Plasminogen activator inhibitor-2 is expressed in different types of congenital ichthyosis:<i>in vivo</i>evidence for its cross-linking into the cornified cell envelope by transglutaminase-1

Abstract

We provide in vivo evidence that PAI-2 is a substrate of TGase-1. The normal expression of PAI-2 in a large group of TGase-1-proficient LI/NCIE patients makes it rather unlikely that PAI-2 alone is a primary molecular cause of LI/NCIE.