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In vivo measurements of ion transport in long-living CF mice.
Biochem Biophys Res Commun 1996; 219(3):753-9BB

Abstract

The Cftr (Cystic Fibrosis Transmembrane Conductance Regulator) gene codes for an epithelial chloride (C1) channel essential for fluid secretion into the respiratory and gastrointestinal tract and from exocrine glands. Mice lacking CFTR function due to a disruption of Cftr exon 10 or exon 1 (Cftr (m1UNC/m1UNC) or Cftr(m1HSC/m1HFC) mice, respectively) generally suffer from severe gastrointestinal disease resulting in death shortly after birth or at the time of weaning. However, a subgroup of the Cftr(m1HSC/m1HSC) mice have been characterized which exhibit relatively mild intestinal pathology resulting in a noncompromised lifespan compared to the more severely affected Cftr(m1UNC/m1UNC) mice. We compared the ion transport capacity of the intestinal mucosa of the mildly and severely affected CF mice using the in vivo technique of rectal potential difference (PD) measurement and found that the net calcium-activated chloride conductance toward the lumen was much greater in the rectum of mildly affected mice than in the severely affected mice. Hence, the milder phenotype may be related to the expression of a factor which enhances the net calcium-activated chloride conductance into the lumen of the intestinal tract.

Authors+Show Affiliations

Department of Cell Biology, University of Toronto, Canada.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Comparative Study
Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

8645253

Citation

Wilschanski, M A., et al. "In Vivo Measurements of Ion Transport in Long-living CF Mice." Biochemical and Biophysical Research Communications, vol. 219, no. 3, 1996, pp. 753-9.
Wilschanski MA, Rozmahel R, Beharry S, et al. In vivo measurements of ion transport in long-living CF mice. Biochem Biophys Res Commun. 1996;219(3):753-9.
Wilschanski, M. A., Rozmahel, R., Beharry, S., Kent, G., Li, C., Tsui, L. C., ... Bear, C. E. (1996). In vivo measurements of ion transport in long-living CF mice. Biochemical and Biophysical Research Communications, 219(3), pp. 753-9.
Wilschanski MA, et al. In Vivo Measurements of Ion Transport in Long-living CF Mice. Biochem Biophys Res Commun. 1996 Feb 27;219(3):753-9. PubMed PMID: 8645253.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - In vivo measurements of ion transport in long-living CF mice. AU - Wilschanski,M A, AU - Rozmahel,R, AU - Beharry,S, AU - Kent,G, AU - Li,C, AU - Tsui,L C, AU - Durie,P, AU - Bear,C E, PY - 1996/2/27/pubmed PY - 1996/2/27/medline PY - 1996/2/27/entrez SP - 753 EP - 9 JF - Biochemical and biophysical research communications JO - Biochem. Biophys. Res. Commun. VL - 219 IS - 3 N2 - The Cftr (Cystic Fibrosis Transmembrane Conductance Regulator) gene codes for an epithelial chloride (C1) channel essential for fluid secretion into the respiratory and gastrointestinal tract and from exocrine glands. Mice lacking CFTR function due to a disruption of Cftr exon 10 or exon 1 (Cftr (m1UNC/m1UNC) or Cftr(m1HSC/m1HFC) mice, respectively) generally suffer from severe gastrointestinal disease resulting in death shortly after birth or at the time of weaning. However, a subgroup of the Cftr(m1HSC/m1HSC) mice have been characterized which exhibit relatively mild intestinal pathology resulting in a noncompromised lifespan compared to the more severely affected Cftr(m1UNC/m1UNC) mice. We compared the ion transport capacity of the intestinal mucosa of the mildly and severely affected CF mice using the in vivo technique of rectal potential difference (PD) measurement and found that the net calcium-activated chloride conductance toward the lumen was much greater in the rectum of mildly affected mice than in the severely affected mice. Hence, the milder phenotype may be related to the expression of a factor which enhances the net calcium-activated chloride conductance into the lumen of the intestinal tract. SN - 0006-291X UR - https://www.unboundmedicine.com/medline/citation/8645253/In_vivo_measurements_of_ion_transport_in_long_living_CF_mice_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0006-291X(96)90306-X DB - PRIME DP - Unbound Medicine ER -