MYB4 in Lilium pumilum affects plant saline-alkaline tolerance.

Lilium pumilum DC (L. pumilum DC) plays an important role in the rational utilization of salinized soil. To explore the molecular mechanism of salt-tolerant L. pumilum, the LpMYB4 was cloned. LpMYB4 close relationship with Bambusa emeiensis and Zea mays MYB4 throughout the phylogenetic tree construction. LpMYB4 protein was found to be localized in the nucleus. Prokaryotic and eukaryotic bacterial solution resistance experiments proved that the exogenous introduction of LpMYB4 made the overexpression strains obtain better survival ability under saline-alkaline stress. Compared with wild-type plants, tobacco plants overexpressing LpMYB4 had better growth and lower leaf wilting and lodging, the content of chlorophyll was higher, the content of hydrogen peroxide and superoxide anion was lower, the activity of peroxidase and superoxide dismutase was higher and the relative conductivity was lower under saline-alkaline stress. The analysis of seed germination and seedling resistance of transgenic plants under salt stress showed that LpMYB4 transgenic seeds were more tolerant to salt stress during germination and growth. Yeast two-hybrid and two-luciferase complementation experiments showed that LpMYB4 interacted with yeast two-hybrid and LpGPX6. The analysis of the role of LpMYB4 in improving plant saline-alkali resistance is helpful to the transformation of plant germplasm resources and has great significance for agriculture and sustainable development.

Clonal integration of stress signal induces morphological and physiological response of root within clonal network.

Clonal integration of defense or stress signal induced systemic resistance in leaf of interconnected ramets. However, similar effects of stress signal in root are poorly understood within clonal network. Clonal fragments of Centella asiaticas with first-young, second-mature, third-old and fourth-oldest ramets were used to investigate transportation or sharing of stress signal among interconnected ramets suffering from low water availability. Compared with control, oxidative stress in root of the first-young, second-mature and third-old ramets was significantly alleviated by exogenous ABA application to the fourth-oldest ramets as well as enhancement of antioxidant enzyme (SOD, POD, CAT and APX) activities and osmoregulation ability. Surface area and volume in root of the first-young ramets were significantly increased and total length in root of the third-old ramets was significantly decreased. POD activity in root of the fourth-oldest and third-old ramets was significantly enhanced by exogenous ABA application to the first-young ramets. Meanwhile, total length and surface area in root of the fourth-oldest and third-old ramets were significantly decreased. Ratio of belowground to aboveground biomass in the whole clonal fragments was significantly increased by exogenous ABA application to the fourth-oldest or first-young ramets. It is suggested that transportation or sharing of stress signal may induce systemic resistance in root of interconnected ramets. Specially, transportation or sharing of stress signal against phloem flow was observed in the experiment. Possible explanation is that rapid recovery of foliar photosynthesis in first-young ramets subjected to exogenous ABA application can partially reverse phloem flow within clonal network. Thus, our experiment provides insight into ecological implication on clonal integration of stress signal.

NiRu-Mo2Ti2C3O2 as an efficient catalyst for alkaline hydrogen evolution reactions: the role of bimetallic site interactions in promoting Volmer-step kinetics.

The Volmer step in alkaline hydrogen evolution reactions (HERs), which supplies H* to the following steps by cleaving H-O-H bonds, is considered the rate-determining step of the overall reaction. The Volmer step involves water dissociation and adsorbed hydroxyl (*OH) desorption; Ru-based catalysts display a compelling water dissociation process in an alkaline HER. Unfortunately, the strong affinity of Ru for *OH blocks the active sites, resulting in unsatisfactory performance during HER processes. Hence, this study investigates a series of key descriptors (ΔG*H2O, ΔG*H-OH, ΔG*H, and ΔG*OH) of TM (Fe, Co, Ni, Ru, Rh, Pd, Os, Ir, or Pt)-Ru/Mo2Ti2C3O2 to systematically explore the effects of bimetallic site interactions on the kinetics of the Volmer step. The results indicate that bimetallic catalysts effectively reduced the strong adsorption of *OH on Ru sites; especially, the NiRu diatomic state shows the highest electron-donating ability, which promoted the smooth migration of *OH from Ru sites to Ni sites. Therefore, Ru, Ni and MXenes are suitable to serve as water adsorption and dissociation sites, *OH desorption sites, and H2 release sites, respectively. Ultimately, NiRu/Mo2Ti2C3O2 promotes Volmer kinetics and has the potential to improve alkaline HERs. This work provides theoretical support for the construction of synergistic MXene-based diatomic catalysts and their wide application in the field of alkaline HERs.

