Article
Serpinopathies and the conformational dementias.
Nature reviews. Genetics - 1 Oct 2002
Lomas David A, Carrell Robin W
Abstract excerpt
The serpin superfamily of serine proteinase inhibitors has a central role in controlling proteinases in many biological pathways in a wide range of species. The inhibitory function of the serpins involves a marked conformational transition, but this inherent molecular flexibility also renders the serpins susceptible to point mutations that result in aberrant intermolecular linkage and polymer formation. The...
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