Interrelationships between serum chemotactic factor inactivator, alpha 1-antitrypsin deficiency, and chronic obstructive lung disease.
Serum chemotactic factor inactivator (CFI) activity was quantitated in 22 subjects with chronic airflow obstruction (CAO); 8 had normal antitrypsin levels (Pi M phenotype), 6 had intermediate antitrypsin deficiency (Pi MZ phenotype), and 8 had severe antitrypsin deficiency (Pi Z phenotype). We studied 19 healthy subjects with normal lung function as controls; 3 had Pi M and 6 had Pi MZ phenotypes. Subjects with CAO, irrespective of the alpha 1-antitrypsin (AAT) phenotype, had significantly lower CFI activity than the normal subjects; the lowest levels were found in those with CAO and Pi Z phenotype. Normal subjects with intermediate AAT deficiency and MZ phenotype had normal levels of CFI. The results suggest that deficiency of serum CFI may be important in the pathogenesis of chronic airflow obstruction, particularly in those with severe AAT deficiency.
D000328:Adult; D000368:Aged; D000626:Aminopeptidases; D002630:Chemotactic Factors / Q000037:antagonists & inhibitors* / Q000097:blood; D002634:Chemotaxis, Leukocyte; D005260:Female; D006801:Humans; D008173:Lung Diseases, Obstructive / Q000097:blood* / Q000150:complications / Q000175:diagnosis; D008297:Male; D008875:Middle Aged; D010641:Phenotype; D012129:Respiratory Function Tests; D000515:alpha 1-Antitrypsin / Q000097:blood; D019896:alpha 1-Antitrypsin Deficiency
D002630:Chemotactic Factors; D000515:alpha 1-Antitrypsin; C010073:chemotactic factor inactivator; D000626:Aminopeptidases