Novel Anti-inflammatory Approaches for Cystic Fibrosis Lung Disease: Identification of Molecular Targets and Design of Innovative Therapies PDF Download
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Author: Carla Maria Pedrosa Ribeiro
Publisher: Frontiers Media SA
ISBN: 2889719413
Category : Science
Languages : en
Pages : 286
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Book Description
Author: Carla Maria Pedrosa Ribeiro
Publisher: Frontiers Media SA
ISBN: 2889719413
Category : Science
Languages : en
Pages : 286
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Book Description
Author: Arata Azuma
Publisher: Springer
ISBN: 3034809778
Category : Medical
Languages : en
Pages : 266
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Book Description
This volume describes the pathogenesis and pathophysiology of several pulmonary diseases as well as their treatment. It also discusses the underlying genetic and molecular biological basis, which opens the way for new treatments for these conditions. It focuses on the treatment of cystic fibrosis including CFTR (cystic fibrosis transmembrane-conductance regulator) modulator therapies, drug therapies that augment airway surface liquid as well as anti-inflammatory and anti-infective therapies. Further topics include long-term, low-dose macrolide therapy for diffuse panbronchiolitis; novel agents for previously untreatable idiopathic pulmonary fibrosis; possible new treatments for pulmonary alveolar proteinosis (PAP); and multiple novel therapeutic targets for treating lymphangiomyomatosis. Research into these conditions has led to major advances in our understanding of the underlying genetic and molecular basis of this disease, and to dramatic improvements in survival and quality of life for affected individuals.
Author: Miquéias Lopes-Pacheco
Publisher: Frontiers Media SA
ISBN: 2889633675
Category :
Languages : en
Pages : 281
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Book Description
Author: Yuanyuan Gu
Publisher:
ISBN:
Category :
Languages : en
Pages : 138
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Book Description
Cystic fibrosis is the most common, lethal autosomal recessive disorder in the United States. Lung disease is the major cause of morbidity and mortality in CF. In the CF lung, chronic infection and dysregulated neutrophilic inflammation lead to progressive airway destruction. Despite the molecular insights afforded by identification of disease-causing gene, CFTR, a clear understanding of the pathogenesis of lung disease in CF remains elusive. There is a poor correlation of genotype with phenotype in lung disease in CF, which strongly suggests that the expression of lung disease in CF is influenced by environmental exposures and/or modifier genes. To search for genes modifying CF lung disease, the Karp lab performed a genome-wide association study in collaboration with GMSG cohort, validating top candidates in collaboration with the CFTSS cohort. Using this approach, genetic variation in IFRD1 was identified and verified as a modifier of lung disease severity in CF. IFRD1 is a HDAC-dependent transcriptional co-activator or co-repressor whose expression is particularly enriched in neutrophils. The goal of my dissertation studies was mechanistic insight into the modulation of CF lung disease by IFRD1. This dissertation research provides evidence in favor of the hypothesis that IFRD1 modulates the course of airway disease in CF through regulation of neutrophil effector function. This study also strongly suggests a mechanism by which IFRD1 modulates neutrophil function in a HDAC-dependent manner to co-suppress the expression of ATF3, a transcriptional repressor of NF-[kappa]B activity in neutrophils. Finally, this research emphasizes the translational implications for therapeutic targeting of neutrophils in CF. This study suggests that the IFRD1/HDAC axis may provide a tractable therapeutic target in CF, and the plethora of other diseases in which neutrophils play an important pathogenic role.
Author: Margarida D. Amaral
Publisher: Humana Press
ISBN: 9781617791192
Category : Science
Languages : en
Pages : 0
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Book Description
Despite the many milestones in cystic fibrosis (CF) research, progress toward curing the disease has been slow, and it is increasingly difficult to grasp and use the already wide and still growing range of diverse methods currently employed to study CF so as to understand it in its multidisciplinary nature. Cystic Fibrosis: Diagnosis and Protocols aims to provide the CF research community and related researchers with a very wide range of high-quality experimental tools, as an easy way to grasp and use classical and novel methods applied to cystic fibrosis. Volume II: Methods and Resources to Understand Cystic Fibrosis focuses on pathophysiology, Omics approaches, and a variety of key resources recently made available for CF research. Written in the highly successful Methods in Molecular BiologyTM series format, chapters include introductions to their respective topics, lists of the necessary materials and reagents, step-by-step, readily reproducible laboratory protocols, and tips on troubleshooting and avoiding known pitfalls. Comprehensive and practical, Cystic Fibrosis: Diagnosis and Protocols will provide readers with optimal working tools to address pressing questions in the best technical way, while helping all of us, as a research and clinical community, to move faster hand-in-hand toward unravelling the secrets of this challenging disorder and cure it.
