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AATD-associated liver disease (AATD-LD) is primarily caused by SERPINA1 protease inhibitor (Pi)*Z variants, of which the homozygous variant Pi*ZZ carries the greatest risk

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In DDC/LA mice, there was a significant increase in Nr5a2 ( LRH1) activation and binding of Nr5a2 to Abcg5/8 and Nr0b2 promoters