16 resultados para "Policy, legislation and regulation".
Filtro por publicador
- JISC Information Environment Repository (3)
- KUPS-Datenbank - Universität zu Köln - Kölner UniversitätsPublikationsServer (1)
- Repository Napier (1)
- Aberdeen University (3)
- Aberystwyth University Repository - Reino Unido (2)
- Academic Archive On-line (Stockholm University; Sweden) (1)
- Academic Research Repository at Institute of Developing Economies (3)
- Acceda, el repositorio institucional de la Universidad de Las Palmas de Gran Canaria. España (1)
- AMS Tesi di Dottorato - Alm@DL - Università di Bologna (6)
- AMS Tesi di Laurea - Alm@DL - Università di Bologna (1)
- Andina Digital - Repositorio UASB-Digital - Universidade Andina Simón Bolívar (1)
- Aquatic Commons (10)
- ArchiMeD - Elektronische Publikationen der Universität Mainz - Alemanha (1)
- Archive of European Integration (76)
- Aston University Research Archive (23)
- Biblioteca Digital da Produção Intelectual da Universidade de São Paulo (3)
- Biblioteca Digital da Produção Intelectual da Universidade de São Paulo (BDPI/USP) (2)
- Biblioteca Digital de la Universidad Católica Argentina (1)
- BORIS: Bern Open Repository and Information System - Berna - Suiça (43)
- Brock University, Canada (4)
- Bulgarian Digital Mathematics Library at IMI-BAS (1)
- CaltechTHESIS (4)
- Cambridge University Engineering Department Publications Database (1)
- CentAUR: Central Archive University of Reading - UK (46)
- Central European University - Research Support Scheme (1)
- Chinese Academy of Sciences Institutional Repositories Grid Portal (5)
- Cochin University of Science & Technology (CUSAT), India (2)
- Coffee Science - Universidade Federal de Lavras (1)
- Comissão Econômica para a América Latina e o Caribe (CEPAL) (44)
- CORA - Cork Open Research Archive - University College Cork - Ireland (7)
- Cornell: DigitalCommons@ILR (1)
- Corvinus Research Archive - The institutional repository for the Corvinus University of Budapest (4)
- Dalarna University College Electronic Archive (2)
- Department of Computer Science E-Repository - King's College London, Strand, London (1)
- DI-fusion - The institutional repository of Université Libre de Bruxelles (2)
- Digital Commons - Michigan Tech (2)
- Digital Commons @ Center for the Blue Economy - Middlebury Institute of International Studies at Monterey (1)
- Digital Commons @ DU | University of Denver Research (2)
- Digital Commons at Florida International University (6)
- Digital Peer Publishing (1)
- DigitalCommons@The Texas Medical Center (24)
- DigitalCommons@University of Nebraska - Lincoln (1)
- DRUM (Digital Repository at the University of Maryland) (2)
- Duke University (6)
- Düsseldorfer Dokumenten- und Publikationsservice (1)
- eResearch Archive - Queensland Department of Agriculture; Fisheries and Forestry (1)
- Greenwich Academic Literature Archive - UK (1)
- Helda - Digital Repository of University of Helsinki (27)
- Indian Institute of Science - Bangalore - Índia (8)
- Memoria Académica - FaHCE, UNLP - Argentina (3)
- Ministerio de Cultura, Spain (2)
- National Center for Biotechnology Information - NCBI (16)
- Nottingham eTheses (1)
- Plymouth Marine Science Electronic Archive (PlyMSEA) (9)
- Portal de Revistas Científicas Complutenses - Espanha (2)
- QSpace: Queen's University - Canada (3)
- QUB Research Portal - Research Directory and Institutional Repository for Queen's University Belfast (85)
- Queensland University of Technology - ePrints Archive (134)
- Repositório Científico do Instituto Politécnico de Lisboa - Portugal (1)
- Repositório digital da Fundação Getúlio Vargas - FGV (9)
- Repositório Institucional UNESP - Universidade Estadual Paulista "Julio de Mesquita Filho" (11)
- Research Open Access Repository of the University of East London. (1)
- RUN (Repositório da Universidade Nova de Lisboa) - FCT (Faculdade de Cienecias e Technologia), Universidade Nova de Lisboa (UNL), Portugal (3)
- SAPIENTIA - Universidade do Algarve - Portugal (2)
- South Carolina State Documents Depository (1)
- Universidad de Alicante (1)
- Universidad del Rosario, Colombia (4)
- Universidad Politécnica de Madrid (3)
- Universidade de Lisboa - Repositório Aberto (2)
- Universidade Federal do Pará (1)
- Universitat de Girona, Spain (1)
- Universitätsbibliothek Kassel, Universität Kassel, Germany (2)
- Université de Lausanne, Switzerland (3)
- Université de Montréal (2)
- Université de Montréal, Canada (7)
- University of Canberra Research Repository - Australia (1)
- University of Michigan (65)
- University of Queensland eSpace - Australia (33)
- University of Washington (4)
- WestminsterResearch - UK (12)
Resumo:
Cystic fibrosis (CF), a disorder of electrolyte transport manifest in the lungs, pancreas, sweat duct, and vas deferens, is caused by mutations in the CF transmembrane conductance regulator (CFTR). The CFTR protein has been shown to function as a cAMP-activated chloride channel and also regulates a separate protein, the outwardly rectifying chloride channel (ORCC). To determine the consequence of disease-producing mutations upon these functions, mutant CFTR was transiently expressed in Xenopus oocytes and in human airway epithelial cells lacking functional CFTR. Both G551D, a mutation that causes severe lung disease, and A455E, a mutation associated with mild lung disease, altered but did not abolish CFTR's function as a chloride channel in Xenopus oocytes. Airway epithelial cells transfected with CFTR bearing either A455E or G551D had levels of chloride conductance significantly greater than those of mock-transfected and lower than those of wild-type CFTR-transfected cells, as measured by chloride efflux. A combination of channel blockers and analysis of current-voltage relationships were used to dissect the contribution of CFTR and the ORCC to whole cell currents of transfected cells. While CFTR bearing either mutation could function as a chloride channel, only CFTR bearing A455E retained the function of regulating the ORCC. These results indicate that CF mutations can affect CFTR functions differently and suggest that severity of pulmonary disease may be more closely associated with the regulatory rather than chloride channel function of CFTR.