| pubmed-article:20147297 | rdf:type | pubmed:Citation | lld:pubmed |
| pubmed-article:20147297 | lifeskim:mentions | umls-concept:C0041538 | lld:lifeskim |
| pubmed-article:20147297 | lifeskim:mentions | umls-concept:C0056889 | lld:lifeskim |
| pubmed-article:20147297 | lifeskim:mentions | umls-concept:C0699900 | lld:lifeskim |
| pubmed-article:20147297 | lifeskim:mentions | umls-concept:C0243125 | lld:lifeskim |
| pubmed-article:20147297 | lifeskim:mentions | umls-concept:C1306673 | lld:lifeskim |
| pubmed-article:20147297 | lifeskim:mentions | umls-concept:C1707271 | lld:lifeskim |
| pubmed-article:20147297 | pubmed:issue | 15 | lld:pubmed |
| pubmed-article:20147297 | pubmed:dateCreated | 2010-4-5 | lld:pubmed |
| pubmed-article:20147297 | pubmed:abstractText | DeltaF508 cystic fibrosis transmembrane conductance regulator (CFTR) degradation involves ubiquitin modification and efficient proteasomal targeting of the nascent misfolded protein. We show that a deubiquitinating enzyme, ubiquitin C-terminal hydrolase-L1 (UCH-L1), is highly expressed in cystic fibrosis (CF) airway epithelial cells in vitro and in vivo. We hypothesized that the elevation in UCH-L1 in CF cells represents a cellular adaptation to counterbalance excessive proteasomal degradation. The bronchial epithelial cell lines IB3-1 (CF, high UCH-L1 expression) and S9 (non-CF, low UCH-L1 expression) were transiently transfected with wild type (WT) or DeltaF508 CFTR, WT UCH-L1 or small interfering RNA-UCH-L1, and a variety of ubiquitin mutants. We observed a positive correlation between UCH-L1 expression and steady state levels of WT- or DeltaF508-CFTR, and this stabilizing effect was confined to the early stages of CFTR synthesis. Immunolocalization of UCH-L1 by confocal microscopy revealed a partial co-localization with a ribosomal subunit and the endoplasmic reticulum. The UCH-L1-associated increase in CFTR levels was correlated with an increase in ubiquitinated CFTR (CFTR-Ub). Co-transfection with mutant ubiquitins and treatment with proteasome inhibitors suggested that UCH-L1 was reducing the proteasomal targeting of CFTR during synthesis by shortening conjugated polyubiquitin chains. Although not sufficient by itself to rescue mutant CFTR therapeutically, the elevation of UCH-L1 and its effect on CFTR processing provides insight into its potential roles in CF and other diseases. | lld:pubmed |
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| pubmed-article:20147297 | pubmed:language | eng | lld:pubmed |
| pubmed-article:20147297 | pubmed:journal | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:20147297 | pubmed:citationSubset | IM | lld:pubmed |
| pubmed-article:20147297 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
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| pubmed-article:20147297 | pubmed:status | MEDLINE | lld:pubmed |
| pubmed-article:20147297 | pubmed:month | Apr | lld:pubmed |
| pubmed-article:20147297 | pubmed:issn | 1083-351X | lld:pubmed |
| pubmed-article:20147297 | pubmed:author | pubmed-author:ZeitlinPamela... | lld:pubmed |
| pubmed-article:20147297 | pubmed:author | pubmed-author:VijNeerajN | lld:pubmed |
| pubmed-article:20147297 | pubmed:author | pubmed-author:HendersonMark... | lld:pubmed |
| pubmed-article:20147297 | pubmed:issnType | Electronic | lld:pubmed |
| pubmed-article:20147297 | pubmed:day | 9 | lld:pubmed |
| pubmed-article:20147297 | pubmed:volume | 285 | lld:pubmed |
| pubmed-article:20147297 | pubmed:owner | NLM | lld:pubmed |
| pubmed-article:20147297 | pubmed:authorsComplete | Y | lld:pubmed |
| pubmed-article:20147297 | pubmed:pagination | 11314-25 | lld:pubmed |
| pubmed-article:20147297 | pubmed:dateRevised | 2011-7-28 | lld:pubmed |
| pubmed-article:20147297 | pubmed:meshHeading | pubmed-meshheading:20147297... | lld:pubmed |
| pubmed-article:20147297 | pubmed:meshHeading | pubmed-meshheading:20147297... | lld:pubmed |
| pubmed-article:20147297 | pubmed:meshHeading | pubmed-meshheading:20147297... | lld:pubmed |
