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Título : | CFTR activity and mitochondrial function | Autor : | Valdivieso, Ángel Gabriel Santa Coloma, Tomás Antonio |
Palabras clave : | MEDICINA; FIBROSIS QUISTICA; CFTR; GENES; ENFERMEDADES; FUNCION 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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