✦ LIBER ✦

Abstracts: Poster session 2

Publisher: Springer
Year: 1998
Tongue: English
Weight: 996 KB
Volume: 8
Category: Article
ISSN: 0959-9851
DOI: 10.1007/bf02267603

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The vast majority of cases of cystic Þbrosis are the result of a single amino acid deletion (DF508) in the sequence of CFTR. The result of this deletion is that the DF-CFTR misfolds and does not obtain its normal residence in the plasma membrane of cells, but instead is proteolyzed. The F508-contain

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## Cystic Fibrosis Conference which occurs in the intermediate and Golgi compartments. This may reßect either a somewhat different route through the secretory pathway or interference with the enzymatic steps in oligosaccharide processing possibly by calnexin. The latter possibility is being explore