Search tips
Search criteria 


Logo of molmedLink to Publisher's site
Mol Med. 1997 June; 3(6): 403–411.
PMCID: PMC2230211

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. MATERIALS AND 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. CONCLUSION: We have identified MUC6 mucin as a significant constituent of the material obstructing the small pancreatic ducts in CF.

Full text

Full text is available as a scanned copy of the original print version. Get a printable copy (PDF file) of the complete article (4.5M), or click on a page image below to browse page by page. Links to PubMed are also available for Selected References.

Images in this article

Click on the image to see a larger version.

Selected References

These references are in PubMed. This may not be the complete list of references from this article.
  • Boué A, Muller F, Nezelof C, Oury JF, Duchatel F, Dumez Y, Aubry MC, Boué J. Prenatal diagnosis in 200 pregnancies with a 1-in-4 risk of cystic fibrosis. Hum Genet. 1986 Nov;74(3):288–297. [PubMed]
  • Riordan JR, Rommens JM, Kerem B, Alon N, Rozmahel R, Grzelczak Z, Zielenski J, Lok S, Plavsic N, Chou JL, et al. Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science. 1989 Sep 8;245(4922):1066–1073. [PubMed]
  • Harris A, Chalkley G, Goodman S, Coleman L. Expression of the cystic fibrosis gene in human development. Development. 1991 Sep;113(1):305–310. [PubMed]
  • Trezise AE, Chambers JA, Wardle CJ, Gould S, Harris A. Expression of the cystic fibrosis gene in human foetal tissues. Hum Mol Genet. 1993 Mar;2(3):213–218. [PubMed]
  • Ho SB, Roberton AM, Shekels LL, Lyftogt CT, Niehans GA, Toribara NW. Expression cloning of gastric mucin complementary DNA and localization of mucin gene expression. Gastroenterology. 1995 Sep;109(3):735–747. [PubMed]
  • Toribara NW, Roberton AM, Ho SB, Kuo WL, Gum E, Hicks JW, Gum JR, Jr, Byrd JC, Siddiki B, Kim YS. Human gastric mucin. Identification of a unique species by expression cloning. J Biol Chem. 1993 Mar 15;268(8):5879–5885. [PubMed]
  • De Bolós C, Garrido M, Real FX. MUC6 apomucin shows a distinct normal tissue distribution that correlates with Lewis antigen expression in the human stomach. Gastroenterology. 1995 Sep;109(3):723–734. [PubMed]
  • Lan MS, Batra SK, Qi WN, Metzgar RS, Hollingsworth MA. Cloning and sequencing of a human pancreatic tumor mucin cDNA. J Biol Chem. 1990 Sep 5;265(25):15294–15299. [PubMed]
  • Batra SK, Metzgar RS, Hollingsworth MA. Human Muc 1 mucin gene expression in the fetal pancreas. Pancreas. 1992;7(3):391–393. [PubMed]
  • Chambers JA, Hollingsworth MA, Trezise AE, Harris A. Developmental expression of mucin genes MUC1 and MUC2. J Cell Sci. 1994 Feb;107(Pt 2):413–424. [PubMed]
  • Gum JR, Hicks JW, Swallow DM, Lagace RL, Byrd JC, Lamport DT, Siddiki B, Kim YS. Molecular cloning of cDNAs derived from a novel human intestinal mucin gene. Biochem Biophys Res Commun. 1990 Aug 31;171(1):407–415. [PubMed]
  • Gum JR, Jr, Hicks JW, Toribara NW, Rothe EM, Lagace RE, Kim YS. The human MUC2 intestinal mucin has cysteine-rich subdomains located both upstream and downstream of its central repetitive region. J Biol Chem. 1992 Oct 25;267(30):21375–21383. [PubMed]
  • Aubert JP, Porchet N, Crepin M, Duterque-Coquillaud M, Vergnes G, Mazzuca M, Debuire B, Petitprez D, Degand P. Evidence for different human tracheobronchial mucin peptides deduced from nucleotide cDNA sequences. Am J Respir Cell Mol Biol. 1991 Aug;5(2):178–185. [PubMed]
  • Balagué C, Audié JP, Porchet N, Real FX. In situ hybridization shows distinct patterns of mucin gene expression in normal, benign, and malignant pancreas tissues. Gastroenterology. 1995 Sep;109(3):953–964. [PubMed]
  • Crawford I, Maloney PC, Zeitlin PL, Guggino WB, Hyde SC, Turley H, Gatter KC, Harris A, Higgins CF. Immunocytochemical localization of the cystic fibrosis gene product CFTR. Proc Natl Acad Sci U S A. 1991 Oct 15;88(20):9262–9266. [PubMed]
  • Anderson MP, Rich DP, Gregory RJ, Smith AE, Welsh MJ. Generation of cAMP-activated chloride currents by expression of CFTR. Science. 1991 Feb 8;251(4994):679–682. [PubMed]
  • Gray MA, Greenwell JR, Argent BE. Secretin-regulated chloride channel on the apical plasma membrane of pancreatic duct cells. J Membr Biol. 1988 Oct;105(2):131–142. [PubMed]
  • Kopelman H, Durie P, Gaskin K, Weizman Z, Forstner G. Pancreatic fluid secretion and protein hyperconcentration in cystic fibrosis. N Engl J Med. 1985 Feb 7;312(6):329–334. [PubMed]

Articles from Molecular Medicine are provided here courtesy of The Feinstein Institute for Medical Research at North Shore LIJ