检索结果-南通市图书馆

Autophagy-targeting modulation to promote peripheral nerve regeneration

Neural Regeneration Research 2025年第7期20卷 1864-1882页

作者： Yan Chen Hongxia Deng Nannan Zhang Department of Obstetrics and Gynecology West China Second HospitalSichuan UniversityChengduSichuan ProvinceChina Key Laboratory of birth defects and Women and Children’s Diseases Ministry of EducationSichuan UniversityChengduSichuan ProvinceChina Laboratory of Reproductive Endocrinology and Reproductive Regulation Sichuan UniversityChengduSichuan ProvinceChina national center for birth Defect Monitoring West China Second University HospitalSichuan UniversityChengduSichuan ProvinceChina

Nerve regeneration following traumatic peripheral nerve injuries and neuropathies is a complex process modulated by diverse factors and intricate molecular *** studies have focused on factors that stimulate axonal outgrowth and myelin ***,recent studies have highlighted the pivotal role of autophagy in peripheral nerve regeneration,particularly in the context of traumatic ***,autophagy-targeting modulation has emerged as a promising therapeutic approach to enhancing peripheral nerve *** current understanding suggests that activating autophagy facilitates the rapid clearance of damaged axons and myelin sheaths,thereby enhancing neuronal survival and mitigating injury-induced oxidative stress and *** actions collectively contribute to creating a favorable microenvironment for structural and functional nerve regeneration.A range of autophagyinducing drugs and interventions have demonstrated beneficial effects in alleviating peripheral neuropathy and promoting nerve regeneration in preclinical models of traumatic peripheral nerve *** review delves into the regulation of autophagy in cell types involved in peripheral nerve regeneration,summarizing the potential drugs and interventions that can be harnessed to promote this *** hope that our review will offer novel insights and perspectives on the exploitation of autophagy pathways in the treatment of peripheral nerve injuries and neuropathies.

关键词： autophagy autophagy related genes Charcot–Marie–Tooth diseases diabetic peripheral neuropathy metformin myelination peripheral nerve injury Schwann cells sciatic nerve Wallerian degeneration

来源：

维普期刊数据库

同方期刊数据库评论

在线全文

学校读者我要写书评

暂无评论

Early Hearing Detection and Intervention in the United States: Achievements and Challenges in the 21^(st) Century

引用

China CDC weekly 2020年第21期2卷 378-382页

作者： Xidong Deng Marcus Gaffney Scott D.Grosse national center on birth defects and developmental disabilities Centers for Disease Control and PreventionAtlantaGAUnited States

INTRODUCTION Congenital hearing loss affects approximately two infants per 1,000 live births in the United States(USA),making it one of the nation’s most common developmental disabilities(1).When left undetected,permanent congenital hearing loss can negatively impact children through delays in their speech,language,social,and emotional development(2).Children who are identified as deaf or hard of hearing(D/HH)should begin receiving intervention services as early as possible to help mitigate the potential adverse effects(3).

关键词： Achievements Challenges emotional

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

AR mutations in 28 patients with androgen insensitivity syndrome(Prader grade 0–3)

引用

Science China(Life Sciences) 2017年第7期60卷 700-706页

作者： Yi Wang Chunxiu Gong Xiou Wang Miao Qin Beo'ing Key Laboratory for Genetics of birth defects Center of Endocrinology Genetics and Metabolism Beo'ing Children's Hospital The Capital Medical University National center for children's health Beijing 100045 China

We investigated the androgen receptor(AR) gene mutation profiles of Chinese patients exhibiting severe androgen insensitivity syndrome(AIS) phenotypes. The present study enrolled 28 patients with genetically diagnosed AIS, who presented with severe phenotypes(Prader grade 0–3). Patients and some family members were screened via amplification and sequencing of their AR exons 1–8, including the corresponding intronic flanking regions. Luteinizing(LH), follicle-stimulating(FSH), and testosterone(T) hormone levels were found to be slightly, but not significantly, higher in patients with complete androgen insensitivity syndrome(CAIS) than in patients with partial androgen insensitivity syndrome(PAIS)(P>0.05). We identified 24 different AR mutations, including 12 that were novel. Ten patients(cases 2, 3, 10, 28, 11, 12, 19, 20, 24, and 25) were found to carry five recurrent mutations(p.Y572 S, p.P914 S, p.S176 R, p.Y782 N, and p.R841H); of these, p.Y572 S, p.S176 R, and p.Y782 N were novel. Among the mutations identified in patients with CAIS, six(66.7%) were characterized as single-nucleotide missense mutations, and six(66.7%) were found to be located in the AR ligand-binding domain(LBD). Among the mutations identified in patients with PAIS, 15(93.8%) were found to be missense, and 11(68.8%) were found to be located in the LBD. Patients 10 and 28 were determined to harbor the same missense mutation(p.P914S), but were diagnosed with CAIS and PAIS, *** hormone levels were slightly, but not significantly, elevated in patients with CAIS compared to those with PAIS. Missense mutations spanning AR exons 1–8 were the predominant form of identified mutations, and these were mostly located in the AR LBD. Approximately 50% of the identified mutations were novel, and have enriched the AR gene-mutation database. Patients harboring identical mutations were in some instances found to exhibit divergent phenotypes.

