Gideon Dreyfuss
School of Medicine, University of Pennsylvania, USA

Gideon Dreyfuss is an Isaac Norris Professor of Biochemistry and Biophysics at the University of Pennysylvania, USA, as well as Howard Hughes Medical Institute Investigator. He studied chemistry and physics at the University of Jerusalem, Israel, breaking with the family tradition of three generations of medical doctors. Dr. Dreyfuss moved then to the USA where he received his PhD in biological chemistry from the Harvard University in Boston. During his postdoctoral studies he became intrigued how proteins and RNA interact in cells. The RNA-protein interactions enable the RNA to perform its function, transcribing the instructions encoded in DNA and translating them into proteins. Gideon Dreyfuss discovered that the generation of mature messenger RNA (mRNA) from pre-messenger RNA (pre-mRNA), that still contain the alternate order of exons and introns, is orchestrated by proteins bound to RNA in cells. The Dreyfuss lab was the first who used UV-light to crosslink RNA and bound proteins to "glue" them together in order to study the delicate interaction between RNA and proteins. Using this approach, Dreyfuss and his co-workers identified the first definitive group of RNA-binding proteins in the early 1980s. In the mid 1990s mutations in the gene called survival of motor neuron (SMN) were discovered to be the cause of the disease spinal muscular atrophy (SMA). Interestingly, the Dreyfuss lab identified the SMN protein slightly earlier as a RNA-binding protein. Unexpectedly, subsequent analysis showed that the SMN protein is not only required for the assembly of ribonucleoproteins (assembly of RNAs with their RNA-binding proteins) but is also involved in splicing. Gideon Dreyfuss proposed that splicing errors might cause SMA. Recently, the Dreyfuss lab showed in an animal model for SMA that the diversity of ribonucleoproteins in different cells is reduced. At the moment, the Dreyfuss lab in collaboration with pharmaceutical companies is searching for compounds, which increase SMN activity in cells from SMA patiens. Gideon Dreyfuss received among others recognitions the Medal of Charles University Faculty of Medicine in Prague (Czech Republic) and the Stanley N. Cohen Award of Excellence in Biomedical Research form the Universtiy of Pennysylvania. Read more...