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Phenotypic characterization of patients with activated PI3Kδ syndrome 1 presenting with features of systemic lupus erythematosus

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Genes & Diseases 2021年第6期8卷 907-917页

作者： Yanping Wang Qiuyun Yang Xuemei Chen Wenjing Tang Lina Zhou Zhi Chen Yunfei An Zhiyong Zhang Xuemei Tang Xiaodong Zhao National clinical Research Center for Child Health and Disorders(Chongqing) Children’s Hospital ofChongqing Medical UniversityChongqing400014PR China China International Science and Technology cooperation Base of Child Development and Critical Disorders Children’s Hospital of Chongqing Medical UniversityChongqing400014PR China Chongqing Key Laboratory of Child Infection and Immunity Children’s Hospital of Chongqing Medical UniversityChongqing400014PR China Division of Rheumatology and Immunology Children’s Hospital of Chongqing Medical UniversityChongqing400014PR China

Activated phosphoinositide 3-kinase d syndrome 1(APDS1)is a primary immunode-ficiency disease caused by gain-of-function mutations in *** features of autoimmune disease have been reported in patients with *** this study,we reported three patients with APDS1 presenting with systemic lupus erythematosus(SLE)*** clinical manifestations included recurrent respiratory tract infection,lymphoproliferation,Coombs-positive hemolytic anemia,decreased complement fractions,positive antinuclear antibodies,renal complications related to SLE associated diseases,which met the clinical spectrum of APDS1 and the classification criteria of *** immunological phenotype included an inversion in the CD4:CD8 ratio,an increase in both non-circulating Tfh CD4^(+)memory T and circulating Tfh populations,a low level of recent thymic emigrant T cells,overexpression of CD57 on T cells,and a decrease in B cells with fewer antibody class switch *** phenotypes detected in patients with APDS1 presenting with SLE were resemble that in patients with APDS1 presenting without ***,we described the effect of glucocorticoids and rapamycin therapy on patients with *** phosphorylation of S6 at Ser235/236 was inhibited in patients with APDS1 who underwent glucocorticoids therapy,including two who presented with SLE *** phosphorylation of AKT at Ser473 and phosphorylation of S6 at Ser235/236 were inhibited in other patients with APDS1 who underwent rapamycin ***,we showed the coexistence of immunodeficiency and SLE phenotype in APDS1,and the inhibition of rapamycin in activated Akt-mTOR signaling pathway.

关键词： Activated phosphoinositide 3-kinase dsyndrome 1 Autoimmune disease Immunosuppressive therapy PIK3CD Systemic lupus erythematosus

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Retinoic acid can improve autophagy through depression of the PI3K-Akt-mTOR signaling pathway via RARαto restore spermatogenesis in cryptorchid infertile rats

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Genes & Diseases 2022年第5期9卷 1368-1377页

作者： Chunlan Long Yu Zhou Lianju Shen Yihang Yu Dong Hu Xing Liu Tao Lin Dawei He Tao Xu Deying Zhang Jing Zhu Guanghui Wei Chongqing Key Laboratory of Children Urogenital Development and Tissue Engineering Ministry of Education Key Laboratory of Child Development and DisordersNational Clinical Research Center for Child Health and DisordersChina International Science and Technology Cooperation Base of Child Development and Critical DisordersChongqing Key Laboratory of PediatricsChongqing 400014PR China Department of Urology Children’s Hospital of Chongqing Medical UniversityChongqing 400014PR China Bio-manufacturing Center Department of Mechanical EngineeringTsinghua UniversityBeijing 100084PR China

Cryptorchidism-caused adult infertility is a common component of idiopathic reasons for male *** acid(RA)has a vital effect on the spermatogenesis ***,we found that the expression of c-Kit,Stra8,and Sycp3 could be up-regulated via the activation of retinoic acid receptorα(RARα)after RA supplementation in neonatal cryptorchid infertile *** also demonstrated that the protein expression of PI3K,p-Akt/pan-Akt,and p-mTOR/mTOR was higher in cryptorchid than in normal testes,and could be suppressed with RA in *** RA treatment in infertile cryptorchid testis in vivo,the levels of the autophagy proteins LC3 and Beclin1 increased and those of P62 *** tracer indicated that the permeability of blood-testis barrier(BTB)in cryptorchid rats decreased after RA ***,after blocking the RARαwith AR7(an RARαantagonist)in testicle culture in vitro,we observed that compared with normal testes,the PI3K-Akt-mTOR signaling pathway and the autophagy pathway was increased and decreased,respectively,which were coincident with cryptorchisd testes in ***,the appropriate concentrations of RA treatment could depress the PI3K-Akt-mTOR signaling pathway and improve the autophagy *** results confirmed that RA can rehabilitate BTB function and drive key protein levels in spermatogonial differentiation through depressing the PI3K-Akt-mTOR signaling pathway via RARα.