关键词： androgen insensitivity syndrome androgen receptor gene novel mutations genotype-phenotype correlation

来源：

维普期刊数据库

同方期刊数据库评论

在线全文

学校读者我要写书评

暂无评论

The effects of obesity on venous thromboembolism: A review

引用

Open Journal of Preventive Medicine 2012年第4期2卷 499-509页

作者： Genyan Yang Christine De Staercke W. Craig Hooper Clinical and Molecular Hemostasis Laboratory Branch Division of Blood Disorders National Center on Birth Defects and Developmental Disabilities Centers for Disease Control and Prevention Atlanta Georgia

Obesity has emerged as a global health issue that is associated with wide spectrum of disorders, including coronary artery disease, diabetes mellitus, hypertension, stroke, and venous thromboembolism (VTE). VTE is one of the most common vascular disorders in the United States and Europe and is associated with significant mortality. Although the association between obesity and VTE appears to be moderate, obesity can interact with other environmental or genetic factors and pose a significantly greater risk of VTE among individuals who are obese and who are exposed simultaneously to several other risk factors for VTE. Therefore, identification of potential interactions between obesity and certain VTE risk factors might offer some critical points for VTE interventions and thus minimize VTE morbidity and mortality among patients who are obese. However, current obesity measurements have limitations and can introduce contradictory results in the outcome of obesity. To overcome these limitations, this review proposes several future directions and suggests some avenues for prevention of VTE associated with obesity as well.

关键词： Obesity Comorbidity Deep Vein Thrombosis Pulmonary Embolism Venous Thromboembolism Risk Factor Prevention

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

national Perinatal Prevalence of Selected Major birth defects--China,2010−2018

引用

China CDC weekly 2020年第37期2卷 711-717页

作者： Wenli Xu Changfei Deng Wenyan Li Ke Wang Jing Tao Yuyang Gao Xiaohong Li Yanping Wang Juan Liang Jun Zhu Hanmin Liu Li Dai national center for birth defects Monitoring West China Second University HospitalSichuan UniversityChengduSichuanChina Key Laboratory of birth defects and Related Diseases of Women and Children(Sichuan University) Ministry of EducationChengduSichuanChina Medical Big Data center Sichuan UniversityChengduSichuanChina.

Introduction:An estimated of 900,000 infants are born with birth defects each year in China causing a substantial disease *** study aimed to depict the epidemiological patterns of selected major birth defects in Chinese perinatal births and provide important baseline data for future ***:Data from the Chinese birth defects Monitoring Network(CBDMN)during 2010–2018 were used to analyze the epidemiological pattern in the prevalence of 15 major birth defects and the trends over ***:In the period of 2010–2018,the top 10 most frequently-occurring birth defects in China included congenital heart diseases(CHDs),polydactyly,cleft lip with or without palate(CL/P),club foot,syndactyly,hydrocephalus,hypospadias,limb reduction defects(LRD),anotia/microtia,and anorectal atresia/*** was a decrease in the prevalence of neural tube defects,CL/P,hydrocephalus,LRD,gastroschisis,and omphalocele,but there were increases in the prevalence of CHDs,cleft palate,hypospadias,club foot,polydactyly,and *** prevalence of most birth defects varied significantly by maternal age,area types(urban/rural),and geographic *** and Implications for Public Health Practice:The findings indicated that the comprehensive prevention of birth defects should focus on these selected birth defects,elderly pregnant women,rural areas,and western regions.

