Table_1_A Novel Variant in Non-coding Region of GJB1 Is Associated With X-Linked Charcot-Marie-Tooth Disease Type 1 and Transient CNS Symptoms.DOC

X-linked Charcot-Marie-Tooth disease type 1 (CMTX1) is a dominantly inherited peripheral neuropathy and is caused by mutations in gap junction beta 1 gene (GJB1). Here, a novel variant of c.-170T>G in GJB1 was identified in a large Chinese CMTX1 pedigree. The proband presented transient “stroke-like” episodes in addition to the peripheral neuropathy. At the time of episode, he had transient hyperthyroidism. To our knowledge, this is the first variant found in non-coding region associated with transient central nervous system (CNS) symptoms and in this case, thyroid dysfunction might contribute to the episode. The mechanism of CMTX1 as well as the transient CNS symptoms waits to be elucidated.</p

3D-QSARs and molecular dynamics simulation studies on induced fit binding of flavones to human aldose reductase

Communicated by Ramaswamy H. Sarma</p

DataSheet1_Immobilized Fe3O4-Polydopamine-Thermomyces lanuginosus Lipase-Catalyzed Acylation of Flavonoid Glycosides and Their Analogs: An Improved Insight Into Enzymic Substrate Recognition.PDF

The conversion of flavonoid glycosides and their analogs to their lipophilic ester derivatives was developed by nanobiocatalysts from immobilizing Thermomyces lanuginosus lipase (TLL) on polydopamine-functionalized magnetic Fe3O4 nanoparticles (Fe3O4-PDA-TLL). The behavior investigation revealed that Fe3O4-PDA-TLL exhibits a preference for long chain length fatty acids (i.e., C10 to C14) with higher reaction rates of 12.6–13.9 mM/h. Regarding the substrate specificity, Fe3O4-PDA-TLL showed good substrate spectrum and favorably functionalized the primary OH groups, suggesting that the steric hindrances impeded the secondary or phenolic hydroxyl groups of substrates into the bonding site of the active region of TLL to afford the product.</p

<i>In Situ</i> Neutron Scattering Studies on the Oxidation and Reduction of CeO₂ and Pt–CeO₂ Nanorods

The oxygen vacancy structure of ceria plays a key role in its performance as a favored material for catalysis applications. Here, we develop an understanding of the effects of Pt loading on the structural evolution of ceria nanorods under redox gas environments that mimic real automotive catalytic converters. In situ neutron scattering studies under redox flow reveal that both CeO2 and Pt–CeO2 nanorods share a bulk fluorite structure with the presence of surface Frenkel-type oxygen defects. However, Pt–CeO2 nanorods are more easily reducible than CeO2 rods as evidenced by an increased concentration of Ce3+, determined by NAP-XPS. Importantly, this work finds no evidence of oxygen vacancy ordered surface reconstruction which has been reported in earlier ex situ investigations. Thus, this work highlights the discrepancy between ex situ and in situ structural observations and emphasizes the need for robust in situ investigations of catalysts to develop industrially relevant materials

Supplementary document for Shortwave Infrared Single-Pixel Spectral Imaging based on GSST Phase-Change Metasurface - 5987482.pdf

Supplementary note

Potassium and Water Coadsorption on TiO₂(110): OH-Induced Anchoring of Potassium and the Generation of Single-Site Catalysts

Potassium deposition on TiO₂(110) results in reduction of the substrate and formation of loosely bound potassium species that can move easily on the oxide surface to promote catalytic activity. The results of density functional calculations predict a large adsorption energy (∼3.2 eV) with a small barrier (∼0.25 eV) for diffusion on the oxide surface. In scanning tunneling microscopy images, the adsorbed alkali atoms lose their mobility when in contact with surface OH groups. Furthermore, K adatoms facilitate the dissociation of water on the titania surface. The K–(OH) species generated are good sites for the binding of gold clusters on the TiO₂(110) surface, producing Au/K/TiO₂(110) systems with high activity for the water–gas shift

Importance of Low Dimensional CeO_<i>x</i> Nanostructures in Pt/CeO_<i>x</i>–TiO₂ Catalysts for the Water–Gas Shift Reaction

