The multisystem degeneration amyotrophic lateral sclerosis - neuropathological staging and clinical translation.

The multisystem degeneration amyotrophic lateral sclerosis - neuropathological staging and clinical translation. Arch Ital Biol. 2017 Dec 01;155(4):118-130 Authors: Verde F, Del Tredici K, Braak H, Ludolph A Abstract Amyotrophic lateral sclerosis (ALS) is traditionally considered a disease affecting exclusively motor neurons. However, much evidence points towards additional involvement of brain systems other than the motor. As much as half of ALS patients display cognitive-behavioral disturbances. ALS shares with a considerable proportion of FTD cases the same neuropathological substrate, namely, inclusions of abnormally phosphorylated protein TDP-43 (pTDP-43). In analogy with pathological staging systems elaborated in the past decades for Alzheimer's disease (AD) and Parkinson's disease (PD), a model of staging of pTDP-43 pathology in sporadic ALS (sALS) has been recently proposed. According to it, 4 stages can be recognized, where pTDP-43 inclusions are found in the agranular motor cortex and α-motor neurons of the brain stem and spinal cord (stage 1), in prefrontal neocortex (middle frontal gyrus), reticular formation, and precerebellar nuclei (stage 2), in further areas of the prefrontal neocortex (gyrus rectus and orbitofrontal gyri), postcentrally located sensory cortex, and basal ganglia (stage 3), and in the anteromedial temporal lobe including the hippocampus (stage 4). Based on this staging effort, a corticofugal axonal model for spreading...
Source: Archives Italiennes de Biologie - Category: Neuroscience Tags: Arch Ital Biol Source Type: research

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Abstract Organophosphorus (OP) compounds have been known as the most widely used pesticides during the past half century and there have been a huge body of literature regarding their association with human chronic diseases. Neurodegenerative and neurodevelopmental disorders including Alzheimer, Parkinson, amyotrophic lateral sclerosis (ALS), attention deficit hyperactivity disorder (ADHD), and autism are among the afflicting neurological diseases which overshadow human life and their higher risk in relation to OP exposures have been uncovered by epidemiological studies. In addition, experimental studies exploring ...
Source: Toxicology - Category: Toxicology Authors: Tags: Toxicology Source Type: research
In this study, we assessed the antioxidant properties of Larrea tridentata, collected specifically from the Chihuahuan desert in the region of El Paso del Norte, TX, USA. LT phytochemicals were obtained from three different extracts (ethanol; ethanol: water (60:40) and water). Then the extracts were evaluated in eight different assays (DPPH, ABTS, superoxide; FRAP activity, nitric oxide, phenolic content, UV visible absorption and cytotoxicity in non-cancerous HS27 cells). The three extracts were not affecting the HS27 cells at concentrations up to 120 µg/mL. Among the three extracts, we found that the mixture of...
Source: Molecules - Category: Chemistry Authors: Tags: Article Source Type: research
In conclusion, we focus on the various newer molecular mechanisms that are associated with the basic understanding of neuroinflammation in neurodegeneration.
Source: Neurochemistry International - Category: Neuroscience Source Type: research
Abstract Protein misfolding and aggregation into fibrillar deposits is a common feature of a large group of degenerative diseases affecting the central nervous system or peripheral organs, termed protein misfolding disorders (PMDs). Despite their established toxic nature, clinical trials aiming to reduce misfolded aggregates have been unsuccessful in treating or curing PMDs. An interesting possibility for disease intervention is the regular intake of natural food or herbal extracts, which contain active molecules that inhibit aggregation or induce the disassembly of misfolded aggregates. Among natural compounds, p...
Source: Cellular and Molecular Life Sciences : CMLS - Category: Cytology Authors: Tags: Cell Mol Life Sci Source Type: research
Abstract Many evidences indicate that oxidative stress plays a significant role in a variety of human disease states, including neurodegenerative diseases. Iron is an essential metal for almost all living organisms due to its involvement in a large number of iron-containing proteins and enzymes, though it could be also toxic. Actually, free iron excess generates oxidative stress, particularly in brain, where anti-oxidative defences are relatively low. Its accumulation in specific regions is associated with pathogenesis in a variety of neurodegenerative diseases (i.e., Parkinson's disease, Alzheimer's disease, Hunt...
