Linked Life Data

12386454

Source:http://linkedlifedata.com/resource/pubmed/id/12386454

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
9
pubmed:dateCreated
2002-10-18
pubmed:abstractText
Structural differences have been reported in the glycosylation patterns of cystic fibrosis glycoproteins. Although the gene mutated in cystic fibrosis (CFTR) has been cloned and characterized as a chloride channel, its relationship to the highly viscous mucus and structural glycoprotein and mucin abnormalities in cystic fibrosis still remains to be defined. We have evaluated O-glycan biosynthesis in CHO and BHK cells that express CFTR and DeltaF508 CFTR as in vitro models, and utilized the cftr knockout mouse as an in vivo model of CFTR dysfunction. Activities of glycosyltransferases and sulfotransferases synthesizing mucin type O-glycan chains were determined in these models. Differences in transferase activity levels were found between tissues and cell types and during mouse development. No specific patterns of activities were associated with the lack of CFTR or with DeltaF508CFTR expression. This suggests that it is not the presence or absence of normal CFTR, or the presence of mutant CFTR alone, but rather cell specific additional factors or pathophysiological consequences that determine the changes in mucin glycosylation in cystic fibrosis.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Sep
pubmed:issn
0282-0080
pubmed:author
pubmed:issnType
Print
pubmed:volume
18
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
685-97
pubmed:dateRevised
2006-11-15
pubmed:meshHeading
pubmed-meshheading:12386454-Amino Acid Sequence, pubmed-meshheading:12386454-Animals, pubmed-meshheading:12386454-CHO Cells, pubmed-meshheading:12386454-Carbohydrate Sequence, pubmed-meshheading:12386454-Cell Line, Transformed, pubmed-meshheading:12386454-Cricetinae, pubmed-meshheading:12386454-Cystic Fibrosis Transmembrane Conductance Regulator, pubmed-meshheading:12386454-Gene Expression Regulation, Developmental, pubmed-meshheading:12386454-Glycosyltransferases, pubmed-meshheading:12386454-Lectins, pubmed-meshheading:12386454-Mice, pubmed-meshheading:12386454-Mice, Inbred CFTR, pubmed-meshheading:12386454-Mice, Knockout, pubmed-meshheading:12386454-Molecular Sequence Data, pubmed-meshheading:12386454-Mucins, pubmed-meshheading:12386454-Polysaccharides, pubmed-meshheading:12386454-Sulfotransferases
pubmed:year
2001
pubmed:articleTitle
Biosynthesis of mucin type O-glycans: lack of correlation between glycosyltransferase and sulfotransferase activities and CFTR expression.
pubmed:affiliation
Department of Medicine, Etherington Hall, Queen's University, Kingston, Ontario, K7L 3N6, Canada. brockhau@post.queensu.ca
pubmed:publicationType
Journal Article, Research Support, Non-U.S. Gov't