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Probe BocBDP with a binding unit suitable for penetrating the BBB and imaging Aβ in vivo.

Lithium-ion battery (LIB) design is the predominant technology to power portable and mobile electric devices/equipment. Fast charging and self-powering of LIBs are significant but challenging features to be addressed for meeting the fast-paced society and emerging demands. Herein, we report a rational photorechargeable lithium-ion battery (photo-LIB) design using LiV

Background Mucopolysaccharidosis (MPS) refers to a group of lysosomal storage disorders for which seven types and 11 subtypes are currently recognized. Targeted next-generation sequencing (NGS) offers an important method of disease typing, diagnosis, prenatal diagnosis, and treatment. Methods Gene variations in 48 Chinese MPS patients were evaluated using NGS, and the pathogenicity of the DNA alterations was evaluated using PolyPhen2, SIFT, and Mutation Taster. The effect of amino acid substitution on protein structure was also assessed. Results Four pedigrees with MPS I (8.3%), 28 with MPS II (58.3%), two with MPS IIIA (4.2%), two with MPS IIIB (4.2%), six with MPS IVA (12.5%), one with MPS IVB (2.1%), and five with MPS VI (10.4%) were identified. Of the 69 variations identified, 11 were novel variants (three in IDUA, five in IDS, and three in GALNS), all of which were predicted to be disease-causing except for one, and were associated with impaired protein structure and function. Conclusions Targeted NGS technology is effective for the gene-based testing of MPS disorders, which show high allelic heterogeneity. MPS II was the predominant form in Chinese. Our study expands the existing variation spectrum of MPS, which is important for disease management and genetic counseling.

Many studies report predictions for cognitive function but there are few predictions in epileptic patients; therefore, we established a workflow to efficiently predict outcomes of both the Mini-Mental State Examination (MMSE) and Montreal Cognitive Assessment (MoCA) in outpatients with epilepsy. Data from 441 outpatients with epilepsy were included; of these, 433 patients met the 12 clinical characteristic criteria and were divided into training (n = 304) and experimental (n = 129) groups. After descriptive statistics were analyzed, cross-validation was used to select the optimal model. The random forest (RF) algorithm was combined with the redundancy analysis (RDA) algorithm; then, optimal feature selection and resampling were carried out after removing linear redundancy information. The features that contributed more to multiple outcomes were selected. Finally, the external traceability of the model was evaluated using the follow-up data. The RF algorithm was the best prediction model for both MMSE and MoCA outcomes. Finally, seven markers were screened by overlapping the top ten important features for MMSE ranked by RF modeling, those ranked for MoCA ranked by RF modeling, and those for both assessments ranked by RDA. The optimal combination of features were namely, sex, age, age of onset, seizure frequency, brain MRI abnormalities, epileptiform discharge in EEG and usage of drugs. which was the most efficient in predicting outcomes of MMSE, MoCA, and both assessments.

To explore the clinical features and genetic etiology of a child with glycogen storage disease VI (GSD-VI).Clinical data and laboratory results of the patient were collected. Whole exome sequencing (WES) was carried out for the patient. Candidate variant and its parental origin was verified by Sanger sequencing.The patient was a 3-year-and-9-month old boy whom has featured abdominal distention, hepatomegaly, short stature and elevated hepatic transaminase. WES revealed the he has harbored compound heterozygous variants of the PYGL gene, namely c.697GA (p.Gly233Ser) and c.320dupA (p.Asn107fs). Sanger sequencing has verified that the two variants have derived from his father and mother, respectively. The c.320dupA (p.Asn107fs) variant was unreported previously.The compound heterozygous variants of the PYGL gene probably underlay the GSD-VI in this patient. Above finding has enriched the spectrum of PYGL gene variants and provided a basis for the treatment and genetic counseling.

