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UTHealth researchers gain insights into severe form of dwarfism
Date:9/3/2014

after birth (which equates to about four years in humans), a large number of chondrocyte cells have died and symptoms worsen. They also found inflammation in the growth plate and cartilage of the joints, suggesting the beginning of osteoarthritis.

To determine if there was a way to reduce the effects of the disorder at its earliest stages, the researchers administered three different medicationslithium, phenylbutyric acid and valproate. They found that the drugs successfully lessened the damage to chondrocyte cells in the growth plate, but each drug resulted in significant side effects. Nevertheless, the results were promising.

"Although these drugs in particular are not viable treatment options, our findings do provide a foundation for the development of a therapy that would reduce inflammation in the growth plate chondrocytes," Posey said. "We also identified an optimal treatment windowstarting around age 2, when most of the cells in the growth plates are still viable and widespread cell death has not yet occurred. Once growth plate chondrocytes have been depleted, generally around age 4, treatments likely would have little effect." Posey said they are now studying other medications.

This work builds on previous research by the paper's senior author Jacqueline T. Hecht, Ph.D., associate dean of research for the UTHealth School of Dentistry, professor and director of the Pediatric Research Center and vice chair for research in the Department of Pediatrics at the UTHealth Medical School. She also serves on the faculty of The University of Texas Graduate School for Biomedical Sciences at Houston. A team led by Hecht discovered the defective COMP gene in 1995.


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Contact: Deborah Mann Lake
deborah.m.lake@uth.tmc.edu
University of Texas Health Science Center at Houston
Source:Eurekalert  

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