Abstract
Normally a thin layer of mucus covers the surface of the gastrointestinal tract protecting the epithelial cells from their environment. In cystic fibrosis (CF), mucus accumulation is abnormally high, resulting in severe intestinal obstruction. The major structural components of mucus are large mucin glycoproteins. We determined specific mucin RNA and protein expression in the gastrointestinal tract of inbred CF transmembrane conductance regulator (CFTR) knockout (CF) mice and correlated expression with histological analyses of tissues. Mucins were detected histochemically using general carbohydrate stains and specific mucin antibodies. Mucin RNA levels were determined by reverse transcription-PCR. Comparisons were made between CF mice and control siblings, all maintained on a liquid diet after weaning. Analyses of the mucins Muc2, Muc3, and MucSac showed lower levels of RNA expression in the CF mice and similar levels of protein. Significantly, there was a sixfold increase in Muc1 RNA expression in the colon of the CF mouse and a moderate increase in Muc1 protein. Further, CF mice lacking Muc1 exhibited greatly diminished intestinal mucus obstruction when compared with Muc1-expressing CF mice and had better survival on solid food. We suggest that Muc1 plays an important role in the mucus obstructions observed in the gastrointestinal tract of the CFTR knockout mouse.
| Original language | English (US) |
|---|---|
| Pages (from-to) | 1798-1806 |
| Number of pages | 9 |
| Journal | Journal of Clinical Investigation |
| Volume | 102 |
| Issue number | 10 |
| State | Published - Nov 15 1998 |
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Keywords
- Gastrointestinal tract
- Immunohistochemistry
- Meconium ileus
- Mouse cystic fibrosis model
- Reverse transcription-polymerase chain reaction
ASJC Scopus subject areas
- Medicine(all)
Cite this
Cystic fibrosis mice lacking Muc1 have reduced amounts of intestinal mucus. / Parmley, Robin R.; Gendler, Sandra J.
In: Journal of Clinical Investigation, Vol. 102, No. 10, 15.11.1998, p. 1798-1806.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Cystic fibrosis mice lacking Muc1 have reduced amounts of intestinal mucus
AU - Parmley, Robin R.
AU - Gendler, Sandra J
PY - 1998/11/15
Y1 - 1998/11/15
N2 - Normally a thin layer of mucus covers the surface of the gastrointestinal tract protecting the epithelial cells from their environment. In cystic fibrosis (CF), mucus accumulation is abnormally high, resulting in severe intestinal obstruction. The major structural components of mucus are large mucin glycoproteins. We determined specific mucin RNA and protein expression in the gastrointestinal tract of inbred CF transmembrane conductance regulator (CFTR) knockout (CF) mice and correlated expression with histological analyses of tissues. Mucins were detected histochemically using general carbohydrate stains and specific mucin antibodies. Mucin RNA levels were determined by reverse transcription-PCR. Comparisons were made between CF mice and control siblings, all maintained on a liquid diet after weaning. Analyses of the mucins Muc2, Muc3, and MucSac showed lower levels of RNA expression in the CF mice and similar levels of protein. Significantly, there was a sixfold increase in Muc1 RNA expression in the colon of the CF mouse and a moderate increase in Muc1 protein. Further, CF mice lacking Muc1 exhibited greatly diminished intestinal mucus obstruction when compared with Muc1-expressing CF mice and had better survival on solid food. We suggest that Muc1 plays an important role in the mucus obstructions observed in the gastrointestinal tract of the CFTR knockout mouse.
AB - Normally a thin layer of mucus covers the surface of the gastrointestinal tract protecting the epithelial cells from their environment. In cystic fibrosis (CF), mucus accumulation is abnormally high, resulting in severe intestinal obstruction. The major structural components of mucus are large mucin glycoproteins. We determined specific mucin RNA and protein expression in the gastrointestinal tract of inbred CF transmembrane conductance regulator (CFTR) knockout (CF) mice and correlated expression with histological analyses of tissues. Mucins were detected histochemically using general carbohydrate stains and specific mucin antibodies. Mucin RNA levels were determined by reverse transcription-PCR. Comparisons were made between CF mice and control siblings, all maintained on a liquid diet after weaning. Analyses of the mucins Muc2, Muc3, and MucSac showed lower levels of RNA expression in the CF mice and similar levels of protein. Significantly, there was a sixfold increase in Muc1 RNA expression in the colon of the CF mouse and a moderate increase in Muc1 protein. Further, CF mice lacking Muc1 exhibited greatly diminished intestinal mucus obstruction when compared with Muc1-expressing CF mice and had better survival on solid food. We suggest that Muc1 plays an important role in the mucus obstructions observed in the gastrointestinal tract of the CFTR knockout mouse.
KW - Gastrointestinal tract
KW - Immunohistochemistry
KW - Meconium ileus
KW - Mouse cystic fibrosis model
KW - Reverse transcription-polymerase chain reaction
UR - http://www.scopus.com/inward/record.url?scp=0032533204&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0032533204&partnerID=8YFLogxK
M3 - Article
C2 - 9819365
AN - SCOPUS:0032533204
VL - 102
SP - 1798
EP - 1806
JO - Journal of Clinical Investigation
JF - Journal of Clinical Investigation
SN - 0021-9738
IS - 10
ER -