Cellular Consequences of Z Alpha-1 Antitrypsin Accumulation and Prospects for Therapy - European Medical Journal

Continue

Cellular Consequences of Z Alpha-1 Antitrypsin Accumulation and Prospects for Therapy

Hepatology
Download PDF
Authors:
Sam Alam,1 Ravi Mahadeva2

1. Senior Post Doctoral Research Fellow, Department of Medicine, University of Cambridge, Cambridge UK
2. Director Cambridge COPD Centre, Cambridge University Hospitals Foundation Trust, Associate Lecturer, Department of Medicine, University of Cambridge, UK

Disclosure:

No potential conflict of interest.

Citation:
EMJ Hepatol. ;1:44-51. DOI/10.33590/emjhepatol/10314058. https://doi.org/10.33590/emjhepatol/10314058.
Keywords:
Alpha-1 Antitrypsin, Emphysema, Liver Disease, Polymerisation

Each article is made available under the terms of the Creative Commons Attribution-Non Commercial 4.0 License.

Abstract

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 the liver or lung disease other than organ transplantation. This review discusses the evolving understanding of the molecular pathogenesis of the condition and how this has led to the emergence of novel treatment strategies for α1- antitrypsin-related liver disease.

Please view the full content in the pdf above.

Rate this content's potential impact on patient outcomes

Average rating / 5. Vote count:

No votes so far! Be the first to rate this content.