Consequences of cystic fibrosis transmembrane regulator mutations on inflammatory cells

Autores
Grumelli, Sandra; Islan, German Abel; Castro, Guillermo Raul
Año de publicación
2016
Idioma
inglés
Tipo de recurso
artículo
Estado
versión publicada
Descripción
Recent studies in cystic fibrosis (CF) transmembrane regulator (CFTR) mutations and function have shed light on its involvement in disease progression. The extent of cell and tissue distribution of CFTR facilitates systemic dysfunction of ion transport in patients carrying a mutation in CFTR, however, its incidences as cofounding risk factor to develop other diseases is not well studied. In this review we differentiate the dysfunctions driven by CFTR mutations in cell of the immune system and their role in CF progression and examine the types of medical treatments available to patients up to date.
Fil: Grumelli, Sandra. Universidad Católica de Córdoba; Argentina
Fil: Islan, German Abel. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico Conicet - La Plata. Centro de Investigación y Desarrollo en Fermentaciones Industriales. Universidad Nacional de La Plata. Facultad de Ciencias Exactas. Centro de Investigación y Desarrollo en Fermentaciones Industriales; Argentina
Fil: Castro, Guillermo Raul. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico Conicet - La Plata. Centro de Investigación y Desarrollo en Fermentaciones Industriales. Universidad Nacional de La Plata. Facultad de Ciencias Exactas. Centro de Investigación y Desarrollo en Fermentaciones Industriales; Argentina
Materia
cystic fibrosis
CFTR-deficiency
CF-immune response
antibiotic therapies
microbioma maintenance
gene therapy
Nivel de accesibilidad
acceso abierto
Condiciones de uso
https://creativecommons.org/licenses/by-nc-sa/2.5/ar/
Repositorio
CONICET Digital (CONICET)
Institución
Consejo Nacional de Investigaciones Científicas y Técnicas
OAI Identificador
oai:ri.conicet.gov.ar:11336/49503
Acceder
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oai_identifier_str oai:ri.conicet.gov.ar:11336/49503
network_acronym_str CONICETDig
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network_name_str CONICET Digital (CONICET)
spelling Consequences of cystic fibrosis transmembrane regulator mutations on inflammatory cellsGrumelli, SandraIslan, German AbelCastro, Guillermo Raulcystic fibrosisCFTR-deficiencyCF-immune responseantibiotic therapiesmicrobioma maintenancegene therapyhttps://purl.org/becyt/ford/3.4https://purl.org/becyt/ford/3Recent studies in cystic fibrosis (CF) transmembrane regulator (CFTR) mutations and function have shed light on its involvement in disease progression. The extent of cell and tissue distribution of CFTR facilitates systemic dysfunction of ion transport in patients carrying a mutation in CFTR, however, its incidences as cofounding risk factor to develop other diseases is not well studied. In this review we differentiate the dysfunctions driven by CFTR mutations in cell of the immune system and their role in CF progression and examine the types of medical treatments available to patients up to date.Fil: Grumelli, Sandra. Universidad Católica de Córdoba; ArgentinaFil: Islan, German Abel. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico Conicet - La Plata. Centro de Investigación y Desarrollo en Fermentaciones Industriales. Universidad Nacional de La Plata. Facultad de Ciencias Exactas. Centro de Investigación y Desarrollo en Fermentaciones Industriales; ArgentinaFil: Castro, Guillermo Raul. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico Conicet - La Plata. Centro de Investigación y Desarrollo en Fermentaciones Industriales. Universidad Nacional de La Plata. Facultad de Ciencias Exactas. Centro de Investigación y Desarrollo en Fermentaciones Industriales; ArgentinaOAT2016-08info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionhttp://purl.org/coar/resource_type/c_6501info:ar-repo/semantics/articuloapplication/pdfapplication/pdfhttp://hdl.handle.net/11336/49503Grumelli, Sandra; Islan, German Abel; Castro, Guillermo Raul; Consequences of cystic fibrosis transmembrane regulator mutations on inflammatory cells; OAT; Pulmonary and Critical Care Medicine; 1; 2; 8-2016; 39-512398-3108CONICET DigitalCONICETenginfo:eu-repo/semantics/altIdentifier/doi/10.15761/PCCM.1000110info:eu-repo/semantics/altIdentifier/url/http://oatext.com/Consequences-of-cystic-fibrosis-transmembrane-regulator-mutations-on-inflammatory-cells.phpinfo:eu-repo/semantics/openAccesshttps://creativecommons.org/licenses/by-nc-sa/2.5/ar/reponame:CONICET Digital (CONICET)instname:Consejo Nacional de Investigaciones Científicas y Técnicas2025-09-10T13:10:24Zoai:ri.conicet.gov.ar:11336/49503instacron:CONICETInstitucionalhttp://ri.conicet.gov.ar/Organismo científico-tecnológicoNo correspondehttp://ri.conicet.gov.ar/oai/requestdasensio@conicet.gov.ar; lcarlino@conicet.gov.arArgentinaNo correspondeNo correspondeNo correspondeopendoar:34982025-09-10 13:10:24.758CONICET Digital (CONICET) - Consejo Nacional de Investigaciones Científicas y Técnicasfalse
dc.title.none.fl_str_mv Consequences of cystic fibrosis transmembrane regulator mutations on inflammatory cells
title Consequences of cystic fibrosis transmembrane regulator mutations on inflammatory cells
