Inhibition of intracellular degradation increases secretion of a mutant form of alpha1-antitrypsin associated with profound deficiency.

doi:10.1172/JCI549

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Article

Inhibition of intracellular degradation increases secretion of a mutant form of alpha1-antitrypsin associated with profound deficiency.

The Journal of clinical investigation - 15 Jun 1998

Novoradovskaya N, Lee J, Yu Z X, Ferrans V J, Brantly M

Abstract excerpt

The mutant Z form of alpha1-antitrypsin (alpha1AT) is responsible for > 95% of all individuals with alpha1AT deficiency, an important inherited cause of emphysema and liver disease. Since secreted Z alpha1AT is a functional antiprotease, we hypothesized that interrupting catabolism of retained Z...

Topics

Animals
Biological Transport
CHO Cells
Cricetinae
Cytoplasmic Granules
Humans
Macrophages, Alveolar
Mutation
Transfection
alpha 1-Antitrypsin

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