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Developmental Changes of ENaC Expression and Function in the Inner Ear of Pendrin Knock-Out Mice as a Perspective on the Development of Endolymphatic Hydrops

DC FieldValueLanguage
dc.contributor.author김보경-
dc.contributor.author김성헌-
dc.contributor.author김진영-
dc.contributor.author김희남-
dc.contributor.author복진웅-
dc.contributor.author최재영-
dc.date.accessioned2015-01-06T16:40:46Z-
dc.date.available2015-01-06T16:40:46Z-
dc.date.issued2014-
dc.identifier.urihttps://ir.ymlib.yonsei.ac.kr/handle/22282913/98556-
dc.description.abstractPendrin mutations cause enlarged vestibular aqueducts and various degrees of sensorineural hearing loss. The selective abolition of pendrin causes dilation of the membranous labyrinth known as endolymphatic hydrops, loss of the endocochlear potential, and consequently loss of hearing function. Because Na+ transport is one of the most important driving forces for fluid transport, the epithelial Na+ channel (ENaC) is believed to play an important role in fluid volume regulation in the inner ear. Therefore, the dysfunction of Na+ transport through ENaC by the acidification of endolymph in Pendred syndrome is one of the potential causes of endolymphatic hydrops. We investigated the changes of ENaC expression and function during the development of the pendrin knock-out mouse. In the cochlea, the expression of β and γENaC was significantly increased at P56 in Pds-/- mice compared with Pds+/+ mice. In the vestibule, the expression of βENaC was significantly increased at P56, and γENaC expression significantly increased from P6 to P56 in Pds-/- mice. The ENaC-dependent trans-epithelial current was not significantly different between Pds+/+ and Pds-/- mice in Reissner's membrane or the saccular extramacular roof epithelium at P0, but the current was significantly increased in Pds-/- mice at P56 compared with Pds+/+ mice. These findings indicate that the expression and function of ENaC were enhanced in Pds-/- mice after the development of endolymphatic hydrops as a compensatory mechanism. This result provides insight into the role of Na+ transport in the development and regulation of endolymphatic hydrops due to pendrin mutations.-
dc.description.statementOfResponsibilityopen-
dc.format.extente95730-
dc.relation.isPartOfPLOS ONE-
dc.rightsCC BY-NC-ND 2.0 KR-
dc.rights.urihttps://creativecommons.org/licenses/by-nc-nd/2.0/kr/-
dc.subject.MESHAnimals-
dc.subject.MESHAnion Transport Proteins/genetics-
dc.subject.MESHAnion Transport Proteins/metabolism*-
dc.subject.MESHEar, Inner/metabolism*-
dc.subject.MESHEndolymphatic Hydrops/genetics-
dc.subject.MESHEndolymphatic Hydrops/metabolism*-
dc.subject.MESHEpithelial Sodium Channels/genetics-
dc.subject.MESHEpithelial Sodium Channels/metabolism*-
dc.subject.MESHHomozygote-
dc.subject.MESHMice-
dc.subject.MESHMice, Knockout-
dc.subject.MESHMutation/genetics-
dc.subject.MESHReverse Transcriptase Polymerase Chain Reaction-
dc.titleDevelopmental Changes of ENaC Expression and Function in the Inner Ear of Pendrin Knock-Out Mice as a Perspective on the Development of Endolymphatic Hydrops-
dc.typeArticle-
dc.contributor.collegeCollege of Medicine (의과대학)-
dc.contributor.departmentYonsei Biomedical Research Center (연세의생명연구원)-
dc.contributor.googleauthorBo Gyung Kim-
dc.contributor.googleauthorJin Young Kim-
dc.contributor.googleauthorHee Nam Kim-
dc.contributor.googleauthorJinwoong Bok-
dc.contributor.googleauthorWan Namkung-
dc.contributor.googleauthorJae Young Choi-
dc.contributor.googleauthorSung Huhn Kim-
dc.identifier.doi10.1371/journal.pone.0095730-
dc.admin.authorfalse-
dc.admin.mappingfalse-
dc.contributor.localIdA00507-
dc.contributor.localIdA00589-
dc.contributor.localIdA01209-
dc.contributor.localIdA01865-
dc.contributor.localIdA04173-
dc.contributor.localIdA01025-
dc.relation.journalcodeJ02540-
dc.identifier.eissn1932-6203-
dc.identifier.pmid24752462-
dc.contributor.alternativeNameKim, Bo Gyung-
dc.contributor.alternativeNameKim, Sung Huhn-
dc.contributor.alternativeNameKim, Jin Young-
dc.contributor.alternativeNameKim, Hee Nam-
dc.contributor.alternativeNameBok, Jin Woong-
dc.contributor.alternativeNameChoi, Jae Young-
dc.contributor.affiliatedAuthorKim, Bo Gyung-
dc.contributor.affiliatedAuthorKim, Sung Huhn-
dc.contributor.affiliatedAuthorKim, Hee Nam-
dc.contributor.affiliatedAuthorBok, Jin Woong-
dc.contributor.affiliatedAuthorChoi, Jae Young-
dc.contributor.affiliatedAuthorKim, Jin Young-
dc.citation.volume9-
dc.citation.number4-
dc.citation.startPagee95730-
dc.identifier.bibliographicCitationPLOS ONE, Vol.9(4) : e95730, 2014-
Appears in Collections:
1. College of Medicine (의과대학) > Yonsei Biomedical Research Center (연세의생명연구원) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Otorhinolaryngology (이비인후과학교실) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Anatomy (해부학교실) > 1. Journal Papers

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