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Objective: To investigate the effectiveness and tolerability of ketogenic diet therapy (KDT) in patients with developmental and epileptic encephalopathy (DEE) associated with genetic etiology which onset within the first 6 months of life, and to explore the association between response to KDT and genotype/clinical parameters. Methods: We retrospectively reviewed data from patients with genetic DEE who started KDT at Beijing Children's Hospital between January 1, 2016, and December 31, 2021. Results: A total of 32 patients were included, involving 14 pathogenic or likely pathogenic single genes, and 16 (50.0%) patients had sodium/potassium channel gene variants. The median age at onset of epilepsy was 1.0 (IQR: 0.1, 3.0) months. The median age at initiation of KDT was 10.0 (IQR: 5.3, 13.8) months and the median duration of maintenance was 14.0 (IQR: 7.0, 26.5) months, with a mean blood β‐hydroxybutyrate of 2.49 ± 0.62 mmol/L. During the maintenance period of KDT, 26 (81.3%) patients had a ≥50% reduction of seizure frequency, of which 12 (37.5%) patients achieved seizure freedom. Better responses were observed in patients with STXBP1 variants, with four out of five patients achieving seizure freedom. There were no statistically differences in the age of onset, duration of epilepsy before KDT, blood ketone values, or the presence of ion channel gene variants between the seizure‐free patients and the others. The most common adverse effects were gastrointestinal side effects, which occurred in 21 patients (65.6%), but all were mild and easily corrected. Only one patient discontinued KDT due to nephrolithiasis. Significance: KDT is effective in treating early onset genetic DEE, and no statistically significant relationship has been found between genotype and effectiveness in this study. KDT is well tolerated in most young patients, with mild and reversible gastrointestinal side effects being the most common, but usually not the reason to discontinue KDT. Plain Language Summary: This study evaluated the response and side effects of ketogenic diet therapy (KDT) in patients who had seizures within the first 6 months of life, and were diagnosed with genetic developmental and epileptic encephalopathy (DEE), a type of severe epilepsy with developmental delay caused by gene variants. Thirty‐two patients involving 14 gene variants who started KDT at Beijing Children's Hospital between were included. KDT was effective in treating early onset genetic DEE in this cohort, and patients with STXBP1 variants responded better; however, no statistically significant relationship was found between gene variant and response. Most young patients tolerated KDT well, with mild and reversible gastrointestinal side effects being the most common. [ABSTRACT FROM AUTHOR]

Importance: CHD2 is a member of the chromodomain helicase DNA‐binding (CHD) family of proteins, which have important roles in the regulation of gene expression. Dysregulation of this protein may lead to various disorders. Objective: To delineate the genotypes and phenotypes of CHD2‐related epilepsy. Methods: We analyzed the medical history, magnetic resonance imaging findings, and video‐electroencephalogram recordings of 17 patients with CHD2 mutations in the Neurology Department of Beijing Children's Hospital from June 2016 to June 2021. Results: Age at seizure onset ranged from 6 months to 10 years; the median age at onset was 4 years. Generalized tonic‐clonic, myoclonic, eyelid myoclonic, atonic, atypical absence, myoclonic‐atonic, and spasm seizures were observed. Ten of the 17 patients had multiple types of seizures. One patient exhibited photosensitivity epilepsy and one patient exhibited grid image‐induced visual reflex epilepsy. Developmental disability was present in 14 patients, while autism features were present in five patients. Sixteen patients had de novo mutations of CHD2; one patient had an inherited variant. Eleven mutations were novel. One patient had two mutations; that patient exhibited development delay and refractory epilepsy. Seizures were controlled in eight patients, improved in seven patients, and resistant to treatment in two patients. Interpretation: Phenotype severity in patients with CHD2 variants ranged from drug‐responsive seizures to severe epileptic encephalopathy. Most patients exhibited developmental disorders. [ABSTRACT FROM AUTHOR]

