In Situ Detection of Active Edge Sites in Single-Layer MoS2_2 Catalysts

MoS2 nanoparticles are proven catalysts for processes such as hydrodesulphurization and hydrogen evolution, but unravelling their atomic-scale structure under catalytic working conditions has remained significantly challenging. Ambient pressure X-ray Photoelectron Spectroscopy (AP-XPS) allows us to follow in-situ the formation of the catalytically relevant MoS2 edge sites in their active state. The XPS fingerprint is described by independent contributions to the Mo3d core level spectrum whose relative intensity is sensitive to the thermodynamic conditions. Density Functional Theory (DFT) is used to model the triangular MoS2 particles on Au(111) and identify the particular sulphidation state of the edge sites. A consistent picture emerges in which the core level shifts for the edge Mo atoms evolve counter-intuitively towards higher binding energies when the active edges are reduced. The shift is explained by a surprising alteration in the metallic character of the edge sites, which is a distinct spectroscopic signature of the MoS2 edges under working conditions

Современные концепции управления высшим учебным заведением

Целью и задачами статьи является исследование современных подходов к управлению вузом, их критический анализ и возможность оптимизации процессов деятельности вуза

Operando Laboratory-Based Multi-Edge X-Ray Absorption Near-Edge Spectroscopy of Solid Catalysts

Laboratory-based X-ray absorption spectroscopy (XAS) and especially X-ray absorption near-edge structure (XANES) offers new opportunities in catalyst characterization and presents not only an alternative, but also a complementary approach to precious beamtime at synchrotron facilities. We successfully designed a laboratory-based setup for performing operando , quasi-simultaneous XANES analysis at multiple K edges, more specifically, operando XANES of mono-, bi-, and trimetallic CO 2 hydrogenation catalysts containing Ni, Fe, and Cu. Detailed operando XANES studies of the multi-element solid catalysts revealed metal-dependent differences in the reducibility and re-oxidation behavior and their influence on the catalytic performance in CO 2 hydrogenation. The applicability of operando laboratory-based XANES at multiple K edges paves the way for advanced multi-element catalyst characterization complementing detailed studies at synchrotron facilities.Peer reviewe

Bi-allelic GAD1 variants cause a neonatal onset syndromic developmental and epileptic encephalopathy.

Developmental and epileptic encephalopathies are a heterogeneous group of early-onset epilepsy syndromes dramatically impairing neurodevelopment. Modern genomic technologies have revealed a number of monogenic origins and opened the door to therapeutic hopes. Here we describe a new syndromic developmental and epileptic encephalopathy caused by bi-allelic loss-of-function variants in GAD1, as presented by 11 patients from six independent consanguineous families. Seizure onset occurred in the first 2 months of life in all patients. All 10 patients, from whom early disease history was available, presented with seizure onset in the first month of life, mainly consisting of epileptic spasms or myoclonic seizures. Early EEG showed suppression-burst or pattern of burst attenuation or hypsarrhythmia if only recorded in the post-neonatal period. Eight patients had joint contractures and/or pes equinovarus. Seven patients presented a cleft palate and two also had an omphalocele, reproducing the phenotype of the knockout Gad1-/- mouse model. Four patients died before 4 years of age. GAD1 encodes the glutamate decarboxylase enzyme GAD67, a critical actor of the γ-aminobutyric acid (GABA) metabolism as it catalyses the decarboxylation of glutamic acid to form GABA. Our findings evoke a novel syndrome related to GAD67 deficiency, characterized by the unique association of developmental and epileptic encephalopathies, cleft palate, joint contractures and/or omphalocele

Operando Laboratory-based X-ray Absorption Spectroscopy: Guidelines for Newcomers in the Field

The new possibility to perform operando X-ray absorption spectroscopy (XAS) in the laboratory expands the potential field of applications towards a broad research community. These applications are multidisciplinary at heart and benefit from joint expertise from different fields, most importantly chemistry, physics, geology, and instrumentation. Hence, a development of collaboration networks that combine skills and knowhow from different fields is highly beneficial in this endeavor. As operando laboratory-based XAS constitutes a highly interesting, advanced, and powerful characterization technique, we provide in this article practical guidelines for newcomers in the field, who would like to employ it. Here, we will describe ten important steps towards a successful operando laboratory-based XAS experiment, which are not only useful for the catalysis community, but for a much wider audience from other research fields, such as environmental chemistry as well as battery and fuel cell research

New SMARCA2 mutation in a patient with Nicolaides–Baraitser syndrome and myoclonic astatic epilepsy

We report a de novo SMARCA2 missense mutation discovered on exome sequencing in a patient with myoclonic astatic epilepsy, leading to reassessment and identification of Nicolaides–Baraitser syndrome. This de novo SMARCA2 missense mutation c.3721C>G, p.Gln1241Glu is the only reported mutation on exon 26 outside the ATPase domain of SMARCA2 to be associated with Nicolaides–Baraitser syndrome and adds to chromatin remodeling as a pathway for epileptogenesis. © 2016 The Authors. American Journal of Medical Genetics Part A published by Wiley Periodicals, Inc

Correction: Unravelling potential reaction intermediates during catalytic pyrolysis of polypropylene with microscopy and spectroscopy

Correction for ‘Unravelling potential reaction intermediates during catalytic pyrolysis of polypropylene with microscopy and spectroscopy’ by Ina Vollmer et al., Catal. Sci. Technol., 2024, 14, 894–902, https://doi.org/10.1039/d3cy01473h

ILAE Genetic Literacy Series: Self-limited familial epilepsy syndromes with onset in neonatal age and infancy

The self-limited (familial) epilepsies with onset in neonates or infants, formerly called benign familial neonatal and/or infantile epilepsies, are autosomal dominant disorders characterized by neonatal- or infantile-onset focal motor seizures and the absence of neurodevelopmental complications. Seizures tend to remit during infancy or early childhood and are therefore called “self-limited”. A positive family history for epilepsy usually suggests the genetic etiology, but incomplete penetrance and de novo inheritance occur. Here, we review the phenotypic spectrum and the genetic architecture of self-limited (familial) epilepsies with onset in neonates or infants. Using an illustrative case study, we describe important clues in recognition of these syndromes, diagnostic steps including genetic testing, management, and genetic counseling

Catalytic upgrading of refinery cracked products by trans-hydrogenation: a review

The production of high premium fuel is an issue of priority to every refinery. The trans-hydrogenation process is devised to convert two low valued refinery cracked products to premium products; the conversion processes involve the combination of dehydrogenation and hydrogenation reaction as a single step process. The paper reviews the recent literature on the use of catalysts to convert low value refinery products (i.e. alkanes and alkynes or alkadienes) to alkenes (olefins) by trans-hydrogenation. Catalysts based on VOx, CrOx and Pt all supported on alumina have been used for the process. However, further studies are still required to ascertain the actual reaction mechanism, mitigating carbon deposition and catalyst deactivation, and the role of different catalysts to optimize the reaction desired products