[HTML][HTML] Folding and stability of the Z and Siiyama genetic variants of human α1-antitrypsin
HA Kang, KN Lee, MH Yu - Journal of Biological Chemistry, 1997 - ASBMB
Z (Glu 342→ Lys) and S iiyama (Ser 53→ Phe) genetic variations of human α 1-antitrypsin (α
1-AT) cause a secretion blockage in the hepatocytes, leading to α 1-AT deficiency in the
plasma. Using in vitro folding analysis, we have shown previously that these mutations
interfere with the proper folding of polypeptides. To understand the fundamental cause for
the secretion defect of the Z and S iiyama variants of α 1-AT, we investigated in vivo folding
and stability of these variant α 1-AT using the secretion system of yeast Saccharomyces …
1-AT) cause a secretion blockage in the hepatocytes, leading to α 1-AT deficiency in the
plasma. Using in vitro folding analysis, we have shown previously that these mutations
interfere with the proper folding of polypeptides. To understand the fundamental cause for
the secretion defect of the Z and S iiyama variants of α 1-AT, we investigated in vivo folding
and stability of these variant α 1-AT using the secretion system of yeast Saccharomyces …
