z-logo
Premium
Recent advances in cystic fibrosis
Author(s) -
McPherson M. A.
Publication year - 1988
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1007/bf01800575
Subject(s) - cystic fibrosis , fibrosis , pathology , secretion , mucin , biology , medicine , endocrinology
Summary Cystic fibrosis, one of the most common lethal inherited disorders in N. European and N. American populations, is characterized by the production of abnormally viscous mucous secretions in the lungs and digestive tract. The pathophysiological basis of the disease is unknown. However, during the last few years, rapid advances in molecular genetics and biochemical and physiological studies on cystic fibrosis epithelial cells have led to optimism that the cystic fibrosis defect will soon be identified. Current evidence suggests that the basic disturbance lies in altered regulation of protein secretion and electrolyte transport leading to an imbalance in composition of epithelial secretions in cystic fibrosis patients. Increasing knowledge of the mechanisms regulating production and secretion of mucins and movement of electrolytes across the cell membrane should lead to development of pharmacological manipulation(s) to correct the cellular abnormality. Ultimately, it is hoped that this will lead to the development of a rational treatment for cystic fibrosis patients.

This content is not available in your region!

Continue researching here.

Having issues? You can contact us here