Source: Biometals - Category: Biochemistry Authors: Tags: Biometals Source Type: research
Publication date: August 2018Source: Molecular Aspects of Medicine, Volume 62Author(s): Susmita Sil, Palsamy Periyasamy, Annadurai Thangaraj, Ernest T. Chivero, Shilpa BuchAbstractPlatelet-derived growth factors (PDGFs) and their receptors (PDGFRs) are expressed in several cell types including the brain cells such as neuronal progenitors, neurons, astrocytes, and oligodendrocytes. Emerging evidence shows that PDGF-mediated signaling regulates diverse functions in the central nervous system (CNS) such as neurogenesis, cell survival, synaptogenesis, modulation of ligand-gated ion channels, and development of specific types o...
Source: Molecular Aspects of Medicine - Category: Molecular Biology Source Type: research
Publication date: July 2018Source: TrAC Trends in Analytical Chemistry, Volume 104Author(s): Bernhard Michalke, Desiree Willkommen, Evgenii Drobyshev, Nikolay SolovyevAbstractElement speciation offers deeper insight into the molecular mechanisms of disease by determining element species pattern. Thus, having great potential for investigating neurodegeneration in Alzheimer's disease, Parkinson's disease, amyotrophic lateral sclerosis, and mild cognitive impairment, speciation is increasingly considered in epidemiological or clinical neurological studies. This review analyses recent speciation findings in neurodegeneration r...
Source: TrAC Trends in Analytical Chemistry - Category: Chemistry Source Type: research
Publication date: Available online 22 April 2018Source: Journal of Clinical Orthopaedics and TraumaAuthor(s): Manoj Kumar, Sanjeev Bhoi, Keshava SharmaAbstractHematopoietic failure (HF) has been observed in trauma hemorrhagic shock (T/HS) patients. Multiple factors are involved. Elevated serum levels of cytokines, catecholamine, granulocyte colony stimulating factor, peripheral blood hematopoietic progenitor cells (HPCs) and decreased expression of erythropoietin receptor are associated with HF among T/HS. HF leads to anaemia, susceptibility to infection, sepsis and multi-organ failure. There is a lack of molecular underst...
Source: Journal of Clinical Orthopaedics and Trauma - Category: Orthopaedics Source Type: research
Publication date: Available online 7 July 2018Source: Pharmacological ResearchAuthor(s): Samira Shirooie, Seyed Fazel Nabavi, Ahmad R. Dehpour, Tarun Belwal, Solomon Habtemariam, Sandro Argüelles, Antoni Sureda, Maria Daglia, Michał Tomczyk, Eduardo Sobarzo-Sanchez, Suowen Xu, Seyed Mohammad NabaviAbstractNeurodegenerative diseases (NDs) such as Parkinson's (PD), Alzheimer's (AD), Huntington's disease (HD), and amyotrophic lateral sclerosis (ALS) cause significant world-wide morbidity and mortality. To date, there is no drug of cure for these, mostly age-related diseases, although approaches in delaying the pathology...
Source: Pharmacological Research - Category: Drugs & Pharmacology Source Type: research
Publication date: Available online 3 April 2018Source: Pharmacology &TherapeuticsAuthor(s): Jan Christoph Koch, Lars Tatenhorst, Anna-Elisa Roser, Kim-Ann Saal, Lars Tönges, Paul LingorAbstractNeurodegenerative disorders like Parkinson's disease, Alzheimer's disease, or amyotrophic lateral sclerosis are affecting a rapidly increasing population worldwide. While common pathomechanisms such as protein aggregation, axonal degeneration, dysfunction of protein clearing and an altered immune response have been characterized, no disease-modifying therapies have been developed so far. Interestingly, a significant involvem...
Source: Pharmacology and Therapeutics - Category: Drugs & Pharmacology Source Type: research
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