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Liver disease in Alpha-1 antitrypsin deficiency (AATD) is caused by the toxic accumulation of mutant Z alpha-1 antitrypsin (Z-AAT) within the endoplasmic reticulum (ER) of hepatocytes. Both liver tissues from AATD patients and livers from PiZ transgenic mice expressing the human Z-AAT exhibit increa...
ORGANISM(S): Mus musculus (Mouse) 
2026-09-21 | PXD066413 | Pride
Alpha-1 Antitrypsin (AAT) is a critical serine protease inhibitor that protects tissue by neutralizing neutrophil elastase and other proteases, particularly in the lungs. Mutations in the SERPINA1 gene lead to Alpha-1 Antitrypsin deficiency (AATD), characterized by low circulating AAT levels and inc...
ORGANISM(S): Mus musculus (Mouse) 
2026-10-05 | PXD065749 | Pride
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