PMID- 16825704
OWN - NLM
STAT- MEDLINE
DCOM- 20061226
LR  - 20061108
IS  - 0193-1857 (Print)
IS  - 0193-1857 (Linking)
VI  - 291
IP  - 6
DP  - 2006 Dec
TI  - Absence of CFTR is associated with pleiotropic effects on mucins in mouse 
      gallbladder epithelial cells.
PG  - G1148-54
AB  - Mucus of cystic fibrosis patients exhibits altered biochemical composition and 
      biophysical behavior, but the causal relationships between altered cystic 
      fibrosis transmembrane conductance regulator (CFTR) function and the abnormal 
      mucus seen in various organ systems remain unclear. We used cultured gallbladder 
      epithelial cells (GBEC) from wild-type and Cftr((-/-)) mice to investigate mucin 
      gene and protein expression, kinetics of postexocytotic mucous granule content 
      expansion, and biochemical and ionic compositions of secreted mucins. Muc1, Muc3, 
      Muc4, Muc5ac, and Muc5b mRNA levels were significantly lower in Cftr((-/-)) GBEC 
      compared with wild-type cells, whereas Muc2 mRNA levels were higher in 
      Cftr((-/-)) cells. Quantitative immunoblotting demonstrated a trend toward lower 
      MUC1, MUC2, MUC3, MUC5AC, and MUC5B mucin levels in Cftr((-/-)) cells compared 
      with cells from wild-type mice. In contrast, the levels of secreted MUC1, MUC3, 
      MUC5B, and MUC6 mucins were significantly higher from Cftr((-/-)) cells; a trend 
      toward higher levels of secreted MUC2 and MUC5AC was also noted from Cftr((-/-)) 
      cells. Cftr((-/-)) cells demonstrated slower postexocytotic mucous granule 
      content expansion. Calcium concentration was significantly elevated in the mucous 
      gel secreted by Cftr((-/-)) cells compared with wild-type cells. Secreted mucins 
      from Cftr((-/-)) cells contained higher sulfate concentrations. Thus absence of 
      CFTR is associated with pleiotropic effects on mucins in murine GBEC.
FAU - Kuver, Rahul
AU  - Kuver R
AD  - Division of Gastroenterology, Department of Medicine, University of Washington, 
      Seattle, WA 98195, USA. kuver@u.washington.edu
FAU - Wong, Thomas
AU  - Wong T
FAU - Klinkspoor, Johanne Henriette
AU  - Klinkspoor JH
FAU - Lee, Sum P
AU  - Lee SP
LA  - eng
PT  - Journal Article
PT  - Research Support, U.S. Gov't, Non-P.H.S.
DEP - 20060706
PL  - United States
TA  - Am J Physiol Gastrointest Liver Physiol
JT  - American journal of physiology. Gastrointestinal and liver physiology
JID - 100901227
RN  - 0 (Mucins)
RN  - 126880-72-6 (Cystic Fibrosis Transmembrane Conductance Regulator)
SB  - IM
MH  - Animals
MH  - Cells, Cultured
MH  - Cystic Fibrosis Transmembrane Conductance Regulator/*metabolism
MH  - Epithelial Cells/*metabolism
MH  - Gallbladder/*metabolism
MH  - Mice
MH  - Mice, Inbred CFTR
MH  - Mice, Knockout
MH  - Mucins/*metabolism
EDAT- 2006/07/11 09:00
MHDA- 2006/12/27 09:00
CRDT- 2006/07/11 09:00
PHST- 2006/07/11 09:00 [pubmed]
PHST- 2006/12/27 09:00 [medline]
PHST- 2006/07/11 09:00 [entrez]
AID - 00547.2005 [pii]
AID - 10.1152/ajpgi.00547.2005 [doi]
PST - ppublish
SO  - Am J Physiol Gastrointest Liver Physiol. 2006 Dec;291(6):G1148-54. doi: 
      10.1152/ajpgi.00547.2005. Epub 2006 Jul 6.