The mechanisms of progression of prion diseases (Creutzfeldt-Jakob and mad cow disease) are now better understood with, finally, a potential target for treatment. A major advance in the research and management of these rapidly progressive and always fatal dementias.
Researchers at the Boston University School of Medicine have found that inhibition of MAPKα p38, a nerve cell enzyme that generally reacts to stress, prevents prions, viral particles responsible for brain degeneration, from altering nerve connections. Inhibition of MAPKα p38 also promotes the recovery of initial damage.
Nerve cells that have a mutation preventing the normal function of p38 MAPKα are also protected, which seems to confirm the role played by the enzyme in this pathological process of slow virus diseases. These results are published in PLOS Pathogens.
Dementia of viral origin
Prion diseases are a group of deadly neurological diseases including Creutzfeldt-Jakob disease and Bovine Spongiform Encephalopathy ("mad cow disease").
They are caused by the spread of "prions", which are altered forms of normal cellular proteins. These abnormal molecules then interact with normal proteins to promote abnormal folding.
An understanding and a therapeutic target
Although researchers understand that this process of converting normal proteins into abnormal proteins is the cause of the symptoms of prion disease (including rapidly progressive dementia, convulsions and personality changes), the exact mechanism of damage to connections neurons in the brain and spinal cord remained misunderstood.
A major step forward
David. A. Harris, a professor in the Department of Biochemistry at the Boston University School of Medicine and author of the study, sees these results as a breakthrough in the research and management of these deadly neurodegenerative diseases.
"Our findings provide new insights into the pathogenesis of prion diseases, uncovering new therapeutic targets for treating these diseases, and allowing us to compare prion diseases with other more common neurodegenerative diseases such as Alzheimer's disease. "