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- A Recombinant Human [alpha]_1-Antitrypsin Variant, M_malton, Undergoes a Spontaneous Conformational Conversion into a Latent Form
- Chan-Hun Jung , Hana Im
- J. Microbiol. 2003;41(4):335-339.
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Abstract
- Many genetic variants of [alpha]_1-antitrypsin have been associated with early onset emphysema and liver cirrhosis. However, the detailed structural basis of pathogenic [alpha]_1-antitrypsin molecules is rarely known. Here we found that a recombinant M_malton variant (Phe52-deleted) lost inhibitory activity by spontaneous conformational conversion into a more stable, inactive form under physiological conditions. Biochemical and spectroscopic data suggested that the variant converts into a reactive center loop-inserted conformation, resembling the latent form of plasminogen activator inhibitor-1.
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