Extracellular pH and lung infections in cystic fibrosis

Abstract: Cystic fibrosis (CF) is an autosomal recessive disease caused by CFTR mutations. It is characterized by high NaCl concentration in sweat and the production of a thick and sticky mucus, occluding secretory ducts, intestine and airways, accompanied by chronic inflammation and infections of t...

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Autores principales: Massip Copiz, María Macarena, Santa Coloma, Tomás Antonio
Formato: Artículo
Lenguaje:Inglés
Publicado: Elsevier 2019
Materias:
GENES CFTR DEPENDIENTES
FIBROSIS QUISTICA
PH
PULMONES
ENFERMEDADES
INFECCIONES
Acceso en línea:https://repositorio.uca.edu.ar/handle/123456789/8916
Aporte de:
Repositorio Institucional de la Universidad Católica Argentina (UCA) de Universidad Católica Argentina
id I33-R139123456789-8916
record_format dspace
institution Universidad Católica Argentina
institution_str I-33
repository_str R-139
collection Repositorio Institucional de la Universidad Católica Argentina (UCA)
language Inglés
topic GENES CFTR DEPENDIENTES
FIBROSIS QUISTICA
PH
PULMONES
ENFERMEDADES
INFECCIONES
spellingShingle GENES CFTR DEPENDIENTES
FIBROSIS QUISTICA
PH
PULMONES
ENFERMEDADES
INFECCIONES
Massip Copiz, María Macarena
Santa Coloma, Tomás Antonio
Extracellular pH and lung infections in cystic fibrosis
topic_facet GENES CFTR DEPENDIENTES
FIBROSIS QUISTICA
PH
PULMONES
ENFERMEDADES
INFECCIONES
description Abstract: Cystic fibrosis (CF) is an autosomal recessive disease caused by CFTR mutations. It is characterized by high NaCl concentration in sweat and the production of a thick and sticky mucus, occluding secretory ducts, intestine and airways, accompanied by chronic inflammation and infections of the lungs. This causes a progressive and lethal decline in lung function. Therefore, finding the mechanisms driving the high susceptibility to lung infections has been a key issue. For decades the prevalent hypothesis was that a reduced airway surface liquid (ASL) volume and composition, and the consequent increased mucus concentration (dehydration), create an environment favoring infections. However, a few years ago, in a pig model of CF, the Na+/K+ concentrations and the ASL volume were found intact. Immediately a different hypothesis arose, postulating a reduced ASL pH as the cause for the increased susceptibility to infections, due to a diminished bicarbonate secretion through CFTR. Noteworthy, a recent report found normal ASL pH values in CF children and in cultured primary airway cells, challenging the ASL pH hypothesis. On the other hand, recent evidences revitalized the hypothesis of a reduced ASL secretion. Thus, the role of the ASL pH in the CF is still a controversial matter. In this review we discuss the basis that sustain the role of CFTR in modulating the extracellular pH, and the recent results sustaining the different points of view. Finding the mechanisms of CFTR signaling that determine the susceptibility to infections is crucial to understand the pathophysiology of CF and related lung diseases.
format Artículo
author Massip Copiz, María Macarena
Santa Coloma, Tomás Antonio
author_facet Massip Copiz, María Macarena
Santa Coloma, Tomás Antonio
author_sort Massip Copiz, María Macarena
title Extracellular pH and lung infections in cystic fibrosis
title_short Extracellular pH and lung infections in cystic fibrosis
title_full Extracellular pH and lung infections in cystic fibrosis
title_fullStr Extracellular pH and lung infections in cystic fibrosis
title_full_unstemmed Extracellular pH and lung infections in cystic fibrosis
title_sort extracellular ph and lung infections in cystic fibrosis
publisher Elsevier
publishDate 2019
url https://repositorio.uca.edu.ar/handle/123456789/8916
work_keys_str_mv AT massipcopizmariamacarena extracellularphandlunginfectionsincysticfibrosis
AT santacolomatomasantonio extracellularphandlunginfectionsincysticfibrosis
bdutipo_str Repositorios
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