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Novel Treatment Strategies for Liver Disease Due to a1-Antitrypsin Deficiency

โœ Scribed by Nicholas Maurice; David H. Perlmutter


Book ID
114902151
Publisher
Wiley (Blackwell Publishing)
Year
2012
Tongue
English
Weight
702 KB
Volume
5
Category
Article
ISSN
1752-8054

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โœ David A. Rudnick; David H. Perlmutter ๐Ÿ“‚ Article ๐Ÿ“… 2005 ๐Ÿ› John Wiley and Sons ๐ŸŒ English โš– 867 KB

Liver disease in alpha-1-antitrypsin (alpha1AT) deficiency is caused by a gain-of-toxic function mechanism engendered by the accumulation of a mutant glycoprotein in the endoplasmic reticulum (ER). The extraordinary degree of variation in phenotypical expression of this liver disease is believed to