scholarly journals Neurodegenerative Diseases and Cholesterol: Seeing the Field Through the Players

2021 ◽  
Vol 13 ◽  
Author(s):  
Frank W. Pfrieger
Keyword(s):  
Multiple Sclerosis ◽  
Amyotrophic Lateral Sclerosis ◽  
Neurodegenerative Diseases ◽  
Biomedical Research ◽  
Economic Burden ◽  
Considerable Portion ◽  
Key Players ◽  
Important Field ◽  
Bibliometric Approach ◽  
Lateral Sclerosis

Neurodegenerative diseases, namely Alzheimer’s (AD), Parkinson’s (PD), and Huntington’s disease (HD) together with amyotrophic lateral sclerosis (ALS) and multiple sclerosis (MS), devastate millions of lives per year worldwide and impose an increasing socio-economic burden across nations. Consequently, these diseases occupy a considerable portion of biomedical research aiming to understand mechanisms of neurodegeneration and to develop efficient treatments. A potential culprit is cholesterol serving as an essential component of cellular membranes, as a cofactor of signaling pathways, and as a precursor for oxysterols and hormones. This article uncovers the workforce studying research on neurodegeneration and cholesterol using the TeamTree analysis. This new bibliometric approach reveals the history and dynamics of the teams and exposes key players based on citation-independent metrics. The team-centered view reveals the players on an important field of biomedical research.

scholarly journals Paraoxonase Role in Human Neurodegenerative Diseases

Antioxidants ◽  
2020 ◽  
Vol 10 (1) ◽  
pp. 11
Author(s):  
Cadiele Oliana Reichert ◽  
Debora Levy ◽  
Sergio P. Bydlowski
Keyword(s):  
Oxidative Stress ◽  
Multiple Sclerosis ◽  
Amyotrophic Lateral Sclerosis ◽  
Neurodegenerative Diseases ◽  
High Density Lipoprotein ◽  
Density Lipoprotein ◽  
Structural Homology ◽  
Redox Systems ◽  
Genetic Cluster ◽  
Lateral Sclerosis

The human body has biological redox systems capable of preventing or mitigating the damage caused by increased oxidative stress throughout life. One of them are the paraoxonase (PON) enzymes. The PONs genetic cluster is made up of three members (PON1, PON2, PON3) that share a structural homology, located adjacent to chromosome seven. The most studied enzyme is PON1, which is associated with high density lipoprotein (HDL), having paraoxonase, arylesterase and lactonase activities. Due to these characteristics, the enzyme PON1 has been associated with the development of neurodegenerative diseases. Here we update the knowledge about the association of PON enzymes and their polymorphisms and the development of multiple sclerosis (MS), amyotrophic lateral sclerosis (ALS), Alzheimer’s disease (AD) and Parkinson’s disease (PD).

scholarly journals Oxidative Stress in Neurodegenerative Diseases: From Preclinical Studies to Clinical Applications

2020 ◽  
Vol 9 (4) ◽  
pp. 1223
Author(s):  
Andrea Tarozzi
Keyword(s):  
Oxidative Stress ◽  
Multiple Sclerosis ◽  
Alzheimer’S Disease ◽  
Parkinson’S Disease ◽  
Amyotrophic Lateral Sclerosis ◽  
Neurodegenerative Diseases ◽  
Huntington's Disease ◽  
Clinical Applications ◽  
Preclinical Studies ◽  
Lateral Sclerosis

Oxidative stress plays an important role in the pathogenesis of several different neurodegenerative diseases (NDDs), such as Alzheimer’s disease (AD), Parkinson’s disease (PD), Huntington’s disease, amyotrophic lateral sclerosis (ALS) and multiple sclerosis (MS) [...]

