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dc.contributor.authorBáez Pagán, Carlos A.
dc.contributor.authorMartínez Ortiz, Yaiza
dc.contributor.authorOtero Cruz, José D.
dc.contributor.authorSalgado Villanueva, Iris K.
dc.contributor.authorVelázquez, Germarie
dc.contributor.authorOrtiz Acevedo, Alejandro
dc.contributor.authorQuesada, Orestes
dc.contributor.authorSilva, Walter I.
dc.contributor.authorLasalde Dominicci, José A.
dc.identifier.citationBáez-Pagán CA, Martínez-Ortiz Y, Otero-Cruz JD, Salgado-Villanueva IK, Velázquez G, Ortiz-Acevedo A, Quesada O, Silva WI, Lasalde-Dominicci JA. Potential role of caveolin-1-positive domains in the regulation of the acetylcholine receptor's activatable pool: implications in the pathogenesis of a novel congenital myasthenic syndrome. Channels (Austin). 2008 May-Jun;2(3):180-90. doi: 10.4161/chan.2.3.6155. Epub 2008 May 18. PMID: 18836288; PMCID: PMC4495657.en_US
dc.description.abstractCholesterol modulates the plasmalemma’s biophysical properties and influences the function and trafficking of membrane proteins. A fundamental phenomenon that remains obscure is how the plasmalemma’s lipid composition regulates the activatable pool of membrane receptors. An outstanding model to study this phenomenon is the nicotinic acetylcholine receptor (nAChR), since the nAChR activatable pool has been estimated to be but a small fraction of the receptors present in the plasmalemma. Studies on the effect of cholesterol depletion in the function of the Torpedo californica nAChR, using the lipid-exposed nAChR mutation (αC418W) that produces a congenital myasthenic syndrome (CMS), demonstrated that cholesterol depletion causes a remarkable increase in the αC418W nAChR’s macroscopic current whereas not in the wild-type (WT). A variety of approaches were used to define the mechanism responsible for the cholesterol depletion mediated-increase in the αC418W nAChR’s macroscopic current. The present study suggests that a substantial fraction of the αC418W nAChRs is located in caveolin-1-positive domains, “trapped” in a non-activatable state, and that membrane cholesterol depletion results in the relocation of these receptors to the activatable pool. Co-fractionation and co-immunoprecipitation of the αC418W nAChR and the membrane raft protein caveolin-1 (cav1) support the notion that interactions at lipid-exposed domains regulate the partition of the receptor into membrane raft microdomains. These results have potential implications as a novel mechanism to fine-tune cholinergic transmission in the nervous system and in the pathogenesis associated to the αC418W nAChR.en_US
dc.publisherNational Library of Medicine. National Center for Biotechnology Informationen_US
dc.relation.ispartofseriesChannels (Austin) 2 (3)
dc.rightsAttribution-NonCommercial-NoDerivs 3.0 United States*
dc.subjectNicotinic acetylcholine receptoren_US
dc.subjectLipid-exposed transmembrane domainsen_US
dc.subjectCongenital Myasthenic syndromeen_US
dc.subjectMembrane raftsen_US
dc.subject.lcshMyasthenia gravisen_US
dc.subject.lcshCell membranesen_US
dc.subject.lcshLipid membranesen_US
dc.titlePotential role of caveolin-1-positive domains in the regulation of the acetylcholine receptor’s activatable pool : implications in the pathogenesis of a novel congenital myasthenic syndromeen_US
dc.title.alternativeImplications in the pathogenesis of a novel congenital myasthenic syndromeen_US
dc.rights.holder©2008, Channels (Austin)en_US
dc.contributor.campusUniversity of Puerto Rico, Río Piedras Campusen_US
dc.description.eventChannels (Austin) 2008. May-June ; 2(3) : 180-190en_US

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