Dr Robert Skoff
Our laboratory is supported by grants from the National Institute of Neurological Diseases and Stroke, the National Multiple Sclerosis Society, and the European Leukodystrophy Association. These grants support research of proteins in the myelin sheath that are critical for normal human function. We study a protein, proteolipid protein, in myelin that is often mutated in humans and is fatal. Using molecular and cell biological techniques, we study the mechanisms by which mutations in this protein lead to cell death and, ultimately, a person's death. Inerestingly, we find that proteolipid protein is inserted into mitochondria where it exerts deleterious effects. We have available for study transgenic mice that mimic closely a human developmental disease. We also study sexual dimorphism of oligodendrocytes, the myelin forming cells in the central nervous system. Our laboratory is the first laboratory in the world to demonstrate morphological and functional differences in male and female oligodendrocytes.
More recently, our lab has been studying the role of proteolipid protein in neonatal hypoxia ischemia. We found that the absence of proteolipid protein is neuroprotective in hypoxia ischemia.
Our laboratory utilizes different molecular and cell biology techniques to study cell lineages and the regulation of myelin formation. These techniques include in situ hybridization, tissue culture, immunocytochemistry, cell transfections, myelin protein synthesis and targeting using enhanced green fluorescent protein as a marker protein, construction of point mutations in myelin protein genes, etc.