Integrated network pharmacology, molecular docking and pharmacodynamic study reveals protective effects and mechanisms of corilagin against cerebral ischemia-induced injury.

BACKGROUND: Stroke is one of the leading causes of death and long-term disability worldwide. Previous studies have found that corilagin has antioxidant, anti-inflammatory, anti-atherosclerotic and other pharmacological activities and has a protective effect against cardiac and cerebrovascular injury. OBJECTIVES: The aim of this study was to investigate the protective effects of corilagin against ischemic stroke and to elucidate the underlying molecular mechanisms using network pharmacology, molecular docking, and animal and cell experiments. METHODS: We investigated the potential of corilagin to ameliorate cerebral ischemia-reperfusion injury using in vivo rat middle cerebral artery occlusion/reperfusion (MCAO/R) and in vitro oxygen-glucose deprivation/reoxygenation (OGD/R) models. RESULTS: Our results suggest that corilagin may exert its anti-ischemic stroke effect by interacting with 92 key targets, including apoptosis-associated proteins (Bcl-2, Bax, caspase-3) and PI3K/Akt signaling pathway-related proteins. In vivo and in vitro experiments showed that corilagin treatment improved neurological deficits, attenuated cerebral infarct volume, and mitigated neuronal damage in MCAO/R rats. Corilagin treatment also enhanced the survival of PC12 cells exposed to OGD/R, reduced the rate of LDH leakage, inhibited cell apoptosis, and activated the PI3K/Akt signaling pathway. Importantly, the effects of corilagin on the PI3K/Akt signaling pathway and apoptosis-associated proteins were reversed by the PI3K-specific inhibitor LY294002. CONCLUSIONS: These results indicate that the molecular mechanism of the anti-ischemic effect of corilagin involves inhibiting neuronal apoptosis and activating the PI3K/Akt signaling pathway. These findings provide a theoretical and experimental basis for the further development and application of corilagin as a potential anti-ischemic stroke agent.

20(R)-ginsenoside Rg3 attenuates cerebral ischemia-reperfusion injury by mitigating mitochondrial oxidative stress via the Nrf2/HO-1 signaling pathway.

Reducing mitochondrial oxidative stress has become an important strategy to prevent neuronal death in ischemic stroke. Previous studies have shown that 20(R)-ginsenoside Rg3 can significantly improve behavioral abnormalities, reduce infarct size, and decrease the number of apoptotic neurons in cerebral ischemia/reperfusion injury rats. However, it remains unclear whether 20(R)-ginsenoside Rg3 can inhibit mitochondrial oxidative stress in ischemic stroke and the potential molecular mechanism. In this study, we found that 20(R)-ginsenoside Rg3 notably inhibited mitochondrial oxidative stress in middle cerebral artery occlusion/reperfusion (MCAO/R) rats and maintained the stability of mitochondrial structure and function. Treatment with 20(R)-ginsenoside Rg3 also decreased the levels of mitochondrial fission proteins (Drp1 and Fis1) and increased the levels of fusion proteins (Opa1, Mfn1, and Mfn2) in MCAO/R rats. Furthermore, we found that 20(R)-ginsenoside Rg3 promoted nuclear aggregation of nuclear factor erythroid2-related factor 2 (Nrf2) but did not affect Kelch-like ECH-associated protein-1 (Keap1), resulting in the downstream expression of antioxidants. In in vitro oxygen-glucose deprivation/reperfusion stroke models, the results of PC12 cells treated with 20(R)-ginsenoside Rg3 were consistent with animal experiments. After transfection with Nrf2 short interfering RNA (siRNA), the protective effect of 20(R)-ginsenoside Rg3 on PC12 cells was reversed. In conclusion, the inhibition of mitochondrial oxidative stress plays a vital position in the anti-cerebral ischemia-reperfusion injury of 20(R)-ginsenoside Rg3, and its neuroprotective mechanism is related to the activation of the nuclear factor erythroid2-related factor 2/heme oxygenase 1 signaling pathway.

