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.
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.
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