Author: Margarida D. Amaral
Publisher: Humana Press
ISBN: 9781617791185
Category : Science
Languages : en
Pages : 528
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Book Description
Despite the many milestones in cystic fibrosis (CF) research, progress towards curing the disease has been slow, and it is increasingly difficult to grasp and use the already wide and still growing range of diverse methods currently employed to study CF so as to understand it in its multidisciplinary nature. Cystic Fibrosis: Diagnosis and Protocols aims to provide the CF research community and related researchers with a very wide range of high-quality experimental tools, as an easy way to grasp and use classical and novel methods applied to cystic fibrosis. Volume I: Approaches to Study and Correct CFTR Defects focuses on the cystic fibrosis transmembrane conductance regulator (CFTR) and its expression, biogenesis, structure, and function in terms of the defects causing CF. Written in the highly successful Methods in Molecular BiologyTM series format, chapters include introductions to their respective topics, lists of the necessary materials and reagents, step-by-step, readily reproducible laboratory protocols, and tips on troubleshooting and avoiding known pitfalls. Comprehensive and practical, Cystic Fibrosis: Diagnosis and Protocols will provide readers with optimal working tools to address pressing questions in the best technical way, while helping all of us, as a research and clinical community, to move faster hand-in-hand toward unravelling the secrets of this challenging disorder and cure it.
Author: Antoinette D. Hillian
Publisher:
ISBN:
Category : Cystic fibrosis
Languages : en
Pages : 179
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Book Description
Pulmonary disease is the most significant manifestation of cystic fibrosis (CF) and is primarily due to continuous and exaggerated inflammation stemming from an inherent dysregulation of the inflammatory processes. Specifically, airway inflammation is characterized by a disproportionate neutrophil load, high levels of neutrophilic enzymes, and excessive neutrophil chemokine expression; thus, the airway inflammation and subsequent damage is considered to be neutrophil-mediated. As interleukin-8 (IL-8) is the primary mediator for neutrophil migration and activation, the progression and maintenance of the neutrophilic inflammation is potentially due to elevated expression of IL-8. This dissertation examines how variations in the expression of IL-8 impact pulmonary disease severity.There is considerable phenotypic variation with regards to pulmonary disease progression among CF patients. It has been suggested that the majority of the observed variation is due to genetic heterogeneity, independent of CFTR genotype. To test this hypothesis, variants in a panel of inflammatory genes were evaluated for a statistical association with lung disease severity in a CF patient population. Variants in the IL8 gene were found to associate with lung disease severity. Specifically, a functional IL-8 promoter variant was identified and shown to result in an allele specific expression difference.To minimize damage caused by inflammation, patients are treated with anti-inflammatory drugs, including ibuprofen. High doses of ibuprofen have been shown to inhibit neutrophil migration, reduce the rate of lung function decline, and slow CF lung disease progression although the molecular mechanism has not been identified. Given its role in neutrophil recruitment and activation, IL-8 was tested as a potential molecular target of ibuprofen. In an epithelial cell line, IL-8 protein and message levels were reduced in response ibuprofen at the level of transcription. Furthermore, ibuprofen was found to correlate with reduced IL-8 message in nasal epithelium from CF patients. Therefore, it appears that the overall reduction of CF lung disease associated with ibuprofen is at least partially mediated through a decrease in IL8 expression. Together these data suggest that alterations in the expression of interleukin-8, either through naturally occurring genetic variation or pharmacological manipulation, modify cystic fibrosis pulmonary disease phenotype.
Author: John Engelhardt
Publisher:
ISBN: 9783039436842
Category :
Languages : en
Pages : 210
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Book Description
Of Genes. Thirty years ago, the gene responsible for cystic fibrosis (CF), a recessive genetic disease caused by mutations in the cystic fibrosis transmembrane conductance regulator gene, was identified. This progress has considerably changed our understanding of the pathophysiology of CF and has paved the way for the development of novel and specific therapies for the disease. The CFTR gene contains 27 exons and is characterized by a frequent three base pair deletion of the p.Phe508del. As a result of collaborative work, today more than 2000 mutations have been reported in the gene, and their impact on protein function is now more evident and useful in designing new strategies to correct the gene defect. The field of gene therapy, as illustrated by Ziying Yan in this book, has worked on identifying an efficient vector system for the delivery of the wild-type CFTR gene to the lung. At the same time, animal models have been developed in mice, rats, rabbits, zebrafish, ferrets, and pigs to establish the efficacity of gene delivery. These animals are also of the utmost importance in testing new molecules as modulators or correctors to improve the CFTR lung function. During the last three decades, the epidemiology of CF has dramatically changed, as today cystic fibrosis is now a chronic adult pulmonary disease.
Author: Scotty McGlothlin Buff
Publisher:
ISBN: 9780549012481
Category :
Languages : en
Pages : 241
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Book Description
These studies support that inflammation is excessive to bacterial infection in CF and demonstrate AAV-based IL-10 gene transfer is a promising anti-inflammatory therapeutic for CF lung disease.
Author: Jun Zhang
Publisher: Springer Nature
ISBN: 3030430405
Category : Medical
Languages : en
Pages : 110
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Book Description
This book is a guide to new and emerging PET technology, instrumentation, and its place in clinical practice. PET technology is currently moving from the conventional photomultiplier tube (PMT) detector based PET to the new generation, solid state light sensor detector. This is a technological leap and holds significant implications for the use of PET imaging. This book introduces and describes the emerging and new generation of PET instrumentations and technologies across manufactures, focusing on solid-state PET detector designs, system characteristics, and clinical practices as well as future advanced Time-of-Flight (TOF) PET technologies. Organized into three sections, the basics of PET imaging; solid state digital PET instrumentation, technology, and clinical practice; and a look to the future of PET imaging, chapters present a full picture of PET imaging, where we are and where we will be. Nuclear medicine physicians, physicists, and technologists can use this book to better understand future PET systems, novel PET technologies, and potential game changes of clinical PET practice.
