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Basics of NUT Midline
Carcinoma


What Causes NUT Midline Carcinoma

Treatment

What Can Families Do


What Causes NUT Midline Carcinoma

As its name implies, NUT midline carcinoma, at least in part, is caused by the NUT gene. However, NUT alone does not cause cancer as far as we know. In NMC, NUT is joined to another gene, most often BRD4, to form a hybrid gene, BRD4-NUT. The protein product, BRD4-NUT, like the mythological Sphinx, does not behave like each component, BRD4 or NUT, would alone. Instead, when joined these proteins act like an entirely different beast, working together to cause cancerous transformation of the cells which harbor them. A unique feature of NMC is the mechanism by which BRD4-NUT causes the cancer to grow. Normally, tissue that makes up our organs forms by the differentiation of rapidly dividing “progenitor cells” into non-proliferative, mature cells which perform the functions of the working organ. If the progenitor cells did not differentiate, they would continue to grow and eventually form a tumor. We have recently shown that BRD4-NUT works by blocking the ability of progenitor cells to differentiate into mature tissue. This results in the uncontrolled growth of progenitor cells which are arrested in a state of perpetual proliferation.

A burning question of many patients and families is “why me”? The formation of NUT-hybrid genes appears to be a sporadic unprovoked event and does not appear to be associated with any specific environmental exposure, such as infection or carcinogen. One goal of the Registry is to better understand NMC by determining common features among patients afflicted with this disease. To try to fully understand the causes of the development of this cancer, we plan to define all the changes that occur during the transformation from a healthy cell to a NUT midline carcinoma cell.

If you would like more detailed information, feel free to read the Doctors section of this website.


 
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