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Topic review
Updated time: 26 Oct 2020
Submitted by: Illana Gozes
Definition: The activity-dependent neuroprotective protein (ADNP), a double-edged sword, sex-dependently regulates multiple genes and was previously associated with the control of early muscle development and aging.
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Topic review
Updated time: 28 Apr 2021
Submitted by: Jacopo Meldolesi
Definition: Biomarkers are molecules that are variable in their origin, nature, and mechanism of action; they are of great relevance in biology and also in medicine because of their specific connection with a single or several diseases. Biomarkers are of two types, which in some cases are operative with each other. Fluid biomarkers, started around 2000, are generated in fluid from specific proteins/peptides and miRNAs accumulated within two extracellular fluids, either the central spinal fluid or blood plasma. The switch of these proteins/peptides and miRNAs, from free to segregated within extracellular vesicles, has induced certain advantages including higher levels within fluids and lower operative expenses. Imaging biomarkers, started around 2004, are identified in vivo upon their binding by radiolabeled molecules subsequently revealed in the brain by positron emission tomography and/or other imaging techniques. A positive point for the latter approach is the quantitation of results, but expenses are much higher.
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Topic review
Updated time: 17 Jun 2021
Submitted by: Maria Xilouri
Definition: Accumulation of the neuronal presynaptic protein alpha-synuclein within proteinaceous inclusions represents the key histophathological hallmark of a spectrum of neurodegenerative disorders, referred to by the umbrella term a-synucleinopathies. Even though alpha-synuclein is expressed predominantly in neurons, pathological aggregates of the protein are also found in the glial cells of the brain. In Parkinson’s disease and dementia with Lewy bodies, alpha-synuclein accumulates mainly in neurons forming the Lewy bodies and Lewy neurites, whereas in multiple system atrophy, the protein aggregates mostly in the glial cytoplasmic inclusions within oligodendrocytes. In addition, astrogliosis and microgliosis are found in the synucleinopathy brains, whereas both astrocytes and microglia internalize alpha-synuclein and contribute to the spread of pathology. The mechanisms underlying the pathological accumulation of alpha-synuclein in glial cells that under physiological conditions express low to non-detectable levels of the protein are an area of intense research. Undoubtedly, the presence of aggregated alpha-synuclein can disrupt glial function in general and can contribute to neurodegeneration through numerous pathways.
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Topic review
Updated time: 22 Apr 2021
ALS
Submitted by: Andrew Eisen
Definition: The site of origin of amyotrophic lateral sclerosis (ALS), although unsettled, is increasingly recognized as being cortico-fugal, which is a dying-forward process primarily starting in the corticomotoneuronal system.
Entry Collection : Neuronal Degeneration
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Topic review
Updated time: 24 Mar 2021
Definition: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by atrophy and paralysis of voluntary muscles as a result of the progressive loss of upper and lower motor neurons. The best characterized inflammasome is NLRP3, which is composed of the NOD-like receptor pyrin domain containing protein 3, the adaptor protein ASC and pro-caspase 1. Although it has been shown that this inflammasome plays an important role in neurodegenerative disorders, such as multiple sclerosis, Alzheimer’s disease and Parkinson’s disease, little is known about its implication in ALS. Since NLRP3 inflammasome plays a pivotal role in several neurodegenerative disorders, we hypothesized that levels of inflammasome components could help in diagnosis or prognosis of amyotrophic lateral sclerosis (ALS).
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Topic review
Updated time: 10 May 2021
Submitted by: Mark Evans
Definition: We live and to do so we must breathe and eat, so are we a combination of what we eat and breathe? Here we will consider this question, and the role in this respect of the AMP-activated protein kinase (AMPK). Emerging evidence suggests that AMPK facilitates central and peripheral reflexes that coordinate breathing and oxygen supply, and contributes to central regulation of feeding and food choice. We propose, therefore, that oxygen supply to the body is aligned with not only the quantity we eat, but also nutrient-based diet selection, and that the cell-specific expression pattern of AMPK subunit isoforms is critical to appropriate system alignment in this respect. If this is the case, then aberrant cell-specific changes in the expression of AMPK subunit isoforms could give rise, in part, to known associations between a wide variety of conditions associated with metabolic disorder.
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Topic review
Updated time: 24 Mar 2021
Submitted by: Nuno Gonçalves-Anjo
Definition: Prion is defined as a “proteinaceous infectious particle” consisting exclusively of a single protein without the involvement of nucleic acids that causes spongiform encephalopathies in mammals. Prion diseases are characterized by the accumulation of abnormal isoforms of PrP glycoprotein.
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Topic review
Updated time: 08 May 2021
Submitted by: Tomasz M. Karpinski
Definition: As the major nonpsychotropic constituent of Cannabis sativa, cannabidiol (CBD) is regarded as one of the most promising therapeutic agents due to its proven effectiveness in clinical trials for many human diseases. Due to the urgent need for more efficient pharmacological treatments for several chronic diseases, in this review, we discuss the potential beneficial effects of CBD for Alzheimer’s disease, epilepsy, multiple sclerosis, and neurological cancers. Due to its wide range of pharmacological activities (e.g., antioxidant, anti-inflammatory, and neuroprotective properties), CBD is considered a multimodal drug for the treatment of a range of neurodegenerative disorders, and various cancer types, including neoplasms of the neural system.
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Topic review
Updated time: 09 Jun 2021
Submitted by: Tue Nielsen
Definition: Myostatin, also known as growth and differentiation factor 8 (GDF-8), was identified in 1997 by McPherron and Lee.
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Topic review
Updated time: 08 Apr 2021
Submitted by: Giacomo Lazzarino
Definition: Due to a multiplicity of causes provoking traumatic brain injury (TBI), TBI is a highly heterogeneous pathology, characterized by high mortality and disability rates. TBI is an acute neurodegenerative event, potentially and unpredictably evolving into sub-chronic and chronic neurodegenerative events, with transient or permanent neurologic, cognitive, and motor deficits, for which no valid standardized therapies are available. A vast body of literature demonstrates that TBI-induced oxidative/nitrosative stress is involved in the development of both acute and chronic neurodegenerative disorders. Cellular defenses against this phenomenon are largely dependent on low molecular weight antioxidants, most of which are consumed with diet or as nutraceutical supplements. A large number of studies have evaluated the efficacy of antioxidant administration to decrease TBI-associated damage in various animal TBI models and in a limited number of clinical trials. Points of weakness of preclinical studies are represented by the large variability in the TBI model adopted, in the antioxidant tested, in the timing, dosages, and routes of administration used, and in the variety of molecular and/or neurocognitive parameters evaluated. The analysis of the very few clinical studies does not allow strong conclusions to be drawn on the real effectiveness of antioxidant administration to TBI patients. Standardizing TBI models and different experimental conditions, as well as testing the efficacy of administration of a cocktail of antioxidants rather than only one, should be mandatory. According to some promising clinical results, it appears that sports-related concussion is probably the best type of TBI to test benefits of antioxidant administration.
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