Next, we evaluated the pathological relevance of USP11 in vivo by conducting experiments with BLM-induced PF in mice (dose 1mg/kg) 38.The immunostaining analysis showed a significant increase in α-SMA in lung tissue from BLM-treated mice, indicating a fibrotic response (Figure 8A; left panel). The gene discussed is ACTA1; the disease is pemphigus foliaceus.