Effects of etidronate on the Enpp1−/− mouse model of generalized arterial calcification of infancy

Huesa, Carmen; Staines, Katherine A.; Mill�n, Jose Luis; MacRae, Vicky E.

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

Citation

Huesa, C., Staines, K. A., Millán, J. L., & MacRae, V. E. (2015). Effects of etidronate on the Enpp1−/− mouse model of generalized arterial calcification of infancy. International Journal of Molecular Medicine, 36(1), 159-165. https://doi.org/10.3892/ijmm.2015.2212

• Journal Article Type: Article
• Acceptance Date: Apr 22, 2015
• Online Publication Date: May 15, 2015
• Publication Date: 2015-07
• Deposit Date: Jan 20, 2017
• Journal: International Journal of Molecular Medicine
• Print ISSN: 1107-3756
• Electronic ISSN: 1791-244X
• Publisher: Spandidos Publications
• Peer Reviewed: Peer Reviewed
• Volume: 36
• Issue: 1
• Pages: 159-165
• DOI: https://doi.org/10.3892/ijmm.2015.2212
• Keywords: mineralization, vascular calcification, ectonucleotide pyrophosphatase/phosphodiesterase 1, etidronate, generalized arterial calcification of infancy
• Public URL: http://researchrepository.napier.ac.uk/Output/466144