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In the classical form of α1antitrypsin deficiency a mutant protein accumulates in a polymerized form in the ER of liver cells causing liver damage and carcinogenesis by a gain-of-toxic function mechanism. Recent studies have indicated that the accumulation of mutant α1antitrypsin Z in the ER spec...
ORGANISM(S): Mus musculus 
In the classical form of α1antitrypsin deficiency a mutant protein accumulates in a polymerized form in the ER of liver cells causing liver damage and carcinogenesis by a gain-of-toxic function mechanism. Recent studies have indicated that the accumulation of mutant α1antitrypsin Z in the ER spec...
ORGANISM(S): Mus musculus 
Alpha1-Antitrypsin Deficiency Registry (AADR-BioLINCC)
Alpha1-Antitrypsin Deficiency Registry (AADR-BioLINCC)
Mice inflammatory responses to inhaled aerosolized LPS: effects of various forms of human alpha1-antitrypsin
Characterize how Balb/c mice responses to aerosolized LPS (lipopolysaccharide) alone or with intraperitoneal (i.p.) delivery of alpha1-antitrypsin (AAT)
ORGANISM(S): Mus musculus 
2022-09-30 | GSE214249 | GEO
α-1-antitrypsin (AAT) regulates protease activity in the lungs and liver. The presence of one or more copies of a mutant Z allele (AAT (E342K), called ATZ), results in low serum levels of ATZ along with intracellular inclusions of polymeric forms, causing lung emphysema and liver cirrhosis. Our expe...
ORGANISM(S): Homo sapiens (Human) 
2020-09-24 | PXD020562 | Pride
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