Background Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease with neuronal cell inclusions composed of neurofilaments and other abnormal aggregative proteins as pathological hallmarks. Approximately 90% of patients have sporadic cases (sALS), and at least 4 genes, i.e. C9orf72, SOD1, FUS and TARDBP, have been identified as the main causative genes, while many others have been proposed as potential risk genes. However, these mutations could explain only ~ 10% of sALS cases. The neurofilament polypeptides encoded by NEFH, NEFM, and NEFL are promising protein biomarkers for ALS and other degenerative diseases. However, whether the genetic variants of these genes were associated with ALS remain ambiguous. Methods Here, we used PCR-Sanger to sequence the exons of these three genes in a cohort of 371 sALS patients and 711 healthy controls (Phase I) and validated the risk variant in another 300 sALS patients and 1076 controls (Phase II). Results A total of 92 variants were identified, including 36 rare heterozygous variants in NEFH, 27 in NEFM, and 16 in NEFL, and only rs568759161 (p.Ser787Arg) in NEFH reached nominal statistical power (P = 0.02 at Phase I, P = 0.009 at Phase II) in the case–control comparison. Together, the Phase I and II studies showed the significantly higher frequency of the variant in cases (9/1342, 0.67%) than in controls (2/3574, 0.07%) (OR 12.06; 95% CI 2.60–55.88; P = 0.0003). No variants passed multiple testing in the discovery cohort, but rs568759161 was associated with ALS in a replication cohort. Conclusions Our results confirmed that NEFH Ser787Arg is a novel sALS risk variant in Chinese subjects, but NEFM and NEFL were not associated with sALS. These data may have implications for genetic counselling and for understanding the pathogenesis of sALS.

Customer clustering technology for distribution process is widely used in location selection, distribution route optimization and vehicle scheduling optimization of power logistics distribution center. Aiming at the problem of customer clustering with unknown distribution center location, this paper proposes a clustering algorithm considering distribution network structure and distribution volume constraint, which makes up for the defect that the classical Euclidean distance does not consider the distribution road information. This paper proposes a logistics distribution customer clustering algorithm, which improves CLARANS algorithm to make the clustering results meet the constraints of customer distribution volume. By using the single vehicle load rate, the sufficient conditions for logistics distribution customer clustering to be solvable under the condition of considering the ubiquitous and constraints are given, which effectively solves the problem of logistics distribution customer clustering with sum constraints. The results state clearly that the clustering algorithm can effectively deal with large-scale spatial data sets, and the clustering process is not affected by isolated customers, The clustering results can be effectively applied to the distribution center location, distribution cost optimization, distribution route optimization and distribution area division of vehicle scheduling optimization.

To carry out genetic analysis for a family with a fetus manifesting micrognathia and a previous history for fetal micromandibular deformity.Systematic ultrasound examination was carried out for the fetus, and the prenatal and postnatal phenotype of the first fetus were retrospectively analyzed. The fetus and his parents were subjected to whole exome sequencing (WES) to identify potential pathogenic variants. Candidate variants were verified by Sanger sequencing.Fetal ultrasound has indicated micrognathia. The first fetus was found to have micrognathia by prenatal ultrasonography and have featured macrosomia and dyspnea due to with tongue retraction, high palatal arch and small mandibular deformity. WES revealed that the fetus has a harbored a c.3GC (p.Met1?) variant of the COL2A1 gene, which was inherited from the father who had myopia and retinal detachment.Stickler syndrome is mainly characterized prenatally by micrognathia, in addition with a variety of postnatal anomalies. The c.3GC (p.Met1?) variant probably underlay the Stickler syndrome in this pedigree.

