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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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| Main Authors: | , |
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| Format: | Artigo |
| Language: | Inglês |
| Published: |
BMJ Group
2004
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| 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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