Please use this identifier to cite or link to this item: https://repositorio.uca.edu.ar/handle/123456789/8676
Título : CFTR activity and mitochondrial function
Autor : Valdivieso, Ángel Gabriel 
Santa Coloma, Tomás Antonio 
Palabras clave : MEDICINAFIBROSIS QUISTICACFTRGENESENFERMEDADESFUNCION MITOCONDRIAL
Fecha de publicación : 2013
Editorial : Elsevier B.V.
Cita : Valdivieso AG, Santa Coloma TA. (2013) CFTR activity and mitochondrial function. Redox Biology 1(1). Disponible en: https://repositorio.uca.edu.ar/handle/123456789/8676
Resumen : Abstract: 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.
URI : https://repositorio.uca.edu.ar/handle/123456789/8676
ISSN : 2213-2317
Disciplina: MEDICINA
DOI: http://dx.doi.org/10.1016/j.redox.2012.11.007
Derechos: Acceso abierto
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