Summary: Pathogen effectors are crucial players during plant colonisation and infection. Plant resistance mostly relies on effector recognition to activate defence responses. Understanding how effector proteins escape from plant surveillance is important for plant breeding and resistance deployment.Here we examined the role of genetic diversity of the stem rust (Puccinia graminis f. sp. tritici (Pgt)) AvrSr50 gene in determining recognition by the corresponding wheat Sr50 resistance gene. We solved the crystal structure of a natural variant of AvrSr50 and used site‐directed mutagenesis and transient expression assays to dissect the molecular mechanisms explaining gain of virulence.We report that AvrSr50 can escape recognition by Sr50 through different mechanisms including DNA insertion, stop codon loss or by amino‐acid variation involving a single substitution of the AvrSr50 surface‐exposed residue Q121. We also report structural homology of AvrSr50 to cupin superfamily members and carbohydrate‐binding modules indicating a potential role in binding sugar moieties.This study identifies key polymorphic sites present in AvrSr50 alleles from natural stem rust populations that play important roles to escape from Sr50 recognition. This constitutes an important step to better understand Pgt effector evolution and to monitor AvrSr50 variants in natural rust populations. [ABSTRACT FROM AUTHOR]

Hybrid nanomaterials with controlled dimensions, intriguing components and ordered structures have attracted significant attention in nanoscience and technology. Herein, we report a facile and green polyoxometallate (POM)‐assisted hydrothermal carbonization strategy for synthesis of carbonaceous hybrid nanomaterials with molecularly dispersed POMs and ordered mesopores. By using various polyoxometallates such as ammonium phosphomolybdate, silicotungstic acid, and phosphotungstic acid, our approach can be generalized to synthesize ordered mesoporous hybrid nanostructures with diverse compositions and morphologies (nanosheet‐assembled hierarchical architectures, nanospheres, and nanorods). Moreover, the ordered mesoporous nanosheet‐assembled hierarchical hybrids with molecularly dispersed POMs exhibit remarkable catalytic activity toward the dehydration of tert‐butanol with the high isobutene selectivity (100 %) and long‐term catalytic durability (80 h). [ABSTRACT FROM AUTHOR]

Summary: Leaf rust, caused by Puccinia hordei, is a devastating fungal disease affecting barley (Hordeum vulgare subsp. vulgare) production globally. Despite the effectiveness of genetic resistance, the deployment of single genes often compromises durability due to the emergence of virulent P. hordei races, prompting the search for new sources of resistance. Here we report on the cloning of Rph15, a resistance gene derived from barley's wild progenitor H. vulgare subsp. spontaneum. We demonstrate using introgression mapping, mutation and complementation that the Rph15 gene from the near‐isogenic line (NIL) Bowman + Rph15 (referred to as BW719) encodes a coiled‐coil nucleotide‐binding leucine‐rich repeat (NLR) protein with an integrated Zinc finger BED (ZF‐BED) domain. A predicted KASP marker was developed and validated across a collection of Australian cultivars and a series of introgression lines in the Bowman background known to carry the Rph15 resistance. Rph16 from HS‐680, another wild barley derived leaf rust resistance gene, was previously mapped to the same genomic region on chromosome 2H and was assumed to be allelic with Rph15 based on genetic studies. Both sequence analysis, race specificity and the identification of a knockout mutant in the HS‐680 background suggest that Rph15‐ and Rph16‐mediated resistances are in fact the same and not allelic as previously thought. The cloning of Rph15 now permits efficient gene deployment and the production of resistance gene cassettes for sustained leaf rust control. [ABSTRACT FROM AUTHOR]

A compact dual‐band circularly polarized (CP) filtering patch antenna is proposed by adopting the dispersive delay line (DDL) in this article. The in‐band and out‐of‐band phase differences of two orthogonal components are effectively controlled by the DDL to realize opposite polarization states. As perfect isolation is achieved between left‐hand circular polarization and the right‐hand one in CP antenna, by controlling the parameters of DDL, four radiation nulls are presented simultaneously, and their locations are directly determined by the phase difference, thus obtains good filtering performance with compact structure. For demonstration, dual‐band CP patch antenna is designed at 2.4 and 5.8 GHz for WLAN applications based on DDL. The simulated and measured results of fabricated prototypes show good agreement. Good out‐of‐band rejection is observed in this design, which verifies the feasibility of the proposed CP filtering strategy. [ABSTRACT FROM AUTHOR]

