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Background:ATP8A2 mutations have only recently been associated with human disease. We present the clinical features from the largest cohort of patients with this disorder reported to date. Methods: An observational study of 9 unreported and 2 previously reported patients with biallelic ATP8A2 mutations was carried out at multiple centres. Results: The mean age of the cohort was 9.4 years old (range: 2.5-28 yrs). All patients demonstrated developmental delay, severe hypotonia and movement disorders: chorea/choreoathetosis (100%), dystonia (27%) or facial dyskinesia (18%). Hypotonia was apparent at birth (70%) or before 6 months old (100%). Optic atrophy was observed in 75% of patients who had a funduscopic examination. MRI of the brain was normal for most patients with a small proportion showing mild cortical atrophy (30%), delayed myelination (20%) and/or hypoplastic optic nerves (20%). Epilepsy was seen in two older patients. Conclusions:ATP8A2 gene mutations have emerged as a cause of a novel phenotype characterized by developmental delay, severe hypotonia and hyperkinetic movement disorders. Optic atrophy is common and may only become apparent in the first few years of life, necessitating repeat ophthalmologic evaluation. Early recognition of the cardinal features of this condition will facilitate diagnosis of this disorder.
Forest carbon sequestration plays an important role in reducing the build-up of greenhouse gases that are known to contribute to global climate change. However, private landowners will supply less carbon sequestration than would be socially desirable if they are unable to capture the economic value of sequestration. We examine the viability of offering landowners property tax subsidies for forest carbon sequestration (referred to as a ‘tax-based subsidy approach’). Waiving property taxes on forestland provides incentives for landowners to afforest non-forested land and/or sustain forests that are at risk of deforestation. We focus on 17 Tennessee counties and one Kentucky county, constituting one of 179 Bureau of Economic Analysis areas in the United States, as a case study. Higher forestland net return from waiving property taxes increases the share of forestland in the 18 counties, which in turn increases the accumulation of carbon in the forest ecosystem, suggesting that this is a viable approach. The annualized county-level cost of supplying forest carbon sequestration using a tax-based subsidy ranges between US$15.56 and US$563.58 per carbon tonne across the 18 counties. Relevant government agencies can use these estimates to target selected counties for more cost-effective adoption of the county-level tax-based subsidy approach.
Coaxially nested intense
sheared flow realized an upgraded stable mirror plasma regime. After such an external control of high vorticity formation due to electron cyclotron heating, significantly unstable plasmas appeared. Thereby, the associated cross-field transport caused a crash of plasmas. Its generalized physics and interpretation could prepare or extend to another possibility of stability in a field-reversed configuration (FRC), for instance. Such underlying physics bases of vorticity formation were essentially or partially performed in tokamaks and stellarators (solved problems). Nevertheless, it remains to be seen whether this mirror-based experimental evidence is applicable or not to open ended FRC devices. This open issue may give a solution of one of unsolved important problems, and possibly provide more generalized and externally controllable opportunities for not only FRC but wider plasma confinement improvements.
Sparganosis is one of the top three tissue-dwelling heterologous helminthic diseases, along with cysticercosis and paragonimiasis, in Korea. Due to a lack of effective early diagnosis and treatment methods, this parasitic disease is regarded as a public health threat. This study evaluated reactivity, against sparganum extracts, of sera from inhabitants of Cheorwon-gun, Goseong-gun and Ongjin-gun in Korea. The sera from 836 subjects were subjected to enzyme-linked immunosorbent assay and immunoblot analysis. The sera from 18 (5.8%) and 15 (5.1%) inhabitants in Cheorwon-gun (n = 312) and Goseong-gun (n = 294), respectively, exhibited highly positive reactions to the sparganum antigen, whereas only two (0.9%) inhabitants in Ongjin-gun (n = 230) showed positivity. We sought antigenic proteins for serodiagnosis of positive sera by immunoproteomic approaches. Total sparganum lysates were separated by two-dimensional electrophoresis and then subjected to immunoblot analysis with mixed sparganosis-positive sera. We found seven antigenic spots and identified paramyosin as an antigenic protein by liquid chromatography–mass spectrometry. By two-dimensional (2D)-based mass analysis and immunoblotting against sparganosis-positive sera, paramyosin was identified as a candidate antigen for serodiagnosis of sparganosis.
Schizophrenia patients have a higher prevalence of type 2 diabetes mellitus with impaired glucose tolerance (IGT) than normals. We examined the relationship between IGT and clinical phenotypes or cognitive deficits in first-episode, drug-naïve (FEDN) Han Chinese patients with schizophrenia.
A total of 175 in-patients were compared with 31 healthy controls on anthropometric measures and fasting plasma levels of glucose, insulin and lipids. They were also compared using a 75 g oral glucose tolerance test and the homeostasis model assessment of insulin resistance (HOMA-IR). Neurocognitive functioning was assessed using the MATRICS Consensus Cognitive Battery (MCCB). Patient psychopathology was assessed using the Positive and Negative Syndrome Scale (PANSS).
