Ontology highlight
ABSTRACT:
ORGANISM(S): Mus musculus
SUBMITTER: Tunda Hidvegi
PROVIDER: E-GEOD-8599 | biostudies-arrayexpress |
REPOSITORIES: biostudies-arrayexpress
The Journal of biological chemistry 20070717 38
In the classical form of alpha(1)-antitrypsin deficiency, a mutant protein accumulates in a polymerized form in the endoplasmic reticulum (ER) of liver cells causing liver damage and carcinogenesis by a gain-of-toxic function mechanism. Recent studies have indicated that the accumulation of mutant alpha(1)-antitrypsin Z in the ER specifically activates the autophagic response but not the unfolded protein response and that autophagy plays a critical role in disposal of insoluble alpha(1)-antitryp ...[more]