Carmen Huesa
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.
Authors
Katherine A. Staines
Jose Luis Mill�n
Vicky E. MacRae
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 |
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