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CFTR and Cystic Fibrosis
Titre de l'éditeur : CFTR and Cystic Fibrosis
CARLOS M. FARINHA
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EN SAVOIR PLUS
Résumé
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 defect, most of which aim at repairing CFTR dysfunction, are covered. Through this integrated perspective, readers will obtain a unique insight into this fascinating membrane-bound protein and its associated disease. This Brief appeals to an audience interested in human genetics, protein folding, protein trafficking and physiology.
Détails
Prix :
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74,69 $
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Catégorie :
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Auteur :
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CARLOS M. FARINHA
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Titre :
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CFTR and Cystic Fibrosis
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Date de parution :
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octobre 2017
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Éditeur :
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LIVRES NUMÉRIQUES DIVERS
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Sujet :
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NUL DIVERS
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ISBN :
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9783319654942 (3319654942)
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Référence Renaud-Bray :
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2399331
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No de produit :
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2399331
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Droits numériques
Format :
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PDF
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Disponibilité :
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Canada,
consultez la liste des pays autorisés.
CFTR and Cystic Fibrosis
De
FARINHA , CARLOS M.
ANDORRE
ÉMIRATS ARABES UNIS
ARMÉNIE
ANTARCTIQUE
ARGENTINE
SAMOA AMÉRICAINES
AUTRICHE
AUSTRALIE
BOSNIE-HERZÉGOVINE
BARBADE
BELGIQUE
BULGARIE
BAHREÏN
BERMUDES
BRUNÉI DARUSSALAM
BOLIVIE
BRÉSIL
BAHAMAS
BOUVET, ÎLE
BELIZE
Canada
SUISSE
COOK, ÎLES
CHILI
CHINE
COLOMBIE
COSTA RICA
CUBA
CHRISTMAS, ÎLE
CHYPRE
TCHÈQUE, RÉPUBLIQUE
ALLEMAGNE
DANEMARK
DOMINIQUE
DOMINICAINE, RÉPUBLIQUE
ALGÉRIE
ÉQUATEUR
ESTONIE
ESPAGNE
FINLANDE
FALKLAND, ÎLES (MALVINAS)
FÉROÉ, ÎLES
FRANCE
GRENADE
GUYANE FRANÇAISE
GIBRALTAR
GROENLAND
GUADELOUPE
GRÈCE
GUATEMALA
GUYANA
HONG-KONG
HEARD, ÎLE ET MCDONALD, ÎLES
HONDURAS
CROATIE
HAÏTI
HONGRIE
INDONÉSIE
IRLANDE
ISRAËL
INDE
OCÉAN INDIEN, TERRITOIRE BRITANNIQUE DE
IRAN, RÉPUBLIQUE ISLAMIQUE D'
ISLANDE
ITALIE
JAMAÏQUE
JAPON
CAMBODGE
COMORES
SAINT-KITTS-ET-NEVIS
CORÉE, RÉPUBLIQUE DE
CAÏMANES, ÎLES
LAO, RÉPUBLIQUE DÉMOCRATIQUE POPULAIRE
LIBAN
SAINTE-LUCIE
LIECHTENSTEIN
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Gestion des droits numériques :
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Adobe DRM
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Entrepôt numérique :
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NUMILOG
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Nombre d'appareils autorisés :
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3
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CFTR and Cystic Fibrosis
,
FARINHA , CARLOS M.
©
LIVRES NUMÉRIQUES DIVERS
2017
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