Decreased serum antioxidant capacity in patients with Wilson disease is associated with neurological symptoms

Background &; Aims

Wilson disease (WD) is an inherited disorder of copper disposition caused by an ATP7B transporter gene mutation, leading to copper accumulation in predisposed tissues. In addition to a genetic predisposition, other factors are likely to contribute to its clinical manifestation. The aim of the study was to assess whether oxidative stress affects the phenotypic manifestation of WD.

Methods

In 56 patients with WD (29 men; 26 with the hepatic form, 22 with the neurologic form, and eight asymptomatic; mean age 38.5?±?12 years), total serum antioxidant capacity (TAC) and inflammatory parameters (hs-CRP, IL-1??, IL-2, IL-6, IL-10, and TNF-??) were analyzed and related to the clinical manifestation, and mutations of the ATP7B gene. The control group for the TAC and inflammatory parameters consisted of 50 age- and gender-matched healthy individuals.

Results

WD patients had a significantly lower TAC (p?Conclusions Data from our study suggest that the increased oxidative stress contributes significantly to the clinical manifestation of WD; as a lower TAC is associated with the neurological symptoms in WD patients.     

Pulmonary arterial hypertension associated with systemic sclerosis in the Czech Republic

Background  

Systemic sclerosis (SSc) is an important cause of pulmonary arterial hypertension (PAH), with an estimated prevalence of 7.85–26.7%.     

Genetic mapping of habitual substance use, obesity-related traits, responses to mental and physical stress, and heart rate and blood pressure measurements reveals shared genes that are overrepresented in the neural synapse

Links between substance use habits, obesity, stress and the related cardiovascular outcomes can be, in part, because of loci with pleiotropic effects. To investigate this hypothesis, we performed genome-wide mapping in 119 multigenerational families from a population in the Saguenay-Lac-St-Jean region with a known founder effect using 58,000 single-nucleotide polymorphisms and 437 microsatellite markers to identify genetic components of the following factors: habitual alcohol, tobacco and coffee use; response to mental and physical stress; obesity-related traits; and heart rate (HR) and blood pressure (BP) measures. Habitual alcohol and/or tobacco users had attenuated HR responses to mental stress compared with non-users, whereas hypertensive individuals had stronger HR and systolic BP responses to mental stress and a higher obesity index than normotensives. Genetic mappings uncovered numerous shared genes among substance use, stress response, obesity and hemodynamic traits, including CAMK4, CNTN4, DLG2, FHIT, GRID2, ITPR2, NOVA1 and PRKCE, forming network of interacting proteins, sharing synaptic function and display higher and patterned expression profiles in brain-related tissues; moreover, pathway analysis of shared genes pointed to long-term potentiation. Subgroup genetic mappings uncovered additional shared synaptic genes, including CAMK4, CNTN5 and DNM3 (hypertension-specific); CNTN4, DNM3, FHIT and ITPR1 (sex-specific), having protein interactions with genes driven from general analysis. In summary, consistent with the observed phenotypic correlations, we found substantial overlap among genomic determinants of these traits in synapse, which supports the notion that the neural synapse may be a shared interface behind substance use, stress, obesity, HR, BP as well as the observed sex- and hypertension-specific genetic differences.     

In situ production of a two-dimensional molybdenum disulfide/graphene hybrid nanosheet anode for lithium-ion batteries

A solvent-free, low-cost, high-yield and scalable single-step ball milling process is developed to construct 2D MoS₂/graphene hybrid electrodes for lithium-ion batteries. Electron microscopy investigation reveals that the obtained hybrid electrodes consist of numerous nanosheets of MoS₂ and graphene which are randomly distributed. The MoS₂/graphene hybrid anodes exhibit excellent cycling stability with high reversible capacities (442 mA h g⁻¹ for MoS₂/graphene (40 h); 553 mA h g⁻¹ for MoS₂/graphene (20 h); 342 mA h g⁻¹ for MoS₂/graphene (10 h)) at a high current rate of 250 mA g⁻¹ after 100 cycles, whereas the pristine MoS₂ electrode shows huge capacity fading with a retention of 37 mA h g⁻¹ at 250 mA g⁻¹ current after 100 cycles. The incorporation of graphene into MoS₂ has an extraordinary effect on its electrochemical performance. This work emphasises the importance of the construction of the 2D MoS₂/graphene hybrid structure to prevent capacity fading issues with the MoS₂ anode in lithium-ion batteries.

A solvent-free, low-cost, high-yield and scalable single-step ball milling process is developed to construct 2D MoS₂/graphene hybrid electrodes for lithium-ion batteries.     

Hybrid fluorescent liquid crystalline composites: directed assembly of quantum dots in liquid crystalline block copolymer matrices

Hybrid fluorescent liquid crystalline (LC) composites containing inorganic quantum dots (QDs) are promising materials for many applications in optics, nanophotonics and display technology, combining the superior emission capability of QDs with the externally controllable optical properties of LCs. In this work, we propose the hybrid LC composites that were obtained by embedding CdSe/ZnS QDs into a series of host LC block copolymers of different architectures by means of a two-stage ligand exchange procedure. The ABA/BAB triblock copolymers and AB diblock copolymers with different polymerization degrees are composed of nematogenic phenyl benzoate acrylic monomer units and poly(4-vinylpyridine) blocks, which are capable of binding to the QD surface. Our results clearly show that the spatial distribution of QDs within composite films as well as the formation of QD aggregates can be programed by varying the structure of the host block copolymer. The obtained composites form a nematic LC phase, with isotropization temperatures being close to those of the initial host block copolymers. In addition, the influence of the molecular architecture of the host block copolymers on fluorescence properties of the obtained composites is considered. The described strategy for the QD assembly should provide a robust and conventional route for the design of highly ordered hierarchical hybrid materials for many practical applications.

Spatial distribution of QDs within hybrid composite films was programed by varying the molecular architecture of the host LC block copolymers.