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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...
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. |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Public Library of Science
2012
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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 |
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