Cellular Consequences of Z Alpha-1 Antitrypsin Accumulation and Prospects for Therapy
The Z variant (Glu342Lys) of α1-antitrypsin polymerises and accumulates in the hepatocyte endoplasmic reticulum predisposing to neonatal hepatitis and liver cirrhosis. The resultant secretory defect leaves the lungs vulnerable to elastolysis and early-onset emphysema. There is currently no cure for...
| Main Authors: | , |
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| Format: | Article |
| Language: | English |
| Published: |
European Medical Journal
2013-12-01
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| Series: | European Medical Journal Hepatology |
| Subjects: | |
| Online Access: | http://emjreviews.com/wp-content/uploads/6.-SamAlam-etal..pdf |