiologic process of gastrointestinal tract in the stress condition.

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现代生物医学进展 2024年第12期24卷 2396-2400页

作者：李峰王布徐凯伦武伟泽张志华赵建清河北北方学院附属第一医院呼吸与危重症医学科河北张家口075000 河北北方学院附属第一医院病理科河北张家口075000

目的:探讨非小细胞肺癌(NSCLC)组织有丝分裂相关激酶2(NEK2)、促红细胞生成素产生肝细胞受体A5(EPHA5)表达情况,分析其与临床病理特征、表皮生长因子受体(EGFR)突变和预后的关系。方法:选取自2019年10月至2020年10月期间我院诊治的151例... 详细信息

目的:探讨非小细胞肺癌(NSCLC)组织有丝分裂相关激酶2(NEK2)、促红细胞生成素产生肝细胞受体A5(EPHA5)表达情况,分析其与临床病理特征、表皮生长因子受体(EGFR)突变和预后的关系。方法:选取自2019年10月至2020年10月期间我院诊治的151例NSCLC患者作为研究对象,术中取其癌组织及癌旁正常组织(距离癌组织5cm以上)。采用实时荧光定量聚合酶链式反应(RT-PCR)检测EGFR基因表达。免疫组织化学法检测癌组织和癌旁组织中NEK2、EPHA5表达。比较癌组织和癌旁组织NEK2、EPHA5表达情况。分析不同临床病理特征NSCLC患者NEK2、EPHA5表达情况。分析EGFR突变型与野生型不同NEK2、EPHA5表达情况。Kaplan-Meier生存曲线分析不同NEK2、EPHA5表达对NSCLC患者的预后情况。结果:与癌旁组织比较,癌组织NEK2、EPHA5阳性表达率明显更高(均P<0.05);与TNM分期Ⅰ~Ⅱ期、无淋巴结转移和肿瘤直径<5 cm患者相比,TNM分期ⅢA期、淋巴结转移和肿瘤直径≥5 cm患者的NEK2、EPHA5阳性表达率均明显更高(均P<0.05);EGFR突变型组NEK2、EPHA5阳性表达率显著高于EGFR野生型组(均P<0.05);NEK2阳性表达组和阴性表达组3年总生存率(OS)分别为40.40%(40/99),57.69%(30/52),EPHA5阳性表达组和阴性表达组患者3年OS分别为41.90%(44/105),56.52%(26/46),各阴性表达组患者累积生存显著高于阳性表达组(P<0.05)。结论:NSCLC癌组织中NEK2、EPHA5阳性表达率升高,与TNM分期ⅢA期、淋巴细胞转移、肿瘤直径有关,还可能导致EGFR突变和不良预后。

关键词：非小细胞肺癌 NEK2 EPHA5 临床病理特征 EGFR突变预后

Einstein Probe discovery of EP240408a:A peculiar X-ray transient with an intermediate timescale

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Science china(Physics,Mechanics & Astronomy) 2024年

