Hepatocyte proteomes reveal the role of protein disulfide isomerase 4 in alpha 1-antitrypsin deficiency

Esra Karatas,Anne-Aurélie Raymond,Céline Leon,Jean-William Dupuy,Sylvaine Di-Tommaso, Nathalie Senant,Sophie Collardeau-Frachon,Mathias Ruiz,Alain Lachaux,Frédéric Saltel,Marion Bouchecareilh

JHEP Reports（2021）

引用 6|浏览9

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摘要

•PDIA4 is upregulated and involved in alpha 1-antitrypsin deficiency (AATD)-mediated liver disease in adults.•Knockdown of PDIA4 by siRNA or inhibition upon cysteamine treatment leads to improvements in features of AATD.•RNA interference against PDIA4 or cysteamine represent approaches for treatment of AATD-mediated liver disease.

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关键词

Alpha 1-antitrypsin deficiency,Liver damage,Protein disulfide isomerase,PDIA4,Cysteamine,Treatment

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