Consequences of cystic fibrosis transmembrane regulator mutations on inflammatory cells

Autores
Grumelli, Sandra; Islan, Germán Abel; Castro, Guillermo Raúl
Año de publicación
2016
Idioma
inglés
Tipo de recurso
reseña 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.
Centro de Investigación y Desarrollo en Fermentaciones Industriales
Materia
Ciencias Médicas
Cystic fibrosis
CFTR-deficiency
CF-immune response
Antibiotic therapies
Microbioma maintenance
Gene therapy
Nivel de accesibilidad
acceso abierto
Condiciones de uso
http://creativecommons.org/licenses/by-nc-sa/4.0/
Repositorio
SEDICI (UNLP)
Institución
Universidad Nacional de La Plata
OAI Identificador
oai:sedici.unlp.edu.ar:10915/100520

id SEDICI_fe11c46d7eb1816e571a96b518801ea5
oai_identifier_str oai:sedici.unlp.edu.ar:10915/100520
network_acronym_str SEDICI
repository_id_str 1329
network_name_str SEDICI (UNLP)
spelling Consequences of cystic fibrosis transmembrane regulator mutations on inflammatory cellsGrumelli, SandraIslan, Germán AbelCastro, Guillermo RaúlCiencias MédicasCystic fibrosisCFTR-deficiencyCF-immune responseAntibiotic therapiesMicrobioma maintenanceGene therapyRecent 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.Centro de Investigación y Desarrollo en Fermentaciones Industriales2016-08info:eu-repo/semantics/reviewinfo:eu-repo/semantics/publishedVersionRevisionhttp://purl.org/coar/resource_type/c_dcae04bcinfo:ar-repo/semantics/resenaArticuloapplication/pdf39-51http://sedici.unlp.edu.ar/handle/10915/100520enginfo:eu-repo/semantics/altIdentifier/url/https://ri.conicet.gov.ar/11336/49503info:eu-repo/semantics/altIdentifier/url/http://oatext.com/Consequences-of-cystic-fibrosis-transmembrane-regulator-mutations-on-inflammatory-cells.phpinfo:eu-repo/semantics/altIdentifier/issn/2398-3108info:eu-repo/semantics/altIdentifier/doi/10.15761/PCCM.1000110info:eu-repo/semantics/altIdentifier/hdl/11336/49503info:eu-repo/semantics/openAccesshttp://creativecommons.org/licenses/by-nc-sa/4.0/Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International (CC BY-NC-SA 4.0)reponame:SEDICI (UNLP)instname:Universidad Nacional de La Platainstacron:UNLP2025-10-15T11:12:00Zoai:sedici.unlp.edu.ar:10915/100520Institucionalhttp://sedici.unlp.edu.ar/Universidad públicaNo correspondehttp://sedici.unlp.edu.ar/oai/snrdalira@sedici.unlp.edu.arArgentinaNo correspondeNo correspondeNo correspondeopendoar:13292025-10-15 11:12:00.776SEDICI (UNLP) - Universidad Nacional de La Platafalse
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
Ciencias Médicas
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, Germán Abel
Castro, Guillermo Raúl
author Grumelli, Sandra
author_facet Grumelli, Sandra
Islan, Germán Abel
Castro, Guillermo Raúl
author_role author
author2 Islan, Germán Abel
Castro, Guillermo Raúl
author2_role author
author
dc.subject.none.fl_str_mv Ciencias Médicas
Cystic fibrosis
CFTR-deficiency
CF-immune response
Antibiotic therapies
Microbioma maintenance
Gene therapy
topic Ciencias Médicas
Cystic fibrosis
CFTR-deficiency
CF-immune response
Antibiotic therapies
Microbioma maintenance
Gene therapy
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.
Centro de Investigación y Desarrollo en Fermentaciones Industriales
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/review
info:eu-repo/semantics/publishedVersion
Revision
http://purl.org/coar/resource_type/c_dcae04bc
info:ar-repo/semantics/resenaArticulo
format review
status_str publishedVersion
dc.identifier.none.fl_str_mv http://sedici.unlp.edu.ar/handle/10915/100520
url http://sedici.unlp.edu.ar/handle/10915/100520
dc.language.none.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv info:eu-repo/semantics/altIdentifier/url/https://ri.conicet.gov.ar/11336/49503
info:eu-repo/semantics/altIdentifier/url/http://oatext.com/Consequences-of-cystic-fibrosis-transmembrane-regulator-mutations-on-inflammatory-cells.php
info:eu-repo/semantics/altIdentifier/issn/2398-3108
info:eu-repo/semantics/altIdentifier/doi/10.15761/PCCM.1000110
info:eu-repo/semantics/altIdentifier/hdl/11336/49503
dc.rights.none.fl_str_mv info:eu-repo/semantics/openAccess
http://creativecommons.org/licenses/by-nc-sa/4.0/
Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International (CC BY-NC-SA 4.0)
eu_rights_str_mv openAccess
rights_invalid_str_mv http://creativecommons.org/licenses/by-nc-sa/4.0/
Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International (CC BY-NC-SA 4.0)
dc.format.none.fl_str_mv application/pdf
39-51
dc.source.none.fl_str_mv reponame:SEDICI (UNLP)
instname:Universidad Nacional de La Plata
instacron:UNLP
reponame_str SEDICI (UNLP)
collection SEDICI (UNLP)
instname_str Universidad Nacional de La Plata
instacron_str UNLP
institution UNLP
repository.name.fl_str_mv SEDICI (UNLP) - Universidad Nacional de La Plata
repository.mail.fl_str_mv alira@sedici.unlp.edu.ar
_version_ 1846064177497505792
score 13.22299