Early Growth and Development Impairments in Patients with Ganglioside GM3 Synthase Deficiency

Ganglioside GM3 synthase is a key enzyme involved in the biosynthesis of gangliosides. GM3 synthase deficiency (GSD) causes a complete absence of GM3 and all downstream biosynthetic derivatives. The individuals affected by this disorder manifest severe irritability, intractable seizures and profound intellectual disability. However, we have found that most newborns seem symptom-free for a period of time after birth. In order to further understand the onset of the disease, we investigated the early growth and development of patients with this condition through this study. We compared 37 affected individuals with their normal siblings and revealed that all children with GSD had relatively normal intrauterine growth and development, as their weight, length and head circumference were similar to their normal siblings at birth. However, the disease progresses quickly after birth and causes significant constitutional impairments of growth and development by 6 months of age. Neither breastfeeding nor gastrostomy tube placement made significant difference on growth and development as all groups of patients showed the similar pattern. We conclude that GSD causes significant postnatal growth and developmental impairments and the amount of gangliosides in breast milk and general nutritional intervention do not seem to alter these outcomes

City of Cuero Site Analysis and Redevelopment Recommendations

Cuero is a small south Texas town which prides itself on preserving the history and heritage of its community while providing southern hospitality to its residents and visitors.Cuero Development Corporation Master Plan- Developed a plan for the best use of a 4.519-acre property. Conducted a comprehensive assessment of options for the development of the property.Texas Target Communitie

Polytypism of Incommensurately Modulated Structures of Crystalline Bromine upon Molecular Dissociation under High Pressure

Polytypism of incommensurately modulated structures was hitherto unobserved. Here, we found the phenomenon in simple halogen systems of bromine and iodine upon molecular dissociation in the solids under pressure. Single-crystal synchrotron X-ray diffraction in laser heated diamond anvil cells pressurised up to 112 GPa revealed a number of allotropes of bromine and iodine including polytypes of Br-III{\gamma} (Fmmm(00{\gamma})s00) with {\gamma} varying within 0.18 to 0.3

Association between hearing aid use and all-cause and cause-specific dementia: an analysis of the UK Biobank cohort.

BACKGROUND: Dementia and hearing loss are both highly prevalent conditions among older adults. We aimed to examine the association between hearing aid use and risk of all-cause and cause-specific dementia among middle-aged and older-aged adults, and to explore the roles of mediators and moderators in their association. METHODS: We used data from the UK Biobank, a population-based cohort study, which recruited adults aged 40-69 years between 2006 and 2010 across 22 centres in England, Scotland, and Wales. We used Cox proportional hazards models to estimate hazard ratios (HRs) and 95% CIs between self-reported hearing aid use status (hearing loss with or without hearing aids) at baseline and risk of dementia (all-cause dementia, Alzheimer's disease, vascular dementia, and non-Alzheimer's disease non-vascular dementia). Dementia diagnoses were ascertained using hospital records and death-register data. We also analysed the roles of mediators (self-reported social isolation, loneliness, and mood) and moderators (self-reported education and income, smoking, morbidity, and measured APOE allele status). FINDINGS: After the exclusion of people who did not answer the question on hearing difficulties (n=25 081 [5·0%]) and those with dementia at baseline visit (n=283 [0·1%]), we included 437 704 people in the analyses. Compared with participants without hearing loss, people with hearing loss without hearing aids had an increased risk of all-cause dementia (HR 1·42 [95% CI 1·29-1·56]); we found no increased risk in people with hearing loss with hearing aids (1·04 [0·98-1·10]). The positive association of hearing aid use was observed in all-cause dementia and cause-specific dementia subtypes (Alzheimer's disease, vascular dementia, and non-Alzheimer's disease non-vascular dementia). The attributable risk proportion of dementia for hearing loss was estimated to be 29·6%. Of the total association between hearing aid use and all-cause dementia, 1·5% was mediated by reducing social isolation, 2·3% by reducing loneliness, and 7·1% by reducing depressed mood. INTERPRETATION: In people with hearing loss, hearing aid use is associated with a risk of dementia of a similar level to that of people without hearing loss. With the postulation that up to 8% of dementia cases could be prevented with proper hearing loss management, our findings highlight the urgent need to take measures to address hearing loss to improve cognitive decline. FUNDING: National Natural Science Foundation of China and Shandong Province, Taishan Scholars Project, China Medical Board, and China Postdoctoral Science Foundation

Self-assembling subnanometer pores with unusual mass-transport properties

A long-standing aim in molecular self-assembly is the development of synthetic nanopores capable of mimicking the mass-transport characteristics of biological channels and pores. Here we report a strategy for enforcing the nanotubular assembly of rigid macrocycles in both the solid state and solution based on the interplay of multiple hydrogen-bonding and aromatic π − π stacking interactions. The resultant nanotubes have modifiable surfaces and inner pores of a uniform diameter defined by the constituent macrocycles. The self-assembling hydrophobic nanopores can mediate not only highly selective transmembrane ion transport, unprecedented for a synthetic nanopore, but also highly efficient transmembrane water permeability. These results establish a solid foundation for developing synthetically accessible, robust nanostructured systems with broad applications such as reconstituted mimicry of defined functions solely achieved by biological nanostructures, molecular sensing, and the fabrication of porous materials required for water purification and molecular separations

