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It has been proposed that the PI Z variant may aggregate into polymers that precipitate and accumulate in cutaneous soft tissues, leading to inflammation and necrotizing skin lesions ( A1AT is the most abundant serum serine protease inhibitor with anti-protease property ( Figure 1 ) ( Figure 1 Data on the safety of A1AT replacement therapy remain limited due to the rarity of the underlying condition, which currently has only one approved indication for treatment

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