Protease binding by alpha 2 macroglobulin in cystic fibrosis.
The interaction of alpha 2 macroglobulin (alpha 2M) with exogenous proteases has been reported by others to be abnormal in cystic fibrosis (CF). We have re-examined these claims. Four parameters were considered:(1) the molar protease binding of alpha 2M; (2) the interaction of bovine cationic trypsin (BCT), complexed to alpha 2M, with low molecular mass substrate, benzoyl arginine ethyl ester (BAEE); (3) the stability of formed alpha 2 M-BCT complexes; and (4) the subunit structure of alpha 2M. We have found CF alpha 2M to be similar to control alpha 2M in every respect studied.
D000293:Adolescent; D000880:Anthraquinones; D001120:Arginine / Q000031:analogs & derivatives / Q000378:metabolism; D002648:Child; D004396:Coloring Agents; D003550:Cystic Fibrosis / Q000378:metabolism*; D005260:Female; D006801:Humans; D007700:Kinetics; D046911:Macromolecular Substances; D008297:Male; D010447:Peptide Hydrolases / Q000378:metabolism*; D014357:Trypsin / Q000378:metabolism; D014360:Trypsin Inhibitor, Kunitz Soybean / Q000378:metabolism; D000511:alpha-Macroglobulins / Q000378:metabolism*
D000880:Anthraquinones; D004396:Coloring Agents; D046911:Macromolecular Substances; D000511:alpha-Macroglobulins; D014360:Trypsin Inhibitor, Kunitz Soybean; D001120:Arginine; C009727:benzoylarginine ethyl ester; D010447:Peptide Hydrolases; D014357:Trypsin; C024758:Remazol Brilliant Blue R