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The Duct Cell in Cystic Fibrosis
Author(s) -
HARRIS ANN
Publication year - 1999
Publication title -
annals of the new york academy of sciences
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.712
H-Index - 248
eISSN - 1749-6632
pISSN - 0077-8923
DOI - 10.1111/j.1749-6632.1999.tb09506.x
Subject(s) - cystic fibrosis , cystic fibrosis transmembrane conductance regulator , in utero , pancreatic duct , pathology , medicine , pancreatic disease , pancreas , duct (anatomy) , mucin , biology , anatomy , fetus , genetics , pregnancy
A bstract : The pancreatic duct cell is central to the etiology of cystic fibrosis (CF) and is the site where pathology commences in utero . We have evaluated expression of the cystic fibrosis transmembrane conductance regulator gene ( CFTR ) through human development and shown it to be expressed from the early mid‐trimester, with highest levels in the most distal portion of the developing duct system and in centroacinar cells. The precise cause of pancreatic destruction in CF is thought to be the obstruction of pancreatic ducts with inspissated secretions. We have shown that the MUC6 mucin is a significant component of the material that obstructs the ducts and that the MUC6 gene shows a very similar pattern of expression to that of CFTR in the developing pancreas. These observations provide a starting point for investigating how mutations in CFTR lead to obstruction of the pancreatic ducts in CF.