Of the patients, 24.5% had IGT compared with none of the controls, and they also had significantly higher levels of fasting blood glucose and 2-h glucose after an oral glucose load, and were more insulin resistant. Compared with those patients with normal glucose tolerance, the IGT patients were older, had a later age of onset, higher waist or hip circumference and body mass index, higher levels of low-density lipoprotein and triglycerides and higher insulin resistance. Furthermore, IGT patients had higher PANSS total and negative symptom subscale scores, but no greater cognitive impairment except on the emotional intelligence index of the MCCB.
IGT occurs with greater frequency in FEDN schizophrenia, and shows association with demographic and anthropometric parameters, as well as with clinical symptoms but minimally with cognitive impairment during the early course of the disorder.
We present recent observation results of Sgr A* at millimeter obtained with VLBI arrays in Korea and Japan.
7 mm monitoring of Sgr A* is part of our AGN large project. The results at 7 epochs during 2013-2014, including high resolution maps, flux density and two-dimensional size measurements are presented. The source shows no significant variation in flux and structure related to the G2 encounter in 2014. According to recent MHD simulations by kawashima et al., flux and magnetic field energy can be expected to increase several years after the encounter; We will keep our monitoring in order to test this prediction.
Astrometric observations of Sgr A* were performed in 2015 at 7 and 3.5 millimeter simultaneously. Source-frequency phase referencing was applied and a combined ”core-shift” of Sgr A* and a nearby calibrator was measured. Future observations and analysis are necessary to determine the core-shift in each source.
Obsessive–compulsive disorder (OCD) has been associated with abnormal cognitive and emotional functions and these dysfunctions may be dependent on the disruption of dynamic interactions within neuronal circuits associated with emotion regulation. Although several studies have shown the aberrant cognitive–affective processing in OCD patients, little is known about how to characterize effective connectivity of the disrupted neural interactions. In the present study, we applied effective connectivity analysis using dynamic causal modeling to explore the disturbed neural interactions in OCD patients.
A total of 20 patients and 21 matched healthy controls performed a delayed-response working memory task under emotional or non-emotional distraction while undergoing functional magnetic resonance imaging.
During the delay interval under negative emotional distraction, both groups showed similar patterns of activations in the amygdala. However, under negative emotional distraction, the dorsolateral prefrontal cortex (DLPFC) and the orbitofrontal cortex (OFC) exhibited significant differences between groups. Bayesian model averaging indicated that the connection from the DLPFC to the OFC was negatively modulated by negative emotional distraction in patients, when compared with healthy controls (p < 0.05, Bonferroni-corrected).
Exaggerated recruitment of the DLPFC may induce the reduction of top-down prefrontal control input over the OFC, leading to abnormal cortico-cortical interaction. This disrupted cortico-cortical interaction under negative emotional distraction may be responsible for dysfunctions of cognitive and emotional processing in OCD patients and may be a component of the pathophysiology associated with OCD.
Background: The hedgehog pathway (Hh) is an important developmental signaling pathway that is commonly dysregulated in brain tumors, most notably in medulloblastomas. To identify novel therapeutic targets within the Hh pathway, we performed the first quantitative proteome-wide evaluation of phosphorylation events resulting from in vitro SHH administration and occurring throughout Hh-driven cerebellar development in vivo. Methods: Multiplexed quantitative mass spectrometry was done using Tandem Mass Tags 10-plex reagents, TiO2 phosphopeptide enrichment and HPLC-MS/MS/MS. Results: Motif analysis of 2-fold changing phosphorylation events suggested casein kinase 2 (CK2) was responsible for mediating 45% of all changes in phosphorylation. Epistasis studies revealed that CK2 activity is necessary for hedgehog signaling and affects terminal signaling components, thereby circumventing challenges of emergence of resistance and a priori resistance that are commonly encountered with existing small molecule inhibitors in medulloblastoma. In vivo, mice harboring MB allografts resistant to current therapies showed near-complete cessation of tumor growth in response to a CK2 inhibitor. Conclusion: Our use of developmental phosphoproteomics revealed casein kinase 2 as a key regulator of hedgehog signaling and therapeutic target in medulloblastoma. Our success establishes a foundation for us, and others, to apply a similar approach in different tumor initiating pathways.
We discuss the advantages of V2O5-P2O5-Fe2O3-Li2O glass-ceramics as a cathode for lithium-ion batteries. The glass was prepared by using the melt quenching method. The glass-ceramics were produced by heat treatment in air. LixV2O5 crystal was only confirmed as the precipitated phase and the degree of crystallinity was approximately 90%. The total capacity of the glass-ceramics was 340 Ah/kg at a C/20 rate for 1.5-4.2 V cutoff ranges. It is 10% higher than the capacity of the glass cathode. Moreover, the charge-discharge performance of the glass-ceramics cathode showed good cycleability similar to that of the glass. The glass-ceramics had a 83% capacity retention after 40 cycles. These results show that glass-ceramics is a potential candidate for lithium-ion cathode materials.
