In VivoMeasurements of Ion Transport in Long-Living CF Mice
| Autor: | Lap-Chee Tsui, Geraldine Kent, Peter R. Durie, Richard Rozmahel, Christine E. Bear, Satti Beharry, Canhui Li, M. Wilschanski |
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| Rok vydání: | 1996 |
| Předmět: |
Male
Exocrine gland medicine.medical_specialty Cystic Fibrosis Biophysics Cystic Fibrosis Transmembrane Conductance Regulator Lumen (anatomy) Mice Inbred Strains Biology Biochemistry Epithelium Membrane Potentials Amiloride Mice Chlorides Intestinal mucosa In vivo Internal medicine medicine Animals Secretion Intestinal Mucosa Respiratory system Molecular Biology Crosses Genetic Gastrointestinal tract Homozygote Rectum Exons Cell Biology Mice Mutant Strains Cystic fibrosis transmembrane conductance regulator Nasal Mucosa medicine.anatomical_structure Endocrinology Immunology biology.protein Female |
| Zdroj: | Biochemical and Biophysical Research Communications. 219:753-759 |
| ISSN: | 0006-291X |
| DOI: | 10.1006/bbrc.1996.0306 |
| Popis: | 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/m1HSC 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. |
| Databáze: | OpenAIRE |
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