Molecular mechanism of hepatocellular injury in alpha1 antitrypsin deficiency
β Scribed by Frederick K. Askari
- Publisher
- John Wiley and Sons
- Year
- 1995
- Tongue
- English
- Weight
- 387 KB
- Volume
- 21
- Category
- Article
- ISSN
- 0270-9139
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β¦ Synopsis
Liver injury in PiZZ cY,-antitrypsin &-AT) deficiency probably results from toxic effects of the abnormal a,-
AT molecule accumulating within the ER of liver cells.
π SIMILAR VOLUMES
In recent years, we have witnessed several important paradigm shifts in understanding the molecular basis of liver disease in alpha-1-antitrypsin (AT) deficiency. These shifts have become possible as a result of a number of advances in research on the cell biology of aggregation-prone mutant protein
alpha-1-antitrypsin; PiZZ, a1-ATZ phenotype; ER, endoplasmic acts with the target P 1 -P 1 residues of the reactive center loop, reticulum; MHC, major histocompatibility complex. the loop inserts further into the gap in the A sheet, generating From the: Departments of Pediatrics, Cell Biology, and
β£-1-Antitrypsin (β£1-AT) deficiency is the most common cause of metabolic pediatric liver disease. Hepatocellular injury is caused by toxicity of the mutant β£-1-antitrypsin Z (β£1-ATZ) molecule retained within hepatocytes. In these studies, we used the PiZ transgenic mouse model of β£1-AT deficiency to
Alpha-1 antitrypsin (AAT; HUGO symbol, SERPINA1) is one of the major serine protease inhibitors (serpins) in human plasma. Deficiency of AAT is a recognised risk factor for chronic obstructive pulmonary disease (COPD), attributed to uninhibited neutrophil elastase released into the lung tissue durin