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Article

Pathogenesis and pathology of liver disease associated with alpha 1-antitrypsin deficiency.

Chest - 1 Dec 1996

Massi G

Abstract excerpt

alpha 1-Antitrypsin (alpha 1-AT) accumulates in the rough endoplasmic reticulum through a mechanism of polymerization. Polymerization is favored by the incorrect tertiary structure of the alpha 1-AT caused by a point mutation at position 342 of the protein. Accumulation of alpha 1-AT in the liver cells (and in hepatocytes and colangiocytes) is not sufficient per se to explain the liver disease that is manifested...

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PMID: 8989160
DOI: 10.1378/chest.110.6_supplement.251s

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