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Receptor Partners Alter Amplitude of BMP Signals in FOP Cells

An important paper was published from the FOP
laboratory in 2007 documenting the role of cell surface sugar-
protein complexes (called heparan sulfate proteoglycans or
HSPGs) in modulating the activity of the mutant FOP receptor.

The paper, "HSPG Modulation of BMP Signaling in FOP Cells,"
was published in The Journal of Cellular Biochemistry. The
significance of the study is that although the FOP mutation causes
tremendous harm, FOP cells attempt to modulate the harm, albeit
ineffectively. Thus, FOP cells try to block the abnormal signal
using HSPGs, but cannot quite muster the resources to do it.
These findings provide important insight into the body's ability
to buffer harm from mutations in signaling pathways and provide
avenues that might be exploited in developing drugs to prevent or
treat FOP.

Dr. Kaplan (far left), Dr. Shore (third from left) and
Ruth McCarrick- Walmsley (far right) meet with the
Henke family during a visit to the FOP Lab.
Justin Henke is at the microscope.

The ACVR1 gene and the associated BMP signaling pathways
have evolved over 500 million years, and are hard- wired
into cells. Thus, there has been ample time for the cell to
figure-out ways to bypass attempts to block it. With these caveats
in mind, we have been conducting experiments in conjunction
with research scientists at a major pharmaceutical company to
identify possible detours that could frustrate drug development.
So, FOP cells beware; we have discovered some of your little
tricks, and we are on to you!