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Inflammasome links traumatic brain injury, chronic traumatic encephalopathy, and Alzheimer's disease

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Neural Regeneration Research 2025年第6期20卷 1644-1664页

作者： Gabriela Seplovich Yazan Bouchi Juan Pablo de Rivero Vaccari Jennifer C.Munoz Pareja Andrew Reisner Laura Blackwell Yehia Mechref Kevin K.Wang J.Adrian Tyndall Binu Tharakan Firas Kobeissy department of Surgery Morehouse School of MedicineAtlantaGAUSA department of Neurobiology Center for NeurotraumaMultiomics&Biomarkers(CNMB)Neuroscience InstituteMorehouse School of MedicineAtlantaGAUSA department of Neurological Surgery and the Miami Project to Cure Paralysis University of Miami Miller School of MedicineMiamiFLUSA Division of Pediatric Critical Care Department of PediatricsUniversity of Miami Miller School of MedicineMiamiFLUSA department of pediatrics Emory UniversityAtlantaGAUSA department of Neurosurgery Children’s Healthcare of AtlantaAtlantaGAUSA department of Chemistry and Biochemistry Texas Tech UniversityLubbockTXUSA Morehouse School of Medicine AtlantaGAUSA

Traumatic brain injury, chronic traumatic encephalopathy, and Alzheimer's disease are three distinct neurological disorders that share common pathophysiological mechanisms involving neuroinflammation. One sequela of neuroinflammation includes the pathologic hyperphosphorylation of tau protein, an endogenous microtubule-associated protein that protects the integrity of neuronal cytoskeletons. Tau hyperphosphorylation results in protein misfolding and subsequent accumulation of tau tangles forming neurotoxic aggregates. These misfolded proteins are characteristic of traumatic brain injury, chronic traumatic encephalopathy, and Alzheimer's disease and can lead to downstream neuroinflammatory processes, including assembly and activation of the inflammasome complex. Inflammasomes refer to a family of multimeric protein units that, upon activation, release a cascade of signaling molecules resulting in caspase-induced cell death and inflammation mediated by the release of interleukin-1β cytokine. One specific inflammasome, the NOD-like receptor protein 3, has been proposed to be a key regulator of tau phosphorylation where it has been shown that prolonged NOD-like receptor protein 3 activation acts as a causal factor in pathological tau accumulation and spreading. This review begins by describing the epidemiology and pathophysiology of traumatic brain injury, chronic traumatic encephalopathy, and Alzheimer's disease. Next, we highlight neuroinflammation as an overriding theme and discuss the role of the NOD-like receptor protein 3 inflammasome in the formation of tau deposits and how such tauopathic entities spread throughout the brain. We then propose a novel framework linking traumatic brain injury, chronic traumatic encephalopathy, and Alzheimer's disease as inflammasomedependent pathologies that exist along a temporal continuum. Finally, we discuss potential therapeutic targets that may intercept this pathway and ultimately minimize long-term neurological decline.

关键词： Alzheimer's disease caspase-1 chronic traumatic encephalopathy inflammasomes neurodegeneration neuroinflammation NLRP1 NLRP3 pyroptosis tauopathy traumatic brain injury

Population-based frequency of surfactant dysfunction mutations in a native Chinese cohort

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World Journal of pediatrics 2016年第2期12卷 190-195页

作者： Yu-Jun Chen Jennifer Anne Wambach Kelcey DePass Daniel James Wegner Shao-Ke Chen Qun-Yuan Zhang Hillary Heins Francis Sessions Cole Aaron Hamvas Division of Neonatology Department of Pediatricsthe First Affi liated Hospital of Guangxi Medical UniversityNanningChina Division of Newborn Medicine Edward Mallinckrodt Department of PediatricsWashington University School of MedicineSt.LouisUSA department of pediatrics Guangxi Maternal and Child Health HospitalNanningChina Division of Statistical Genomics Department of GeneticsWashington University School of MedicineSt.LouisUSA

