Toxic Prion Protein Helps Unravel Neuronal Death Mechanism Linked to Neurodegenerative Disorders

Toxic Prion Protein Helps Unravel Neuronal Death Mechanism Linked to Neurodegenerative Disorders
brainResearchers at the Florida Campus of The Scripps Research Institute (TSRI) recently discovered a mechanism responsible for neuronal death that could be linked to several neurodegenerative diseases like Alzheimer’s, Parkinson’s and amyotrophic lateral sclerosis (ALS). Their findings entitled “Neuronal death induced by misfolded prion protein is due to NAD+ depletion and can be relieved in vitro and in vivo by NAD+ replenishment” were published in the journal Brain. Prion diseases are a family of rare, progressive neurodegenerative disorders that are caused by prions – abnormal, pathogenic agents able to induce anomalous folding of specific proteins usually in the brain. Bovine spongiform encephalopathy (also known as “mad cow disease”) and its human equivalent, Creutzfeldt-Jakob disease are examples of prion diseases. Neurodegenerative disorders result from abnormalities in the processing of specific neuronal proteins. Neuronal death is a natural process, part of the brain development, that when not properly regulated can lead to neurodegenerative diseases. The mechanisms underlying neuronal death in protein misfolding ne
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