Why Disease Turns Soft Tissue Into Bone?

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2008-12-09 09:17

Scientists have created a new mouse model that may help researchers explain how a rare disease causes otherwise supple soft tissue and joints to turn into bone.

Fibrodysplasia ossificans progressiva (FOP) is a rare congenital disease that affects the connective tissue, causing muscles, ligaments, and other soft tissues to turn to bone. The process of ossification often occurs following traumatic injury to the soft tissue, and can progress over time to cause nearly all joints of the body to become permanently frozen in place. Affected individuals develop problems speaking, breathing, and eating. There is no treatment, and many people who have FOP die in their twenties or thirties.

Using genetically modified mice, Paul Yu and his colleagues have developed a model that mirrors many aspects of the bone-forming disease. The scientists have shown that treating these mice with a drug that blocks the overactive receptor that causes FOP can reduce abnormal bone growth and preserve joint function.

In 2008, Yu received an Early Career Physician-Scientist Award from the Howard Hughes Medical Institute (HHMI) that provides promising physician-scientists with the financial support needed to develop their research programs at the beginning of their academic careers. The award is part of a long-time push by HHMI to increase the number researchers who translate basic science discoveries into improved treatment for patients.

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