作者： Wenda Zhang Weimin Yuan Zhixing ling Yong Chen Nanda Rea Arne Rau Zhiming Cai huaqing Cheng Francesco Coti Zelati lixin Dai Jingwei Hu Shumei Jia Chichuan Jin Dongyue li Paul O’Brien Rongfeng Shen Xinwen Shu Shengli Sun Xiaojin Sun Xiaofeng Wang Lei Yang Bing Zhang Chen Zhang Shuang-Nan Zhang Yonghe Zhang Jie An David Buckley Alexis Coleiro Bertrand Cordier liming Dou Rob Eyles-Ferris Zhou Fan hua feng Shaoyu Fu Johan P. U. Fynbo Lluis Galbany Saurabh W. Jha Shuaiqing Jiang Albert Kong Erik Kuulkers Weihua Lei Wenxiong li Bifang liu Mingjun liu Xing liu Yuan liu Zhu liu Chandreyee Maitra Alessio Marino Itumeleng Monageng Kirpal Nandra Jeremy Sanders Roberto Soria lian Tao Junfeng Wang Song Wang Tinggui Wang Zhongxiang Wang Qingwen Wu Xuefeng Wu Dong Xu Yanjun Xu Suijian Xue Yongquan Xue Zijian Zhang Zipei Zhu Hu Zou Congying Bao Fansheng Chen Houlei Chen Tianxiang Chen Wei Chen Yehai Chen Yifan Chen Chenzhou Cui Weiwei Cui Yanfeng Dai Dongwei Fan Ju Guan Dawei Han Dongjie Hou Haibo Hu Maohai huang Jia Huo Zhenqing Jia Bowen Jiang Ge Jin Chengkui li Junfei li Longhui li Maoshun li Wei li Zhengda li Tianying lian Congzhan liu Heyang liu huaqiu liu Fangjun Lu Laidan Luo Jia Ma Xuan Mao Haiwu Pan Xin Pan liming Song Hui Sun Yunyin Tan Qingjun Tang Yihan Tao Hao Wang Juan Wang Lei Wang Wenxin Wang Yilong Wang Yusa Wang Qinyu Wu Haitao Xu Jingjing Xu Xinpeng Xu Yunfei Xu Zhao Xu Changbin Xue Yulong Xue Ailiang Yan Haonan Yang Xiongtao Yang Yanji Yang Juan Zhang Mo Zhang Wenjie Zhang Zhen Zhang Zhen Zhang Ziliang Zhang Donghua Zhao Haisheng Zhao Xiaofan Zhao Zijian Zhao Hongyan Zhou Yilin Zhou Yuxuan Zhu Zhencai Zhu Institut d’Estudis Espacials de Catalunya (IEEC) Max Planck Institute for Extraterrestrial Physics Innovation Academy for Microsatellites Chinese Academy of Sciences department of Physics university of Hong Kong School of Physics and Astronomy university of Leicester School of Physics and Astronomy Sun Yat-sen university National Astronomical Observatories department of Physics Anhui normal university Shanghai Institute of Technical Physics Chinese Academy of Sciences Physics department Tsinghua university Nevada Center for Astrophysics university of Nevada Las Vegas department of Physics and Astronomy university of Nevada Las Vegas Chinese Academy of Sciences South African Astronomical Observatory department of Astronomy university of Cape Town department of Physics university of the Free State Universite' Paris Cite' CNRS Astroparticule et Cosmologie Universite' Paris Cite' CEA Paris-Saclay School of Astronomy and Space Sciences IRFU/DAp-AIM department of Astronomy Guangzhou university Cosmic Dawn Center (DAWN) Niels Bohr Institute university of Copenhagen department of Physics and Astronomy Rutgers the State university of New Jersey Institute for Cosmic Ray Research university of Chinese Academy of Sciences The university of Tokyo ESA/ESTEC Noordwijk 2201 AZ The Netherlands department of Astronomy School of Physics huazhong university of Science and Technology INAF-Osservatorio Astrofisico di Torino Sydney Institute for Astronomy School of Physics Key Laboratory of Particle Astrophysics The university of Sydney department of Astronomy Xiamen university Institute for Frontiers in Astronomy and Astrophysics Beijing normal university department of Astronomy university of Science and Technology Hefei School of Astronomy and Space Sciences university of Science and Technology of china Institute of High Energy Physics Institute of Deep Space Sciences Deep Space Exploration Laboratory department of Astronomy School of Physics and Astronomy Yunnan university Shanghai Astronomical Observatory Chinese Academy of Science

