Phenotype-dependent inhibition of human alpha 1-antitrypsin by 1,6-hexane diamine in vitro.
Blood donors and patients from pulmonary wards gave serum specimens for the assay of their alpha 1-antitrypsin activity and phenotype. The same specimens were then incubated at the room temperature overnight with increasing concentrations of 1,6-hexane diamine. The 5 mM amine concentration caused a significant decrease in heterozygous antitrypsins (M1M2, M1M3, M2M3 and M1S) activity while it was less in the homozygous (M1M1 and M2M2) antitrypsin phenotypes. The SS and ZZ phenotypes showed a very low initial activity which was, however, further reduced. Analysis for the antitrypsin protein showed a simultaneous loss of its activity. The data suggest that heterozygous antitrypsin carriers may be more prone to the effects of inhaled amines despite their 'normal' phenotypic constitution.
Berode
M
M
Institute of Occupational Health Sciences, Lausanne, Switzerland.
Leuenberger
P
P
Savolainen
H
H
D003959:Diamines / Q000494:pharmacology*; D006579:Heterozygote; D006720:Homozygote; D006801:Humans; D066298:In Vitro Techniques; D007525:Isoelectric Focusing; D010641:Phenotype; D000515:alpha 1-Antitrypsin / Q000235:genetics*
D003959:Diamines; D000515:alpha 1-Antitrypsin; C007297:1,6-diaminohexane