| pubmed-article:11733566 | rdf:type | pubmed:Citation | lld:pubmed |
| pubmed-article:11733566 | lifeskim:mentions | umls-concept:C0037047 | lld:lifeskim |
| pubmed-article:11733566 | lifeskim:mentions | umls-concept:C0010674 | lld:lifeskim |
| pubmed-article:11733566 | lifeskim:mentions | umls-concept:C0041427 | lld:lifeskim |
| pubmed-article:11733566 | lifeskim:mentions | umls-concept:C0019904 | lld:lifeskim |
| pubmed-article:11733566 | lifeskim:mentions | umls-concept:C0031437 | lld:lifeskim |
| pubmed-article:11733566 | lifeskim:mentions | umls-concept:C0596019 | lld:lifeskim |
| pubmed-article:11733566 | lifeskim:mentions | umls-concept:C0314603 | lld:lifeskim |
| pubmed-article:11733566 | lifeskim:mentions | umls-concept:C1705241 | lld:lifeskim |
| pubmed-article:11733566 | lifeskim:mentions | umls-concept:C0439097 | lld:lifeskim |
| pubmed-article:11733566 | lifeskim:mentions | umls-concept:C1547348 | lld:lifeskim |
| pubmed-article:11733566 | lifeskim:mentions | umls-concept:C1706907 | lld:lifeskim |
| pubmed-article:11733566 | pubmed:issue | 11 | lld:pubmed |
| pubmed-article:11733566 | pubmed:dateCreated | 2001-12-4 | lld:pubmed |
| pubmed-article:11733566 | pubmed:abstractText | To investigate the impact of chloride (Cl(-)) permeability, mediated by residual activity of the cystic fibrosis transmembrane conductance regulator (CFTR) or by other Cl(-) channels, on the manifestations of cystic fibrosis (CF), we determined Cl(-) transport properties of the respiratory and intestinal tracts in Delta F508 homozygous twins and siblings. In the majority of patients, cAMP and/or Ca(2+)-regulated Cl(-) conductance was detected in the airways and intestine. Our finding of cAMP-mediated Cl(-) conductance suggests that, in vivo, at least some Delta F508 CFTR can reach the plasma membrane and affect Cl(-) permeability. In respiratory tissue, the expression of basal CFTR-mediated Cl(-) conductance, demonstrated by 30% of Delta F508 homozygotes, was identified as a positive predictor of milder CF disease. In intestinal tissue, 4,4'-diisothiocyanatostilbene-2,2'-disulfonic acid-insensitive (DIDS-insensitive) Cl(-) secretion, which is indicative of functional CFTR channels, correlated with a milder phenotype, whereas DIDS-sensitive Cl(-) secretion was observed mainly in more severely affected patients. The more concordant Cl(-) secretory patterns within monozygous twins compared with dizygous pairs imply that genes other than CFTR significantly influence the manifestation of the basic defect. | lld:pubmed |
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| pubmed-article:11733566 | pubmed:language | eng | lld:pubmed |
| pubmed-article:11733566 | pubmed:journal | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:11733566 | pubmed:citationSubset | AIM | lld:pubmed |
| pubmed-article:11733566 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
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| pubmed-article:11733566 | pubmed:status | MEDLINE | lld:pubmed |
| pubmed-article:11733566 | pubmed:month | Dec | lld:pubmed |
| pubmed-article:11733566 | pubmed:issn | 0021-9738 | lld:pubmed |
| pubmed-article:11733566 | pubmed:author | pubmed-author:ThomasSS | lld:pubmed |
| pubmed-article:11733566 | pubmed:author | pubmed-author:HalleyD JDJ | lld:pubmed |
| pubmed-article:11733566 | pubmed:author | pubmed-author:MastellaGG | lld:pubmed |
| pubmed-article:11733566 | pubmed:author | pubmed-author:de JongeH RHR | lld:pubmed |
| pubmed-article:11733566 | pubmed:author | pubmed-author:TümmlerBB | lld:pubmed |
| pubmed-article:11733566 | pubmed:author | pubmed-author:BijmanJJ | lld:pubmed |
| pubmed-article:11733566 | pubmed:author | pubmed-author:EllemunterHH | lld:pubmed |
| pubmed-article:11733566 | pubmed:author | pubmed-author:VeezeH JHJ | lld:pubmed |
| pubmed-article:11733566 | pubmed:author | pubmed-author:BallmannMM | lld:pubmed |
| pubmed-article:11733566 | pubmed:author | pubmed-author:MekusFF | lld:pubmed |
| pubmed-article:11733566 | pubmed:author | pubmed-author:BronsveldII | lld:pubmed |
| pubmed-article:11733566 | pubmed:author | pubmed-author:LaabsUU | lld:pubmed |
| pubmed-article:11733566 | pubmed:issnType | Print | lld:pubmed |
| pubmed-article:11733566 | pubmed:volume | 108 | lld:pubmed |
| pubmed-article:11733566 | pubmed:owner | NLM | lld:pubmed |
| pubmed-article:11733566 | pubmed:authorsComplete | Y | lld:pubmed |
| pubmed-article:11733566 | pubmed:pagination | 1705-15 | lld:pubmed |
| pubmed-article:11733566 | pubmed:dateRevised | 2009-11-18 | lld:pubmed |
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| pubmed-article:11733566 | pubmed:year | 2001 | lld:pubmed |
