Cited 355 times in
Aberrant CFTR-dependent HCO3- transport in mutations associated with cystic fibrosis
DC Field | Value | Language |
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dc.contributor.author | 이민구 | - |
dc.date.accessioned | 2016-02-19T11:19:21Z | - |
dc.date.available | 2016-02-19T11:19:21Z | - |
dc.date.issued | 2001 | - |
dc.identifier.issn | 0028-0836 | - |
dc.identifier.uri | https://ir.ymlib.yonsei.ac.kr/handle/22282913/142888 | - |
dc.description.abstract | Cystic fibrosis (CF) is a disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR). Initially, Cl- conductance in the sweat duct was discovered to be impaired in CF, a finding that has been extended to all CFTR-expressing cells. Subsequent cloning of the gene showed that CFTR functions as a cyclic-AMP-regulated Cl- channel; and some CF-causing mutations inhibit CFTR Cl- channel activity. The identification of additional CF-causing mutants with normal Cl- channel activity indicates, however, that other CFTR-dependent processes contribute to the disease. Indeed, CFTR regulates other transporters, including Cl(-)-coupled HCO3- transport. Alkaline fluids are secreted by normal tissues, whereas acidic fluids are secreted by mutant CFTR-expressing tissues, indicating the importance of this activity. HCO3- and pH affect mucin viscosity and bacterial binding. We have examined Cl(-)-coupled HCO3- transport by CFTR mutants that retain substantial or normal Cl- channel activity. Here we show that mutants reported to be associated with CF with pancreatic insufficiency do not support HCO3- transport, and those associated with pancreatic sufficiency show reduced HCO3- transport. Our findings demonstrate the importance of HCO3- transport in the function of secretory epithelia and in CF. | - |
dc.description.statementOfResponsibility | open | - |
dc.format.extent | 94~97 | - |
dc.relation.isPartOf | NATURE | - |
dc.rights | CC BY-NC-ND 2.0 KR | - |
dc.rights.uri | https://creativecommons.org/licenses/by-nc-nd/2.0/kr/ | - |
dc.subject.MESH | Bicarbonates/metabolism* | - |
dc.subject.MESH | Biological Transport | - |
dc.subject.MESH | Cell Line | - |
dc.subject.MESH | Chlorides/metabolism | - |
dc.subject.MESH | Cystic Fibrosis/metabolism* | - |
dc.subject.MESH | Cystic Fibrosis Transmembrane Conductance Regulator/genetics | - |
dc.subject.MESH | Cystic Fibrosis Transmembrane Conductance Regulator/metabolism* | - |
dc.subject.MESH | Humans | - |
dc.subject.MESH | Membrane Potentials | - |
dc.subject.MESH | Membranes/metabolism | - |
dc.subject.MESH | Mutagenesis, Site-Directed | - |
dc.subject.MESH | Mutation | - |
dc.subject.MESH | Pancreas/metabolism | - |
dc.subject.MESH | Transfection | - |
dc.title | Aberrant CFTR-dependent HCO3- transport in mutations associated with cystic fibrosis | - |
dc.type | Article | - |
dc.contributor.college | College of Medicine (의과대학) | - |
dc.contributor.department | Dept. of Pharmacology (약리학) | - |
dc.contributor.googleauthor | Joo Young Choi | - |
dc.contributor.googleauthor | Daniella Muallem | - |
dc.contributor.googleauthor | Kirill Kiselyov | - |
dc.contributor.googleauthor | Min Goo Lee | - |
dc.contributor.googleauthor | Philip J. Thomas | - |
dc.contributor.googleauthor | Shmuel Muallem | - |
dc.identifier.doi | 10.1038/35065099 | - |
dc.admin.author | false | - |
dc.admin.mapping | false | - |
dc.contributor.localId | A02781 | - |
dc.relation.journalcode | J02289 | - |
dc.identifier.eissn | 1476-4687 | - |
dc.identifier.pmid | 11242048 | - |
dc.contributor.alternativeName | Lee, Min Goo | - |
dc.contributor.affiliatedAuthor | Lee, Min Goo | - |
dc.rights.accessRights | free | - |
dc.citation.volume | 410 | - |
dc.citation.number | 6824 | - |
dc.citation.startPage | 94 | - |
dc.citation.endPage | 97 | - |
dc.identifier.bibliographicCitation | NATURE, Vol.410(6824) : 94-97, 2001 | - |
dc.identifier.rimsid | 39551 | - |
dc.type.rims | ART | - |
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