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A homozygous nonsense mutation in DNAJC30 causes Leber’s hereditary optic neuropathy with Leigh-like phenotypes

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Genes & Diseases 2023年第4期10卷 1165-1168页

作者： Chao Shen Kitty Wang Wei Li Alvaro Serrano Kelly Powers Chengwan Zhang Jianjun Chen miao sun Department of Systems Biology Beckman Research Institute of City of HopeMonroviaCA 91016USA Division of Medical Genetics Department of PediatricsChildren’s Hospital Los Angeles/Keck School of Medicine of USCLos AngelesCA 90027USA Division of Neurology Children’s Hospital Los AngelesLos AngelesCA 90027USA Division of Genomic Medicine Department of Pathology and Laboratory MedicineChildren’s Hospital Los Angeles/Keck School of Medicine of USCLos AngelesCA 90027USA

Nuclear encoded genes can cause early-onset mitochon-dria-related disorders such as Leigh or Leigh-like *** in DNAJC30 have been implicated in mitochondria-related diseases such as Leber’s hereditary optic neuropa-thy(LHON)and Williams syndrome(WS).However,the role of DNAJC30 in disease progression concerning mitochon-drial dysfunction has yet to be fully *** we report a 12-year-old boy with acute dystonia onset at age 10.

关键词： Leigh Leber acute

AN ELECTROCHEMICALLY PREPARED SKY-BLUE LIGHT EMITTING ETHER FUNCTIONALIZED POLYFLUORENE AS CHEMOSENSOR FOR METAL IONS

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Chinese Journal of Polymer Science 2013年第11期31卷 1579-1589页

作者： Wei Liu 孙明亮 Qian Liu Ben-lin He Hong-lun Ge miao sun Qin-jie Zhang 王玮 Institute of Material Science and Engineering Ocean University of China

Synthesis and electrochemical polymerization of 9,9-bis（2-（2-（2-methoxy ethoxy）ethoxy）ethyl）-fluorene （EO-F） into poly[9,9-bis（2-（2-（2-methoxy ethoxy）ethoxy）ethyl）-fluorene] （EO-PF） films are reported. The boron trifluoride diethyl etherate electrolyte enables facile preparation of EO-PF films at lower potential compared to LiCIOa/MeCN and the electrochemical polymerizations are discussed. The EO-PF shows good electrochemical behavior and can be dissolved in solvents such as DMSO and THF. The solubility of EO-PF in THF is 2 ***-1 and the number average molecular weight is 35300 with a polydispersity index of 1.65. The side chains on C9 position of the monomer maintain unchanged aRer electrooxidation into corresponding polymer. The EO-PF dissolved in THF under 365 nm ultraviolet light is sky blue light emitting with the Commission Internationale de L＇Eclairage-CIE coordinates of （0.19, 0.15）. The electropolymerized EO-PF is used for the first time in chemosensing metal ions, demonstrating fluorescence quenching for Mn2＋ and Fe3＋ while fluorescence enhancement for Cr6＋ ions.

关键词： 9,9-Bis(2-(2-(2-methoxyethoxy)ethoxy)ethyl)-fluorene Boron trifluoride diethyl etherate Chemosensoryresponse Electrochemical polymerization.

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任意排列的空心管桩屏障对SH波的多重散射

岩土力学 2014年第4期35卷 943-950,958页

作者：孙苗苗浙江省水利河口研究院杭州310020 浙江省水利防灾减灾重点实验室杭州310020

提出1种可计算任意排列、任意圆截面尺寸的多排空心管桩对平面SH波多重散射的理论求解方法。考虑第1重散射即为通常所谓的单重散射,对特定管桩第2重散射的入射波为在各管桩所受的第1重散射波之和,亦即第1重散射所激发的次生波源,以此类... 详细信息

提出1种可计算任意排列、任意圆截面尺寸的多排空心管桩对平面SH波多重散射的理论求解方法。考虑第1重散射即为通常所谓的单重散射,对特定管桩第2重散射的入射波为在各管桩所受的第1重散射波之和,亦即第1重散射所激发的次生波源,以此类推至第m重散射。假设每重散射在桩-土边界以及管桩内部均满足弹性边界条件,将入射波、散射波及透射波采用波函数展开,并运用Graf加法定理移轴获得待定的各重散(透)射复系数,得到总散射及管桩内部透射波场的理论解。算例中讨论了散射重数、桩径与桩(排)间距之比、桩-土剪切模量之比、管桩内外径之比以及多排管桩的几何参数等对屏障隔离效果的影响,结果表明,管桩可取得比实心桩更好的屏蔽效果,同时对管桩非连续屏障的工程设计提供了有价值的理论参考依据。

