TLR4 and Bloom syndrome: Importantly, the administration of hESC-MSC-IMRCs or hESC-MSC-IMRC-CM demonstrated the inhibitory effects on the amelioration of BLM-induced inflammatory responses in a late stage of PF progression (treatment was conducted at d7, d14, and d21 after the first dose of BLM challenge) by suppressing the expression of Tlr4, Myd88, and p-NF-κB and TNF-α protein, as assessed by immunoblotting assay (Figure 7a,b) and immunofluorescence staining of TNF-α (Figure 7c), and the secretion of IL-1β, TNF-α, and IL-6 in lungs of PF mice determined by ELISA (Figure 7d–f).