Article
FBG1 Is the Final Arbitrator of A1AT-Z Degradation.
PloS one - 1 Jan 2015
Wen John H, Wen Hsiang, Gibson-Corley Katherine N, Glenn Kevin A
Abstract excerpt
Alpha-1 antitrypsin deficiency is the leading cause of childhood liver failure and one of the most common lethal genetic diseases. The disease-causing mutant A1AT-Z fails to fold correctly and accumulates in the endoplasmic reticulum (ER) of the liver, resulting in hepatic fibrosis and hepatocellular carcinoma in a subset of patients. Furthermore, A1AT-Z sequestration in hepatocytes leads to a reduction in A1AT...
Topics
- Animals
- Apoptosis Regulatory Proteins
- Autophagy
- Beclin-1
- Cell Line, Tumor
- Disease Models, Animal
- Endoplasmic Reticulum
- Female
- Gene Expression Regulation
- Half-Life
- Hepatocytes
- Humans