We report the discovery of a peculiar X-ray transient,EP240408a,by Einstein Probe （EP） and follow-up studies made with EP,Swift,NICER,GROND,ATCA and other ground-based multiwavelength *** new transient was first detected with Wide-field X-ray Telescope （WXT） on board EP on April 8th,2024,manifested in an intense yet brief X-ray flare lasting for *** flare reached a peak flux of 3.9×10-9erg cm-2s-1in 0.5-4 keV,300 times brighter than the underlying X-ray emission detected throughout the *** and more precise follow-up observations by EP/FXT,Swift and NICER confirmed the finding of this new *** X-ray spectrum is non-thermal in 0.5-10 keV,with a power-law photon index varying *** X-ray light curve shows a plateau lasting for4d,followed by a steep decay till becoming undetectable10 d after the initial *** on its temporal property and constraints from previous EP observations,an unusual timescale in the range of 7-23 d is found for EP240408a,which is intermediate between the commonly found fast and long-term *** counterparts have been found in optical and near-infrared,with the earliest observation at 17 h after the initial X-ray detection,suggestive of intrinsically weak emission in these *** demonstrate that the remarkable properties of EP240408a are inconsistent with any of the transient types known so far,by comparison with,in particular,jetted tidal disruption events,gamma-ray bursts,X-ray binaries and fast blue optical *** nature of EP240408a thus remains an *** suggest that EP240408a may represent a new type of transients with intermediate timescales of the order of10 *** detection and follow-ups of more of such objects are essential for revealing their origin.

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Genome size evolution of the extant lycophytes and ferns

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Plant Diversity 2022年第2期44卷 141-152页

作者： Fa-Guo Wang Ai-hua Wang Cheng-Ke Bai Dong-Mei Jin li-Yun Nie AJ Harris Le Che Juan-JuanWang Shi-Yu li Lei Xu Hui Shen Yu-feng Gu Hui Shang Lei Duan Xian-Chun Zhang Hong-feng Chen Yue-Hong Yan Key Laboratory of Plant Resources Conservation and Sustainable Utilization Guangdong Provincial Key Laboratory of Applied BotanySouth China Botanical GardenChinese Academy of SciencesGuangzhou510650China College of life Sciences Shaanxi Normal UniversityXi'an710062China Eastern china Conservation Centre for Wild Endangered Plant Resources Shanghai Chenshan Botanical GardenShanghai201602China Key Laboratory of Environment Change and Resources Use in Beibu Gulf Ministry of EducationNanning Normal UniversityNanning530001China department of Biology Oberlin CollegeOberlinOH44074USA State Key Laboratory of Systematic and Evolutionary Botany Institute of BotanyChinese Academy of SciencesBeijing100093China Key Laboratory of National Forestry and Grassland Administration for Orchid Conservation and Utilization the National Orchid Conservation Center of China and the Orchid Conservation&Research Center of Shenzhen518114ShenzhenChina life Science and Technology College Harbin Normal UniversityHarbin150025China

Ferns and lycophytes have remarkably large ***,little is known about how their genome size evolved in fern *** explore the origins and evolution of chromosome numbers and genome size in ferns,we used flow cytometry to measure the genomes of 240 species(255 samples)of extant ferns and lycophytes comprising 27 families and 72 genera,of which 228 species(242 samples)represent new *** analyzed correlations among genome size,spore size,chromosomal features,phylogeny,and habitat type preference within a phylogenetic *** also applied ANOVA and multinomial logistic regression analysis to preference of habitat type and genome *** the phylogeny,we conducted ancestral character reconstruction for habitat types and tested whether genome size changes simultaneously with shifts in habitat *** found that 2 C values had weak phylogenetic signal,whereas the base number of chromosomes(x)had a strong phylogenetic ***,our analyses revealed a positive correlation between genome size and chromosome traits,indicating that the base number of chromosomes(x),chromosome size,and polyploidization may be primary contributors to genome expansion in ferns and *** sizes in different habitat types varied significantly and were significantly correlated with habitat types;specifically,multinomial logistic regression indicated that species with larger 2 C values were more likely to be *** habitat is inferred to be ancestral for both extant ferns and lycophytes,whereas transitions to other habitat types occurred as the major clades *** in habitat types appear be followed by periods of genomic *** on these results,we inferred that habitat type changes and multiple whole-genome duplications have contributed to the formation of large genomes of ferns and their allies during their evolutionary history.

