Translational research - Paper in AJP just accepted

After many years of work on cardiovascular calcification - from the phenotype to causal gene identification and understanding of the underlying patho-mechanisms - our recent paper describes how the underlying causal gene Abcc6 leads to a deficiency of its protein product and as a result to reduced inorganic pyrophosphate (PPi) in the plasma of mice. 
This deficincy predisposes mice to calcification as well as to Pseudoxamthoma Elasticum (PXE) in human patients.



In the just accepted paper we demonstrate how to rescue the phenotype by supplementing mice with PPi or its analogue Etidronate, a drug used to treat osteoporosis.

Thanks to Olivier and Chris for the many successful years of collaboration between both groups in Lübeck and Hawaii. 





ZA


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