Literature DB >> 9234245

Cystic fibrosis of the pancreas: involvement of MUC6 mucin in obstruction of pancreatic ducts.

C J Reid1, K Hyde, S B Ho, A Harris.   

Abstract

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.

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Year:  1997        PMID: 9234245      PMCID: PMC2230211     

Source DB:  PubMed          Journal:  Mol Med        ISSN: 1076-1551            Impact factor:   6.354


  18 in total

1.  Immunocytochemical localization of the cystic fibrosis gene product CFTR.

Authors:  I Crawford; P C Maloney; P L Zeitlin; W B Guggino; S C Hyde; H Turley; K C Gatter; A Harris; C F Higgins
Journal:  Proc Natl Acad Sci U S A       Date:  1991-10-15       Impact factor: 11.205

2.  Evidence for different human tracheobronchial mucin peptides deduced from nucleotide cDNA sequences.

Authors:  J P Aubert; N Porchet; M Crepin; M Duterque-Coquillaud; G Vergnes; M Mazzuca; B Debuire; D Petitprez; P Degand
Journal:  Am J Respir Cell Mol Biol       Date:  1991-08       Impact factor: 6.914

3.  Cloning and sequencing of a human pancreatic tumor mucin cDNA.

Authors:  M S Lan; S K Batra; W N Qi; R S Metzgar; M A Hollingsworth
Journal:  J Biol Chem       Date:  1990-09-05       Impact factor: 5.157

4.  Secretin-regulated chloride channel on the apical plasma membrane of pancreatic duct cells.

Authors:  M A Gray; J R Greenwell; B E Argent
Journal:  J Membr Biol       Date:  1988-10       Impact factor: 1.843

5.  Molecular cloning of cDNAs derived from a novel human intestinal mucin gene.

Authors:  J R Gum; J W Hicks; D M Swallow; R L Lagace; J C Byrd; D T Lamport; B Siddiki; Y S Kim
Journal:  Biochem Biophys Res Commun       Date:  1990-08-31       Impact factor: 3.575

6.  Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA.

Authors:  J R Riordan; J M Rommens; B Kerem; N Alon; R Rozmahel; Z Grzelczak; J Zielenski; S Lok; N Plavsic; J L Chou
Journal:  Science       Date:  1989-09-08       Impact factor: 47.728

7.  Generation of cAMP-activated chloride currents by expression of CFTR.

Authors:  M P Anderson; D P Rich; R J Gregory; A E Smith; M J Welsh
Journal:  Science       Date:  1991-02-08       Impact factor: 47.728

8.  In situ hybridization shows distinct patterns of mucin gene expression in normal, benign, and malignant pancreas tissues.

Authors:  C Balagué; J P Audié; N Porchet; F X Real
Journal:  Gastroenterology       Date:  1995-09       Impact factor: 22.682

9.  Pancreatic fluid secretion and protein hyperconcentration in cystic fibrosis.

Authors:  H Kopelman; P Durie; K Gaskin; Z Weizman; G Forstner
Journal:  N Engl J Med       Date:  1985-02-07       Impact factor: 91.245

10.  Expression of the cystic fibrosis gene in human development.

Authors:  A Harris; G Chalkley; S Goodman; L Coleman
Journal:  Development       Date:  1991-09       Impact factor: 6.868

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  12 in total

1.  Identification of disease-specific genes in chronic pancreatitis using DNA array technology.

Authors:  H Friess; J Ding; J Kleeff; Q Liao; P O Berberat; J Hammer; M W Büchler
Journal:  Ann Surg       Date:  2001-12       Impact factor: 12.969

Review 2.  Cystic fibrosis: an inherited disease affecting mucin-producing organs.

Authors:  Camille Ehre; Caroline Ridley; David J Thornton
Journal:  Int J Biochem Cell Biol       Date:  2014-03-28       Impact factor: 5.085

3.  Cystic fibrosis and mucins.

Authors:  A Harris; C Reid
Journal:  J Med Genet       Date:  1998-01       Impact factor: 6.318

4.  Genetic variation in CFTR and modifier loci may modulate cystic fibrosis disease severity.

Authors:  Alekh Paranjapye; Manon Ruffin; Ann Harris; Harriet Corvol
Journal:  J Cyst Fibros       Date:  2019-11-14       Impact factor: 5.482

Review 5.  Animal models of gastrointestinal and liver diseases. Animal models of cystic fibrosis: gastrointestinal, pancreatic, and hepatobiliary disease and pathophysiology.

Authors:  Alicia K Olivier; Katherine N Gibson-Corley; David K Meyerholz
Journal:  Am J Physiol Gastrointest Liver Physiol       Date:  2015-01-15       Impact factor: 4.052

6.  The characterization of the first anti-mouse Muc6 antibody shows an increased expression of the mucin in pancreatic tissue of Cftr-knockout mice.

Authors:  Valérie Gouyer; Shih-Hsing Leir; Daniel Tetaert; Yamin Liu; Frédéric Gottrand; Ann Harris; Jean-Luc Desseyn
Journal:  Histochem Cell Biol       Date:  2010-03-23       Impact factor: 4.304

7.  A sheep model of cystic fibrosis generated by CRISPR/Cas9 disruption of the CFTR gene.

Authors:  Zhiqiang Fan; Iuri Viotti Perisse; Calvin U Cotton; Misha Regouski; Qinggang Meng; Chaim Domb; Arnaud J Van Wettere; Zhongde Wang; Ann Harris; Kenneth L White; Irina A Polejaeva
Journal:  JCI Insight       Date:  2018-10-04

Review 8.  CFTR, mucins, and mucus obstruction in cystic fibrosis.

Authors:  Silvia M Kreda; C William Davis; Mary Callaghan Rose
Journal:  Cold Spring Harb Perspect Med       Date:  2012-09-01       Impact factor: 6.915

Review 9.  Human Molecular Genetics and the long road to treating cystic fibrosis.

Authors:  Ann Harris
Journal:  Hum Mol Genet       Date:  2021-10-01       Impact factor: 5.121

10.  Smad4 loss synergizes with TGFα overexpression in promoting pancreatic metaplasia, PanIN development, and fibrosis.

Authors:  Dario Garcia-Carracedo; Chih-Chieh Yu; Nathan Akhavan; Stuart A Fine; Frank Schönleben; Naoki Maehara; Dillon C Karg; Chuangao Xie; Wanglong Qiu; Robert L Fine; Helen E Remotti; Gloria H Su
Journal:  PLoS One       Date:  2015-03-24       Impact factor: 3.240

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