The Anderson insulating states in Au nanoparticle assembly are identified and studied under the application of magnetic fields and gate voltages. When the inter-nanoparticle tunneling resistance is smaller than the quantum resistance, the system showing zero Mott gap can be insulating at very low temperature. In contrast to Mott insulators, Anderson insulators exhibit great negative magnetoresistance, inferring charge de-localization in a strong magnetic field. When probed by the electrodes spaced by ~200 nm, they also exhibit interesting gate-modulated current similar to the multi-dot single electron transistors. These results reveal the formation of charge puddles due to interplay of disorder and quantum interference at low temperatures.

Fabry disease (FD) is a recessive monogenic inheritance disease linked to chromosome X, secondary to mutations in the GLA gene. Its prevalence is estimated between 1:8,454 and 1:117,000 among males and is probably underdiagnosed. Mutations in the GLA gene lead to the progressive accumulation of globotriaosylceramide (Gb3). Gb3 accumulates in lysosomes of different types of cells of the heart, kidneys, skin, eyes, central nervous system, and gastrointestinal system, and may lead to different clinical scenarios. The impairment of the nervous system, in addition to the acroparesthesias, is characterized by hypohidrosis and cerebrovascular accidents. Gb3-accumulation supports the hypothesis that Gb3 is not solely responsible for disease manifestation. Overloading of lysosomes with Gb3 may simply lead to the rupture of cytoplasma and in consequence to cell death .Secondary effects of Gb3 accumulation which might be responsible for disease pathology include also inflammatory processes. Perturbed leukocyte function in Fabry disease compared to healthy controls and abnormal numbers of different immune cells, including lymphocytes, monocytes, CD8+ cells, B cells and dendritic cells . Noteworthy other studies reported no correlation between CRP-levels as a global inflammatory marker and the Mainz-Severity-Score-Index (MSSI) as a marker for disease severity . Other authors reported on auto-immunological reactions in α-galactosidase A deficiency. Others researchers found higher expressions of neuronal apoptosis inhibiting protein as a key anti-apoptotic mediator in children with Fabry disease compared to healthy controls. Noteworthy, this upregulation did not change after institution of ERT, whereas apoptosis inducing factor appears to be upregulated under ERT. The authors were not able to explain their observations, but further research has to be directed on this topic. Gb3-accumulation has been reported to induce oxidative stress and/or the formation reactive oxygen species (ROS). Lipids and proteins may be oxidised and may be unable to function. Of note, ERT increased the generation of ROS in vitro, and up-regulated the intracellular adhesion molecule ICAM 1. The authors hypothesized that ERT may enhance endothelial damage, allowing to understand continuously occurring strokes in patients on treatment. Furthermore, Gb3-accumulation has been reported to induce oxidative stress and/or the formation reactive oxygen species (ROS). Lipids and proteins may be oxidised and may be unable to function. Of note, ERT increased the generation of ROS in vitro, and up-regulated the intracellular adhesion molecule ICAM 1. Another gateway into alteration of endothelial function may be given by the Nitric-Oxide-Synthase-3-genotypes. Endothelium-derived nitric oxide is a key regulator of vessel wall function and cardiovascular homeostasis. Thus, neurological complications seem to be linked to different pathogenetic molecular mechanisms.Progressive accumulation of GB3 represent a possible pathogenetic event of peripheral nerve involvement, whereas central nervous involvement in the clinical setting of cerebrovascular ischemic events seem to be due to the endotheliopathy of Anderson-Fabry Disease with lacunar lesions and white matter hyperintensities ( WMHs) . In this review manuscript we revised molecular mechanisms of peripheral and central neurological complications of Anderson-Fabry Disease.