关键词： Cryptorchidism Male infertility Retinoic acid Spermatogenesis Testis

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Trends in Mortality of Cirrhosis in China: An Analysis of the China Death Surveillance Database from 2008 to 2020

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Journal of clinical and Translational Hepatology 2024年第3期12卷 236-244页

作者： Xiaoxiao Wang Huixin Liu Jinlei Qi Lijun Wang Peng Yin Feng Liu Lai Wei Yu Wang Maigeng Zhou Huiying Rao Peking University People’s Hospital Peking University Hepatology InstituteBeijing Key Laboratory of Hepatitis C and Immunotherapy for Liver DiseasesBeijing International Cooperation Base for Science and Technology on NAFLD DiagnosisBeijingChina Department of clinical Epidemiology and Biostatistics Peking University People’s HospitalBeijingChina National Center for Chronic Noncommunicable Disease Control and Prevention Chinese Center for Disease Control and PreventionBeijingChina Beijing Tsinghua Changgung Hospital Tsinghua UniversityBeijingChina Chinese Foundation for Hepatitis Prevention and Control BeijingChina

Background and Aims:China accounts for 14.9%of total cirrhosis deaths worldwide.A detailed and comprehensive understanding of the contemporary status of cirrhosis mortality in China is crucial for establishing strategies for intervention and decreasing the disease burden of cirrhosis *** study aimed to report the cirrhosis mortality rates in our whole country or province over ***:Mortality data from 2008 to 2020 were retrieved from the Disease Surveillance Point System(DSPs)of the Chinese Center for Disease Control and *** crude mortality rate and age-standardized mortality rate of patients with cirrhosis were stratified by sex,residential location,and *** average annual percentage change(AAPC)in cirrhosis mortality rates from 2008 to 2020 was also ***:The crude mortality rate of cirrhosis was 4.57/100,000 people in *** with females and individuals living in urban areas,males and people living in rural areas had greater age-standardized *** crude mortality rate and age-standardized mortality rate in provinces in Southwest China(Guangxi,Yunnan,Guizhou,and Qinghai)were greater than those in other ***,with increasing age,the age-specific mortality rate increased *** 2008 to 2020,the mortality rate of cirrhosis in China decreased except for in males aged 50–59 years,females aged 45–49 years and females aged 80–84 ***:The mortality rate of patients with cirrhosis in China decreased from 2008 to *** the future,interventions of cirrhosis mortality control need to pay more attention to all males,females aged 45–49 and 80–84 years,and people living in rural areas and in provinces in Southwest China.

关键词： Cirrhosis Mortality Trend China

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Diagnosis,treatment,outcome,and reasons for delayed treatment of cryptorchidism with torsion in children:a 16-year retrospective study in a large pediatric medical center

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Asian Journal of Andrology 2022年第4期24卷 386-389页

作者： Meng Yang Sheng Wen Tao Lin Xing Liu De-Ying Zhang Feng Liu Sheng-De Wu Guang-Hui Wei Pei-Ru He Yi Hua Department of Urology Children's Hospital of Chongqing Medical UniversityChongqing 400000China Pediatrics Research Institute Children's Hospital of Chongqing Medical UniversityMinistry of Education Key Laboratory of Child Development and DisordersNational Clinical Research Center for Child Health and DisordersChina International Science and Technology Cooperation Base of Child Development and Critical DisordersChildren's Hospital of Chongqing Medical UniversityChongqing 400000China Chongqing Key Laboratory of Pediatrics Chongqing Key Laboratory of Children Urogenital Development and Tissue EngineeringChongqing 400000China.

