Alpha1-antitrypsin deficiency. 4: Molecular pathophysiology.
review · Level V
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- Record sourced from PubMed, PMID 15170041.
- Also identified by PMC identifier 1747018.
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Abstract
The molecular basis of alpha(1)-antitrypsin deficiency is reviewed and is shown to be due to the accumulation of mutant protein as ordered polymers within the endoplasmic reticulum of hepatocytes. The current goals are to determine the cellular response to polymeric alpha(1)-antitrypsin and to develop therapeutic strategies to block polymerisation in vivo.
Medical subject headings
- Liver Diseases
- Lung Diseases
- alpha 1-Antitrypsin
- alpha 1-Antitrypsin Deficiency