Impaired word and face recognition in older adults with type 2 diabetes

Background: Older adults with type 2 diabetes mellitus (DM2) exhibit accelerated decline in some domains of cognition, including verbal episodic memory. Few studies have investigated the influence of DM2 status in older adults on recognition memory for more complex stimuli, such as faces. Aims: In the present study, we sought to compare recognition memory performance for words, objects and faces under conditions of relatively low and high cognitive load. Methods: Healthy older adults with good glucoregulatory control (n = 13) and older adults with DM2 (n = 24) were administered recognition memory tasks in which stimuli (faces, objects and words) were presented under conditions of either i) low (stimulus presented without a background pattern), or ii) high (stimulus presented against a background pattern) cognitive load. Results: In a subsequent recognition phase, the DM2 group recognised fewer faces than healthy controls. Further, the DM2 group exhibited word recognition deficits in the low cognitive load condition. Conclusions: The recognition memory impairment observed in the patients with DM2 has clear implications for day-to-day functioning. While these deficits were not amplified under conditions of increased cognitive load, the present study emphasises that recognition memory impairment for both words and more complex stimuli, such as faces, are a feature of DM2 in older adults

Effect of particle size on silver nanoparticle deposition onto dielectric barrier discharge (DBD) plasma functionalized polyamide fabric

The effect on the deposition of three different size silver nanoparticles (AgNPs) onto a polyamide 6,6 (PA) fabric pre-treated using air dielectric barrier discharge (DBD) plasma was investigated. The SEM, EDS, and XPS analysis confirm that the smaller is the diameter of AgNPs, the higher the amount of adsorbed NPs on the PA. The DBD treatment on PA induces a threefold increase in Ag adsorption. The result confirms a dual effect on the wettability of the plasma treated PA substrate. AgNPs slightly enhance hydrophobicity of the PA surface and, at the same time, protect it against the plasma aging effect. The effect on the deposition of three different size silver nanoparticles (AgNPs) onto a Polyamide 6,6 (PA) fabric pre-treated using air dielectric barrier discharge (DBD) plasma was investigated. The smaller is the size, the higher the loaded AgNPs. The DBD treatment induces a threefold increase in Ag adsorption. AgNPs enhance hydrophobicity of the PA surface and reduce the plasma aging effect.Fundação para a Ciência e a Tecnologia (FCT

Seroprevalence of Pandemic (H1N1) 2009 in Pregnant Women in China: An Observational Study

BACKGROUND: We investigated the seropositive rates and persistence of antibody against pandemic (H1N1) 2009 virus (pH1N1) in pregnant women and voluntary blood donors after the second wave of the pandemic in Nanjing, China. METHODOLOGY/PRINCIPAL FINDINGS: Serum samples of unvaccinated pregnant women (n = 720) and voluntary blood donors (n = 320) were collected after the second wave of 2009 pandemic in Nanjing. All samples were tested against pH1N1 strain (A/California/7/2009) with hemagglutination inhibition assay. A significant decline in seropositive rates, from above 50% to about 20%, was observed in pregnant women and voluntary blood donors fifteen weeks after the second wave of the pandemic. A quarter of the samples were tested against a seasonal H1N1 strain (A/Brisbane/59/2007). The antibody titers against pH1N1 strain were found to correlate positively with those against seasonal H1N1 strain. The correlation was modest but statistically significant. CONCLUSIONS AND SIGNIFICANCE: The high seropositive rates in both pregnant women and voluntary blood donors suggested that the pH1N1 virus had widely spread in these two populations. Immunity derived from natural infection seemed not to be persistent well

Measurement of the inclusive and dijet cross-sections of b-jets in pp collisions at sqrt(s) = 7 TeV with the ATLAS detector

The inclusive and dijet production cross-sections have been measured for jets containing b-hadrons (b-jets) in proton-proton collisions at a centre-of-mass energy of sqrt(s) = 7 TeV, using the ATLAS detector at the LHC. The measurements use data corresponding to an integrated luminosity of 34 pb^-1. The b-jets are identified using either a lifetime-based method, where secondary decay vertices of b-hadrons in jets are reconstructed using information from the tracking detectors, or a muon-based method where the presence of a muon is used to identify semileptonic decays of b-hadrons inside jets. The inclusive b-jet cross-section is measured as a function of transverse momentum in the range 20 < pT < 400 GeV and rapidity in the range |y| < 2.1. The bbbar-dijet cross-section is measured as a function of the dijet invariant mass in the range 110 < m_jj < 760 GeV, the azimuthal angle difference between the two jets and the angular variable chi in two dijet mass regions. The results are compared with next-to-leading-order QCD predictions. Good agreement is observed between the measured cross-sections and the predictions obtained using POWHEG + Pythia. MC@NLO + Herwig shows good agreement with the measured bbbar-dijet cross-section. However, it does not reproduce the measured inclusive cross-section well, particularly for central b-jets with large transverse momenta.Comment: 10 pages plus author list (21 pages total), 8 figures, 1 table, final version published in European Physical Journal