Dense thin β/β’’-alumina electrolyte films of less than 50 μm thickness were fabricated using vacuum dip-coating on porous substrate tubes. The porous substrate tubes were fabricated using a slip casting method. Fine Na-β/β’’-alumina powder was obtained via traditional solid state reaction processing. It was found that vacuum dip-coating is an effective method for fabricating thin dense layers coated on the porous tube. The mechanical properties of the porous tube, with and without the dense layer, were tested using a C-ring method. The optimized sintering process was also studied.
Three types of silica materials with different morphology, specifically SiO2 hollow microspheres, mesoporous silica, and silica aerogel were tested as potential precursors for synthesis of silicon nano- and meso-structures that resemble the original morphology of the precursors. In the optimized magnesium thermal reduction process, magnesium vapor was delivered to silica surface through a stainless steel mesh placed on top of a zirconia boat filled with silica precursor. This approach allowed for better control of silicon nanostructure formation by minimizing reaction by-products that can affect performance of lithium ion battery anode. Material morphological properties of the reduced silica precursors are discussed in terms of X-ray diffraction, BET, BJH pore size distribution, Raman spectroscopy, and TGA.
Recent experimental evidence on nano-particle and nano-wire silicon anodes showed an initial rapid velocity of reaction front at the initial stage of lithiation, followed by an apparent slowing or even halting of the reaction front propagation. This intriguing phenomenon is attributed to the lithiation-induced mechanical stresses across the reaction front which is believed to play an important role in the kinetics of reaction at the front. Here, through theoretical formulation, we presented a comprehensive study on lithiation-induced stress field and its contribution to the driving force of lithiation in hollow spherical anodes with different boundary conditions at the inner surface of the particle. Our results reveal that hollow spherical silicon anodes can be lithiated more easily than solid spherical silicon particles and thus may serve as an optimal design of high performance anodes of lithium-ion battery.
Silicon is emerging as a very attractive anode material for lithium ion batteries due to its low discharge potential, natural abundance, and high theoretical capacity of 4200 mAh/g, more than ten times that of graphite (372 mAh/g). This high charge capacity is the result of silicon’s ability to incorporate 4.4 lithium atoms per silicon atom; however, the incorporation of lithium also leads to a 300-400% volume expansion during charging, which can cause pulverization of the material and loss of access to the silicon. The architecture of the anode must therefore be able to adapt to this volume increase. Here we present a layered carbon nanotube and silicon nanoparticle electrode structure, fabricated using directed assembly techniques. The porous carbon nanotube layers maintain electrical connectivity through the active material and increase the surface area of the current collector. Using this architecture, we obtain an initial capacity in excess of 4000 mAh/g, as well as increased power and energy density as compared to anodes fabricated using the standard procedure of slurry casting.
In the present work nanostructures of manganese dioxide have been synthesized and characterized as potential catalysts for Li-air batteries. The R-MnO2 nanourchin-shaped catalyst was synthesized by mild hydrothermal conditions under autogeneous pressure. X-ray powder diffraction (XRD) was used to confirm the formation of single R-MnO2. The microstructure of the obtained nanostructures was investigated by scanning and transmission electron microscopy (SEM and TEM) showing the presence of acicular manganese oxide aggregates (5-10 nm wide) which tend to form spherical clusters, taking on an urchin-shaped form of roughly 6 microns diameter. The cyclability analyses reveal an enhanced performance and efficiency for the batteries with higher amounts of catalyst. This catalyst is thought to promote alternative reaction pathways in the Li2CO3 decomposition which attenuate the instability of the electrolyte and/or carbon electrode during the discharge resulting in an improved cyclability.
Enterotoxigenic Escherichia coli (ETEC) is now recognized as a common cause of foodborne outbreaks. This study aimed to describe the first ETEC O169 outbreak identified in Korea. In this outbreak, we identified 1642 cases from seven schools. Retrospective cohort studies were performed in two schools; and case-control studies were conducted in five schools. In two schools, radish kimchi was associated with illness; and in five other schools, radish or cabbage kimchi was found to have a higher risk among food items. Adjusted relative risk of kimchi was 5·87–7·21 in schools that underwent cohort studies; and adjusted odds ratio was 4·52–12·37 in schools that underwent case-control studies. ETEC O169 was isolated from 230 affected students, and was indistinguishable from the isolates detected from the kimchi product distributed by company X, a food company that produced and distributed kimchi to all seven schools. In this outbreak, we found that the risk of a kimchi-borne outbreak of ETEC O169 infection is present in Korea. We recommend continued monitoring regarding food safety in Korea, and strengthening surveillance regarding ETEC O169 infection through implementation of active laboratory surveillance to confirm its infection.