CO₂ and H₂ production from the water–gas shift (WGS) reaction was studied over Pt/CeO_<i>x</i>–TiO₂ catalysts with incremental loadings of CeO_<i>x</i>, which adopts variations in the local morphology. The lowest loading of CeO_<i>x</i> (1 wt % to 0.5 at. %) that is configured in its smallest dimensions exhibited the best WGS activity over larger dimensional structures. We attribute this to several factors including the ultrafine dispersed one-dimensional nanocluster geometry, a large concentration of Ce³⁺ and enhanced reducibility of the low loadings. We utilized several in situ experiments to monitor the active state of the catalyst during the WGS reaction. X-ray diffraction (XRD) results showed lattice expansion that indicated reduced ceria was prevalent during the WGS reaction. On the surface, Ce³⁺ related hydroxyl groups were identified by diffuse reflectance infrared Fourier transform spectroscopy (DRIFTS). The enhanced reducibility of the catalyst with the introduction of ceria was further revealed by H₂-temperature programed reduction (H₂-TPR) and good thermal stability was confirmed by <i>in situ</i> environmental transmission electron microscopy (ETEM). We also investigated the formation of the low dimensional structures during catalyst preparation, through a two-stage crystal growth of ceria crystallite on TiO₂ nanoparticle: fine crystallites ∼1D formed at ∼250 °C, followed by crystal growth into 2D chain and 3D particle from 250–400 °C

Mechanistic Insights of Ethanol Steam Reforming over Ni–CeO_<i>x</i>(111): The Importance of Hydroxyl Groups for Suppressing Coke Formation

We have studied the reaction of ethanol and water over Ni–CeO_2‑<i>x</i>(111) model surfaces to elucidate the mechanistic steps associated with the ethanol steam reforming (ESR) reaction. Our results provide insights about the importance of hydroxyl groups to the ESR reaction over Ni-based catalysts. Systematically, we have investigated the reaction of ethanol on Ni–CeO_2‑<i>x</i>(111) at varying Ce³⁺ concentrations (CeO_1.8–2.0) with absence/presence of water using a combination of soft X-ray photoelectron spectroscopy (sXPS) and temperature-programmed desorption (TPD). Consistent with previous reports, upon annealing, metallic Ni formed on reduced ceria while NiO was the main component on fully oxidized ceria. Ni⁰ is the active phase leading to both the C–C and C–H cleavage of ethanol but is also responsible for carbon accumulation or coking. We have identified a Ni₃C phase that formed prior to the formation of coke. At temperatures above 600 K, the lattice oxygen from ceria and the hydroxyl groups from water interact cooperatively in the removal of coke, likely through a strong metal–support interaction between nickel and ceria that facilitates oxygen transfer

Striving Toward Noble-Metal-Free Photocatalytic Water Splitting: The Hydrogenated-Graphene–TiO₂ Prototype

Graphane, graphone, and hydrogenated graphene (HG) have been extensively studied in recent years due to their interesting properties and potential use in commercial and industrial applications. The present study reports investigation of hydrogenated graphene/TiO_2–<i>x</i> (HGT) nanocomposites as photocatalysts for H₂ and O₂ production from water without the assistance of a noble metal cocatalyst. By combination of several techniques, the morphologies, bulk/atomic structure, and electronic properties of all the powders were exhaustively interrogated. Hydrogenation treatment efficiently reduces TiO₂ nanoparticles, while the graphene oxide sheets undergo the topotactic transformation from a graphene-like structure to a mixture of graphitic and turbostratic carbon (amorphous/disordered) upon altering the calcination atmosphere from a mildly reducing to a H₂-abundant environment. Remarkably, the hydrogenated graphene–TiO_2–<i>x</i> composite that results upon H₂-rich reduction exhibits the highest photocatalytic H₂ evolution performance equivalent to low loading of Pt (∼0.12 wt %), whereas the addition of HG suppresses the O₂ production. We propose that such an enhancement can be attributed to a combination of factors including the introduction of oxygen vacancies and Ti³⁺ states, retarding the recombination of charge carriers, and thus, facilitating the charge transfer from TiO_2–<i>x</i> to the carbonaceous sheet