Hierarchically porous MOFs (HP-MOFs) present advantageous synergism of micro- and mesopore but challenging in synthetic control at molecular scale. Herein, we present the first example of reversible and controllable mesopore generation and renovation in a microporous MOF of HKUST-1 via synthetic manipulation at molecular scale. An ammonia-gas etching strategy is proposed to create mesopores in carboxylate-based microporous MOFs and thus produce HP-MOFs. Gas-phase etching ensures uniform mesopore formation inside the MOF crystals via plane-oriented cutting the carboxylate-metal bonds off without affecting the crystal size and morphology. The mesopore size is controlled by the etching temperature, while the mesopore volume could be tuned by adjusting etchant pressure. The generated mesopores could be renovated using MOF precursors solutions so that to achieve controllable mesopore generation/closure, and encapsulation of the adsorbed molecules. This work demonstrates a powerful protocol for precisely tailoring and tuning the properties of MOF materials at molecular scale.

Room-temperature phosphorescent (RTP) carbon dots offer unique opportunities in information security applications, but the delay time of such materials reported thus far is typically shorter than 10 s and thus affects readout of the encoded information. Herein, a fast processable and one-step synthetic approach is developed for the preparation of N and P codoped carbonized polymer dots (NP-CPDs) exhibiting ultralong-lived RTP (URTP) responses. The URTP emission with the delay time exceeding 23 s (lifetime of ∼1.48 s) can be clearly identified with the naked eye. The presence of self-protective covalently crosslinked frameworks with codoped N and P atoms and the formation of interdot/intradot hydrogen-bonded skeletons are suggested to account for the observed URTP phenomena. The NP-CPDs exhibit excellent water solubility and color conservation during repeated liquid–solid transformations, thus allowing for a fast and large-scale ink-jet print of such nanodots to encode information. Consequently, the potential application of such NP-CPDs in anticounterfeiting and information encryption is demonstrated. This study provides a facile strategy to prepare URTP nanostructures for intriguing applications.

The surface state of all-inorganic cesium lead halide (CsPbX3) perovskite quantum dots (QDs) is a crucial factor for the performance of CsPbX3 QD-based optoelectronic devices. In this research, we ...

Efficient cleavage of aryl–ether linkages is a key strategy for generating aromatic chemicals and fuels from lignin. Currently, a popular method to depolymerize native/technical lignin employs a combination of Lewis acid and hydrogenation metal. However, a clear mechanistic understanding of the process is lacking. Thus, a more thorough understanding of the mechanism of lignin depolymerization in this system is essential. Herein, we propose a detailed mechanistic study conducted with lignin model compounds (LMC) via a synergistic Co–Zn/Off-Al H-beta catalyst that mirrors the hydrogenolysis process of lignin. The results suggest that the main reaction paths for the phenolic dimers exhibiting α-O-4 and β-O-4 ether linkages are the cleavage of aryl–ether linkages. Particularly, the conversion was readily completed using a Co–Zn/Off-Al H-beta catalyst, but 40% of α-O-4 was converted and β-O-4 did not react in the absence of a catalyst under the same conditions. In addition, it was found that the presence of hydroxyl groups on the side chain, commonly found in native lignin, greatly promotes the cleavage of aryl–ether linkages activated by Zn Lewis acid, which was attributed to the adsorption between Zn and the hydroxyl group. Followed by the cobalt catalyzed hydrogenation reaction, the phenolic dimers are degraded into monomers that maintain aromaticity.

Fluorescent carbon dots (CDs) have emerged as promising materials for white light-emitting diodes (WLEDs) on account of carbon's intrinsic merits of high stability, low cost, and environment-friendliness. However, CDs usually give relatively narrow emission with full width at half maximum within 70–90 nm, which fundamentally restrains construction of CDs-based WLEDs with high color rendering index (CRI). Here, we demonstrate tricolor ultrabroad-band (109–120 nm) emissive CDs (Tri-CDs) with a quantum yield (QY) up to 21–64% by a one-pot solvothermal route. Experimental investigations reveal that tunable emissions of these CDs are based on differently sized sp2 conjugated domains and the content of graphitic nitrogen, which can be modulated by controlling the carbonization and deamination processes during solvothermal reactions. The ultrabroad-band emission spectra of Tri-CDs originate from broad distribution of the particle size. A WLED with Commission Internationale de l’Eclairage (CIE) color coordinate of (0.362, 0.370) and high CRI of 96.5 is fabricated by integrating Tri-CDs films with an ultraviolet LED chip. This work opens up a new opportunity for the development of high CRI CDs-based WLEDs.