关键词： prevention defects birth

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Physiological functions and clinical implications of fibrinogen-like 2:A review

引用

World Journal of Clinical Infectious Diseases 2013年第3期3卷 37-46页

作者： Genyan Yang W Craig Hooper Clinical and Molecular Hemostasis Laboratory Branch Division of Blood Disorders National Center on Birth Defects and Developmental DisabilitiesCenters for Disease Control and Prevention Atlanta GA 30301United States

Fibrinogen-like 2(FGL2) encompasses a transmembrane(m FGL2) and a soluble(s FGL2) form with differential tertiary structure and biological activities. Typically, m FGL2 functions as prothrombinase that is capable of initiating coagulation in tissue without activation of the blood clotting cascade, whereas s FGL2 largely acts as an immunosuppressor that can repress proliferation of alloreactive T lymphocytes and maturation of bone marrow dendritic cells. Protein sequences of FGL2 exhibit evolutionary conservation across wide variety of species, especially at the carboxyl terminus that contains fibrinogen related domain(FRED). The FRED of FGL2 confers specificity and complexity in the action of FGL2, including receptor recognition, calcium affiliation, and substrate binding. Constitutive expression of FGL2 during embryogenesis and in mature tissues suggests FGL2 might be physiologically important. However, excessive induction of FGL2 under certain medical conditions(e.g. pathogen invasion) could trigger complement activation, inflammatory response,cellular apoptosis, and immune dysfunctions. On the other hand, complete absence of FGL2 is also detrimental as lack of FGL2 can cause autoimmune glomerulonephritis and acute cellular rejection of xenografts. All these roles involve m FGL2, s FGL2, or their combination. Although it is not clear how m FGL2 is cleaved off its host cells and secreted into the blood, circulating s FGL2 has been found correlated with disease severity and viral loading among patients with human hepatitis B virus or hepatitis C virus infection. Further studies are warranted to understand how FGL2 expression is regulated under physiological and pathological conditions. Even more interesting is to determine whether m FGL2 can fulfill an immunoregulatory role through its FRED at carboxyl end of the molecule and, and vice versa, whether s FGL2 is procoagulant upon binding to a target cell. Knowledge in this area should shed light on development of s FG

关键词： Fibrinogen-like 2 Prothrombinase Immunosuppressor Infectious disease

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Advances in the conventional clinical treatment for hepatoblastoma and therapeutic innovation

引用

World Journal of Pediatric Surgery 2021年第3期4卷 9-15页

作者： Zhixue Chen Rui Dong national Children's Medical center Department of OncologyChildren's Hospital of Fudan UniversityShanghai Key Laboratory of Birth DefectsShanghaiChina

Background Hepatoblastoma(HB)is a rare malignancy usually occurring in children under 3 years *** advancements in surgical techniques and molecular biology,new treatments have been *** resources The recent literatures on new treatments,molecular mechanisms and clinical trials for HB were searched and *** Surgical resection remains the main option for treatment of *** complete resection is recommended,a resection with microscopical positive margins(R1)may have similar 5-year overall survival and 5-year event-free survival(EFS)rates after cisplatin chemotherapy and the control of metastasis,as only once described so *** green-guided surgery can help achieve precise ***,associating liver partition and portal vein ligation for staged hepatectomy can rapidly increase future liver remnant volume compared with portal vein ligation or ***-containing chemotherapies slightly differ among the guidelines from the International Childhood Liver Tumors Strategy Group(SIOPEL),Children’s Oncology Group(COG)and Chinese Anti-Cancer Association Pediatric Committee(CCCG),and the 3-year EFS rate of patients in SIOPEL and CCCG studies was recently shown to be higher than that in COG *** transplantation is an option for patients with unresectable HB,and successful cases of autologous liver transplantation have been *** addition,effective inhibitors of important targets,such as the mTOR(mammalian target of rapamycin)inhibitor rapamycin,β-catenin inhibitor celecoxib and EpCAM(epithelial cell adhesion molecule)inhibitor catumaxomab,have been demonstrated to reduce the activity of HB cells and to control metastasis in experimental research and clinical *** These advances in surgical and medical treatment provide better outcomes for children with HB,and identifying novel targets may lead to the development of future targeted therapies and immunotherapies.

关键词： clinical metastasis treatment

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

Unrecognized congenital heart disease in rural school-age children:getting to the root of the problem

引用

World Journal of Pediatrics 2022年第5期18卷 305-307页

作者： Qu-Ming Zhao Guo-Ying Huang Pediatric Heart center Children's Hospital of Fudan UniversityNational Children's Medical Center399 Wan Yuan RoadShanghai 201102China Shanghai Key Laboratory of birth defects ShanghaiChina

Congenital heart disease(CHD)is an important contributor to morbidity and mortality in children,and the global burden of this illness has raised unique challenges to the healthcare system[1,2].China is among the countries with the highest burden of this disorder despite having achieved huge advances in cardiac surgery or interventional therapies in CHD during the past two decades[3].Interestingly and unexpected is the lower prevalence rate of CHD reported in *** prevalence of CHD at live birth is estimated to be 9.4 per 1000 worldwide[4],However,this figure is much lower in China,with only 4.9 per 1000 live births between 2015 and 2019[5].

