The prion protein (PrP) is a normal cellular glycophosphatidylinositol anchored sialoglycoprotein encoded by the Prnp gene. PrP is expressed in many tissues (with relatively high levels in the brains of animals and humans) and has been shown to be necessary for clinical disease. PrP can display at least two conformations: a normal cellular conformation which is predominantly alphahelical (PrPC) and a pathological beta-pleated conformation (PrPSc) associated with prion diseases. Although prion diseases are characterized clinically as a result of neurodegeneration, PrPC expression has been shown to exert a neuroprotective role. It is the goal of Rubenstein's lab to halt and reverse this neurodegeneration by establishing stem cells that are resistant to the infectious agent, and using these stem cells in replacement therapy.
Friedland RP, Petersen RB, Rubenstein R. Bovine spongiform encephalopathy and aquaculture. J Alzheimers Dis. 2009 Jun;17(2):277-9.
Chang B, Gray P, Piltch M, Bulgin MS, Sorensen-Melson S, Miller MW, Davies P, Brown DR, Coughlin DR, Rubenstein R. Surround optical fiber immunoassay (SOFIA): an ultra-sensitive assay for prion protein detection. J Virol Methods. 2009 Jul;159(1):15-22.
Kolecki R, Lafauci G, Rubenstein R, Mazur-Kolecka B, Kaczmarski W, Frackowiak J. The effect of amyloidosis-beta and ageing on proliferation of neuronal progenitor cells in APP-transgenic mouse hippocampus and in culture.
Acta Neuropathol. 2008 Oct;116(4):419-24.
Rubenstein, R., Gray, P.C., Cleland, T.J.J., Piltch, M.S., Hlavacek, W.S., Ambrosiano, J.J., Kim, J-I., Roberts, R.M.: Dynamics of the nucleated polymerization model of prion replication. Biophys. Chem. 125: 360-367, 2007.
Stobart MJ, Parchaliuk D, Simon SL, Lemaistre J, Lazar J, Rubenstein R, Knox JD. Differential expression of interferon responsive genes in rodent models of transmissible spongiform encephalopathy disease. Mol Neurodegener. 2007 Mar 16;2:5.