Search results for " Aggregation"
showing 10 items of 441 documents
Protein Aggregation in Muscle Fibers and Respective Neuromuscular Disorders
2007
Protein aggregation in muscle fibers may be a nonspecific phenomenon such as occurring in cores or ragged red fibers. However, it may also be a disease-specific and disease-significant phenomenon constituting protein aggregate myopathies (PAMs). These may be divided into two classes: The first one is marked by impaired extralysosomal degradation of proteins, catabolic PAM, encompassing desmin-related myopathies. Mutant proteins, that is, desmin, myotilin, or α-B crystallin, defy protein degradation, aggregate and associate with other proteins within muscle fibers, hence marking desminopathies, myotilinopathies, and α-B crystallinopathies. A second class of PAM encompasses those apparently a…
Glial and neuronal expression of polyglutamine proteins induce behavioral changes and aggregate formation inDrosophila
2004
Patients with polyglutamine expansion diseases, like Huntington's disease or several spinocerebellar ataxias, first present with neurological symptoms that can occur in the absence of neurodegeneration. Behavioral symptoms thus appear to be caused by neuronal dysfunction, rather than cell death. Pathogenesis in polyglutamine expansion diseases is largely viewed as a cell-autonomous process in neurons. It is likely, however, that this process is influenced by changes in glial physiology and, at least in the case of DRPLA glial inclusions and glial cell death, seems to be an important part in the pathogenesis. To investigate these aspects in a Drosophila model system, we expressed polyglutami…
Nucleation mechanisms and spatial hetereogeneity in insulin amyloid fibrils formation
2008
An algorithm for simulating end-user behaviour in a real time pricing market
2015
The energy market has changed radically over the last decade, mainly due to an increased penetration of renewable energies. Now the end users have directly access to the energy market and can actively take part to the electricity market. Electricity customers can indeed modify their behavior through Demand Response, namely by means of pricing strategies that support a change in the end-users habits. This can be accomplished through a 'loads aggregator', a third party that collects the requests and signals for Active Demand-based services coming from the markets and the different actors of energy market. This paper describes a simulation framework to generate the simulated optimal behavior o…
Real-time pricing for aggregates energy resources in the Italian energy market
2015
Abstract Over the last decade, the architecture of the energy market has radically changed. In many countries end-users are now able to directly access the market, which has given rise to the question of how they can actively participate in that market. End-users can comprise a critical mass through aggregation that is carried out by a third party – to wit the “loads aggregator.” This paper proposes a new framework for generating feasible real-time price curves for those end-users in a demand-response management process. The underlying algorithm generates output curves as the solution to a constrained optimization problem whose objective function is the aggregator's economic benefit. A case…
P53 Aggregates and Interacts with Tau in Alzheimer's Disease
2020
Molecular mechanisms linking amyloid β toxicity and Tau hyperphosphorylation in Alzheimer׳s disease
2015
Neurofibrillary tangles (aggregates of cytoskeletal Tau protein) and senile plaques (aggregates mainly formed by amyloid β peptide) are two landmark lesions in Alzheimer׳s disease. Some researchers have proposed tangles, whereas others have proposed plaques, as primary lesions. For a long time, these were thought of as independent mechanisms. However, experimental evidence suggests that both lesions are intimately related. We review here some molecular pathways linking amyloid β and Tau toxicities involving, among others, glycogen synthase kinase 3β, p38, Pin1, cyclin-dependent kinase 5, and regulator of calcineurin 1. Understanding amyloid β and Tau toxicities as part of a common pathophys…
Microvascular in vivo assessment of reperfusion injury: significance of prostaglandin E1 and I2 in postischemic “no-reflow” and “reflow-paradox”
2004
Microvascular ischemia-reperfusion (I/R) injury is characterized by failure of capillary perfusion ("no-reflow") and reoxygenation-associated phenomena ("reflow-paradox"), including activation of leukocyte-endothelium interaction with cytotoxic mediator-induced loss of endothelial integrity. The objectives of this study were to elucidate the impact of both prostaglandins E(1) (PGE(1)) and I(2) (PGI(2)) in microvascular reperfusion injury, with special focus on the distinct pathophysiology of no-reflow- and reflow-paradox phenomena.By use of the hamster dorsal skinfold preparation and in vivo fluorescence microscopy, the microcirculation of a striated skin muscle was assessed before 4 h of p…
C-reactive protein and efficacy of antiplatelet therapy in (intracranial) atherosclerosis
2018
C-reactive protein (CRP) and other inflammatory biomarkers can indicate both the severity and extent of atherosclerosis, reflecting the inflammatory nature of the disease process.1 Atherogenesis begins with an inflammatory response to vascular injury with cells and mediators initiating the healing response and later inducing growth of atherosclerotic plaques. Inflammation then increases plaque instability, promoting rupture, fissuring, or erosion—the pathogenetic milieu of thrombosis in atherothrombotic ischemic strokes.