spellingShingle Consequences of cystic fibrosis transmembrane regulator mutations on inflammatory cells
Grumelli, Sandra
cystic fibrosis
CFTR-deficiency
CF-immune response
antibiotic therapies
microbioma maintenance
gene therapy
title_short Consequences of cystic fibrosis transmembrane regulator mutations on inflammatory cells
title_full Consequences of cystic fibrosis transmembrane regulator mutations on inflammatory cells
title_fullStr Consequences of cystic fibrosis transmembrane regulator mutations on inflammatory cells
title_full_unstemmed Consequences of cystic fibrosis transmembrane regulator mutations on inflammatory cells
title_sort Consequences of cystic fibrosis transmembrane regulator mutations on inflammatory cells
dc.creator.none.fl_str_mv Grumelli, Sandra
Islan, German Abel
Castro, Guillermo Raul
author Grumelli, Sandra
author_facet Grumelli, Sandra
Islan, German Abel
Castro, Guillermo Raul
author_role author
author2 Islan, German Abel
Castro, Guillermo Raul
author2_role author
author
dc.subject.none.fl_str_mv cystic fibrosis
CFTR-deficiency
CF-immune response
antibiotic therapies
microbioma maintenance
gene therapy
topic cystic fibrosis
CFTR-deficiency
CF-immune response
antibiotic therapies
microbioma maintenance
gene therapy
purl_subject.fl_str_mv https://purl.org/becyt/ford/3.4
https://purl.org/becyt/ford/3
dc.description.none.fl_txt_mv Recent studies in cystic fibrosis (CF) transmembrane regulator (CFTR) mutations and function have shed light on its involvement in disease progression. The extent of cell and tissue distribution of CFTR facilitates systemic dysfunction of ion transport in patients carrying a mutation in CFTR, however, its incidences as cofounding risk factor to develop other diseases is not well studied. In this review we differentiate the dysfunctions driven by CFTR mutations in cell of the immune system and their role in CF progression and examine the types of medical treatments available to patients up to date.
Fil: Grumelli, Sandra. Universidad Católica de Córdoba; Argentina
Fil: Islan, German Abel. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico Conicet - La Plata. Centro de Investigación y Desarrollo en Fermentaciones Industriales. Universidad Nacional de La Plata. Facultad de Ciencias Exactas. Centro de Investigación y Desarrollo en Fermentaciones Industriales; Argentina
Fil: Castro, Guillermo Raul. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico Conicet - La Plata. Centro de Investigación y Desarrollo en Fermentaciones Industriales. Universidad Nacional de La Plata. Facultad de Ciencias Exactas. Centro de Investigación y Desarrollo en Fermentaciones Industriales; Argentina
description Recent studies in cystic fibrosis (CF) transmembrane regulator (CFTR) mutations and function have shed light on its involvement in disease progression. The extent of cell and tissue distribution of CFTR facilitates systemic dysfunction of ion transport in patients carrying a mutation in CFTR, however, its incidences as cofounding risk factor to develop other diseases is not well studied. In this review we differentiate the dysfunctions driven by CFTR mutations in cell of the immune system and their role in CF progression and examine the types of medical treatments available to patients up to date.
publishDate 2016
dc.date.none.fl_str_mv 2016-08
dc.type.none.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
http://purl.org/coar/resource_type/c_6501
info:ar-repo/semantics/articulo
format article
status_str publishedVersion
dc.identifier.none.fl_str_mv http://hdl.handle.net/11336/49503
Grumelli, Sandra; Islan, German Abel; Castro, Guillermo Raul; Consequences of cystic fibrosis transmembrane regulator mutations on inflammatory cells; OAT; Pulmonary and Critical Care Medicine; 1; 2; 8-2016; 39-51
2398-3108
CONICET Digital
CONICET
url http://hdl.handle.net/11336/49503
identifier_str_mv Grumelli, Sandra; Islan, German Abel; Castro, Guillermo Raul; Consequences of cystic fibrosis transmembrane regulator mutations on inflammatory cells; OAT; Pulmonary and Critical Care Medicine; 1; 2; 8-2016; 39-51
2398-3108
CONICET Digital
CONICET
dc.language.none.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv info:eu-repo/semantics/altIdentifier/doi/10.15761/PCCM.1000110
info:eu-repo/semantics/altIdentifier/url/http://oatext.com/Consequences-of-cystic-fibrosis-transmembrane-regulator-mutations-on-inflammatory-cells.php
dc.rights.none.fl_str_mv info:eu-repo/semantics/openAccess
https://creativecommons.org/licenses/by-nc-sa/2.5/ar/
eu_rights_str_mv openAccess
rights_invalid_str_mv https://creativecommons.org/licenses/by-nc-sa/2.5/ar/
dc.format.none.fl_str_mv application/pdf
application/pdf
dc.publisher.none.fl_str_mv OAT
publisher.none.fl_str_mv OAT
dc.source.none.fl_str_mv reponame:CONICET Digital (CONICET)
instname:Consejo Nacional de Investigaciones Científicas y Técnicas
reponame_str CONICET Digital (CONICET)
collection CONICET Digital (CONICET)
instname_str Consejo Nacional de Investigaciones Científicas y Técnicas
repository.name.fl_str_mv CONICET Digital (CONICET) - Consejo Nacional de Investigaciones Científicas y Técnicas
repository.mail.fl_str_mv dasensio@conicet.gov.ar; lcarlino@conicet.gov.ar
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score 12.993085

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