ABSTRACT
Knowledge of the physiological function of cellular prion protein has been acquired from prion diseases such as Creutzfeldt-Jakob disease, as well as PRNP knock out and transgenic mice. Recent progress in neurobiology has further delineated the neuroprotective role played by cellular prion protein. In this paper, we review the role of cellular prion protein in cell survival including its antiapoptotic effect on Bax-mediated cell death and its responses to various environmental stresses including oxidative stress, and ischemia. Finally, we discuss the significance of cellular prion protein in different neurodegenerative diseases and the possible development of future therapies.
Subject(s)
Cell Survival/physiology , Neuroprotective Agents/metabolism , Oxidative Stress , PrPC Proteins/metabolism , Animals , Brain Ischemia/metabolism , Cell Death/physiology , Epilepsy/metabolism , GPI-Linked Proteins , Heat-Shock Response/physiology , Hypoglycemia/metabolism , Inflammation/metabolism , Neuroglia/metabolism , Neuronal Plasticity/physiology , Prions/metabolism , bcl-2-Associated X Protein/metabolismABSTRACT
The authors investigated a 40-year-old woman who presented with ataxia and dementia with little progression for over 40 months. The results of a CSF 14-3-3 protein and EEG study did not reveal major abnormalities. Brain MRI showed increased signal intensity over the occipital cortex in diffusion-weighted imaging. To our knowledge, this is the longest MM-type sporadic Creutzfeldt-Jakob disease case with cortical kuru-type plaques.