Background: Rare mutations in surfactant-associatedgenes contribute to neonatal respiratory distress *** frequency of mutations in these genes in the Chinesepopulation is ***: We obtained blood spots from the GuangxiNeonatal Screening Center in Nanning, China thatincluded Han (n=443) and Zhuang (n=313) ethnic *** resequenced all exons of the surfactant proteins-B(SFTPB), -C (SFTPC), and the ATP-binding cassettemember A3 (ABCA3) genes and compared the frequenciesof 5 common and all rare ***: We found minor differences in thefrequencies of the common variants in the Han andZhuang cohorts. We did not find any rare mutations inSFTPB or SFTPC, but we found three ABCA3 mutationsin the Han [minor allele frequency (MAF)=0.003] and 7 inthe Zhuang (MAF=0.011) cohorts (P=0.10). The ABCA3mutations were unique to each cohort;five were *** collapsed carrier rate of rare ABCA3 mutations inthe Han and Zhuang populations combined was 1.3%,which is signifi cantly lower than that in the United States(P<0.001).Conclusions: The population-based frequency ofmutations in ABCA3 in south China newborns is signifi cantlylower than that in United States. The contribution of theserare ABCA3 mutations to disease burden in the south Chinapopulation is still unknown.

关键词： genetic epidemiology human population genetics neonatal respiratory distress syndrome pulmonary surfactant

Evaluation and management of inherited disorders of surfactant metabolism

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Chinese Medical Journal 2010年第20期123卷 2943-2947页

作者： Aaron Hamvas edward mallinckrodt department of pediatrics Washington University School of Medicine and St. Louis Children's Hospital St. Louis Missouri 63110 USA

Objective To review the pathophysiology, evaluation, management, and outcomes of children with inherited disorders of surfactant metabolism due to mutations in the genes encoding surfactant proteins-B or -C（SFTPB, SFTPC）, ATP binding cassette member A3 （ABCA3）, and thyroid transcription factor （NKX2.1）.Data sources Review of the literature, previous work from the author＇s and collaborators＇ laboratories, St. Louis Children＇s Hospital Lung Transplant *** selection Key articles in the field, author＇s *** Inherited disorders of surfactant metabolism present as acute, severe respiratory dysfunction in the neonatal period （SFTPB, ABCA3, NKX2.1） or as chronic respiratory insufficiency in later infancy and childhood which is of variable onset, severity, and course （SFTPC, ABCA3, NKX2.1）. Diagnosis is established with sequencing the relevant genes; lung biopsy with electron microscopy is a useful adjunct. For surfactant protein-B and ABCA3 deficiency presenting with acute neonatal disease, treatment options are limited to lung transplantation or compassionate care. For the more chronic presentations of surfactant protein-C, ABCA3, and NKX2.1 associated disease, the natural history is variable and therefore individualized, supportive care is appropriate,Conclusions Inherited disorders of surfactant metabolism are rare, but informative diseases that provide unique opportunities for understanding mechanisms of respiratory disease in newborns and children.

关键词： respiratory distress syndrome newborn genetics lung transplantation

FUBP3 mediates the amyloid-β-induced neuronal NLRP3 expression

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Neural Regeneration Research 2025年第7期20卷 2068-2083页

作者： Jing Yao Yuan Li Xi Liu Wenping Liang Yu Li Liyong Wu Zhe Wang Weihong Song The National Clinical Research Center for Geriatric Disease Xuanwu HospitalCapital Medical UniversityBeijingChina department of Neurology The First Affiliated Hospital of Zhengzhou UniversityZhengzhouHenan ProvinceChina department of Neurosurgery Xuanwu HospitalCapital Medical UniversityBeijingChina department of pediatrics The Second Affiliated Hospital and Yuying Children’s HospitalKey Laboratory of Alzheimer’s Disease of Zhejiang ProvinceInstitute of AgingWenzhou Medical UniversityWenzhouZhejiang ProvinceChina Oujiang Laboratory(Zhejiang Laboratory for Regenerative Medicine Visionand Brain Health)WenzhouZhejiang ProvinceChina

