Malignant rhabdoid tumors are highly aggressive tumors that attack the brain or the kidneys and occur
primarily in children under two years old. There are about 20 to 25 new
cases diagnosed each year, suggested the Dana-Farber Cancer
Little is known about these tumors, so finding a cure is incredibly
difficult. Researchers investigating the disease are focused on learning
how the body functions and why these tumors occur at all.
‘How a complex protein called Snr1, the homologue of human SMARCB1/hSNF5/INI1, acts as a tumor suppressor in an unconventional manner in fruit flies has been described by researchers.’
A team of Florida State University researchers has discovered that a
common household pest can tell us a lot about an aggressive tumor that
attacks young children.
Professor of Biological Science Wu-Min Deng and postdoctoral researcher Gengqiang Xie published a new paper in the journal Cancer Research
that describes how a complex protein called Snr1, the homologue of
human SMARCB1/hSNF5/INI1, acts as a tumor suppressor in an
unconventional manner in fruit flies.
Fruit flies are often used as a model to determine the basic
fundamentals of several diseases in humans, including cancer, because
about 75% of human disease-causing genes have a counterpart in
"Basically, we used a fly's imaginal tissue, composed of cells
similar to human epithelia, to understand this cancer gene," Xie said.
"There's no treatment for this cancer, so we need to understand how it
Xie and Deng are specifically looking at malignant rhabdoid tumors. In studying the fruit flies, Xie and Deng homed in on a specific
protein that was generally known as part of a larger protein complex
crucial to normal growth and development. They found this protein, which
is analogous to one in humans, was molecularly and functionally
different than other components of the protein complex.
In carefully orchestrated experiments, researchers removed that
protein from several fruit flies. When they did this, the fruit flies
immediately experienced tumor growth in the tissues that line the
"It really has a lot of potential because if you want to find
treatments, you need to understand how it works," Deng said. "We knew
this information would be very useful in understanding human tumor
Deng and Xie hope to further investigate this protein to find out
what causes it to occasionally misfire and stop suppressing the tumor
growth. Their long-term goal is to find drug molecules to test on these
proteins to see if they can develop a treatment.