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.
Source: Handbook of Clinical Neurology - Category: Neurology Source Type: research

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Publication date: Available online 14 December 2018Source: Neurochemistry InternationalAuthor(s): Bruk Getachew, Antonei B. Csoka, Michael Aschner, Yousef TizabiAbstractManganese (Mn) and iron (Fe) are trace elements that are essential for proper growth and physiological functions as both play critical role in a variety of enzymatic reactions. At high concentrations, however, they can be toxic and cause neurodegenerative disorders, particularly Parkinson-like syndromes. Nicotine, on the other hand, has been shown to have neuroprotective effects against various endogenous or exogenous toxins that selectively damage the dopa...
Source: Neurochemistry International - Category: Neuroscience Source Type: research
Authors: Delyon J, Arangalage D, Baroudjian B, Lebbe C, Brohl AS, Saller J PMID: 30545987 [PubMed - in process]
Source: Journal of the National Comprehensive Cancer Network : JNCCN - Category: Cancer & Oncology Tags: J Natl Compr Canc Netw Source Type: research
CONCLUSION: Spousal and nonspousal MCI dyads may approach clinical trial decisions differently. Future research should investigate how AD biomarker testing affects participants' willingness to enroll in trials. PMID: 30522811 [PubMed - as supplied by publisher]
Source: The American Journal of Geriatric Psychiatry - Category: Psychiatry Tags: Am J Geriatr Psychiatry Source Type: research
Abstract Malignancies were one of the earliest recognized manifestations that led to the description of the acquired immune deficiency syndrome (AIDS). The majority of cancers in AIDS patients are associated with coinfection with oncogenic viruses, such as Epstein-Barr virus, human herpesvirus 8, and human papillomavirus, with resulting malignancies occurring secondary to diminished immune surveillance against viruses and virus-infected tumor cells. Over 50% of AIDS lymphomas are associated with Epstein-Barr virus (EBV) and/or HHV8 infection. HHV8-associated diseases include Kaposi sarcoma (KS), primary ...
Source: Cell Research - Category: Cytology Authors: Tags: Cancer Treat Res Source Type: research
Postural control asymmetry is an important aspect of Parkinson's disease (PD) that may be associated with falls. The aim of this study was to compare the postural control asymmetry during postural tasks between fallers and non-fallers in people with PD and...
Source: SafetyLit - Category: International Medicine & Public Health Tags: Age: Elder Adults Source Type: news
Despite the development of several modern imaging modalities, radiography remains the first and the most important mode of investigation for evaluating jaw lesions. Osteosarcoma is the most common primary malignant bone tumor, accounting for approximately 20% of the sarcomas, but only 5% of the osteosarcomas occur in the jaws. Malignant lesions are known to quickly grow downward into the periodontal ligament (PDL) space, resulting in an irregular widening and destruction of the lamina dura. Often, root fractures can result in a similar radiographic appearance of the surrounding periodontium.
Source: Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontics - Category: ENT & OMF Authors: Source Type: research
Publication date: Available online 13 December 2018Source: NeuroImageAuthor(s): Lin Chen, Zhiliang Wei, Kannie Chan, Shuhui Cai, Guanshu Liu, Hanzhang Lu, Philip C. Wong, Peter C.M. van Zijl, Tong Li, Jiadi XuAbstractThe goal of this study was to develop a molecular biomarker for the detection of protein aggregation involved in Alzheimer's disease (AD) by exploiting the features of the water saturation transfer spectrum (Z-spectrum), the CEST signal of which is sensitive to the molecular configuration of proteins. A radial-sampling steady-state sequence based ultrashort echo time (UTE) readout was implemented to image the ...
Source: NeuroImage - Category: Neuroscience Source Type: research
Publication date: Available online 13 December 2018Source: The Lancet NeurologyAuthor(s): Nilo Riva, Gabriele Mora, Gianni Sorarù, Christian Lunetta, Ottavia E Ferraro, Yuri Falzone, Letizia Leocani, Raffaella Fazio, Mauro Comola, Giancarlo Comi, Fabio Formaglio, Paolo Rossi, Marta Clerici, Yuri Matteo Falzone, Laura Pozzi, Daniele Martinelli, Federica Cerri, Ignazio Diego Lopez, Filippo Martinelli-Boneschi, Angelo QuattriniSummaryBackgroundSpasticity is a major determinant of disability and decline in quality of life in patients with motor neuron disease. Cannabinoids have been approved for symptomatic treatment of...
Source: The Lancet Neurology - Category: Neurology Source Type: research
DiscussionSCD can precede both AD and non-AD dementia. Despite their younger age, individuals with SCD in a memory clinic setting have a higher risk of dementia than those in community-based cohorts.
Source: Alzheimer's and Dementia: The Journal of the Alzheimer's Association - Category: Geriatrics Source Type: research
Publication date: Available online 13 December 2018Source: Alzheimer's &DementiaAuthor(s): Ganesh M. Babulal, Yakeel T. Quiroz, Benedict C. Albensi, Eider Arenaza-Urquijo, Arlene J. Astell, Claudio Babiloni, Alex Bahar-Fuchs, Joanne Bell, Gene L. Bowman, Adam M. Brickman, Gaël Chételat, Carrie Ciro, Ann D. Cohen, Peggye Dilworth-Anderson, Hiroko H. Dodge, Simone Dreux, Steven Edland, Anna Esbensen, Lisbeth Evered, Michael EwersAbstractAlzheimer's disease and related dementias (ADRDs) are a global crisis facing the aging population and society as a whole. With the numbers of people with ADRDs predicted to ri...
Source: Alzheimer's and Dementia: The Journal of the Alzheimer's Association - Category: Geriatrics Source Type: research
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