We describe and summarize the diagnosis,treatment,and reasons for delayed treatment of children with cryptorchidism torsion in Children's Hospital of Chongqing Medical *** study included 19 cases of cryptorchidism *** age of the children ranged from 16 days to 12 years(median:6 years).The interval from diagnosis to surgery varied from 4 h to 16 days(median:3 days).Ultrasound was performed in all *** cases had cryptorchidism torsion,2 cases had a soft tissue mass in the inguinal region,and 2 cases had an inguinal/abdominal *** cases were treated with an orchidopexy,12 cases were treated with orchiectomy,and 2 cases received resection of a testicular *** 5 children with an orchidopexy were followed up from 1 month to 7 years(median:3 years),with 1 child having a testis retraction and no blood *** the 12 children who had an orchiectomy,three had delayed diagnosis due to family unawareness of the condition,while other delays were due to delayed referral from primary care *** relative rarity and insufficient awareness of cryptorchidism torsion resulted in a low rate of testicular ***,hospitals of all levels should be fully aware of cryptorchidism with torsion and ensure a male child's genital system and inguinal region are examined to improve the success rate of testicular salvage.

关键词： children cryptorchidism testicular torsion

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NLRP3 inflammasome as a novel therapeutic target for Alzheimer’s disease

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Signal Transduction and Targeted Therapy 2020年第1期5卷 2098-2099页

作者： Yun Zhang Zhifang Dong Weihong Song Townsend Family Laboratories Department of PsychiatryThe University of British Columbia2255 Wesbrook MallVancouverBC V6T 1Z3Canada Pediatric Research Institute Ministry of Education Key Laboratory of Child Development and DisordersNational Clinical Research Center for Child Health and DisordersChina International Science and Technology Cooperation Base of Child Development and Critical DisordersChongqing Key Laboratory of Translational Medical Research in Cognitive Development and Learning and Memory DisordersChildren’s Hospital of Chongqing Medical UniversityChongqingChina

In a recent publication in Nature,Ising et *** the effect of the NLRP3 inflammasome on Aβ-induced tau pathology in Alzheimer’s disease(AD).The findings indicate that NLRP3 may be a novel target for the treatment of AD.

关键词： Alzheimer Aβ tau

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Dual-directional regulation of spinal cord injury and the gut microbiota

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Neural Regeneration Research 2024年第3期19卷 548-556页

作者： Yinjie Cui Jingyi Liu Xiao Lei Shuwen Liu Haixia Chen Zhijian Wei Hongru Li Yuan Yang Chenguang Zheng Zhongzheng Li Academy of Medical Engineering and Translational Medicine Tianjin UniversityTianjinChina School of Medical Technology Tianjin University of Traditional Chinese MedicineTianjinChina Research Center of Experimental Acupuncture Science Tianjin University of Traditional Chinese MedicineTianjinChina International cooperation and Exchange Office Chengdu University of Traditional Chinese MedicineChengduSichuan ProvinceChina International cooperation Base of Spinal Cord Injury Tianjin Key Laboratory of Spine and Spinal Cord InjuryDepartment of OrthopedicsTianjin Medical University General HospitalTianjinChina Department of Orthopedics Qilu Hospital of Shandong UniversityJinanShandong ProvinceChina

There is increasing evidence that the gut microbiota affects the incidence and progression of central nervous system diseases via the brain-gut *** spinal cord is a vital important part of the central nervous system;however,the underlying association between spinal cord injury and gut interactions remains *** studies suggest that patients with spinal cord injury frequently experience intestinal dysfunction and gut *** in the gut microbiota can cause disruption in the intestinal barrier and trigger neurogenic inflammatory responses which may impede recovery after spinal cord *** review summarizes existing clinical and basic research on the relationship between the gut microbiota and spinal cord *** research identified three key ***,the gut microbiota in patients with spinal cord injury presents a key characteristic and gut dysbiosis may profoundly influence multiple organs and systems in patients with spinal cord ***,following spinal cord injury,weakened intestinal peristalsis,prolonged intestinal transport time,and immune dysfunction of the intestine caused by abnormal autonomic nerve function,as well as frequent antibiotic treatment,may induce gut ***,the gut microbiota and associated metabolites may act on central neurons and affect recovery after spinal cord injury;cytokines and the Toll-like receptor ligand pathways have been identified as crucial mechanisms in the communication between the gut microbiota and central nervous *** microbiota transplantation,probiotics,dietary interventions,and other therapies have been shown to serve a neuroprotective role in spinal cord injury by modulating the gut *** targeting the gut microbiota or associated metabolites are a promising approach to promote functional recovery and improve the complications of spinal cord injury.

