The Experts below are selected from a list of 86211 Experts worldwide ranked by ideXlab platform
Roland Riek - One of the best experts on this subject based on the ideXlab platform.
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the fold of alpha synuclein fibrils
Proceedings of the National Academy of Sciences of the United States of America, 2008Co-Authors: Marcal Vilar, Huiting Chou, Thorsten Luhrs, Samir K Maji, Dominique Riekloher, Rene Verel, Gerard Manning, Henning Stahlberg, Roland RiekAbstract:The aggregation of proteins into amyloid fibrils is associated with several neurodegenerative Diseases. In Parkinson's Disease it is believed that the aggregation of α-synuclein (α-syn) from monomers by intermediates into amyloid fibrils is the Toxic Disease-causative mechanism. Here, we studied the structure of α-syn in its amyloid state by using various biophysical approaches. Quenched hydrogen/deuterium exchange NMR spectroscopy identified five β-strands within the fibril core comprising residues 35–96 and solid-state NMR data from amyloid fibrils comprising the fibril core residues 30–110 confirmed the presence of β-sheet secondary structure. The data suggest that β1-strand interacts with β2, β2 with β3, β3 with β4, and β4 with β5. High-resolution cryoelectron microscopy revealed the protofilament boundaries of ≈2 × 3.5 nm. Based on the combination of these data and published structural studies, a fold of α-syn in the fibrils is proposed and discussed.
Mauricio Castillo - One of the best experts on this subject based on the ideXlab platform.
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Acute Marchiafava-Bignami Disease: MR Findings in Two Patients
AJNR. American journal of neuroradiology, 2003Co-Authors: Andres Arbelaez, Adriana Pajon, Mauricio CastilloAbstract:Marchiafava-Bignami is a rare Toxic Disease seen mostly in chronic alcoholics that results in progressive demyelination and necrosis of the corpus callosum. The process may extend laterally into the neighboring white matter and occasionally as far as the subcortical regions. We present the MR imaging findings in two patients who presented acutely and review the features of the Disease and of other acute alcohol-related disorders.
Marcal Vilar - One of the best experts on this subject based on the ideXlab platform.
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the fold of alpha synuclein fibrils
Proceedings of the National Academy of Sciences of the United States of America, 2008Co-Authors: Marcal Vilar, Huiting Chou, Thorsten Luhrs, Samir K Maji, Dominique Riekloher, Rene Verel, Gerard Manning, Henning Stahlberg, Roland RiekAbstract:The aggregation of proteins into amyloid fibrils is associated with several neurodegenerative Diseases. In Parkinson's Disease it is believed that the aggregation of α-synuclein (α-syn) from monomers by intermediates into amyloid fibrils is the Toxic Disease-causative mechanism. Here, we studied the structure of α-syn in its amyloid state by using various biophysical approaches. Quenched hydrogen/deuterium exchange NMR spectroscopy identified five β-strands within the fibril core comprising residues 35–96 and solid-state NMR data from amyloid fibrils comprising the fibril core residues 30–110 confirmed the presence of β-sheet secondary structure. The data suggest that β1-strand interacts with β2, β2 with β3, β3 with β4, and β4 with β5. High-resolution cryoelectron microscopy revealed the protofilament boundaries of ≈2 × 3.5 nm. Based on the combination of these data and published structural studies, a fold of α-syn in the fibrils is proposed and discussed.
Ephrem Eggermont - One of the best experts on this subject based on the ideXlab platform.
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reye syndrome revisited a descriptive term covering a group of heterogeneous disorders
European Journal of Pediatrics, 2000Co-Authors: Maria Casteelsvan Daele, Christel Van Geet, Carine Wouters, Ephrem EggermontAbstract:Reye syndrome, characterised by the combination of liver Disease and non-inflammatory encephalopathy, is a non-specific clinicopathological entity and a descriptive term covering a group of heterogeneous disorders. Nowadays, some of these patients are diagnosed more correctly as having infectious, metabolic, Toxic or other Disease. The non-specific case definition implies that the epidemiological studies suggesting a link with acetylsalicylic acid have been performed on a heterogeneous group of children, whereby the value of these studies and their ensuing hypothesis is weakened. Moreover, a detailed analysis of the epidemiological surveys of the Centers for Disease Control, the Yale study and of the British risk factor study provides evidence that not only the use of acetylsalicylic acid but also that of phenothiazines and other anti-emetics is significantly greater in Reye syndrome cases than in controls. As to the decline of Reye syndrome, recent literature data reveal that this is related to more accurate modern diagnosis of infectious, metabolic or Toxic Disease, reducing the percentage of idiopathic or true cases of Reye syndrome.
Chang Geon Chung - One of the best experts on this subject based on the ideXlab platform.
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Mechanisms of protein Toxicity in neurodegenerative Diseases
Cellular and Molecular Life Sciences, 2018Co-Authors: Chang Geon ChungAbstract:Protein Toxicity can be defined as all the pathological changes that ensue from accumulation, mis-localization, and/or multimerization of Disease-specific proteins. Most neurodegenerative Diseases manifest protein Toxicity as one of their key pathogenic mechanisms, the details of which remain unclear. By systematically deconstructing the nature of Toxic proteins, we aim to elucidate and illuminate some of the key mechanisms of protein Toxicity from which therapeutic insights may be drawn. In this review, we focus specifically on protein Toxicity from the point of view of various cellular compartments such as the nucleus and the mitochondria. We also discuss the cell-to-cell propagation of Toxic Disease proteins that complicates the mechanistic understanding of the Disease progression as well as the spatiotemporal point at which to therapeutically intervene. Finally, we discuss selective neuronal vulnerability, which still remains largely enigmatic.