关键词：隔振非连续屏障管桩多重散射任意排列

Relationships among KRAS mutation status, expression of RAS pathway signaling molecules, and clinicopathological features and prognosis of patients with colorectal cancer

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World Journal of Gastroenterology 2019年第7期25卷 808-823页

作者： Xiang-Bin Wan Ai-Qin Wang Jian Cao Zhi-Chuang Dong Ning Li Sen Yang miao-miao sun Zhi Li Su-Xia Luo Department of General Surgery the Affiliated Tumor Hospital of Zhengzhou University Department of Pharmacy The Second Affiliated Hospital of Xinxiang Medical University Department of Medical Oncology the Affiliated Tumor Hospital of Zhengzhou University China-US (Henan) Hormel Cancer Institute Department of Pathology the Affiliated Tumor Hospital of Zhengzhou University

BACKGROUND The RAS/RAF/MEK/ERK and PI3 K/AKT/mTOR signaling pathways all belong to mitogen-activated protein kinase(MAPK) signaling pathways, Mutations in any one of the upstream genes(such as the RAS gene or the BRAF gene) may be transmitted to the protein through transcription or translation, resulting in abnormal activation of the signaling pathway. This study investigated the relationship between the KRAS gene mutation and the clinicopathological features and prognosis of colorectal cancer(CRC), and the effect of KRAS mutations on its associated proteins in CRC, with an aim to clarify the cause of tumor progression and drug resistance caused by mutation of the KRAS *** To investigate the KRAS gene and RAS pathway signaling molecules in CRC and to analyze their relationship with clinicopathological features and prognosis METHODS Colorectal cancer tissue specimens from 196 patients were analyzed for KRAS mutations using quantitative polymerase chain reaction and for KRAS, BRAF,MEK, and ERK protein expression levels using immunohistochemistry of tumor microarrays. To analyze differences of RAS pathway signaling molecule expression levels in different KRAS gene status, the relationships between these parameters and clinicopathological features, 4-year progression-free survival, and overall survival were analyzed by independent sample t test, Kaplan-Meier plots,and the log-rank test. Predictors of overall and disease-free survival were assessed using a Cox proportional hazards *** Of the 196 patients, 62(32%) carried mutations in codon 12(53/62) or codon 13(9/62) in exon 2 of the KRAS gene. KRAS, BRAF, ERK, and MEK protein expression was detected in 71.4%, 78.8%, 64.3%, and 50.8% of CRC tissues,respectively. There were no significant differences between KRAS mutation status and KRAS, BRAF, MEK, or ERK protein levels. Positive expression of KRAS and ERK was associated with poor tumor differentiation, and KRAS expression was also associated with age < 56

关键词： Colorectal cancer KRAS gene KRAS protein BRAF protein MEK protein ERK protein

北京西山门头沟地区主要地质遗迹分布特征及形成演化

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水文地质工程地质 2024年第3期51卷 210-221页

作者：贺瑾瑞徐尚智宋庆伟郝春燕董颖冉淑红吴家悦于兆林郑艺龙孙淼北京市地质灾害防治研究所北京100120 中国地质科学院北京100037 中国地质环境监测院北京100081

北京门头沟区处于燕山与太行山的交汇处,山地面积占98.5%,属北京西山的核心地区,在漫长的地质历史长河中,留下了丰富的地质遗迹。针对该区地质遗迹分布特征、形成演化缺乏系统研究,并制约其保护利用等问题,在充分利用前人成果的基础上,... 详细信息