关键词： chemokines cytokines gut microbiota NLRP3 spinal cord injury Toll-like receptor ligand tryptophan

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BMP9 induces osteogenic differentiation through up-regulating LGR4 via the mTORC1/ Stat3 pathway in mesenchymal stem cells

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Genes & Diseases 2024年第3期11卷 468-483页

作者： Jie Zhang Jinhai Jiang Hang Liu Shiyu Wang Kaixin Ke Siyuan Liu Yue Jiang Lu Liu Xiang Gao Baicheng He Yuxi Su Department of Pharmacology School of PharmacyChongqing Medical UniversityChongqing 400016China Key Laboratory of Biochemistry and Molecular Pharmacology of Chongqing Chongqing Medical UniversityChongqing 400016China Department of Orthopedics Second Affiliated Hospital of Chongqing Medical UniversityChongqing 400016China orthopedics Department Children's Hospital of Chongqing Medical UniversityChongqing 400014China China International Science and Technology cooperation Base of Child Development and Critical Disorders Jiangxi Hospital Affiliated Children's Hospital of Chongqing Medical UniversityJiangxi 330000China National clinical Research Center for Child Health and Disorders China

Bone defects and non-union are prevalent in clinical orthopedy,and the outcomes of current treatments are often *** tissue engineering offers a promising approach to treating these conditions *** morphogenetic protein 9(BMP9)can commit mesenchymal stem cells to osteogenic lineage,and a knowledge of the underlying mechanisms may help advance the field of bone tissue ***-rich repeats con-taining G protein-coupled receptor 4(LGR4),a member of G protein-coupled receptors,is essential for modulating bone *** study is aimed at investigating the impact of LGR4 on BMP9-induced osteogenesis in mesenchymal stem cells as well as the underlying *** marrow stromal cells from BMp9-knockout mice exhibited diminished LGR4 expression,and exogenous LGR4 clearly restored the impaired osteogenic potency of the bone marrow stromal ***,LGR4 expression was increased by BMP9 in C3H10T1/2 ***4 augmented the benefits of BMP9-induced osteogenic markers and bone formation,whereas LGR4 inhibition restricted these ***,the BMP9-induced li-pogenic markers were increased by LGR4 *** protein levels of Raptor and p-Stat3 were elevated by *** knockdown or p-Stat3 suppression attenuated the osteoblastic markers and LGR4 expression brought on by ***4 significantly reversed the blocking ef-fect of Raptor knockdown or p-Stat3 suppression on the BMP9-induced osteoblastic *** interacts with p-Stat3,and p-Stat3 activates the LGR4 promoter *** conclusion,LGR4 boosts BMP9 osteoblastic potency in mesenchymal stem cells,and BMP9 may up-regulate LGR4 via the mTORC1/Stat3 signal activation.

关键词： BMP9 LGR4 mTORC1 Osteogenic differentiation Stat3

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Incidence characteristics of testicular microlithiasis and its association with risk of primary testicular tumors in children: a systematic review and meta-analysis

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World Journal of Pediatrics 2020年第6期16卷 585-597页

作者： Cheng-Jun Yu Jian-Dong Lu Jie Zhao Yi Wei Tian-Xin Zhao Tao Lin •Da-Wei He Sheng-De Wu Guang-Hui Wei Department of Urology Children's Hospital of Chongqing Medical UniversityChongqingChina Chongqing Key Laboratory of Children Urogenital Development and Tissue Engineering ChongqingChina National clinical Research Center for Child Health and Disorders ChongqingChina Chongqing Key Laboratory of Pediatrics Chongqing Room 806.Kejiao Building(No.6)No.136.Zhongshan 2nd RoadYuzhong DistrictChongqing.China China International Science and Technology cooperation Base of Child Development and Critical Disorders ChongqingChina Ministry of Education Key Laboratory of Child Development and Disorders ChongqingChina

Background To systematically evaluate the incidence characteristics of testicular microlithiasis(TM)in children and its association with primary testicular tumors(PTT).Methods A systematic review and meta-analysis were conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-analysis(PRISMA)statement.A priori protocol was registered in the PROSPERO database(CRD42018111119),and a literature search of all relevant studies published until February 2019 was ***,retrospective cohort,or cross-sectional studies containing ultrasonography(US)data on the incidence of TM or the association between TM and PTT were eligible for *** Of the 102 identified articles,18 studies involving 58,195 children were included in the final *** overall incidence of TM in children with additional risk factors for FTT was 2.1%.In children,the proportion of left TM in unilateral cases was 55.7%,the frequency of bilateral TM was 69.0%,and proportion of classic TM was 71.8%[95%confidence interval(Cl)62.4-81.1%,P=0.0,72=0.0%].About 93.5%of TM remained unchanged,and newly detected PTT rate was very low(4/296)during *** overall risk ratio of TM in children with a concurrent diagnosis of PTT was 15.46(95%Cl 6.93-34.47,P<0.00001).Conclusions The incidence of TM in children is highly ***,TM is usually bilateral,of the classic type,and remains stable or unchanged at *** patients with TM and contributing factors for PTT have an increased risk for PTT;however,there is no evidence to support mandatory US surveillance of children with TM.

