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Titlebook: CFTR and Cystic Fibrosis; From Structure to Fu Carlos M.‘Farinha Book 2018 The Author(s) 2018 CFTR.cystic Fibrosis.protein folding.protein

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發(fā)表于 2025-3-21 18:54:28 | 只看該作者 |倒序?yàn)g覽 |閱讀模式
書目名稱CFTR and Cystic Fibrosis
副標(biāo)題From Structure to Fu
編輯Carlos M.‘Farinha
視頻videohttp://file.papertrans.cn/221/220264/220264.mp4
概述Discusses the relationship between the CFTR protein and cystic fibrosis.Explains how the protein structure and structure-defects affect the function of CFTR.Presents CFTR-based and independent therapi
叢書名稱SpringerBriefs in Molecular Science
圖書封面Titlebook: CFTR and Cystic Fibrosis; From Structure to Fu Carlos M.‘Farinha Book 2018 The Author(s) 2018 CFTR.cystic Fibrosis.protein folding.protein
描述.This Brief is devoted to the CFTR protein and cystic fibrosis, and it provides an updated perspective of the genetic, functional and cellular processes involved in this conformational disorder.?Starting with a historical perspective on cystic fibrosis and its clinical features, the author departs into an in-depth description of the biology of the CFTR protein, ending with a discussion on the latest approaches aimed at developing corrective therapies for cystic fibrosis..First the basic aspects of cystic fibrosis as a disorder are addressed, focusing on genetics and mutation prevalence. Then the CFTR protein is discussed in detail: its structure and classification within the ABC transporter superfamily, its biogenesis with membrane insertion and chaperone assisted folding, its glycosylation and how it regulates the endoplasmatic reticulum quality control mechanisms that assess CFTR folding status. Extra attention is given to post-ER trafficking and regulation of membrane stability and anchoring, and to CFTR functions. This is linked to the molecular mechanisms through which different CFTR mutations cause cystic fibrosis. Finally, the different efforts aiming at rescuing the basic d
出版日期Book 2018
關(guān)鍵詞CFTR; cystic Fibrosis; protein folding; protein trafficking; membrane proteins; ion channels; ABC transpor
版次1
doihttps://doi.org/10.1007/978-3-319-65494-2
isbn_softcover978-3-319-65493-5
isbn_ebook978-3-319-65494-2Series ISSN 2191-5407 Series E-ISSN 2191-5415
issn_series 2191-5407
copyrightThe Author(s) 2018
The information of publication is updating

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發(fā)表于 2025-3-21 23:26:28 | 只看該作者
SpringerBriefs in Molecular Sciencehttp://image.papertrans.cn/c/image/220264.jpg
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CFTR and Cystic Fibrosis,Cystic fibrosis (CF) is the most common autosomal recessive disorder in Caucasians and appears mostly in childhood. The first medical reports of CF symptoms date to the seventeenth century with descriptions of the meconium ileus but the condition was known previously with some medieval references such as the one that states: .
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2191-5407 function of CFTR.Presents CFTR-based and independent therapi.This Brief is devoted to the CFTR protein and cystic fibrosis, and it provides an updated perspective of the genetic, functional and cellular processes involved in this conformational disorder.?Starting with a historical perspective on cys
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發(fā)表于 2025-3-22 22:37:52 | 只看該作者
Book 2018es involved in this conformational disorder.?Starting with a historical perspective on cystic fibrosis and its clinical features, the author departs into an in-depth description of the biology of the CFTR protein, ending with a discussion on the latest approaches aimed at developing corrective thera
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Book 2018ng status. Extra attention is given to post-ER trafficking and regulation of membrane stability and anchoring, and to CFTR functions. This is linked to the molecular mechanisms through which different CFTR mutations cause cystic fibrosis. Finally, the different efforts aiming at rescuing the basic d
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