关键词： mortality congenital raised

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

A De Novo Variant Identified in the PPP2R1A Gene in an Infant Induces Neurodevelopmental Abnormalities

引用

Neuroscience Bulletin 2020年第2期36卷 179-182页

作者： Yanghui Zhang Haoxian Li Hua Wang Zhengjun Jia Hui Xi Xiao Mao national Health Commission Key Laboratory of birth defects Research Prevention and TreatmentHunan Provincial Maternal and Child Health Care HospitalChangsha 410008China

Dear Editor,Neurodevelopmental disorders include a wide range of conditions such as epilepsy,intellectual disability,and autism spectrum *** disorders commonly cooccur in patients,which suggests that they share a common etiology[1,2].Genetic advances resulting from sequencing techniques over the past few years have greatly expanded our understanding of neurodevelopmental disorders.

关键词： epilepsy expanded patients

来源：

维普期刊数据库

同方期刊数据库评论

在线全文

学校读者我要写书评

暂无评论

Highly efficient fermentation of glycerol and 1,3-propanediol using a novel starch as feedstock

引用

Food Bioscience 2022年第2期46卷 200-208页

作者： You Yang Ying Chen Yanling Jin Jing Liu Xiang Qin Wenjun Liu Ling Guo Department of Pediatrics Children Hematological Oncology and Birth Defects LaboratoryThe Affiliated Hospital of Southwest Medical UniversitySichuan Clinical Research Center for Birth DefectsLuzhouSichuan646000China CAS Key Laboratory of Environmental and Applied Microbiology Environmental Microbiology Key Laboratory of Sichuan ProvinceChengdu Institute of BiologyChinese Academy of SciencesChengduSichuan610041China national Engineering Research center for Non-Food Biorefinery Guangxi Academy of SciencesNanningGuangxi530007China

As a potential cost-effective feedstock for highly efficient fermentation of glycerol and its downstream product 1,3-propanediol(1,3-PD),duckweed starch was characterized and used for glycerol fermentation,for the first time,in this *** involved in glycerol biosynthesis(gpd1 and gpp2)were overexpressed in Escherichia coli,and genes involved in glycerol catabolism(glpK and gldA)were disrupted,which led to significantly decreased residual sugar levels and dramatically increased glycerol *** maximum glycerol concentration in fed-batch fermentation reached 102.72 g L^(-1) at 28 h,and the glycerol productivity was 3.67 g L^(-1) h^(-1),which,to our knowledge,is the highest productivity thus far ***,glycerol broth was fermented into 1,3-PD by Klebsiella *** concentration,conversion rate and productivity of 1,3-PD reached 35.54 g L^(-1),40.28%and 0.89 g L^(-1) h^(-1),respectively,without *** summary,the duckweed starch-to-glycerol-to-1,3-PD process is feasible and shows potential for improving glycerol industry competitiveness.

关键词： Duckweed starch Glycerol 1,3-Propanediol Fed-batch fermentation Biosynthesis

来源：

维普期刊数据库评论

在线全文

维普期刊数据库

学校读者我要写书评

暂无评论

欢迎您,

建议与咨询留下您的常用邮箱和电话号码，以便我们向您反馈解决方案和替代方法

时间限定

文献类型

馆藏选择

核心期刊

语言

文献类型

帮助

文字说明：

检索规则说明：

检索范例：

分类表

所选分类

限定检索结果

文献类型

馆藏范围

日期分布

学科分类号

主题

机构

作者

语言

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

请选择保存的检索档案：

请选择收藏分类：

通借通还

欢迎您,

建议与咨询 留下您的常用邮箱和电话号码，以便我们向您反馈解决方案和替代方法

时间限定

文献类型

馆藏选择

核心期刊

语言

文献类型

帮助

文字说明：

检索规则说明：

检索范例：

分类表

所选分类

限定检索结果

文献类型

馆藏范围

日期分布

学科分类号

主题

机构

作者

语言

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

在线全文

请选择保存的检索档案： 新增检索档案 确定 取消

请选择收藏分类： 新增自定义分类 确定 取消

通借通还

建议与咨询留下您的常用邮箱和电话号码，以便我们向您反馈解决方案和替代方法

请选择保存的检索档案：

请选择收藏分类：