Abstract
CYSTIC FIBROSIS (CF) is an autosomal recessive genetic disorder with a frequency of about 1/1,600 among newborn infants of European descent. In spite of extensive investigation its primary molecular abnormality remains unknown. It has been observed that most CF patients lack an α2 macroglobulin–protease complex1 and that binding of trypsin and other proteases to α2 macroglobulin (α2M) is lower in plasma of CF patients than in that of normal controls2. We therefore decided to study the interaction of trypsin with plasma proteins of CF patients. Here we report that trypsin-binding IgG (TbIgG) is present in 80% of CF patients and in 30% of their mothers, but not in 97% of normal controls or in other relatives of CF patients. Experimental evidence supports the antibody nature of TbIgG, which is presumably produced against the porcine trypsin ingested by CF patients as pancreatic extracts for substitutive enzyme therapy.
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ROMEO, G., PARSONS, M., BOSSEN, A. et al. Trypsin-binding IgG in cystic fibrosis. Nature 274, 909–911 (1978). https://doi.org/10.1038/274909a0
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DOI: https://doi.org/10.1038/274909a0
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