History and Prevalence of Involuntary Emotional Expression Disorder

CNS Spectrums ◽  
2007 ◽  
Vol 12 (S5) ◽  
pp. 6-10 ◽  
Author(s):  
John E. Duda
Keyword(s):  
Multiple Sclerosis ◽  
Traumatic Brain Injury ◽  
Amyotrophic Lateral Sclerosis ◽  
Brain Injury ◽  
Neurodegenerative Diseases ◽  
Emotional Expression ◽  
Neurological Disorders ◽  
Prevalence Rates ◽  
Neurological Injuries ◽  
Lateral Sclerosis

AbstractThe syndrome now known as involuntary emotional expression disorder (IEED) is a condition characterized by uncontrollable episodes of laughing and/or crying. It has been known for more than a century, but confusing and conflicting terminology may have hampered the progress of physicians in recognizing this condition. IEED is associated with various neurological disorders and neurodegenerative diseases, including amyotrophic lateral sclerosis, multiple sclerosis, Parkinson's disease, Alzheimer's disease and other dementias, and neurological injuries such as stroke and traumatic brain injury. It is hoped that better defined terminology for IEED may help in the future diagnosis of this debilitating condition, the establishment of accurate prevalence rates for IEED in the varying underlying conditions, and also in removing blame and stigma from sufferers by providing reassurance about the nature of their condition.

scholarly journals Therapeutic Assay with the Non-toxic C-Terminal Fragment of Tetanus Toxin (TTC) in Transgenic Murine Models of Prion Disease

2021 ◽  
Author(s):  
Marina Betancor ◽  
Laura Moreno-Martínez ◽  
Óscar López-Pérez ◽  
Alicia Otero ◽  
Adelaida Hernaiz ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Neurodegenerative Diseases ◽  
Prion Disease ◽  
Tetanus Toxin ◽  
Neuronal Survival ◽  
Prion Diseases ◽  
Cellular Prion Protein ◽  
Murine Models ◽  
Terminal Fragment ◽  
Lateral Sclerosis

AbstractThe non-toxic C-terminal fragment of the tetanus toxin (TTC) has been described as a neuroprotective molecule since it binds to Trk receptors and activates Trk-dependent signaling, activating neuronal survival pathways and inhibiting apoptosis. Previous in vivo studies have demonstrated the ability of this molecule to increase mice survival, inhibit apoptosis and regulate autophagy in murine models of neurodegenerative diseases such as amyotrophic lateral sclerosis and spinal muscular atrophy. Prion diseases are fatal neurodegenerative disorders in which the main pathogenic event is the conversion of the cellular prion protein (PrPC) into an abnormal and misfolded isoform known as PrPSc. These diseases share different pathological features with other neurodegenerative diseases, such as amyotrophic lateral sclerosis, Parkinson’s disease or Alzheimer’s disease. Hitherto, there are no effective therapies to treat prion diseases. Here, we present a pilot study to test the therapeutic potential of TTC to treat prion diseases. C57BL6 wild-type mice and the transgenic mice Tg338, which overexpress PrPC, were intracerebrally inoculated with scrapie prions and then subjected to a treatment consisting of repeated intramuscular injections of TTC. Our results indicate that TTC displays neuroprotective effects in the murine models of prion disease reducing apoptosis, regulating autophagy and therefore increasing neuronal survival, although TTC did not increase survival time in these models.

scholarly journals Occupational Therapy Interventions in Adults with Multiple Sclerosis or Amyotrophic Lateral Sclerosis: A Scoping Review

2021 ◽  
Vol 18 (4) ◽  
pp. 1432
Author(s):  
Luis De-Bernardi-Ojuel ◽  
Laura Torres-Collado ◽  
Manuela García-de-la-Hera
Keyword(s):  
Multiple Sclerosis ◽  
Amyotrophic Lateral Sclerosis ◽  
Occupational Therapy ◽  
Scoping Review ◽  
Adult Population ◽  
Falls Prevention ◽  
Targeted Interventions ◽  
Therapy Interventions ◽  
Occupational Therapy Interventions ◽  
Lateral Sclerosis