北京门头沟区处于燕山与太行山的交汇处,山地面积占98.5%,属北京西山的核心地区,在漫长的地质历史长河中,留下了丰富的地质遗迹。针对该区地质遗迹分布特征、形成演化缺乏系统研究,并制约其保护利用等问题,在充分利用前人成果的基础上,通过遥感解译、地面调查、岩矿鉴定等,对该区地质遗迹类型进行系统梳理,深入探讨了区内地质遗迹分布特征和形成演化。结果表明:(1)该区地质遗迹丰富、类型多样;目前已查明的各类地质遗迹有100处,涵盖地质剖面、地质构造、古生物化石、地貌景观等多个类型。(2)青白口系、马兰黄土等地质剖面及古生物化石类地质遗迹的分布与中新元古界—第四系地层出露区密切相关;褶皱、断裂等主要分布在妙峰山镇—斋堂镇的中高山地区;燕山期酸性岩体地质遗迹主要沿大型断裂分布;黄土台地、河流阶地、峡谷以及水体景观类主要沿永定河干流及大型支流分布。(3)区内发生多次海侵,形成了诸多地层地质剖面遗迹,芹峪运动、蓟县运动、燕山运动等留下了多处不整合面、断层、褶皱等构造形迹,也主导了多期多种矿床形成。研究结果可为区内地质遗迹资源深入研究及其保护利用提供重要依据。

关键词：地质遗迹分布特征形成演化保护利用北京西山

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乳及乳制品中嗜冷菌多样性的研究进展

食品工业 2024年第1期45卷 201-206页

作者：孙苗邵伟刘政宇马宪兰陈贺赵艳坤新疆农业科学院农业质量标准与检测技术研究所农业农村部农产品质量安全风险评估实验室新疆农产品质量安全实验室乌鲁木齐830091 新疆农业大学动物科学学院新疆肉乳用草食动物营养实验室乌鲁木齐8300522 中国农业科学院北京畜牧兽医研究所农业农村部奶及奶制品质量安全控制重点实验室北京100193

嗜冷菌是一种适应低温环境的外界污染菌,其种类繁多并广泛存在于乳品加工的各个环节。低温冷藏是控制乳中微生物繁殖和保持乳品质量的常用方法,这却为嗜冷菌提供了有利的生存环境。其生长过程中会产生具有高度热稳定性的腐败酶,这些酶... 详细信息

嗜冷菌是一种适应低温环境的外界污染菌,其种类繁多并广泛存在于乳品加工的各个环节。低温冷藏是控制乳中微生物繁殖和保持乳品质量的常用方法,这却为嗜冷菌提供了有利的生存环境。其生长过程中会产生具有高度热稳定性的腐败酶,这些酶会对乳品的质量与安全造成极大的威胁。嗜冷菌的菌群结构和多样性也易受到多种因素影响,这就为乳中嗜冷菌的分离鉴定带来了困难。结合国内外文献,综述了国内外乳及乳制品中嗜冷菌多样性的研究现状,比较了传统培养法、分子生物学方法、高通量测序等检测技术在研究嗜冷菌多样性方面的优势和缺陷,以期为牧场及乳制品加工厂从污染源头保障原料乳及乳制品的安全生产提供理论依据,并为判定原料乳潜在的细菌污染风险和乳品产业加工原材料风险评估提供背景信息。

关键词：嗜冷菌微生物多样性乳及乳制品食品检测技术高通量测序

RETREATMENT WITH FLUDARABINE AND CYCLOSPORINE FOR ONE CASE OF REFRACTORY PURE RED CELL APLASIA

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Chinese Medical Sciences Journal 2008年第1期23卷 60-62页

作者： Guang-sheng He Xiang Zhang De-pei Wu Ai-ning sun miao miao Xiu-li Wang Zheng-ming Jin Department of Clinical Hematology Key Laboratory of Thrombosis and Hemostasis of Ministry of Health Jiangsu Insititute of Hematology the First Affiliated Hospital of Soochow University Suzhou 215006

MANY cases of pure red cell aplasia （PRCA） were mediated by over-function of immune cells, and responded well to immunosuppressive therapy. Sometimes refractory cases also arose. Fludarabine is an analogue of adenosine resistant to deamination which is widely used for B-chronic lymphocytic leukemia （CLL） and other hematological malignancies.^2 As a strong immunosuppressive agent, fludarabine has generally been used in nonmyeloblative conditioning regimens for hematopoietic stem cells transplantation for hematological malignancies and severe aplastic anemia recently.^3 In this study,

关键词： pure red cell aplasia refractory fludarabine

Effects of Nitrogen Rate and Split Application Ratio on Nitrogen Use and Soil Nitrogen Balance in Cotton Fields

