Alpha-1 Antitrypsin Inclusions Sequester GRP78 in a Bile Acid-Inducible Manner.

doi:10.1111/liv.16207

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Article

Alpha-1 Antitrypsin Inclusions Sequester GRP78 in a Bile Acid-Inducible Manner.

Liver international : official journal of the International Association for the Study of the Liver - 1 Jan 2025

Spivak Igor, Guldiken Nurdan, Usachov Valentyn, Schaap Frank, Damink Steven W M Olde, Bouchecareilh Marion, Lehmann Alexandra, Fu Lei, Mo Fa-Rong, Ensari Gökce Kobazi, Hufnagel Franziska, Fromme Malin, Preisinger Christian, Strnad Pavel

Abstract excerpt

BACKGROUND AND AIMS: The homozygous PiZ mutation (PIZZ genotype) constitutes the predominant cause of severe alpha-1 antitrypsin (AAT) deficiency and leads to liver disease via hepatocellular AAT aggregation. We systematically analysed the composition of AAT aggregates and studied the impact of bile acids. METHODS: AAT inclusions were isolated from livers of PiZ overexpressing mice and PIZZ humans via...

Topics

Endoplasmic Reticulum Chaperone BiP
Animals
Bile Acids and Salts
Humans
alpha 1-Antitrypsin
Heat-Shock Proteins
alpha 1-Antitrypsin Deficiency
Mice
Hepatocytes

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Male

Liver

Female

Inclusion Bodies

Adult

Middle Aged

Mice, Transgenic

Mutation

Source

Open in PubMed Open full text

Identifiers

PMID: 39665869
DOI: 10.1111/liv.16207
PMCID: PMC11636636

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