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Mechanisms Underlying the Cellular Clearance of Antitrypsin Z: Lessons from Yeast Expression Systems

The most frequent cause of α(1)-antitrypsin (here referred to as AT) deficiency is homozygosity for the AT-Z allele, which encodes AT-Z. Such individuals are at increased risk for liver disease due to the accumulation of aggregation-prone AT-Z in the endoplasmic reticulum of hepatocytes. However, th...

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Autori principali: Gelling, Cristy L., Brodsky, Jeffrey L.
Natura: Artigo
Lingua:Inglês
Pubblicazione: American Thoracic Society 2010
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Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC3136956/
https://ncbi.nlm.nih.gov/pubmed/21030514
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1513/pats.201001-007AW
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