Figure 5.

Targeting C-X-C chemokine receptor 4 (CXCR4) using AMD3100 ameliorates lung remodeling in nonobese diabetic severe combined immunodeficiency γ (NSG) mice that were injected with lung explant cells isolated from a patient with rapidly or slowly progressing idiopathic pulmonary fibrosis (IPF). A total of 5 × 10⁵ biobanked slowly or rapidly progressing IPF lung explant cells were injected intravenously into NSG mice. At day 35 after injection, one group of mice were injected daily with 5 mg/kg of AMD3100 (5 days per week) for an additional 4 weeks (63 days total), and the mice were then sacrificed for histologic and biochemical analysis. A–E: Representative Masson's trichrome staining of naive NSG mouse lung (A) or lungs from NSG mice injected with explant cells from a patient with slowly progressing IPF (B and C) or from a patient with rapidly progressing IPF (D and E) untreated (Unt) (B and E) or AMD3100 treated (C and F) for 4 weeks. The mean hydroxyproline (HYP) content in naive and slowly progressing (D) or rapidly progressing (G) groups Unt or therapeutically treated with AMD3100. n = 5 per group (A–G). ^∗P < 0.05, ^∗∗P < 0.01 (unpaired parametric t-test). Original magnification: ×50 (A–C, E, and F).