Alzheimer's disease is characterized by deposition of amyloid-β,which forms extracellular neuritic plaques,and accumulation of hyperphosphorylated tau,which aggregates to form intraneuronal neurofibrillary tangles,in the *** NLRP3 inflammasome may play a role in the transition from amyloid-βdeposition to tau phosphorylation and *** NLRP3 is primarily found in brain microglia,and tau is predominantly located in neurons,it has been suggested that NLRP3 expressed by microglia indirectly triggers tau phosphorylation by upregulating the expression of pro-inflammatory ***,we found that neurons also express NLRP3 in vitro and in vivo,and that neuronal NLRP3 regulates tau *** biochemical methods,we mapped the minimal NLRP3 promoter and identified FUBP3 as a transcription factor regulating NLRP3 expression in *** primary neurons and the neuroblastoma cell line Neuro2A,FUBP3 is required for endogenous NLRP3 expression and tau phosphorylation only when amyloid-βis *** the brains of aged wild-type mice and a mouse model of Alzheimer's disease,FUBP3 expression was markedly increased in cortical *** analysis suggested that FUBP3 plays a role in neuron-mediated immune *** also found that FUBP3 trimmed the 5′end of DNA fragments that it bound,implying that FUBP3 functions in stress-induced *** findings suggest that neuronal NLRP3 may be more directly involved in the amyloid-β-to–phospho-tau transition than microglial NLRP3,and that amyloid-βfundamentally alters the regulatory mechanism of NLRP3 expression in *** that FUBP3 was only expressed at low levels in young wild-type mice and was strongly upregulated in the brains of aged mice and Alzheimer's disease mice,FUBP3 could be a safe therapeutic target for preventing Alzheimer's disease progression.

关键词： 5′end trimming Alzheimer's disease amyloid-beta amyloid-β-dependent transcription FUBP3 inflammasome inflammation neuron NLRP3 tau transcription factor

A mutation in signal peptide of rat resistin gene inhibits differentiation of 3T3-L1 preadipocytes

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Acta Pharmacologica Sinica 2004年第12期25卷 139-145页

作者： Xi-rong GUO2,3, Hai-xia GONG2, Yan-qin GAO4, Li FEI, Yu-hui NI, Rong-hua CHEN department of pediatrics, Center of Human Functional Genomics, Nanjing Medical University, Nanjing 210029, China 4department of pediatrics, the Third Affiliated Hospital of Nanjing Medical University, Yizheng 211900, China department of pediatrics Center of Human Functional Genomics Nanjing Medical University department of pediatrics the Third Affiliated Hospital of Nanjing Medical University

AIM: To detect the resistin expression of white adipose tissue in diet-induced obese (DIO) versus diet-resistant (DR)rats, andto investigatethe relationshipof mutatedresistin and 3T3-L1 preadipocytes differentiation. METHODS: RT-PCR and Western Blot were used to detect gene /protein expression. 3T3-L1 cells were cultured, transfected, and induced to differentiation using 0.5 mmol/L 3-isobutyl-1-methyxanthine (MIX), 1 mg/L insulin, and 1 μmol/L dexamethasone. Oil red O staining was applied to detect the degree of preadipocytes differentiation. RESULTS: Expression of resistin mRNA was upregulated in DIO rats and downregulated in DR rats. However, the expression levels varied greatly within the groups. Sequencing of the resistin genes from DIO and DR rats revealed a Leu9Val (C25G) missense mutation within the signal peptide in one DR rat. The mutant resistin inhibited preadipocyte differentiation. Local experiments and Western blotting with tagged resistin fusion proteins identified both mutant and wild type proteins in the cytoplasm and secreted into the culture medium. Computer predictions using the Proscan and Subloc programs revealed four putative phosphorylation sites and a possible leucine zipper motif within the rat resistin protein. CONCLUSION: Resistin-increased differentiation may be inhibited by the mutation- containing precursor protein, or by the mutant non-secretory resistin isoform.

关键词： resistin mutation adipocyte differentiation obesity

Molecular docking studies of human MCT8 protein with soy isoflavones in Allan-Herndon-Dudley syndrome(AHDS)

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Journal of Pharmaceutical Analysis 2018年第5期8卷 318-323页

作者： Divya Shaji department of pediatrics Aichi Medical University

Monocarboxylate transporter-8 （MCT8） is a specific thyroid hormone transporter, essential for the uptake of thyroid hormone into target tissues. Mutations in the MCT8 gene have been identified as the cause of Allan-Herndon-Dudley syndrome （AHDS）. It has been reported that soy isoflavones influence thyroid hormone system and can interact with thyroid hormone transporter proteins. Therefore, the present study aimed to find out whether soy isoflavones （genistein, daidzein and glycitein） can be used as a natural inhibitor to target MCT8 in AHDS. Docking studies were performed for soy isoflavones in order to evaluate their binding affinity to MCT8 protein using AutoDock4 （version 4.2.6） and AutoDock Vina. After docking, the ligands were ranked according to their binding energy and the best lead compound was selected based on the least binding energy. The docking results indicated that daidzein possesses the lowest binding energy against MCT8. Moreover, it was found that the residues PRO-338, HIS-341, and GLU-348 were involved in hydrogen bond interactions with genistein and daidzein. This study suggests that daidzein is a promising natural inhibitor to target MCT8 in AHDS.