Abstract: The realization of asymmetric hollow carbonaceous nanostructures remains a great challenge, especially when biomass is chosen as the carbon resource through hydrothermal carbonization (HTC). Herein, a simple and straightforward solvent‐induced buckling strategy is demonstrated for the synthesis of asymmetric spherical and bowl‐like carbonaceous nanomaterials. The formation of the bowl‐like morphology was attributed to the buckling of the spherical shells induced by the dissolution of the oligomers. The bowl‐like particles prepared through this solvent‐driven approach demonstrated a well‐controlled morphology and a uniform particle size of approximately 360 nm. The obtained nanospheres and nanobowls were loaded with CoS2 nanoparticles to act as heterogeneous catalysts for the selective hydrogenation of aromatic nitro compounds. As expected, the CoS2/nanobowls catalyst showed good tolerance to a wide scope of reducible groups and afforded both high activity and selectivity in almost all the tested substrates. [ABSTRACT FROM AUTHOR]

Summary: The effector protein AvrP is secreted by the flax rust fungal pathogen (Melampsora lini) and recognized specifically by the flax (Linum usitatissimum) P disease resistance protein, leading to effector‐triggered immunity. To investigate the biological function of this effector and the mechanisms of specific recognition by the P resistance protein, we determined the crystal structure of AvrP. The structure reveals an elongated zinc‐finger‐like structure with a novel interleaved zinc‐binding topology. The residues responsible for zinc binding are conserved in AvrP effector variants and mutations of these motifs result in a loss of P‐mediated recognition. The first zinc‐coordinating region of the structure displays a positively charged surface and shows some limited similarities to nucleic acid‐binding and chromatin‐associated proteins. We show that the majority of the AvrP protein accumulates in the plant nucleus when transiently expressed in Nicotiana benthamiana cells, suggesting a nuclear pathogenic function. Polymorphic residues in AvrP and its allelic variants map to the protein surface and could be associated with differences in recognition specificity. Several point mutations of residues on the non‐conserved surface patch result in a loss of recognition by P, suggesting that these residues are required for recognition. [ABSTRACT FROM AUTHOR]

The tedious and costly route of the general nanocasting method hinders the commercial application of mesoporous non-precious metal catalysts based on transition metal-coordinating nitrogen-doped carbons (MN/C). Here, a simple, economic and sustainable hydrothermal carbonization (HTC)/soft templating method is demonstrated for the direct preparation of mesoporous FeN/C materials (FeN/MC). By this method, FeN/MC materials with high specific surface area, hierarchically mesoporous structure, and appropriate iron and nitrogen contents are prepared. The optimized FeN/MC materials exhibit superior catalytic performance in the oxygen reduction reaction (ORR) to commercial Pt/C in 0.1 M KOH. This confirms that the materials are one of the most efficient non-precious metal catalysts for the ORR in alkaline electrolyte. In addition, considerable durability and ORR activity are demonstrated by the FeN/MC materials in 0.1 M HClO4. Thus, this HTC/soft templating method has great promise for the practical application of this kind of non-precious metal catalysts in the ORR. [ABSTRACT FROM AUTHOR]

Summary: Using a simple oligo selection procedure, we have previously identified a tobacco sequence-specific DNA-binding activity, TDBA12, that increases markedly during the tobacco mosaic virus (TMV)-induced hypersensitive response (HR). Based on the binding specificity and the two cDNA clones isolated, TDBA12 is related to a novel class of DNA-binding factors containing WRKY domains. In the present study, we report that TDBA12 could be induced not only by TMV infection but also by treatment with salicylic acid (SA) or its biologically active analogs capable of inducing pathogenesis-related (PR) genes and enhanced resistance. TDBA12 was sensitive to temperature and the protein dissociating agent sodium deoxycholate, suggesting that it may be a multimeric factor in which protein–protein interaction is important for the enhanced DNA-binding activity. Pre-treatment of nuclear extracts with alkaline phosphatase abolished TDBA12, suggesting that protein phosphorylation is important for its high DNA-binding activity. TDBA12 specifically recognized the elicitor response element of the tobacco class I basic chitinase gene promoter. The increase in the levels of TDBA12 following TMV infection or SA treatment preceded the induced expression of the tobacco chitinase gene. These results strongly suggest that certain WRKY DNA-binding proteins may be activated by enhanced protein phosphorylation and regulate inducible expression of defense-related genes during pathogen- and SA-induced plant defense responses. [ABSTRACT FROM AUTHOR]