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Pedosphere 2017年第4期27卷 769-777页

作者： LI Pengcheng DONG Helin LIU Aizhong LIU Jingran sun miao LI Yabing LIU Shaodong ZHAO Xinhua MAO Shuchun State Key Laboratory of Cotton Biology Institute of Cotton Research of Chinese Academy of Agricultural SciencesAnyang 455000China

The Yellow River valley is one of the three largest cotton production areas in *** experiment was performed in cotton fields of Anyang,China from 2013 to 2014 to investigate the effects of nitrogen(N) application rate and the ratio between basal and topdressing N fertilizer on N balance in a soil-plant system,N use efficiency,and cotton *** N application rates as treatments were applied with the same split application *** of the N(50% basal fertilizer) was applied at pre-planting and the other half(50% topdressing fertilizer) at the initial flowering *** treatments were:zero N(N0,control),90 kg N ha^(-1)(N90(5/5)),180 kg N ha^(-1)(N180(5/5)),270 kg N ha^(-1)(N270(5/5),a reduced N rate),and 360 kg N ha^(-1)(N360(5/5),a conventional N rate).Additional 2 split application ratios as treatments were applied with the same N rate of 270 kg N ha^(-1).The split application ratios between basal N and topdressing N were 30%:70%(N270(3/7)) and 70%:30%(N270(7/3)).Results demonstrated that soil NH_4-N content in the 0–60 cm layer and NO3-N content in the 0–20 cm layer increased with increased N rate at the squaring and boll-opening stages and then decreased to lower levels at the initial flowering and harvest *** NO_3-N content in the 20–60 cm layer after the initial flowering stage increased with the increase of topdressing N *** apparent N surplus varied at different growth stages,while the soil apparent N surplus over the entire growth period exhibited a positive relationship at N rates over 180 kg ha^(-1).Seed cotton yield of N270(3/7) was the highest of all *** N uptake,N agronomic efficiency,and apparent N recovery efficiency of N270(3/7) were significantly higher than those of N270(5/5) and N270(7/3) in both growing *** suggest both economic and ecological benefits in cotton production in the Yellow River valley could be created,by appropriately reducing total N application rate and increasing the ratio of top

关键词： apparent N recovery efficiency, crop yield, N agronomic efficiency, N fertilizer, plant growth, soil apparent N surplus

中医药治疗青春期多囊卵巢综合征的现代机制研究进展及优势

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中国医药导报 2024年第9期21卷 41-44,49页

作者：张春兰侯丽辉李威孙淼王伟明黑龙江中医药大学附属第一医院妇科黑龙江哈尔滨150040 黑龙江省中医药科学院中药研究所黑龙江哈尔滨150036

多囊卵巢综合征(PCOS)是与内分泌异常、生殖功能障碍关系密切的一种疾病,以雄激素过高、持续无排卵或胰岛素抵抗为重要特征。PCOS多于青春期起病,青春期PCOS患病率逐年升高,发病年龄逐渐提前,患者除了有闭经、肥胖、痤疮、多毛等常见的... 详细信息

多囊卵巢综合征(PCOS)是与内分泌异常、生殖功能障碍关系密切的一种疾病,以雄激素过高、持续无排卵或胰岛素抵抗为重要特征。PCOS多于青春期起病,青春期PCOS患病率逐年升高,发病年龄逐渐提前,患者除了有闭经、肥胖、痤疮、多毛等常见的症状外,还有可能产生焦虑、自卑等心理障碍,这给许多青春期女性带来困扰,应积极地予以临床干预。传统医学认识青春期PCOS注重脏腑,认为本病病机为肾虚,兼以脾虚、肝郁;现代医学的认识则进展为信号通路靶向的细胞进程,主要涉及Wnt/β-catenin、PI3K/Akt/mTOR等信号通路。目前,对于青春期PCOS目前尚无统一标准的诊疗方案,在临床中,除常用的西药治疗外,可以采用中药及中医特色治疗进行积极、及时的干预。本文对青春期PCOS的中医药治疗方法及现代机制研究进展进行综述,以期对青春期PCOS的治疗提供多种途径的治疗方法。

关键词：青春期多囊卵巢综合征机制研究进展中医中药治疗优势