The biochemical basis of cystic fibrosis: an hypothesis based upon the polyelectrolytes of mucus.
The evidence shows that the pathology of cystic fibrosis (CF) is related to mucoid secretions. Consequently, the question of what governs the viscosity of mucus is examined in detail. It is generally agreed that the mucin gel consists of polyanionic glycoproteins and a hitherto unidentified poly cationic cross-linking protein. It is proposed that larger amounts of polycations increase viscosity and, in excess, cause mucus to become a polycation. As these compounds are known to inhibit the ductal reabsorption of sodium, the two principal aspects of the pathology of CF can be related to the same mechanism. The mucus globule membrane (MGM) has been proposed as the cross-linking protein.
D003550:Cystic Fibrosis / Q000378:metabolism* / Q000503:physiopathology; D004573:Electrolytes / Q000378:metabolism*; D006801:Humans; D008565:Membrane Proteins / Q000378:metabolism; D008566:Membranes / Q000378:metabolism; D009093:Mucus / Q000378:metabolism*; D014783:Viscosity
D004573:Electrolytes; D008565:Membrane Proteins