New Researches On The Relationship Between Amyotrophic Lateral Sclerosis And Prion Protein

Amyotrophic lateral sclerosis(ALS),also known as Lou Gehrig's disease,is a kind of motor neuron disease.ALS is a deadly,adult-onset neurodegenerative disorder characterized by selectively violating upper and lower motor neurons,causing progressive degeneration and death of upper and lower motor neurons.ALS is a rapidly progressive fatal neuromuscular condition.Ultimately,death is caused by destruction of the motor neurons which control muscles that innervate breathing 3-5 years after the onset of initial sympotems.The etiology of ALS remains to be resolved and several pathogenic mechanisms may be involved:(1)abnormal aggregating of protein(2)oxidativestress(3)excitotoxicity(4)genetic factors(5)autoimmunity dysfunction(6)cytoskeleton abnormal(7)mitochondria dysfunction.Prion diseses is caused by the configuration change of cellular prion protein into misfolded isoform of cellular prion protein which abnormally aggregates into the cells.There are a lot of researches about the etiology of ALS on the abnormal aggregation of prion-like protein recently.Some new research progress had been made which allow us to have a more profound insight into the etiology and the theraputic of ALS.In this review we will summarize the pathogenesis related prion-like proteins and the latest research about the therapy of ALS.