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has rapidly spread and poses a major threat to public health worldwide. The whole genome sequencing plays a crucial role in virus surveillance and evolutionary analysis. In this study, five genome sequences of SARS-CoV-2 were obtained from nasopharyngeal swab samples from Zhengzhou, China. Following RNA extraction and cDNA synthesis, multiplex PCR was performed with two primer pools to produce the overlapped amplicons of ~1,200 bp. The viral genomes were obtained with 96% coverage using nanopore sequencing. Forty-five missense nucleotide mutations were identified; out of these, 5 mutations located at Nsp2, Nsp3, Nsp14, and ORF10 genes occurred with a

A large fraction of energy, including solar energy, is dissipated into ambient atmosphere as low-grade waste heat. Efficient utilization of such energy is critical to address the current energy crisis and global warming issue. Herein, the efficient near-IR (NIR)-photothermal, thermoelectric, and thus photo-thermo-electric conversion of polyoxovanadate compound {[Ni(1,10-phenanthroline)3 ][V14 O34 Cl]Cl, NiV14 } in ionic liquid was achieved. The solution displayed a NIR-photothermal efficiency of 16.04 and 23.43 % at 808 and 1064 nm, respectively. Taking advantage of the synergetic thermodiffusive and thermogalvanic effects of various ion species in NiV14 solution, an open circuit voltage of approximately 0.45 V was obtained at ΔT=70 K generated by physical heating or NIR irradiation, indicating a large Seebeck coefficient of 6.38 mV K-1 and an optimized thermal power at 1.2 W m-2 . The polyoxovanadate-ionic liquid system offers a new platform for efficiently utilizing not only low-grade thermal energy but also solar energy for electricity generation.

To review the clinical data of a fetus with false positive result of non-invasive prenatal testing (NIPT) due to confined placental mosaicism (CPM).Amniotic fluid sample was taken from a pregnant women with high risk for chromosome 16 aneuploidy for karyotyping analysis, single nucleotide polymorphism array (SNP array) and interphase fluorescence in situ hybridization (FISH). Genetic testing was also conducted on the fetal and maternal surface of the placenta, root of umbilical cord and fetal skin tissue after induced abortion.Cytogenetic analysis of the amniotic fluid sample yielded a normal karyotype. SNP array revealed mosaicism (20%) of trisomy 16 in the fetus. FISH confirmed the presence of mosaicism (25%) for trisomy 16. After induced labor, all sampled sites of placenta were confirmed to contain trisomy 16 by SNP array, while the analysis of fetal skin tissue yielded a negative result.CPM is an important factor for false positive NIPT result. Prenatal identification of CPM and strengthened pregnancy management are important to reduce adverse pregnancy outcomes.

To detect gene inversion in two pedigrees affected with Hemophilia A by using Nanopore sequencing technology.Peripheral blood samples were taken from members of the two pedigrees. Following extraction of genome DNA, genetic variants of the carriers were detected by Nanopore sequencing and subjected to bioinformatic analysis.Nanopore sequencing has identified the niece of the proband of the pedigree 1 as carrier of Hemophilia A Inv22, and the mother of the proband of the pedigree 2 as carrier of Hemophilia A Inv1, which was consistent with clinical findings. Breakpoint sites in both pedigrees were accurately mapped. Statistical analysis of the sequencing results revealed a large number of variations in the carriers' genomes including deletions, duplications, insertions, inversions and translocations.Nanopore sequencing can be used to analyze gene inversions associated with Hemophilia A, which also provided a powerful tool for the diagnosis of diseases caused by gene inversions.

