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Science 9 October 1987:
Vol. 238. no. 4824, pp. 209 - 211
DOI: 10.1126/science.2958938
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Articles

Science, Vol 238, Issue 4824, 209-211
Copyright © 1987 by American Association for the Advancement of Science

articles

Alpha 2-antiplasmin Enschede: alanine insertion and abolition of plasmin inhibitory activity

WE Holmes, HR Lijnen, L Nelles, C Kluft, HK Nieuwenhuis, DC Rijken, and D Collen

Center for Thrombosis and Vascular Research, University of Leuven, Belgium.

An abnormal alpha 2-antiplasmin that is associated with a serious bleeding tendency has been found in a Dutch family and is referred to as alpha 2-antiplasmin Enschede. This abnormal alpha 2-antiplasmin is converted from an inhibitor of plasmin to a substrate. The molecular defect of alpha 2-antiplasmin Enschede, as revealed by sequencing of cloned genomic DNA fragments, consists of an alanine insertion near the active site region of the molecule. Substitution of this fragment into complementary DNA for a wild-type alpha 2-antiplasmin yields a translation product with physical and functional properties typical of the abnormal alpha 2-antiplasmin Enschede. The naturally occurring mutant may serve as a model for investigating the structures that determine the properties of an inhibitor versus those of a substrate in serine protease inhibitors.


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Science. ISSN 0036-8075 (print), 1095-9203 (online)