关键词： Genome size Ferns Chromosome numbers Habitat type Whole-genome duplications Evolution

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皖南低山丘陵区水土保持综合评价

水土保持学报 2001年第6期15卷 20-23,77页

作者：张理华周秉根万荣荣刘万青宿州师范专科学校地理系安徽宿州234000 安徽师范大学国土资源与旅游学院安徽芜湖241000

为了更好地做好皖南低山丘陵区的水土保持工作 ,在建立区内水土保持综合评价模型的基础上 ,对区内水土保持状况进行综合评价 ,其结果中山区为 6 3.0 0 ,低山区为 6 0 .0 3,丘陵区为 5 7.40 ,盆谷区为 6 5 .38。根据评价因子的匹配系数 (... 详细信息

为了更好地做好皖南低山丘陵区的水土保持工作 ,在建立区内水土保持综合评价模型的基础上 ,对区内水土保持状况进行综合评价 ,其结果中山区为 6 3.0 0 ,低山区为 6 0 .0 3,丘陵区为 5 7.40 ,盆谷区为 6 5 .38。根据评价因子的匹配系数 (Cc) ,提出不同的水土保持对策 ,以促进区内生态环境的改善。

关键词：皖南低山丘陵区水土保持综合评价匹配系数保护对策

Ruminal in situ disappearance kinetics of six nutritive ingredients in leaves and stems of young tetraploid black locust in growing steers

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Forestry Studies in china 2009年第3期11卷 168-173页

作者： JIANG Jin-zhong SUN Peng li Yun ZHANG Guo-jun liU Jian-hua HAN feng-bao NIU Chen College of Biological Sciences and Biotechnology Beijing Forestry University Key Laboratory of Genetics and Breeding of Forest Trees and Ornamental Plants of Ministry of Education Beijing 100083 P. R. China department of Chemistry Guizhou Normal College Guiyang 550018 P. R. China Forestry Bureau of Xianghe County Hebei Province Xianghe 065400 P. R. China department of Plant and Soil Science Texas Tech University Lubbock 79409 USA

Tetraploid black locust （Robinia pseudoacacia） is a woody plant with larger leaves and richer nutritional content than normal diploid black locust, with greater biomass and wider adaptability than herbaceous plant forage. However, currently no stock- breeders have adopted it as a major forage in china and other countries for a lack of information on the evaluation of nutritional ingredients of leaves and stems of young tetraploid black locust （YTBL） by in situ digesting tests. The objectives of this study were： 1） to assess the nutritive value of YTBL that bourgeoned from root stumps in the spring by determining the ruminal in situ disappearance kinetics of dry matter （DM）, organic matter （OM）, crude protein （CP）, ether extract （EE）, neutral detergent fiber （NDF） and acid detergent fiber （ADF） from leaves, stems and a mixture of both leaves and stems （MLS） of YTBL; 2） to compare these kinetic estimates with those of Leymus chinensis, a widely used ruminant forage in china. All samples were evaluated in situ by using five ruminally cannulated crossbred steers （550±22.7 kg BW）. The results of 48 hours, the representative incubation period, indicate that in situ disappearance rates of DM and OM in YTBL leaves, stems and MLS are on average about 40% and can attain the level of digestibility of L. chinensis. In situ disappearance rates of leaves, stems and MLS of YTBL for CP and EE were 41% and 58% respectively, rates higher than those of L. chinensis. But the disappearance rates of NDF and ADF were, on average, respectively 30.62% and 24.89% and lower than those ofL. chinensis. By analyzing synthetically all of our results it could be inferred that MLS of YTBL offers effective ruminal degradability of DM, NDF, ADF, CP, EE and OM, similar to the nutrients of L. chinensis. This supports our contention that the YTBL leaves, stems and MLS are suitable feed for those ruminants requiring high levels of nutrients, such as dairy cows and Boer goats, instead of plant forage, whi

关键词： tetraploid black locust in situ disappearance kinetics ruminant nutritive analyses