Anderson – Fabry disease (AFD) is a rare, X-linked lysosomal storage disorder caused by a mutation in the α-Galactosidase A gene resulting in α-Galactosidase A enzyme (α-Gal A) deficiency. The metabolic defect leads to progressive accumulation of glycosphingolipids and structural and functional impairment of affected organs. Due to the inheritance pattern, male patients are hemizygous with more severe manifestations of the disease as compared to females who in most cases are heterozygous with delayed and variable clinical presentation caused by uneven X – chromosome inactivation. Fabry disease cases are often identified by targeted screening programs in high-risk groups, such as in patients with end-stage renal disease, premature stroke or unexplained cardiomyopathy. Here we describe a unique case of homozygous female patient identified by a nationwide screening program in hypertrophic cardiomyopathy patients. Before the systematic screening, the patient had a diagnosis of hypertrophic obstructive cardiomyopathy and was treated accordingly including alcohol septal ablation to reduce the obstructive gradient. The confirmation of Fabry disease has led to discovery of the same variant in several members of her family. The identified variant was c.644A>G, p.Asn215Ser (N2015S) known to cause predominant cardiac involvement with late onset of the disease. The variant is amenable for an oral therapy by the small molecular chaperone migalastat which was started, then interrupted for recurrence of patient´s migraine and then re-initiated again after two years. During this period the patient received enzyme replacement therapy by agalsidase beta but developed a progressively worsening venous access. Our case illustrates the importance of systematic screening of patients with clinical evidence of hypertrophic cardiomyopathy in whom routine diagnostic process fails to discover Fabry disease, in particular variants with late onset cardiac manifestations. Many of the late onset variants are amenable for orally active therapy by migalastat which significantly improves the comfort of the treatment. Its long term results are analysed by a large international “Follow-me” registry which was designed to verify the validity of pivotal trials with migalastat in Fabry disease.

From the mid-19th century, Collegiate Gothic architecture gained prominence among American universities. Subsequently, through the efforts of American missionaries, the Collegiate Gothic style spread to Christian universities in Asia. Specifically, this study concentrates on the nuanced relationship between the transnational missionary routes and adopting the Collegiate Gothic style, primarily focusing on Anderson Hall at Soochow University, China. The investigation meticulously dissects the multifaceted factors influencing the choice of architectural styles, considering the perspectives of missionaries, architects, and other key stakeholders. Through an intricate analysis of Anderson Hall, this research illuminates the unique characteristics of Collegiate Gothic architecture. By unraveling the complex layers of East Asian campus heritage, this study enriches our comprehension of its origins. It establishes a robust foundation for guiding the preservation and revitalization of architectural styles within university campuses. This scholarly inquiry holds particular significance for experts in academic heritage conservation.

We show that the compound samarium hexaboride is a strong topological insulator using the eigenvalues of the space inversion operator in the low-energy limit of the periodic Anderson model. Additionally, we assume the presence of the ferromagnetic exchange interaction (M). A Dirac cone like feature in the surface state energy spectra is observed for M equals zero in a certain parameter range. For M not equal to zero, there is no Kramers degeneracy. We have been able to show that this phase corresponds to the quantum anomalous Hall state by calculating Berry curvature and the Chern number. Using Floquet theory, we further show that the access to a novel state with broken time reversal symmetry is possible due to the normal incidence of circularly polarized optical field on the surface of the compound despite M being absent.

The basic equation for estimating the aerodynamic power captured by an Anderson Vertical Axis Wind Turbine (AVAWT) is a solution of the Navier-Stokes(N-S) equations for a baroclinic, inviscid flow. In a nutshell, the pressure difference across the AVAWT is derived from Bernoulli’s equation; an upshot of the integration of the N-S momentum equation for a baroclinic inviscid flow, Euler’s momentum equation. The resulting expression for the pressure difference across the AVAWT rotor is plotted as a function of freestream speed. Experimentally determined airstream speeds at the AVAWT inlet and outlet, coupled with corresponding freestream speeds are used in estimating the aerodynamic power captured. The aerodynamic power is subsequently used in calculating the aerodynamic power coefficient of the AVAWT. The actual power coefficient is calculated from the power generated by the AVAWT at various free stream speeds and plotted as a function of the latter. Experimental results show that, at all free stream speeds and tip speed ratios, the aerodynamic power coefficient is higher than the actual power coefficient of the AVAWT. Consequently, the power generated by the AVAWT prototype is lower than the aerodynamic power captured, given the same inflow wind condition.