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Variable phenotypes and outcomes associated with the MMACHC c.482G>A mutation:follow-up in a large CblC disease cohort

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World Journal of Pediatrics 2024年第8期20卷 848-858页

作者： sheng-Nan Wu Hui-Shu E Yue Yu Shi-Ying Ling Li-Li Liang Wen-Juan Qiu Hui-Wen Zhang Rui-Xue Shuai Hai-Yan Wei Chi-Ju Yang Peng Xu Xi-Gui Chen Hui Zou Ji-Zhen Feng Ting-Ting Niu Hai-Li Hu Kai-Chuang Zhang De-Yun Lu Zhu-Wen Gong Xia Zhan Wen-Jun Ji Xue-Fan Gu Yong-xing Chen Lian-Shu Han Department of Endocrinology and Metabolism Henan Key Laboratory of Children’s Genetics and Metabolic DiseasesChildren’s Hospital Affiliated to Zhengzhou UniversityHenan Children’s HospitalZhengzhou Children’s HospitalNo.255 Gangdu StreetZhengzhouChina Department of Pediatric Endocrinology and Genetics Fujian Children’s HospitalFuzhouChina The Center for Pediatric Liver Diseases Children’s HospitalFudan UniversityShanghaiChina Department of Pediatric Endocrinology/Genetics Shanghai Institute for Pediatric ResearchXinhua HospitalSchool of MedicineShanghai Jiao Tong UniversityNo.1665 Kongjiang RoadShanghaiChina Department of Pediatrics Second Affiliated Hospital of Naval Medical UniversityShanghaiChina Center of Neonatal Disease Screening Jining Maternal and Child Health Care HospitalJiningChina Center of Neonatal Disease Screening Jinan Maternal and Child Health Care HospitalJinanChina Center of Neonatal Disease Screening Shijiazhuang Maternal and Child Health Care HospitalShijiazhuangChina Center of Neonatal Disease Screening Shandong Maternal and Child Health Care HospitalJinanChina Center of Neonatal Disease Screening Hefei Maternal and Child Health Care HospitalHefeiChina

Background The aim of this study was to characterize the variable phenotypes and outcomes associated with the methylmalonic aciduria and homocystinuria type C protein gene(MMACHC)c.482G>A mutation in 195 Chinese cases with CblC *** We carried out a national,retrospective multicenter study of 195 Chinese patients with CblC disease attributable to the MMACHC c.482G>A variant either in a homozygous or compound heterozygous *** control group consisted of 200 patients diagnosed with CblC disease who did not possess the c.482G>A *** features,including disease onset,symptoms,biochemical metabolites,gene mutation,and follow-up outcomes were reviewed and analyzed in *** median follow-up period spanned 3 years and 8 months,with a range of 1 year and 2 months to 12 years and 10 *** Among 195 patients carrying the c.482G>A variant,125(64.1%)cases were diagnosed by newborn screening(NBS),60(30.8%)cases were detected due to disease onset,and 10(5.1%)cases were identified from sibling *** hundred and seventeen(93.6%)individuals who were diagnosed by NBS,and nine patients who came from sibling diagnoses remained asymptomatic in this *** 69 symptomatic patients of the c.482G>A group,more patients presented with later onset,and the top six common clinical symptoms at disease onset were developmental delay(59.4%),lower limb weakness and poor exercise tolerance(50.7%),cognitive decline(37.7%),gait instability and abnormal posture(36.2%),seizures(26.1%),and psychiatric and behavioral disturbances(24.6%).In the 159 symptomatic patients lacking c.482G>A variants,the most frequently observed clinical manifestations at disease onset included developmental delay(81.8%),lethargy and feeding difficulty(62.9%),lower limb weakness and poor exercise tolerance(54.7%),prolonged neonatal jaundice(51.6%),vomiting(47.2%),and seizures(32.7%).Before treatment,the levels of blood propionylcarnitine,propionylcarnitine/acetylcarnitine ratio

关键词： c.482G>A CblC disease MMACHC gene Newborn screening Outcome Use our pre-submission checklist Avoid common mistakes on your manuscript.

