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α(1)-Antitrypsin deficiency • 4: Molecular pathophysiology

The molecular basis of α(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 α(1)-antitrypsin and to develop thera...

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Bibliographic Details
Main Authors: Lomas, D, Parfrey, H
Format: Artigo
Language:Inglês
Published: BMJ Group 2004
Subjects:
Online Access:https://ncbi.nlm.nih.gov/pmc/articles/PMC1747018/
https://ncbi.nlm.nih.gov/pubmed/15170041
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1136/thx.2003.006528
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