This scoping review aims to describe occupational therapy interventions carried out with multiple sclerosis (MS) and amyotrophic lateral sclerosis (ALS) patients in occupational therapy. A peer review of the literature was conducted in different databases: Pubmed, Scopus, Web of Science and Embase, and in some occupational therapy journals. A search of the literature published was carried out before December 2019. The inclusion criteria were as follows: (1) articles evaluating the intervention of occupational therapy in MS or ALS including experimental, randomized, nonrandomized and exploratory studies; (2) written in English or Spanish; (3) adult population (over 18 years old). The initial search identified 836 articles of which we included 32 divided into four areas of intervention: fatigue-targeted interventions, cognitive interventions, physical interventions and others. Only 16 studies were carried out exclusively by occupational therapists. Most occupational therapy interventions are aimed at fatigue and physical rehabilitation. The majority of the studies in our review included MS patients, with little representation from the ALS population. These interventions have shown an improvement in perceived fatigue, manual dexterity, falls prevention and improvement in cognitive aspects such as memory, communication, depression and quality of life in the MS and ALS populations.

scholarly journals Proline/arginine dipeptide repeat polymers derail protein folding in amyotrophic lateral sclerosis

2021 ◽  
Vol 12 (1) ◽  
Author(s):  
Maria Babu ◽  
Filippo Favretto ◽  
Alain Ibáñez de Opakua ◽  
Marija Rankovic ◽  
Stefan Becker ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Protein Folding ◽  
Neurodegenerative Diseases ◽  
Catalyst Activity ◽  
Coding Region ◽  
Prolyl Isomerase ◽  
X Ray Crystallography ◽  
Hexanucleotide Repeat ◽  
Lateral Sclerosis ◽  
Dipeptide Repeat

AbstractAmyotrophic lateral sclerosis and frontotemporal dementia are two neurodegenerative diseases with overlapping clinical features and the pathological hallmark of cytoplasmic deposits of misfolded proteins. The most frequent cause of familial forms of these diseases is a hexanucleotide repeat expansion in the non-coding region of the C9ORF72 gene that is translated into dipeptide repeat polymers. Here we show that proline/arginine repeat polymers derail protein folding by sequestering molecular chaperones. We demonstrate that proline/arginine repeat polymers inhibit the folding catalyst activity of PPIA, an abundant molecular chaperone and prolyl isomerase in the brain that is altered in amyotrophic lateral sclerosis. NMR spectroscopy reveals that proline/arginine repeat polymers bind to the active site of PPIA. X-ray crystallography determines the atomic structure of a proline/arginine repeat polymer in complex with the prolyl isomerase and defines the molecular basis for the specificity of disease-associated proline/arginine polymer interactions. The combined data establish a toxic mechanism that is specific for proline/arginine dipeptide repeat polymers and leads to derailed protein homeostasis in C9orf72-associated neurodegenerative diseases.

scholarly journals Conservative iron chelation for neurodegenerative diseases such as Parkinson’s disease and amyotrophic lateral sclerosis

2020 ◽  
Vol 127 (2) ◽  
pp. 189-203 ◽  
Author(s):  
David Devos ◽  
◽  
Z. Ioav Cabantchik ◽  
Caroline Moreau ◽  
Véronique Danel ◽  
...  
Keyword(s):  
Parkinson’S Disease ◽  
Parkinson's Disease ◽  
Amyotrophic Lateral Sclerosis ◽  
Neurodegenerative Diseases ◽  
Iron Chelation ◽  
Lateral Sclerosis

Co-occurrence of multiple sclerosis and amyotrophic lateral sclerosis: A case report

2021 ◽  
Vol 429 ◽  
pp. 118159
Author(s):  
Paola Ajdinaj ◽  
Marianna Gabriella Rispoli ◽  
Laura Ferri ◽  
Maria D'Apolito ◽  
Deborah Farina ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Amyotrophic Lateral Sclerosis ◽  
Case Report ◽  
Lateral Sclerosis
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