Fig. 2 | Bone Research

Fig. 2

From: Extra-skeletal manifestations in mice affected by Clcn7-dependent autosomal dominant osteopetrosis type 2 clinical and therapeutic implications

Fig. 2

Lung, kidney, and muscle phenotype. Lungs, kidneys, and muscles were harvested from 12-month-old WT and ADO2 CD1 male mice. a Analysis of fibrosis gene signature in RNA-dSeq data sets of 1-month-old WT and ADO2 C57BL6/J male mice lungs. NES normalized enrichment score. b Masson’s trichrome staining of WT and ADO2 lung sections. White arrows: perivascular collagen (blue) accumulation. V: vessel. c Quantification of perivascular collagen. d Immunofluorescence analyses of ClC7 (green) and e the macrophage marker F4/80 (purple) in WT and ADO2 lung sections. N·mm−2, number per mm2. f Real time RT-PCR for the indicated genes in WT and ADO2 lungs. g Immunofluorescence analysis of phospho (p)-Smad2/3 (red) in WT and ADO2 lung sections. h Enrichment plots for fibrosis gene signature in ADO2 kidneys. NES normalized enrichment score. i Masson’s trichrome staining of WT and ADO2 kidney sections. White arrows: perivascular collagen (blue) accumulation. V: vessel. j Quantification of perivascular collagen. k Immunohistochemistry for ClC7 (brown) in WT and ADO2 kidney sections. l Immunofluorescence analysis of the macrophage marker F4/80 (purple) in WT and ADO2 kidney sections. m Real time RT-PCR for the indicated genes in WT and ADO2 kidneys. n Immunofluorescence analysis of p-Smad2/3 (red) in WT and ADO2 kidney sections. o Enrichment plots of fibrosis gene signature in ADO2 muscles. NES normalized enrichment score. p Masson’s trichrome staining of WT and ADO2 muscle sections. White arrows: perivascular collagen (blue) accumulation. V: vessel. q Quantification of perivascular collagen. r Immunohistochemistry for ClC7 (brown) in WT and ADO2 muscle sections. s Immunofluorescence analysis for the macrophage marker F4/80 (purple) in WT and ADO2 muscle sections. t Real time RT-PCR for the indicated genes in WT and ADO2 muscles. u Immunofluorescence analysis of p-Smad1/2 (red) in WT and ADO2 muscle sections. Nuclei are stained in blue with DAPI. Images are representative, and data are the mean ± S.D of five mice per group (Student’s t test)

Back to article page