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大港油区稠油井开采工艺现状及发展趋向

特种油气藏 2005年第6期12卷 3-7页

作者：张勇邢金生王长在周俊杰孙连杰中油大港油田公司天津大港300280

大港油区立足于稠油冷采,形成了稠油井开采系列配套工艺技术,其中地层处理配套技术主要包括单井化学吞吐、微生物采油、有机溶剂清洗等,举升工艺主体技术包括常规有杆泵、电热杆、螺杆泵、电潜泵等,同时广泛配套环空掺水加药降粘工艺。... 详细信息

大港油区立足于稠油冷采,形成了稠油井开采系列配套工艺技术,其中地层处理配套技术主要包括单井化学吞吐、微生物采油、有机溶剂清洗等,举升工艺主体技术包括常规有杆泵、电热杆、螺杆泵、电潜泵等,同时广泛配套环空掺水加药降粘工艺。目前的工艺配套技术能够满足大港油田稠油尤其是普通稠油的开采需要,但在今后的稠油开采中,应该对各项工艺技术进行更深入的研究、优化、配套,提高特稠油和稠油井的开采工艺配套水平,降低稠油井开采成本。

关键词：稠油开采工艺现状发展趋向大港油区

Nitric Oxide Signaling Molecules in Acupoints:Toward Mechanisms of Acupuncture

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Chinese Journal of Integrative Medicine 2017年第11期23卷 812-815页

作者： MA sheng-xing Los Angeles Biomedical Research Institute at Harbor-UCLAMedical Center and Department of Obstetrics and Gynecology David Geffen School of MedicineUniversity of California at Los Angeles and Harbor-UCLA Medical CenterTorranceCA(90502)United States

Recent clinical trial studies have demonstrated that the effects of acupuncture on pain improvement are small and no difference between acupoints and non-points. Whether acupuncture needles must be inserted in specific points depends on whether acupoint specificity exists that is still not resolved, and is now urgent. Previous anatomical studies have demonstrated that acupoints exist higher number of nerve fibers/trunks, blood vessels, hair follicles, and sweat glands as well as density of the gap junction. Recent evidence shows that nitric oxide（NO） level is elevated in the acupoints/meridians and is associated with an enhanced expression of NO synthase endowed with transient receptor potential vanilloid type-1. There is growing evidence from international groups showing that acupuncture induces NO-mediated vasodilatation, which increases local blood flow and allows for a flush of algesic or sensitizing substances, leading to pain relief. Previous studies, using a novel biocapture system, have demonstrated that NOx–（total nitrite and nitrate） and cyclic guanosine monophosphate（c GMP） concentrations are consistently increased over skin acupoints compared to non-meridian control regions（NMCR） in humans. Dermal microdialysis in humans showed that NO-c GMP releases in the subcutaneous tissue of acupoint are higher than those in NMCR and increased by electroacupuncture（EA）. Recent studies have demonstrated that low-frequency electrical stimulation and manual acupuncture with low stimulating force and rate produce an elevation of NO release predominantly over acupoints. In contrast, NO levels over the areas of the skin regions are moderately reduced by high-frequency EA stimulation. The results from anatomical and biochemical studies consistently show that acupoints exist higher levels of NO signaling molecules, and stimulus-evoked NO release is also with a higher level at acupoints. Results suggest that NO signaling molecules contribute to the specificity of acupoints,

关键词： acupoint nitric oxide electroacupuncture manual acupuncture reinforcement methods

卤化物钙钛矿金属位铋离子掺杂的调控研究进展

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物理学进展 2024年第2期44卷 73-95页

作者：黄小芮孙悦贺圣荣邢军新疆固体物理与器件重点实验室新疆大学乌鲁木齐830017 物理科学与技术学院新疆大学乌鲁木齐830017

高性能金属卤化物钙钛矿太阳能电池目前正从实验室研发向工业生产转型,并在未来全球能源领域展示了其不可估量的应用价值。为了实现高性能的金属卤化物钙钛矿太阳能电池,钙钛矿材料作为有源工作层,其性能的提高和优化过程经过了十几年,... 详细信息

高性能金属卤化物钙钛矿太阳能电池目前正从实验室研发向工业生产转型,并在未来全球能源领域展示了其不可估量的应用价值。为了实现高性能的金属卤化物钙钛矿太阳能电池,钙钛矿材料作为有源工作层,其性能的提高和优化过程经过了十几年,并已取得巨大的进步。本文以高性能卤化物钙钛矿中金属位铋离子掺杂的调制研究为重点,系统总结了卤化物钙钛矿薄膜的发展和常规制备工艺。研究表明,三元阳离子钙钛矿可以抑制黄相的形成、改变晶粒尺寸、提高稳定性等特点,成为未来高性能钙钛矿光电器件的首选。文章还对铋离子(Bi^(3+))掺杂钙钛矿体系进行了讨论,研究发现Bi3+的特殊电子结构和无毒化学性质,不仅能够提高钙钛矿的稳定性,还能灵活调控钙钛矿的光电性能,从而实现铋离子掺杂的高性能钙钛矿太阳能电池。结合目前卤化物钙钛矿太阳能电池的研究现状,我们可以预见在未来的器件优化过程中,可以将铋离子掺杂策略与三元混合阳离子钙钛矿相互结合。最后,我们展望了实现高性能金属卤化物钙钛矿太阳能电池的几个潜在研究方向。

