Studies on the Mechanism of Inhibition of Sodium Transport in Cystic Fibrosis of the Pancreas

Cystic Fibrosis: Isolation and Physical Properties of a Salivary Cystic Fibrosis Factor

“…Since the isolated factor has basic or cationic groups (20), it may be electrostatiscally bound to the heparin which is anionic. This effect has been observed for heparin and other polyanions when C F factors from sweat and saliva have been tested using the Mangos retrograde perfusion method (29,31) and is explicable on the basis of their postulated cationic character (13,30). The heparin effect is also consistent with the in vivo and in vitro findings of Doggett and Harrison (15,16) who used ca.…”

“…These factors have in general been described as immunoglobulins, complement components, abnormal proteins, peptides ranging from 1,000-10,000 in molecular weight, as well as abnormal enzymes and polyamines and/or their metabolites (7, 8, 10-12, 15, 20, 22, 29, 31, 33,35). Various bioassays have been developed for the detection of these CF factors and include the oyster gill ciliary assay developed by Bowman et al (7) and modified by Schmoyer et al (39), a fresh water mussel assay by Besley et al (4), a modification of the original rabbit tracheal assay (40) described by Conover et al (9), the retrograde perfusion assay by Mangos (29)(30)(31), and its modification as described by Taylor et al (41), and recently a short-circuit current membrane system by Araki et al (1) adapted from Asano (2). In addition, a means of detection utilizing isoelectric focusing has been described by Wilson and Fudenberg (43).…”

Studies on Cystic Fibrosis Using Isoelectric Focusing. I. An Assay for Detection of Cystic Fibrosis Homozygotes and Heterozygote Carriers from Serum

“…ciliary dyskinesis or stasis. Reports by various investigators indicate that this biologic activity attributed to C / F factor is mimicked by other cationic molecules such as bradykinin (12) and polyamines (6,9,16,23). and therefore, is not specific for the presence of C / F factor in a given serum sample.…”

Studies on Cystic Fibrosis Using Isoelectric Focusing. II. Demonstration of Deficient Proteolytic Cleavage of α2-Macroglobulin in Cystic Fibrosis Plasma

“…53). Other I'actors :Ire found in saliva and sueat froni C F homo/)gotes (31) and have been produced froni cultured cells derived from C F honiorygotes and hetero/ygotes (4 6, 13). Partial characteriration of the various C F factors suggests that the!…”