Cystic fibrosis of the pancreas: involvement of MUC6 mucin in obstruction of pancreatic ducts.
BACKGROUND
Cystic fibrosis (CF) is characterized by pancreatic destruction following the gradual obstruction of small pancreatic ducts, from the mid-trimester of gestation onwards. To date, the material causing the obstruction has not been identified. The MUC6 mucin cDNA was isolated from human stomach and has been shown to be expressed in a number of other tissues in the gastrointestinal tract, including the gall bladder and parts of the ileum and colon.


METHODS
We have examined the expression of MUC6 mucin in the human pancreas, both during development and postnatally, by mRNA in situ hybridization and immunocytochemistry.


RESULTS
In this report we establish that MUC6 transcripts are abundant in pancreatic epithelial cells and show a very similar pattern of expression in the epithelium lining small ducts and centroacinar cells to that shown by the cystic fibrosis transmembrane conductance regulator gene (CFTR). In addition, material obstructing the pancreatic ducts of CF pancreas was shown to contain MUC6 mucin.


CONCLUSIONS
We have identified MUC6 mucin as a significant constituent of the material obstructing the small pancreatic ducts in CF.
Reid
C J
CJ
Institute of Molecular Medicine, University of Oxford, John Radcliffe Hospital, U.K.
Hyde
K
K
Ho
S B
SB
Harris
A
A
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000473:pathology*; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000096:biosynthesis / Q000235:genetics; D005260:Female; D005333:Fetus / Q000473:pathology; D015870:Gene Expression; D006801:Humans; D007150:Immunohistochemistry; D017403:In Situ Hybridization; D008297:Male; D055272:Mucin-6; D009077:Mucins / Q000096:biosynthesis* / Q000235:genetics; D010183:Pancreatic Ducts / Q000473:pathology*; D011247:Pregnancy; D011262:Pregnancy Trimester, Second
C505032:CFTR protein, human; C524734:MUC6 protein, human; D055272:Mucin-6; D009077:Mucins; D019005:Cystic Fibrosis Transmembrane Conductance Regulator