[HTML][HTML] 6-mer peptide selectively anneals to a pathogenic serpin conformation and blocks polymerization: implications for the prevention of Z α1-antitrypsin-related …
R Mahadeva, TR Dafforn, RW Carrell… - Journal of Biological …, 2002 - ASBMB
Conformational diseases such as amyloidosis, Alzheimer's disease, prion diseases, and the
serpinopathies are all caused by structural rearrangements within a protein that transform it
into a pathological species. These diseases are typified by the Z variant of α 1-antitrypsin
(E342K), which causes the retention of protein within hepatocytes as inclusion bodies that
are associated with neonatal hepatitis and cirrhosis. The inclusion bodies result from the Z
mutation perturbing the conformation of the protein, which facilitates a sequential interaction …
serpinopathies are all caused by structural rearrangements within a protein that transform it
into a pathological species. These diseases are typified by the Z variant of α 1-antitrypsin
(E342K), which causes the retention of protein within hepatocytes as inclusion bodies that
are associated with neonatal hepatitis and cirrhosis. The inclusion bodies result from the Z
mutation perturbing the conformation of the protein, which facilitates a sequential interaction …