Revealing the detrimental CO2 reduction effect of La0.6Sr0.4FeO3-δ-derived heterostructure in solid oxide electrolysis cells.

Solid oxide electrolysis cells hold unique Faraday efficiency and favored thermodynamic/kinetics for CO2 reduction to CO. Perovskite oxide-based composite materials are promising alternatives to Ni-based cermet electrodes in SOECs. However, contrary results of the electrocatalytic activity over single-phase perovskite oxide exist and the rationale of the negative effect is not well revealed. In this work, two-phase perovskite materials with various complementary properties and unique interfaces are self-assembled, which was realized by "subtractive" defect-driven phase separation. The obtained heterostructure electrodes showed reduced performance over that of single-phase materials although the cyclic stability was improved. The main reasons for the performance degradation are the decrease of electrical conductivity, oxygen vacancy concentration while increasing the average valence state of B-site Fe cations, and electrode surface Sr aggregation. This work highlights the self-assembly method and insight into the rational design and synthesis of active electrodes/catalysts for CO2 conversion in solid oxide cells.

Fabrication of MnO2 coating on aluminum honeycomb for fast catalytic decomposition of ozone at room temperature.

Herein, the coating of MnO2 nanomaterials on the surface of aluminum honeycomb was carried out to meet the requirements of high air velocity, low pressure drop and high activity in ozone removal scenarios. A commercially readily available waterborne silica sol mixed with waterborne acrylate latex was creatively utilized as the binder. A series of coating samples were prepared by spray coating method and evaluated focusing on their adhesion strength and catalytic activity towards ozone decomposition in an air duct at room temperature, by varying MnO2/binder mass ratio and number of sprayings. It was found that the adhesion strength of the catalytic coatings on the aluminum honeycomb increased with the increase of binder mass ratio, but the increased binder made the catalyst particles closely packed, resulting in reduced exposure of active sites and decrease of ozone conversion. Accordingly, catalyst slurry with 81.8 wt.% MnO2 in dry coating and spraying times of two were determined as the optimal process parameters. As-prepared aluminum honeycomb filter with MnO2 layer of 50 µm thickness achieved ozone conversion of 29.3%±1.7% under conditions of air velocity 3.0 m/sec, relative humidity ∼50%, room temperature (26°C) and initial ozone concentration of 200 ppbV. This filter can be well adaptable to indoor air purification equipment operating at high air velocity with low wind resistance.

Dual diagnosis of osteogenesis imperfecta (OI) and short stature and advanced bone age with or without early-onset osteoarthritis and/or osteochondritis dissecans (SSOAOD) reveals a cumulative effect on stature caused by mutations in COL1A1 and ACAN genes.

Short stature is a feature when a person's height is more than 2 SDS below the corresponding mean height for a given age, gender and population. It can be influenced by many factors essential to growth plate. Here we report a three-generation family with 13 patients affected by osteogenesis imperfecta (OI) type I, short stature and advanced bone age, with or without early-onset osteoarthritis and/or osteochondritis dissecans (SSOAOD) or both. Panel sequencing of the proband revealed mutations in two extracellular matrix related genes: COL1A1 and ACAN. When comparing the quantitative trait, height within the family of different mutation carrier groups, we found an interesting cumulative effect, the ones with both mutations manifest shortest stature. Dual diagnoses of the family also suggest the necessity of a comprehensive molecular diagnosis method, such as panel/exome sequencing, especially encountering patients with novel phenotype or extreme trait.

A Novel Nonsense Mutation of PHF6 in a Female with Extended Phenotypes of Borjeson-Forssman-Lehmann Syndrome

Borjeson-Forssman-Lehmann syndrome (BFLS) is a rare X-linked disease caused by PHF6 mutations. Classic BFLS has been associated with intellectual disability (ID), developmental delay (DD), obesity, epilepsy, typical facial features and anomalies of fingers and toes. Endocrinological phenotypes and outcome of treatment in this condition remain to be delineated. Here we report a patient who exhibited complete growth hormone deficiency who responded to hormonal treatment but with adverse effects. Horseshoe kidney was present in this patient, which is also atypical in BFLS. A heterozygous nonsense mutation c.673C>T (p.R225X) of PHF6 gene was identified in the patient, inherited from her unaffected mother. Both the patient and her mother showed highly skewed X-inactivation. We reviewed the phenotypes of all reported BFLS cases, and summarized their endocrine presentations. This first report of an Asian patient with BFLS further delineated the genetic and phenotypic spectrum of the syndrome. The adverse effect experienced by the patient suggests caution in the use of growth hormone treatment in this condition.

Biallelic UNC80 mutations caused infantile hypotonia with psychomotor retardation and characteristic facies 2 in two Chinese patients with variable phenotypes.

Biallelic UNC80 mutations cause infantile hypotonia with psychomotor retardation and characteristic facies 2 (IHPRF2), which is characterized by hypotonia, developmental delay (DD)/intellectual disability (ID), intrauterine growth retardation, postnatal growth retardation and characteristic facial features. We report two unrelated Chinese patients with compound heterozygous UNC80 mutations inherited from their parents, as identified by whole-exome sequencing (WES). Mutations c.3719G>A (p.W1240*)/c.4926_4937del (p.N1643_L1646del) and c.4963C>T (p.R1655C)/c.8385C>G (p.Y2795*) were identified in patient 1 and patient 2, respectively. Although both patients presented with DD/ID and hypotonia, different manifestations also occurred. Patient 1 presented with infantile hypotonia, epilepsy and hyperactivity without growth retardation, whereas patient 2 presented with persistent hypotonia, growth retardation and self-injury without epilepsy. Furthermore, we herein summarize the genotypes and phenotypes of patients with UNC80 mutations reported in the literature, revealing that IHPRF2 is a phenotypically heterogeneous disease. Common facial dysmorphisms include a thin upper lip, a tented upper lip, a triangular face, strabismus and microcephaly. To some extent, the manifestations of IHPRF2 mimic those of Angelman syndrome (AS)-like syndromes.

From force curves to surface nanomechanical properties.

Surface science, which spans the fields of chemistry, physics, biology and materials science, requires information to be obtained on the local properties and property variations across a surface. This has resulted in the development of different scanning probe methods that allow the measurement of local chemical composition and local electrical and mechanical properties. These techniques have led to rapid advancement in fundamental science with applications in areas such as composite materials, corrosion protection and wear resistance. In this perspective article, we focussed on the branch of scanning probe methods that allows the determination of surface nanomechanical properties. We discussed some different AFM-based modes that were used for these measurements and provided illustrative examples of the type of information that could be obtained. We also discussed some of the difficulties encountered during such studies.

Flowerlike CeO2 microspheres coated with Sr2Fe1.5Mo0.5Ox nanoparticles for an advanced fuel cell.

Flowerlike CeO2 coated with Sr2Fe1.5Mo0.5Ox (Sr-Fe-Mo-oxide) nanoparticles exhibits enhanced conductivity at low temperatures (300-600 °C), e.g. 0.12 S cm(-1) at 600 °C, this is comparable to pure ceria (0.1 S cm(-1) at 800 °C). Advanced single layer fuel cell was constructed using the flowerlike CeO2/Sr-Fe-Mo-oxide layer attached to a Ni-foam layer coated with the conducting transition metal oxide. Such fuel cell has yielded a peak power density of 802 mWcm(-2) at 550 °C. The mechanism of enhanced conductivity and cell performance were analyzed. These results provide a promising strategy for developing advanced low-temperature SOFCs.