A fly is like a test tube, but its alive, Fernandez-Funez said. Its a system where you can play with genes quickly and easily, so instead of doing one experiment in one year, you can do five or 10 or 15. If you fail in most of them, thats not really important whats important is that you find one of them that works.
Fernandez-Funezs NIH award funds experiments with fruit flies whose DNA has been genetically modified to produce hamster prion protein. In rodents, cows, humans and other mammals, such proteins can accidentally misfold, taking on an abnormal shape that causes other prion proteins they contact to take a similar shape one that promotes the accumulation of brain-cell killing protein deposits. And by the end of the transgenic fruit flies 40-day lifespan, their tiny brains also show the telltale signs of prion misfolding and neurotoxicity, according to Fernandez-Funez.
Weve produced these flies, we see the biochemical changes on the prion protein and we can see the neurodegeneration, Fernandez-Funez said. Now, we want to figure out how we can stop this process.
Using the new NIH funding, Fernandez-Funez and Rincon-Limas plan to investigate the protective effects in flies, mammalian cells and mice of so-called chaperone proteins, which work to make sure that other proteins take on their proper shapes after they are produced. Their preliminary experiments show that one such protein, known as Hsp70, protects their flies against the neurotoxic effects of prion protein misfolding; they intend to use their flies to determine whether other possibly protective chaperone proteins
|Contact: Jim Kelly|
University of Texas Medical Branch at Galveston