Mg/Al/Fe layered double hydroxide (MAF-LDH1) was prepared by solvothermal method with the sodium dodecyl sulfate as the template, and the ethanol system was benefit to growth of sample. The nature in the resulting MAF-LDH was investigated by X-ray diffraction, field emission scanning electron microscopy, Fourier transformed infrared spectra, thermogravimetric analysis, and N2 adsorption-desorption. The morphology of MAF-LDH1 is petal-like with the size of 400–500 nm and the thickness about 10–20 nm. The adsorption performance of the samples was evaluated by absorption of the Congo red (CR) solutions. Compared with Mg/Al layered double hydroxide (MA-LDH), the maximum adsorption capacities of the MAF-LDH1 samples were 943.4 mg/g which was greatly enhanced. Furthermore, after seven cycling tests, the adsorption performance was still up to 90%. Theoretical calculation results revealed that the adsorption process was spontaneous and followed the pseudo-second-order kinetic model and Freundlich model. This work provides a promising alternative strategy to enhance the adsorptive properties of hydrotalcite-like materials.

A series of non-precious metal/metal oxide nickel-cobalt catalysts was prepared for a highly efficient depolymerization of Kraft lignin (KL) into liquid fuels using amorphous TiZr-oxide (Ti1−yZryO2) as a carrier. The effects of Ni-NiOx, Co-CoOx, NiCo-NiCoOx, NiCoOx and NiCo catalysts supported on amorphous TiZr-oxide carrier on KL depolymerization were investigated. It was found that the NiCo-NiCoOx/Ti1−yZryO2 catalyst is optimal for converting KL to petroleum ether (PE)-soluble product (mainly composed of monomers and dimers) in an 80.2% high yield at 320 °C for 24 h, with excellent reusability and a low formation of char. Under these conditions, the higher heating value (HHV) increased from 25.11 to 33.89 MJ/kg. A meticulous study on NiCo-NiCoOx/Ti1−yZryO2 catalysts revealed that the synergistic effect among Lewis acid sites, basic sites and metal active sites played an important role in obtaining high yields of monomers and low rates of char formation during lignin conversion.

The Pr3+ doped oxyfluoride silicate glasses prepared by melt-quenching method are investigated. It is found that the replacement of CaO with Na2O in glass composition favors the formation of CaF2 crystals during the melt quenching process. The addition of Al2O3 and ZnO into glass composition enhances the 3P0 → 3H4 emission of Pr3+ ions. The self-excitation of Pr3+ is observed under the excitation wavelength of 443 nm, which can be strengthened with the increase of CaF2 crystal content. Furthermore, a unique emission peak at 690 nm is displayed in the glass containing crystalline Al2O3. Time-resolved emission spectra and decay curves reveal that the emission at 481 nm decays faster than that at 600 nm. The Pr3+ doped oxyfluoride silicate glasses have promising prospects in light emitting diode applications.

A series of Ce3+, Dy3+ doped Ca3(P1-xBxO4)2 phosphors were synthesized by a high temperature solid-state reaction method, and their corresponding structure, morphology, and luminescence properties were investigated. It is found that the crystal phase transition from β-Ca3(PO4)2 to Ca5(PO4)3(OH) gradually occurs with increasing the boron content in the phosphors, which can provide various local environments for the doping ions and consequently affect the luminescence properties of the phosphors. A red shift of the emission peak of Ce3+ was revealed upon incorporation of boron into Ce/Dy co-doped phosphors. In addition, the luminescence properties could be well modulated by varying the excitation wavelength. The developed phosphors have great potential as a kind of single component white light-emitting phosphors for white light-emitting diodes.

