Article
Targeting a surface cavity of alpha 1-antitrypsin to prevent conformational disease.
The Journal of biological chemistry - 29 Aug 2003
Parfrey Helen, Mahadeva Ravi, Ravenhill Neil A, Zhou Aiwu, Dafforn Timothy R, Foreman Richard C, Lomas David A
Abstract excerpt
Conformational diseases are caused by a structural rearrangement within a protein that results in aberrant intermolecular linkage and tissue deposition. This is typified by the polymers that form with the Z deficiency variant of alpha 1-antitrypsin (Glu-342 --> Lys). These polymers are retained within hepatocytes to form inclusions that are associated with hepatitis, cirrhosis, and hepatocellular carcinoma. We...
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