Chapter 18 Prion-like mechanisms in amyotrophic lateral sclerosis
Publication date: 2018 Source:Handbook of Clinical Neurology, Volume 153 Author(s): Jacob I. Ayers, Neil R. Cashman The prion hypothesis – a protein conformation capable of replicating without a nucleic acid genome – was heretical at the time of its discovery. However, the characteristics of the disease-misfolded prion protein and its ability to transmit disease, replicate, and spread are now widely accepted throughout the scientific community. In fact, in the last decade a wealth of evidence has emerged supporting similar properties observed for many of the misfolded proteins implicated in other neurodegenerative diseases, such as Alzheimer disease, Parkinson disease, tauopathies, and as described in this chapter, amyotrophic lateral sclerosis (ALS). Multiple studies have now demonstrated the ability for superoxide dismutase-1, 43-kDa transactive response (TAR) DNA-binding protein, fused-in sarcoma, and most recently, C9orf72-encoded polypeptides to display properties similar to those of prions. The majority of these are cell-free and in vitro assays, while superoxide dismutase-1 remains the only ALS-linked protein to demonstrate several prion-like properties in vivo. In this chapter, we provide an introduction to ALS and review the recent literature linking several proteins implicated in the familial forms of the disease to properties of the prion protein.
Conclusion: The Activa PC IPG demonstrates a significantly reduced battery life of 2.1 years, with a median battery life of 4.5 years in comparison to 6.6 years in the Kinetra IPG. Future technology developments should therefore be focused on improving the battery life of the newer IPG systems.Stereotact Funct Neurosurg
Alzheimer's patients older than 65 benefit the most benefit from deep brain stimulation, according to findings from a recent phase II clinical trial.
“As it prepares to turn 70, the NHS is coming towards the worst decade in its existence as far as funding goes,” began the discussion of health and social care. The mover of the motion said that, “services have been repeatedly run down by a government that prefers tax cuts for the wealthy to a well-funded NHS… we now have a permanent winter for our NHS and workloads and stress loads have gone through the roof for many members.” Tracy Holmes, from Sefton local government branch, told delegates about the bad practise in homecare she had witnessed: from visits of just five minutes scheduled...
Condition: Parkinson Disease Interventions: Biological: VY-AADC02; Other: Placebo Sponsor: Voyager Therapeutics Not yet recruiting
Conditions: Alzheimer Disease; Mild Cognitive Impairment Intervention: Diagnostic Test: Pramlintide challenge test Sponsor: Boston University Not yet recruiting
Xueping Chen, Qian-Qian Wei, Yongping Chen, Bei Cao, RuWei Ou, Yanbing Hou, Xiaoqin Yuan, Lingyu Zhang, Hui Liu, Huifang Shang
Nicolas Gigu ère, Samuel Burke Nanni, Louis-Eric Trudeau
Authors: Müller T, Möhr JD Abstract INTRODUCTION: Parkinson's disease is a chronic, neurodegenerative disease. Its symptoms and course are heterogeneous. After several years of investigative drug studies, levodopa remains the most efficacious drug despite its long-term limitations. Consequently, research into new drug delivery modes is ongoing. Areas covered: This review summarizes past and current advances of levodopa therapy with a focus on long-term patient management. Current research aims to increase drug bioavailability and to deliver it to the brain continuously. Reduced fluctuations improve drug e...
Notice NOT-AG-18-019 from the NIH Guide for Grants and Contracts
DEMENTIA symptoms are triggered by a decline in brain function. There are several types of dementia you should be aware of, including Alzheimer ’s disease and vascular dementia. But, do you know the symptoms of the less common types? Watch out for these signs of frontotemporal dementia.