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Measurements of CFTR-Mediated Cl(−) Secretion in Human Rectal Biopsies Constitute a Robust Biomarker for Cystic Fibrosis Diagnosis and Prognosis

BACKGROUND: Cystic Fibrosis (CF) is caused by ∼1,900 mutations in the CF transmembrane conductance regulator (CFTR) gene encoding for a cAMP-regulated chloride (Cl(−)) channel expressed in several epithelia. Clinical features are dominated by respiratory symptoms, but there is variable organ involve...

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Detalles Bibliográficos
Autores principales: Sousa, Marisa, Servidoni, Maria F., Vinagre, Adriana M., Ramalho, Anabela S., Bonadia, Luciana C., Felício, Verónica, Ribeiro, Maria A., Uliyakina, Inna, Marson, Fernando A., Kmit, Arthur, Cardoso, Silvia R., Ribeiro, José D., Bertuzzo, Carmen S., Sousa, Lisete, Kunzelmann, Karl, Ribeiro, Antônio F., Amaral, Margarida D.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2012
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3474728/
https://www.ncbi.nlm.nih.gov/pubmed/23082198
http://dx.doi.org/10.1371/journal.pone.0047708