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Origins of Cystic Fibrosis Airway Disease

Origins of Cystic Fibrosis Airway Disease
囊性纤维化气道疾病的起源
批准号:
10470203
负责人:
DAVID A STOLTZ
金额:
$229.33万
依托单位:
依托单位国家:
美国
项目类别:
财政年份:
2008
资助国家:
美国
项目状态:
已结题
起止时间:
2008-09-05 至 2024-06-30

项目摘要

项目成果

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中文摘要
翻译
CFTR基因被发现28年后,导致气道囊性纤维化(CF)的原因
英文摘要
Twenty-eight years after the discovery of the CFTR gene, the causes of cystic fibrosis (CF) airway disease remain controversial, we still lack answers to many critical questions, our therapies are inadequate, and CF remains a life limiting and too often lethal disease. A major impediment to progress has been lack of CF animal models with a lung disease phenotype resembling humans with CF. We developed pigs with targeted alterations of the CFTR gene. CF pigs spontaneously develop the hallmark features of CF lung disease, including airway infection, inflammation, airway wall remodeling, mucus accumulation, and airway obstruction. Within hours of birth, CF pigs fail to eradicate bacteria as effectively as wild-type pigs. At least two host defense defects, reduced antimicrobial activity in airway surface liquid (ASL) and impaired mucociliary transport (MCT) contribute. Both were caused by abnormally acidic airway liquid. In this Program three highly accomplished investigators will use CF pigs to answer fundamental questions about CF lung disease. Together, the three projects will discover how loss of CFTR function affects: a) submucosal gland function, the properties of mucus, and MCT; b) ASL pH and proton secretion via ATP12A; and, c) small airways function, a likely site of CF disease pathogenesis. The Project Leaders and their teams have an outstanding track record of collaborative CF research, and here they sharpen their focus to a common goal. Their highly creative research is supported by five cores that provide innovative infrastructure and services. By further educating CF pathogenesis, it will accelerate discovery of novel therapies for this lethal disease.
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DOI: 10.1016/j.jcf.2014.02.004
发表时间: 2014-09
期刊: Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
影响因子: --
作者: [Reznikov LR, Abou Alaiwa MH, Dohrn CL, Gansemer ND, Diekema DJ, Stoltz DA, Welsh MJ]
通讯作者: Welsh MJ
Cystic fibrosis transmembrane conductance regulator with a shortened R domain rescues the intestinal phenotype of CFTR-/- mice.
具有缩短的 R 结构域的囊性纤维化跨膜电导调节剂可挽救 CFTR-/- 小鼠的肠道表型。
DOI: 10.1073/pnas.1019752108
发表时间: 2011
期刊: Proceedings of the National Academy of Sciences of the United States of America
影响因子: 11.1
作者: [Ostedgaard,LyndaS, Meyerholz,DavidK, Vermeer,DanielW, Karp,PhilipH, Schneider,Lindsey, Sigmund,CurtD, Welsh,MichaelJ]
通讯作者: Welsh,MichaelJ
CFTR-deficient pigs display peripheral nervous system defects at birth.
CFTR 缺陷的猪在出生时表现出周围神经系统缺陷。
DOI: 10.1073/pnas.1222729110
发表时间: 2013
期刊: Proceedings of the National Academy of Sciences of the United States of America
影响因子: 11.1
作者: [Reznikov,LeahR, Dong,Qian, Chen,Jeng-Haur, Moninger,ThomasO, Park,JungMin, Zhang,Yuzhou, Du,Jianyang, Hildebrand,MichaelS, Smith,RichardJH, Randak,ChristophO, Stoltz,DavidA, Welsh,MichaelJ]
通讯作者: Welsh,MichaelJ
DOI: 10.1016/j.jcf.2022.08.019
发表时间: 2023-03
期刊: JOURNAL OF CYSTIC FIBROSIS
影响因子: 5.2
作者: [Ehre, Camille, Hansson, Gunnar C., Thornton, David J., Ostedgaard, Lynda S.]
通讯作者: Ostedgaard, Lynda S.
共 53 条
    Climate Change and Lung Health Training Program
    • 批准号:
      10556149
    • 项目类别:
    • 资助金额:
      $28.85万
    • 财政年份:
      2023
    • 负责人:
      DAVID A STOLTZ
    • 依托单位:
    Testing the Contributions of Airway Submucosal Glands and Surface Epithelia to Lung Health
    • 批准号:
      10597111
    • 项目类别:
    • 资助金额:
      $62.95万
    • 财政年份:
      2022
    • 负责人:
      DAVID A STOLTZ
    • 依托单位:
    Animal Models Core
    • 批准号:
      10677590
    • 项目类别:
    • 资助金额:
      $59.97万
    • 财政年份:
      2020
    • 负责人:
      DAVID A STOLTZ
    • 依托单位:
    Animal Models Core
    • 批准号:
      10024664
    • 项目类别:
    • 资助金额:
      $61.06万
    • 财政年份:
      2020
    • 负责人:
      DAVID A STOLTZ
    • 依托单位:
    海外基金