Wilfried Rossoll

 

Department of Neuroscience Mayo Clinic.

New York. EEUU

My career has focused on cellular and molecular mechanisms of motor neuron disease since I became fascinated with the role of mRNA localization in neuronal health and disease at the beginning of my postdoc in 2000 with Dr. Michael Sendtner at the Institute for Clinical Neurobiology in Wuerzburg. During this time, I was involved in the identification of a missense mutation in the tubulin-specific chaperone E (Tbce) gene in the mouse mutant progressive motor neuronopathy (pmn), a model of human motor neuron disease. Additionally, I collaborated on the characterization of the neuromuscular degeneration (nmd) mouse mutant, which carries mutations in the RNA helicase gene Ighmbp2, and is an animal model for distal spinal muscular atrophy with respiratory distress type 1 (SMARD1/DSMA1). During this time I became involved in spinal muscular atrophy (SMA) research, which is one of my main research interests.  

 

Since coming to Emory as instructor and then assistant professor, I have focused on RNA-mediated disease mechanisms in neurodegeneration. I have used my expertise with basic RNA biology, motor neuron culture, and mouse models of neurologic disease, to focus on the following research questions: (1) what are the cellular mechanisms that regulate mRNA trafficking and local protein synthesis in axons, (2) what is the contribution of mRNA binding proteins and mRNA processing defects to the pathomechanisms in neurodegenerative diseases such as SMA and FTD/ALS, (3) can interventions that target mRNA processing defects lead to the development of therapeutic strategies for these diseases? 

 

Due to the limitations of primary motor neuron culture for biochemical analysis, I have developed a great interest in using patient stem cell-derived motor neurons. 2012-2017, I have served as associate director of the Laboratory of Translational Cell Biology (LTCB), an Emory School of Medicine supported initiative, whose objectives were to establish Emory patient stem cell derived models of neurologic diseases, characterize disease mechanisms at the cellular level, and conduct high content microscopy-based drug screening.  

 

In early 2017, I have joined the faculty in the Department of Neuroscience at Mayo Clinic in Jacksonville, to establish my own lab and continue my independent research program on cellular and molecular disease mechanisms in neurodegenerative disorders. Most recently, I have established collaborations with investigators in Spain, Japan, and colleagues at the Mayo Clinic, to investigate repeat-associated non-ATG (RAN) translation-dependent disease mechanisms in the two highly related hexanucleotide-repeat disorders, C9FTD/ALS and SCA36. 

 

SEE MORE »