关键词：钙钛矿铋离子掺杂太阳能电池光电性能

卤化物钙钛矿单晶及光电探测器:研究进展和挑战

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物理学进展 2024年第5期44卷 209-242页

作者：孙悦黄小芮贺圣荣邢军新疆大学新疆固体物理与器件重点实验室乌鲁木齐830017 新疆大学物理科学与技术学院乌鲁木齐830017

近年来,由于金属卤化物钙钛矿材料展现出了优异的光学和电学特性(如高吸收系数、长载流子扩散长度、小激子结合能、高缺陷容限、可调带隙等性质),以及其具有低成本的溶液制备工艺,使其在光探测、光电转换和光发射等领域取得了巨大的科... 详细信息

近年来,由于金属卤化物钙钛矿材料展现出了优异的光学和电学特性(如高吸收系数、长载流子扩散长度、小激子结合能、高缺陷容限、可调带隙等性质),以及其具有低成本的溶液制备工艺,使其在光探测、光电转换和光发射等领域取得了巨大的科研进展。与钙钛矿多晶薄膜相比,钙钛矿单晶具有更长的载流子寿命、更高的载流子迁移率、更长的扩散长度、更低的陷阱密度等优势,从而促进了近十几年来钙钛矿单晶制备和优化的相关研究,并将高质量卤化物钙钛矿单晶广泛应用在光探测等重要应用领域。在这篇综述中,我们聚焦基于不同形式、不同化学组分卤化物钙钛矿单晶(包括单晶块体和单晶薄膜)的光电探测器技术领域的最新进展,首先系统综述了卤化物钙钛矿单晶的制备和优化进展,重点关注了三元阳离子杂化钙钛矿单晶的最新进展,然后全面介绍了基于钙钛矿单晶的各类型光电探测器研究现状,最后总结了卤化物钙钛矿单晶光电探测器研究领域目前面临的挑战并进行了展望,有望推动该领域的快速进步和发展。

关键词：钙钛矿单晶单晶薄膜三元阳离子光电探测器

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基于波普艺术的品牌包装构建研究

绿色包装 2024年第5期 143-147页

作者：汤盛行孙静八马茶业股份有限公司广东深圳518001

包装的设计是品牌塑造中关键的一环。随着消费文化的改变,传统包装越来越难以满足品牌的塑造,品牌包装发展需要找到新的突破口。如何最大程度实现品牌价值,艺术与商业的联袂尤为重要。在高度信息化时代,艺术与商业的融合是时代的必然,... 详细信息

包装的设计是品牌塑造中关键的一环。随着消费文化的改变,传统包装越来越难以满足品牌的塑造,品牌包装发展需要找到新的突破口。如何最大程度实现品牌价值,艺术与商业的联袂尤为重要。在高度信息化时代,艺术与商业的融合是时代的必然,而波普艺术以其商业性与通俗性的兼具特性,得到消费者的认可,并从多个维度参与到品牌包装的构建中。本文从品牌包装的设计视角出发,细述了在艺术精神与商业逻辑的新型关系下,波普艺术的价值所在。

关键词：艺术设计品牌包装消费文化波普艺术包装设计

La/Nd比对AZ91-RE合金显微组织和拉伸性能的影响

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Transactions of Nonferrous Metals Society of China 2024年第5期34卷 1456-1469页

作者：崔鹏星胡茂良吉泽升王晔郭宇许红雨哈尔滨理工大学材料科学与化学工程学院哈尔滨150080 哈尔滨理工大学先进制造智能化技术教育部重点实验室哈尔滨150080 湖州学院智能制造学院湖州313000

研究La/Nd比对AZ91-RE合金显微组织和拉伸性能的影响。采用经典表征方法分析第二相的体积分数、平均最近邻距离(NND)和数量密度。利用第一性原理计算确定第二相的形成焓、力学性能和热力学性质。结果表明,La与Nd的质量分数比(w_(La)/w_(... 详细信息

研究La/Nd比对AZ91-RE合金显微组织和拉伸性能的影响。采用经典表征方法分析第二相的体积分数、平均最近邻距离(NND)和数量密度。利用第一性原理计算确定第二相的形成焓、力学性能和热力学性质。结果表明,La与Nd的质量分数比(w_(La)/w_(Nd))为3/2的合金具有最小的平均晶粒尺寸、最小的平均最近邻距离和最高的第二相数量密度。计算结果表明,Al_(11)La_(3)和Al2Nd的形成在能量上有利。此外,Al_(11)La_(3)和Al_(2)La在抗变形能力、抗剪切变形能力、固体刚度和热稳定性方面都明显优于其他第二相。因此,提高Al_(11)La_(3)和Al_(2)La相的含量有利于改善AZ91-RE合金的力学性能。