关键词： Children Incidence Primary testicular tumors Testicular microlithiasis

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Molecular glue triggers degradation of PHGDH by enhancing the interaction between DDB1 and PHGDH

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Acta Pharmaceutica Sinica B 2024年第9期14卷 4001-4013页

作者： Ziqi Huang Kun Zhang Yurui Jiang Mengmeng Wang Mei Li Yuda Guo Ruolin Gao Ning Li Chenyang Wang Jia Chen Jiefu Wang Ning Liu Xiang Liu Shuangwei Liu Mingming Wei Cheng Yang Guang Yang The State Key Laboratory of Medicinal Chemical Biology College of Life SciencesCollege of PharmacyNankai UniversityTianjin 300071China Tianjin Medical University Cancer Institute and Hospital National Clinical Research Center for CancerTianjin’s Clinical Research Center for CancerKey Laboratory of Cancer Prevention and TherapyTianjin 300060China

Cancer stem cells(CSCs)play a pivotal role in tumor initiation,proliferation,metastasis,drug resistance,and ***,targeting CSCs has emerged as a promising avenue for cancer ***,3-phosphoglycerate dehydrogenase(PHGDH)has been identified as being intricately associated with the regulation of numerous cancer stem ***,reports detailing the functional regulators of PHGDH that can mitigate the stemness across cancer types are *** this study,the novel“molecular glue”LXH-3-71 was identified,and it robustly induced degradation of PHGDH,thereby modulating the stemness of colorectal cancer cells(CRCs)both in vitro and in ***,LXH-3-71 was observed to form a dynamic chimera,between PHGDH and the DDB1-CRL E3 *** insights not only elucidate the anti-CSCs mechanism of the lead compound but also suggest that degradation of PHGDH may be a more viable therapeutic strategy than the development of PHGDH ***,compound LXH-3-71 was leveraged as a novel ligand for the DDB1-CRL E3 ligase,facilitating the development of new PROTAC molecules targeting EGFR and CDK4 degradation.

关键词： Molecular glue Targeted protein degradation PHGDH Cancer stem cells PROTACs

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Metabolic syndrome,ApoE genotype,and cognitive dysfunction in an elderly population:A single-center,case-control study

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World Journal of clinical Cases 2021年第5期9卷 1005-1015页

作者： Jie-Yu Wang Li Zhang Jia Liu Wei Yang Li-Na Ma Department of Geriatrics Xuanwu HospitalCapital Medical UniversityNational Clinical Research Center for Geriatric MedicineBeijing 100053China

BACKGROUND Metabolic syndrome(MetS)is related to poor cognitive ***,the results of previous studies were inconsistent,and whether the ApoEε4 allele modifies the association remains *** To elucidate the relationships among MetS,ApoEε4,and cognitive dysfunction in an elderly population in *** One hundred elderly patients with MetS and 102 age-and gender-matched controls were included in the *** clinical characteristics and biochemical index for glucose and lipid metabolism were *** distribution of ApoEε4 was assessed with PCR restriction fragment length polymorphism *** function was evaluated by mini-mental status examination at the 1-year follow-up *** Compared with controls,MetS patients had worse cognitive function and decreased ability to participate in activities of daily life(P=0.001 and 0.046,respectively).Patients with cognitive dysfunction had higher prevalence of MetS(62.1%vs 36.4%,P<0.001)and were more likely to carry the ApoEε4 allele(22.3%vs 10.1%,P=0.019).Multivariate logistic regression analyses showed that diagnosis with MetS,severe insulin resistance,status as an ApoEε4 carrier,higher systolic blood pressure,and larger waist circumference were risk factors for cognitive dysfunction(P<0.05).Repeated-measures analysis of variance,performed with data collected at the 1-year follow-up,revealed continuous influences of MetS and ApoEε4 on the deterioration of cognitive function(time×team,P<0.001 for both).CONCLUSION Diagnosis of MetS and ApoEε4 carrier status were persistently associated with cognitive dysfunction among an elderly population in China.

关键词： Metabolic syndrome ApoEε4 Cognitive dysfunction Elderly Genotype Case-control study

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