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Keywords: antitrypsin
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Clin Sci (Lond) (1994) 86 (5): 489–495.
Published: 01 May 1994
...David A. Lomas 1. The Z deficiency variant of α 1 -antitrypsin predisposes the homozygote to early-onset panlobular emphysema and results in the accumulation of antitrypsin within the hepatocyte, which leads to hepatocellular damage and cirrhosis. The mechanism of this accumulation has been shown...