Search results for "Ceph"
showing 10 items of 2036 documents
Spike-wave discharges in absence epilepsy: segregation of electrographic components reveals distinct pathways of seizure activity.
2020
Key points The major electrophysiological hallmarks of absence seizures are spike and wave discharges (SWDs), consisting of a sharp spike component and a slow wave component. In a widely accepted scheme, these components are functionally coupled and reflect an iterative progression of neuronal excitation during the spike and post-excitatory silence during the wave. In a genetic rat model of absence epilepsy, local pharmacological inhibition of the centromedian thalamus (CM) selectively suppressed the spike component, leaving self-contained waves in epidural recordings. Thalamic inputs induced activity in cortical microcircuits underlying the spike component, while intracortical oscillations…
Automatic sleep scoring: A deep learning architecture for multi-modality time series
2020
Background: Sleep scoring is an essential but time-consuming process, and therefore automatic sleep scoring is crucial and urgent to help address the growing unmet needs for sleep research. This paper aims to develop a versatile deep-learning architecture to automate sleep scoring using raw polysomnography recordings. Method: The model adopts a linear function to address different numbers of inputs, thereby extending model applications. Two-dimensional convolution neural networks are used to learn features from multi-modality polysomnographic signals, a “squeeze and excitation” block to recalibrate channel-wise features, together with a long short-term memory module to exploit long-range co…
BAX inhibitor-1 is a Ca(2+) channel critically important for immune cell function and survival.
2015
The endoplasmic reticulum (ER) serves as the major intracellular Ca(2+) store and has a role in the synthesis and folding of proteins. BAX (BCL2-associated X protein) inhibitor-1 (BI-1) is a Ca(2+) leak channel also implicated in the response against protein misfolding, thereby connecting the Ca(2+) store and protein-folding functions of the ER. We found that BI-1-deficient mice suffer from leukopenia and erythrocytosis, have an increased number of splenic marginal zone B cells and higher abundance and nuclear translocation of NF-κB (nuclear factor-κ light-chain enhancer of activated B cells) proteins, correlating with increased cytosolic and ER Ca(2+) levels. When put into culture, purifie…
Lack of NFATc1 SUMOylation prevents autoimmunity and alloreactivity
2020
A novel transgenic mouse, in which the transcription factor NFATc1 bears lysine-to-arginine mutations that prevent modification by SUMO, develops normally and is healthy. However, SUMO-insensitive NFATc1 transmits strong tolerogenic signals, thus preventing autoimmune and alloimmune T cell responses.
Lethal systemic and brain infection caused by Prototheca zopfii algae in a patient with acute myeloid leukemia
2021
Systemic protothecosis is an exceptionally rare cause of sepsis with few available therapeutic options. Here, we report on a female patient with newly diagnosed acute myeloid leukemia who died after start of chemotherapy due to a severe septic shock caused by a disseminated systemic infection with Prototheca zopfii including encephalitis.
Transcription factor NRF2 as a therapeutic target for chronic diseases: a systems medicine approach
2018
Systems medicine has a mechanism-based rather than a symptom- or organ-based approach to disease and identifies therapeutic targets in a nonhypothesis-driven manner. In this work, we apply this to transcription factor nuclear factor (erythroid-derived 2)-like 2 (NRF2) by cross-validating its position in a protein-protein interaction network (the NRF2 interactome) functionally linked to cytoprotection in low-grade stress, chronic inflammation, metabolic alterations, and reactive oxygen species formation. Multiscale network analysis of these molecular profiles suggests alterations of NRF2 expression and activity as a common mechanism in a subnetwork of diseases (the NRF2 diseasome). This netw…
A T cell-specific deletion of HDAC1 protects against experimental autoimmune encephalomyelitis.
2017
Multiple sclerosis (MS) is a human neurodegenerative disease characterized by the invasion of autoreactive T cells from the periphery into the CNS. Application of pan-histone deacetylase inhibitors (HDACi) ameliorates experimental autoimmune encephalomyelitis (EAE), an animal model for MS, suggesting that HDACi might be a potential therapeutic strategy for MS. However, the function of individual HDAC members in the pathogenesis of EAE is not known. In this study we report that mice with a T cell-specific deletion of HDAC1 (using the Cd4-Cre deleter strain; HDAC1-cKO) were completely resistant to EAE despite the ability of HDAC1cKO CD4+ T cells to differentiate into Th17 cells. RNA sequencin…
PEX6 is Expressed in Photoreceptor Cilia and Mutated in Deafblindness with Enamel Dysplasia and Microcephaly
2015
Deafblindness is part of several genetic disorders. We investigated a consanguineous Egyptian family with two siblings affected by congenital hearing loss and retinal degeneration, initially diagnosed as Usher syndrome type 1. At teenage, severe enamel dysplasia, developmental delay, and microcephaly became apparent. Genome-wide homozygosity mapping and whole-exome sequencing detected a homozygous missense mutation, c.1238G>T (p.Gly413Val), affecting a highly conserved residue of peroxisomal biogenesis factor 6, PEX6. Biochemical profiling of the siblings revealed abnormal and borderline plasma phytanic acid concentration, and cerebral imaging revealed white matter disease in both. We show …
Nutritional control of IL-23/Th17-mediated autoimmune disease through HO-1/STAT3 activation
2017
AbstractThe nutritional curcumin (CUR) is beneficial in cell-mediated autoimmune diseases. The molecular mechanisms underlying this food-mediated silencing of inflammatory immune responses are poorly understood. By investigating antigen-specific immune responses we found that dietary CUR impairs the differentiation of Th1/Th17 cells in vivo during encephalomyelitis and instead promoted Th2 cells. In contrast, feeding CUR had no inhibitory effect on ovalbumin-induced airway inflammation. Mechanistically, we found that CUR induces an anti-inflammatory phenotype in dendritic cells (DC) with enhanced STAT3 phosphorylation and suppressed expression of Il12b and Il23a. On the molecular level CUR …
Multiple organ failure as onset of Mediterranean spotted fever: a review based on a case
2016
Mediterranean spotted fever (MSF) is an infectious disease endemic in the southern regions of Italy, with an incidence of about 400 cases/year. The bacteria responsible of the disease is <em>Rickettsia conorii</em>, transmitted to humans by <em>Rhipicephalus sanguineus</em>, the common dog tick. The infection usually manifests with a characteristic symptomatologic triad: fever, exanthema and the so called <em>tache noire</em>, which is the typical eschar at the site of the tick bite. Immunoglobulin M (IgM) and IgG enzymelinked immunosorbent assay and the gold standard micro-immunofluorescent assay, allow serological diagnosis. We report the case of a man …