Fig. 2: Osteoidosis and increased expression of pyrophosphate (PPi)-related genes associated with murine neurofibromas.
From: A molecular basis for neurofibroma-associated skeletal manifestations in NF1
(a) Representative images of von Kossa/van Gieson-stained (top) and S100+ nerves (bottom) thoracic vertebral sections from control and Plp-CreERT; Nf1flox/flox mice. N: normal nerve, T: plexiform tumor adjacent to vertebral bone. Osteoid (nonmineralized bone) is stained pink (white arrows) and mineralized bone is stained black. Bar: 50 µm. (b) Osteoid surfaces/bone surfaces (OS/BS), osteoid volume/bone volume (OV/BV) and osteoid width (O. Wi.) in vertebral cancellous bone regions adjacent to normal nerves or neurofibroma (NF) tumors in control and Plp-creERT;Nf1flox/flox (cKO) mice (wild type [WT]: n = 6 vertebral bodies from 3 mice), cKO without adjacent tumor (−, n = 9 vertebral bodies from 4 mice) and adjacent to tumor (+, n = 7 vertebral bodies from 4 mice). *p < 0.05, one-way analysis of variance (ANOVA) and post hoc pair-wise comparison with Bonferroni adjustment. (c, d) Normalized expression levels in neurofibromas from (c) Dhh-cre;Nf1flox/flox (n = 15) and control (normal sciatic nerve, n = 15) mice (microarray) and (d) from Periostin-cre; Nf1flox/flox (n = 6) and control (trigeminal nerve, n = 6) mice (RNAseq). *p < 0.05; **p < 0.01 by two-sided t-test.
