Research NewsGenetic Disease

Protein Builds Second Skeleton

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Science  30 Aug 1996:
Vol. 273, Issue 5279, pp. 1170
DOI: 10.1126/science.273.5279.1170


The villain in a rare inherited disease that relentlessly converts the body's soft connective tissues into bone lurks within the victims' own immune systems, researchers have found. In the disease, called fibrodysplasia ossificans progressiva, the slightest injury to ligaments, tendons, or muscles ultimately causes new bone to appear at the site of the injury. The reason: The white blood cells that invade the tissue in response to injury erroneously manufacture bone morphogenic protein-4 (BMP-4), a powerful signaling protein that ordinarily helps build the skeleton of the developing embryo.