关键词： AZ91合金显微组织拉伸性能第一性原理计算电子结构热力学性质

林分改变驱动的优势菌根真菌类型变化影响土壤有机碳积累

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土壤学报 2024年第1期61卷 223-234页

作者：屠嘉莹金文豪盛卫星邢佳佳彭丽媛陈俊辉徐秋芳秦华浙江农林大学省部共建亚热带森林培育国家重点实验室浙江农林大学环境与资源学院杭州311300 浙江省建德市林业局浙江建德311600

马尾松林(PinusmassonianaLamb.)是典型的外生菌根(Ectomycorrhiza,ECM)优势林,但是近年来受到松材线虫病的影响,生态服务功能下降,逐渐被丛枝菌根(Arbuscularmycorrhiza,AM)占优势的阔叶林所替代,但亚热带地区马尾松林转变为阔叶林过程... 详细信息

马尾松林(PinusmassonianaLamb.)是典型的外生菌根(Ectomycorrhiza,ECM)优势林,但是近年来受到松材线虫病的影响,生态服务功能下降,逐渐被丛枝菌根(Arbuscularmycorrhiza,AM)占优势的阔叶林所替代,但亚热带地区马尾松林转变为阔叶林过程中,优势菌根类型的改变对土壤有机碳积累的影响仍不清楚。以建德市马尾松林和阔叶林为研究对象,通过高效液相色谱和中性脂肪酸、磷脂脂肪酸等技术,测定优势菌根真菌生物量、球囊霉素相关土壤蛋白(GRSP)含量以及土壤胞外酶活性和微生物群落特征。结果表明:AM真菌占优势的阔叶林(AMD)取代ECM真菌占优势的马尾松林(ECMD),土壤有机碳显著提高了36.81%,微生物碳利用效率(CUE)显著提高了53.85%,AM真菌生物量提高了25.57%,ECM真菌生物量下降45.04%,并且ECM真菌占优势的马尾松林受到更严重的氮限制。磷脂脂肪酸分析显示,相比于AM真菌占优势的阔叶林,ECM真菌占优势的马尾松林革兰氏阳性细菌(G^(+))以及革兰氏阳性与阴性细菌之比(G^(+)/G^(–))分别显著下降了21.47%和6.46%。冗余分析(Redundancy analysis,RDA)结果表明,AM真菌占优势和ECM真菌占优势的森林之间土壤微生物群落结构存在显著差异(P<0.05),其中AM真菌生物量和土壤有机碳与微生物群落结构变异显著相关。GRSP含量下降以及不同类型菌根真菌招募微生物类群不同是导致ECM真菌占优势森林土壤有机碳下降的重要原因。因此,亚热带地区马尾松林被阔叶林替代后增加森林土壤有机碳含量,提高森林碳汇功能。

关键词：丛枝菌根真菌外生菌根真菌有机碳磷脂脂肪酸麦角固醇微生物策略

Influence on the Solid−Liquid Two-Phase Flow from Cross-Section Area of Slurry Pumps for Deep-Sea Mining

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China Ocean Engineering 2022年第3期36卷 439-450页

作者： WANG Run-kun ZHU Zu-chao SU Xiang-hui TANG Da-sheng jin xing Maciej GRUSZCZYNSKI The Province Key Lab of Fluid Transmission Technology Zhejiang Sci-Tech UniversityHangzhou 310018China State Key Laboratory of Deep Sea Mineral Resources Development and Utilization Changsha Institute of Mining and MetallurgyChangsha 410000China Wroclaw University of Environmental and Life Sciences Wroclaw 52-124Poland

To explore the mechanism of solid-liquid two-phase flow in deep-sea mining pumps,this paper investigates the influences of the impeller cross-section area on the multi-phase flow in the slurry *** and numerical results are presented for two-phase flow in four impellers with different cross-section *** show that the degree of vortex strength and the passing capacity of particles increase as the cross-section area of the *** addition,the correlations between the two-phase flow and cross-section area have been revealed by a mathematical model taking the force of the flow field into *** simulation results confirm the theoretical analysis,while the experimental pump performances validate the numerical *** influence of the cross-section area on two-phase flow and pump performance could provide theoretical support for the design of high-performance deep-sea mining slurry pumps.

关键词： deep-sea mining slurry pump CFD-DEM solid-liquid two-phase flow Rossby number