Please use this identifier to cite or link to this item: https://repositorio.uca.edu.ar/handle/123456789/8676
DC FieldValueLanguage
dc.contributor.authorValdivieso, Ángel Gabrieles
dc.contributor.authorSanta Coloma, Tomás Antonioes
dc.date.accessioned2019-09-03T15:39:49Z-
dc.date.available2019-09-03T15:39:49Z-
dc.date.issued2013-
dc.identifier.citationValdivieso AG, Santa Coloma TA. (2013) CFTR activity and mitochondrial function. Redox Biology 1(1). Disponible en: https://repositorio.uca.edu.ar/handle/123456789/8676es
dc.identifier.issn2213-2317-
dc.identifier.urihttps://repositorio.uca.edu.ar/handle/123456789/8676-
dc.description.abstractAbstract: Cystic Fibrosis(CF)isafrequentandlethalautosomalrecessivedisease,causedbymutationsinthe gene encodingtheCysticFibrosisTransmembraneConductanceRegulator(CFTR).Beforethediscovery of the CFTR gene, severalhypothesesattemptedtoexplaintheetiologyofthisdisease,includingthe possible roleofachloridechannel,diversealterationsinmitochondrialfunctions,theoverexpressionof the lysosomalenzyme a-glucosidaseandadeficiencyinthecytosolicenzymeglucose6-phosphate dehydrogenase.Becauseofthediversemitochondrialchangesfound,someauthorsproposedthatthe affectedgeneshouldcodifyforamitochondrialprotein.Later,theCFTRcloningandthedemonstration of itschloridechannelactivityturnedthemitochondrial,lysosomalandcytosolichypothesesobsolete. However,inrecentyears,usingnewapproaches,severalinvestigatorsreportedsimilarornew alterationsofmitochondrialfunctionsinCysticFibrosis,thusrediscoveringapossibleroleof mitochondriainthisdisease.Here,wereviewtheseCFTR-drivenmitochondrialdefects,including differentialgeneexpression,alterationsinoxidativephosphorylation,calciumhomeostasis,oxidative stress, apoptosisandinnateimmuneresponse,whichmightexplainsomecharacteristicsofthe complexCFphenotypeandrevealspotentialnewtargetsfortherapy.es
dc.formatapplication/pdf-
dc.language.isoenges
dc.publisherElsevier B.V.es
dc.rightsAcceso abierto*
dc.rights.urihttp://creativecommons.org/licenses/by-nc-sa/4.0/*
dc.sourceRedox Biology Vol. 1, N° 1, 2013es
dc.subjectMEDICINAes
dc.subjectFIBROSIS QUISTICAes
dc.subjectCFTRes
dc.subjectGENESes
dc.subjectENFERMEDADESes
dc.subjectFUNCION MITOCONDRIAL-
dc.titleCFTR activity and mitochondrial functiones
dc.typeArtículoes
dc.identifier.doihttp://dx.doi.org/10.1016/j.redox.2012.11.007-
uca.disciplinaMEDICINA-
uca.issnrd1es
uca.affiliationFil: Valdivieso, Ángel Gabriel. Pontificia Universidad Católica Argentina. Facultad de Ciencias Médicas. Instituto de Investigaciones Biomédicas. Laboratorio de Biología Celular y Molecular; Argentinaes
uca.affiliationFil: Santa Coloma, Tomás Antonio. Pontificia Universidad Católica Argentina. Facultad de Ciencias Médicas. Instituto de Investigaciones Biomédicas. Laboratorio de Biología Celular y Molecular; Argentinaes
uca.affiliationFil: Santa Coloma, Tomás Antonio. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentinaes
uca.versionpublishedVersiones
item.languageiso639-1en-
item.grantfulltextopen-
item.fulltextWith Fulltext-
crisitem.author.deptInstituto de Investigaciones Biomédicas - BIOMED-
crisitem.author.deptLaboratorio de Biología Celular y Molecular-
crisitem.author.deptConsejo Nacional de Investigaciones Científicas y Técnicas-
crisitem.author.deptInstituto de Investigaciones Biomédicas - BIOMED-
crisitem.author.deptLaboratorio de Biología Celular y Molecular-
crisitem.author.deptLaboratorio de Nanotecnología-
crisitem.author.deptConsejo Nacional de Investigaciones Científicas y Técnicas-
crisitem.author.orcid0000-0002-3266-1095-
crisitem.author.parentorgFacultad de Ciencias Médicas-
crisitem.author.parentorgInstituto de Investigaciones Biomédicas - BIOMED-
crisitem.author.parentorgFacultad de Ciencias Médicas-
crisitem.author.parentorgInstituto de Investigaciones Biomédicas - BIOMED-
crisitem.author.parentorgInstituto de Investigaciones Biomédicas - BIOMED-
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