5-Hydroxymethylfurfural (HMF) which is derived from cellulosic biomass is an important platform for value added downstream chemicals and fuels via diversified processes. Nanoceria rods, cubes and octahedra were synthesized by a hydrothermal method and used as supports for preparing Au/CeO2 catalysts via deposition–precipitation. The morphology effect of CeO2 in the Au/CeO2 catalysts was investigated in selective oxidation of HMF to 2,5-furandicarboxylic acid (FDCA). The Au/CeO2-rod was more efficient for the oxidation of HMF and the TOF value of Au/CeO2-rod was 6.3 (min−1), which was 7 times as high as that of Au/CeO2-cube (0.9 min−1) and 32 times as high as that of Au/CeO2-oct (0.2 min−1). The experimental results and catalyst characterization implied that the oxygen vacancies on the Au–CeO2 interface determined the catalytic activity by affecting the size, and valence of Au and the interfacial acidic properties. The Au/CeO2-rod with the highest oxygen vacancies presented the higher proportion of cationic gold and more interfacial Lewis acidic sites between ceria and Au nanoparticles. Moreover, a synergistic effect between the interfacial Lewis acid, homogeneous base and neighboring Au particles promoted the oxidation reaction via efficiently activating the hydroxyl, aldehyde and molecular O2.

A series of Pr3+ and Tm3+ doped oxyfluoride silicate glasses prepared by melt-quenching method are investigated by X-ray diffraction, differential scanning calorimeter, Fourier transform infrared spectra, and photoluminescence spectra. The luminescence of Pr3+ in the as-prepared glasses mainly show two intensified emission peaks in blue (484 nm) and reddish orange (604 nm) regions corresponding to the transitions 3P0→3H4 and 3P0→3H6 (1D2→3H4) of Pr3+ ions. The relative emission intensity of these two emission peaks can be tailored by varying the glass matrix compositions. For certain base glass compositions, the formation of CaF2 crystals occurred during the melt quenching process, which can change the luminescence performance of the glasses. In addition, we find that the Tm3+ ions play a significant role in modifying the luminescence of Pr3+. The Pr3+ and Tm3+ doped oxyfluoride silicate glasses reported here have broad prospects in light emitting diode applications.

In this study, a novel modified nickel/H-beta (Ni/DeAl-beta) catalyst, which has active acidic sites and hydrogen binding sites, was prepared and used to produce liquefied fuel from lignin. The bifunctional Ni/DeAl-beta catalyst efficiently converted kraft lignin into liquefied fuel due to the synergistic effect of aluminum Lewis acid sites and nickel hydrogen binding sites. At a nickel content of 0.6 mmol/gzeolite, the Ni/DeAl-beta catalyst gave a high liquid product yield of 88.6% at 300 °C for 36 h. Most of the liquid product was dissolved in petroleum ether (73% of 88.6%), which was mainly composed of monomeric and dimeric degradation products. Under these conditions, the higher heating values (HHV) increased from 24.9 MJ/kg for kraft lignin to 32.0 MJ/kg for the liquid product. These results demonstrated the bifunctional Ni/DeAl-beta catalyst could be an efficient catalyst for lignin to liquefied fuel conversion.

The Tb3+ and Dy3+ doped phosphosilicate glasses prepared by melt quenching method are studied for light emitting diode applications. For certain base glass compositions, the crystals Ca3(PO4)2 and CaZn2(PO4)2 are precipitated during the melt quenching process, which induce the narrowness of the full width at half maximum (FWHM) of the emission bands peaked at 574 nm of Dy3+ ions. The intensity of emission bands of Tb3+ ions at 489 and 544 nm is significantly enhanced with the increase of P2O5 content. The ratio of the emission located at the yellow wavelength portion to that located at the blue wavelength region of the Dy3+ ions can be apparently tuned by changing the P2O5 content as well. In addition, the energy transfer between Tb3+ and Dy3+ in the as-prepared glasses is also discussed. The Commission International de I'Eclairage (CIE) chromaticity diagram shows that the emission hue is dependent on the glass composition. The Tb3+ and Dy3+ doped phosphosilicate glasses have promising prospects in light emitting diode applications.