Abnormal breakdown of α2-macroglobulin-trypsin complex in cystic fibrosis
1977; Elsevier BV; Volume: 78; Issue: 3 Linguagem: Inglês
10.1016/0009-8981(77)90068-7
ISSN1873-3492
AutoresEmmanuel Shapira, Yoav Ben‐Yoseph, Henry L. Nadler,
Tópico(s)Infant Nutrition and Health
ResumoThe complex of trypsin with purified α2-macroglobulin from normals and patients with cystic fibrosis was studied. The formed complex failed to reveal any proteolytic activity toward a high molecular weight substrate whereas the esterolytic activity towards a low molecular weight substrate was retained. This esterolytic activity was resistant to inhibition by a high molecular weight inhibitor. During incubation at 38° C the complex with normal α2-macroglobulin was slowly inhibited by the high molecular weight inhibitor and regained activity with the high molecular weight substrate. This phenomenon was not obtained when the α2-macroglobulin from cystic fibrosis was examined. These data suggest that the gradual conversion of normal α2-macroglobulin-trypsin complex into an α2-macroglobulin fragment-trypsin complex is deficient in patients with cystic fibrosis.
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