Article
Constitutive activation of MEK1 in osteoprogenitors increases strength of bone despite impairing mineralization.
Bone - 1 Jan 2020
Fowlkes John L, Bunn R Clay, Ray Philip D, Kalaitzoglou Evangelia, Uppuganti Sasidhar, Unal Mustafa, Nyman Jeffry S, Thrailkill Kathryn M
Abstract excerpt
Recent clinical studies have revealed that a somatic mutation in MAP2K1, causing constitutive activation of MEK1 in osteogenic cells, occurs in melorheostotic bone disease in humans. We have generated a mouse model which expresses an activated form of MEK1 (MEK1DD) specifically in osteoprogenitors postnatally. The skeletal phenotype of these mice recapitulates many features of melorheostosis observed in humans,...
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