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Trypsin binding activity of alpha 2-macroglobulin in cystic fibrosis and other lung diseases

D V Schidlow, F Kueppers

    The American Review of Respiratory Disease
    |January 1, 1980
    PubMed

    Insights

    This study found no defect in alpha 2-macroglobulin protease-binding in cystic fibrosis patients. Increased binding activity was observed in cystic fibrosis, heterozygotes, and other lung diseases, suggesting a non-specific effect.

    Area of Science:

    • Biochemistry
    • Pulmonary Medicine
    • Genetics

    Background:

    • Abnormal interactions between alpha 2-macroglobulin and proteases are implicated in cystic fibrosis (CF).
    • Alpha 2-macroglobulin is a key protease inhibitor involved in regulating proteolytic activity.
    • Understanding these interactions is crucial for CF pathogenesis research.

    Purpose of the Study:

    • To investigate the protease-binding activity of alpha 2-macroglobulin in patients with cystic fibrosis.
    • To compare binding activity in CF patients, heterozygotes, and individuals with other pulmonary conditions.
    • To determine if a defect in alpha 2-macroglobulin function contributes to cystic fibrosis.

    Main Methods:

    • Measured the binding activity of trypsin to alpha 2-macroglobulin using a functional assay.
    • Assayed samples from 65 cystic fibrosis patients, 41 heterozygotes, 18 children with asthma, 21 COPD patients, and 21 healthy controls.
    • Utilized the substrate N-alpha-benzoyl-arginine-p-nitroanilide to quantify bound trypsin activity.

    Main Results:

    • A moderately increased molar binding ratio of trypsin to alpha 2-macroglobulin was observed in all patient groups and heterozygotes compared to controls.
    • Absolute alpha 2-macroglobulin concentration correlated with age, not disease status.
    • The increased binding activity was not specific to cystic fibrosis, also appearing in heterozygotes and other pulmonary diseases.

    Conclusions:

    • The study provides evidence against a defect in alpha 2-macroglobulin protease-binding activity in cystic fibrosis.
    • The observed increase in binding activity may be due to structural variations or ligand binding, not a CF-specific defect.
    • Findings suggest the altered binding is a common response in various pulmonary conditions and genetic carriers.

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