[HTML][HTML] The endoplasmic reticulum degradation pathway for mutant secretory proteins α1-antitrypsin Z and S is distinct from that for an unassembled membrane …
JH Teckman, DH Perlmutter - Journal of Biological Chemistry, 1996 - ASBMB
We have theorized that a subset of PiZZ α1-antitrypsin (α1-AT)-deficient individuals is more
susceptible to liver injury by virtue of second inherited trait (s) or environmental factor (s),
which exaggerate the accumulation of mutant α1-AT Z within the endoplasmic reticulum
(ER) of liver cells. Using a complementation approach in which cell lines from PiZZ
individuals with liver disease (" susceptible" hosts) and from PiZZ individuals without liver
disease (" protected" hosts) are transduced with the mutant α1-AT Z gene, we have recently …
susceptible to liver injury by virtue of second inherited trait (s) or environmental factor (s),
which exaggerate the accumulation of mutant α1-AT Z within the endoplasmic reticulum
(ER) of liver cells. Using a complementation approach in which cell lines from PiZZ
individuals with liver disease (" susceptible" hosts) and from PiZZ individuals without liver
disease (" protected" hosts) are transduced with the mutant α1-AT Z gene, we have recently …