ENPP1-Fc prevents mortality and vascular calcifications in rodent model of generalized arterial calcification of infancy

Ronald A. Albright, Paul Stabach, Wenxiang Cao, Dillon Kavanagh, Isabelle Mullen, Alexander A. Braddock, Mariel S. Covo, Martin Tehan, Guangxiao Yang, Zhiliang Cheng, Keith Bouchard, Zhao-Xue Yu, Stephanie Thorn, Xiangning Wang, Ewa J. Folta-Stogniew, Alejandro Negrete, Albert J. Sinusas, Joseph Shiloach, George Zubal, Joseph A. Madri, Enrique M. De La Cruz and Demetrios T. Braddock ()
Additional contact information
Ronald A. Albright: Yale University School of Medicine
Paul Stabach: Yale University School of Medicine
Wenxiang Cao: Yale University
Dillon Kavanagh: Yale University School of Medicine
Isabelle Mullen: Yale University School of Medicine
Alexander A. Braddock: Yale University School of Medicine
Mariel S. Covo: Yale University School of Medicine
Martin Tehan: Yale University School of Medicine
Guangxiao Yang: Yale University School of Medicine
Zhiliang Cheng: Alexion
Keith Bouchard: Alexion
Zhao-Xue Yu: Alexion
Stephanie Thorn: Yale University School of Medicine
Xiangning Wang: Yale University School of Medicine
Ewa J. Folta-Stogniew: WM Keck Biotechnology Research Laboratory, Yale University School of Medicine
Alejandro Negrete: Biotechnology Core Laboratory, National Institute of Diabetes and Digestive Kidney Diseases, National Institutes of Health
Albert J. Sinusas: Yale University School of Medicine
Joseph Shiloach: Biotechnology Core Laboratory, National Institute of Diabetes and Digestive Kidney Diseases, National Institutes of Health
George Zubal: Z-Concepts LLC
Joseph A. Madri: Yale University School of Medicine
Enrique M. De La Cruz: Yale University
Demetrios T. Braddock: Yale University School of Medicine

Nature Communications, 2015, vol. 6, issue 1, 1-11

Abstract: Abstract Diseases of ectopic calcification of the vascular wall range from lethal orphan diseases such as generalized arterial calcification of infancy (GACI), to common diseases such as hardening of the arteries associated with aging and calciphylaxis of chronic kidney disease (CKD). GACI is a lethal orphan disease in which infants calcify the internal elastic lamina of their medium and large arteries and expire of cardiac failure as neonates, while calciphylaxis of CKD is a ubiquitous vascular calcification in patients with renal failure. Both disorders are characterized by vascular Mönckeburg’s sclerosis accompanied by decreased concentrations of plasma inorganic pyrophosphate (PPi). Here we demonstrate that subcutaneous administration of an ENPP1-Fc fusion protein prevents the mortality, vascular calcifications and sequela of disease in animal models of GACI, and is accompanied by a complete clinical and biomarker response. Our findings have implications for the treatment of rare and common diseases of ectopic vascular calcification.

Date: 2015
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DOI: 10.1038/ncomms10006

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