关键词： Molecular docking MCT8 AHDS Soy isoflavones Daidzein Genistein

Imperative for long-term management and surveillance in Kawasaki disease

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World Journal of Clinical Cases 2025年第4期13卷 61-63页

作者： Yan Pan Fu-Yong Jiao department of pediatrics The First Affiliated Hospital of Yangtze UniversityJingzhou 434000Hubei ProvinceChina Shaanxi Kawasaki Disease Diagnosis and Treatment Center Children’s HospitalShaanxi Provincial People’s Hospital of Xi’an Jiaotong UniversityXi’an 710000Shaanxi ProvinceChina

Kawasaki disease(KD)is a significant pediatric vasculitis known for its potential to cause severe coronary artery *** the effectiveness of initial treatments,such as intravenous immunoglobulin,KD patients can experience long-term cardiovascular issues,as evidenced by a recent case report of an adult who suffered a ST-segment elevation myocardial infarction due to previous KD in the World Journal of Clinical *** editorial emphasizes the critical need for long-term management and regular surveillance to prevent such *** drawing on recent research and case studies,we advocate for a structured approach to follow-up care that includes routine cardiac evaluations and preventive measures.

关键词： Kawasaki disease Long-term management Coronary artery aneurysm Surveillance Preventive care

Hepatitis B vaccine in celiac disease:Yesterday,today and tomorrow

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World Journal of Gastroenterology 2013年第6期19卷 838-845页

作者： Giovanna Vitaliti Andrea Domenico Praticò Carla Cimino Giovanna Di Dio Elena Lionetti Mario La Rosa Salvatore Leonardi department of pediatrics University of Catania

Some studies showed that in celiac patients the immunological response to vaccination is similar to that one found in general population except for vaccine against hepatitis B virus (HBV).The non-responsiveness to HBV vaccine has also been described in healthy people,nevertheless the number of non-responders has been demonstrated to be higher in celiac disease (CD) patients than in healthy *** hypothesis explaining this higher rate of unresponsiveness to HBV vaccine in CD patients have been described,such as the genetic hypothesis,according with CD patients carrying the disease-specific haplotype HLA-B8,DR3,and DQ2,show a lower response to HBV vaccine both in clinical expressed CD patients and in healthy people carrying the same *** the other hand,it has been demonstrated that the gluten intake during the vaccination seems to influence the response to the same ***,it has been demonstrated a possible genetic predisposition to hepatitis B vaccine nonresponsiveness likely due to the presence of specific human leukocyte antigen haplotypes and specific single nucleotide polymorphism in genes of cytokine/cytokinereceptors and toll like receptors,but the pathogenic mechanism responsible for this low responsiveness still remains *** aim of this review is to focus on the possible pathogenic causes of unresponsiveness to HBV vaccine in CD patients and to propose an alternative vaccination schedule in order to improve the responsiveness to HBV vaccine in this at-risk patients.

关键词： Celiac disease Non responders Hepatitis B vaccine Vaccination schedules

Case Report:Congenital atresia of the left main coronary artery in an infant

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Journal of Zhejiang University-Science B(Biomedicine & Biotechnology) 2010年第7期11卷 539-541页

作者： Su Ye SOHN Gi Young JANG Byung Min CHOI department of pediatrics Korea University Hospital

Congenital atresia of the left main coronary artery is a rare occurrence, and surgical revascularizationbypass graft is required. We here report a rare case of congenital coronary anomaly in an infant. A lO-month-old male infant was admitted to the hospital with heart failure symptoms. Echocardiographic examinations revealed mitral valve regurgitation and ischemic changes of the anterolateral atresia of the left main coronary artery with a severe papillary muscle and chordae. Coronary angiography showed hypoplastic left anterior descending artery and a circumflex coronary artery. Unfortunately, sudden cardiac arrest occurred after catheterization and the infant did not recover despite of immediate cardiopulmonary resuscitation. Further studies are needed to find a newer diagnostic method to detect coronary anomaly in an infant, and coronary angiography, if necessary, has to be performed very carefully.

关键词： Conqenital heart disease Anomalous coronaries Infant