INTERNATIONAL JOURNAL OF MOLECULAR MEDICINE 36: 159-165, 2015
Effects of etidronate on the Enpp1-/- mouse model of generalized arterial calcification of infancy CARMEN HUESA1*, KATHERINE A. STAINES1*, JOSE LUIS MILLÁN2 and VICKY E. MacRAE1 1
Roslin Institute and R(D)SVS, The University of Edinburgh, Edinburgh, UK; Sanford Children's Health Research Center, Sanford-Burnham Medical Research Institute, La Jolla, CA, USA
2
Received February 27, 2015; Accepted April 22, 2015 DOI: 10.3892/ijmm.2015.2212 Abstract. Generalized arterial calcification of infancy (GACI) is an autosomal recessive disorder of spontaneous infantile arterial and periarticular calcification which is attributed to mutations in the ectonucleotide pyrophosphatase/phosphodiesterase 1 (Enpp1) gene. Whilst the bisphosphonate, etidronate, is currently used off-label for the treatment for GACI, recent studies have highlighted its detrimental effects on bone mineralisation. In the present study, we used the Enpp1-/mouse model of GACI to examine the effects of etidronate treatment (100 µg/kg), on vascular and skeletal calcification. Micro-computed tomography (µCT) analysis revealed a significant decrease in trabecular bone mass, as reflected by the decrease in trabecular bone volume/tissue volume (BV/ TV; %), trabecular thickness, trabecular separation, trabecular number and pattern factor (P
Effects of etidronate on the Enpp1-/- mouse model of generalized arterial calcification of infancy CARMEN HUESA1*, KATHERINE A. STAINES1*, JOSE LUIS MILLÁN2 and VICKY E. MacRAE1 1
Roslin Institute and R(D)SVS, The University of Edinburgh, Edinburgh, UK; Sanford Children's Health Research Center, Sanford-Burnham Medical Research Institute, La Jolla, CA, USA
2
Received February 27, 2015; Accepted April 22, 2015 DOI: 10.3892/ijmm.2015.2212 Abstract. Generalized arterial calcification of infancy (GACI) is an autosomal recessive disorder of spontaneous infantile arterial and periarticular calcification which is attributed to mutations in the ectonucleotide pyrophosphatase/phosphodiesterase 1 (Enpp1) gene. Whilst the bisphosphonate, etidronate, is currently used off-label for the treatment for GACI, recent studies have highlighted its detrimental effects on bone mineralisation. In the present study, we used the Enpp1-/mouse model of GACI to examine the effects of etidronate treatment (100 µg/kg), on vascular and skeletal calcification. Micro-computed tomography (µCT) analysis revealed a significant decrease in trabecular bone mass, as reflected by the decrease in trabecular bone volume/tissue volume (BV/ TV; %), trabecular thickness, trabecular separation, trabecular number and pattern factor (P