Incidence, associated factors, and outcomes of delirium in critically ill children in china: a prospective cohort study.

BACKGROUND: Delirium occurs frequently in critically ill children and has been reported in many countries, but delirium is not well-characterized in China. The aim of this study was to represent the incidence of delirium in critically ill children in China, its associated factors, and the influence of delirium on in-hospital outcomes. METHODS: This observational prospective cohort study was set up in a large academic medical center with a 57-bed PICU in southwestern China. Critically ill children who required PICU stays over 24 h and were admitted between November 2019 and February 2022 were included in this study. The Cornell Assessment of Pediatric Delirium was used twice daily for delirium evaluation by bedside nurses, and twenty-four clinical features were collected from medical and nursing records during hospitalization. RESULTS: The incidence of delirium was 26.0% (n = 410/1576). Multivariate analysis revealed that seven independent risk factors including days of mechanical ventilation and physical restraints, admission diagnosis (neurologic disorder), sleep deprivation, use of benzodiazepines and dexmedetomidine, liver failure/liver dysfunction associated with delirium in critically ill children. One potentially protective factor was the watching television /listening to music/playing with toys. Children with delirium had longer lengths of stay in the PICU (median 11 vs. 10 days, p < 0.001) and hospital (median 18 vs. 15 days, p < 0.001) compared to those without delirium. Additionally, the in-hospital mortality rates were 4.63% and 0.77% in patients with and without delirium (p < 0.05). CONCLUSIONS: Delirium is common in critically ill children in China and related to poor outcomes. Interventional studies are warranted to determine the best practices to reduce delirium exposure in at-risk children.

ARF6 promotes hepatocellular carcinoma proliferation through activating STAT3 signaling.

BACKGROUND: Hepatocellular Carcinoma (HCC) possesses the high mortality in cancers worldwide. Nevertheless, the concrete mechanism underlying HCC proliferation remains obscure. In this study, we show that high expression of ARF6 is associated with a poor clinical prognosis, which could boost the proliferation of HCC. METHODS: Immunohistochemistry and western blotting were used to detect the expression level of ARF6 in HCC tissues. We analyzed the clinical significance of ARF6 in primary HCC patients. We estimated the effect of ARF6 on tumor proliferation with in vitro CCK8, colony formation assay, and in vivo nude mouse xenograft models. Immunofluorescence was conducted to investigate the ARF6 localization. western blotting was used to detect the cell cycle-related proteins with. Additionally, we examined the correlation between ARF6 and STAT3 signaling in HCC with western blotting, immunohistochemistry and xenograft assay. RESULTS: ARF6 was upregulated in HCC tissues compared to adjacent normal liver tissues. The increased expression of ARF6 correlated with poor tumor differentiation, incomplete tumor encapsulation, advanced tumor TNM stage and poor prognosis. ARF6 obviously promoted HCC cell proliferation, colony formation, and cell cycle progression. In vivo nude mouse xenograft models showed that ARF6 enhanced tumor growth. Furthermore, ARF6 activated the STAT3 signaling and ARF6 expression was positively correlated with phosphorylated STAT3 level in HCC tissues. Furthermore, after intervening of STAT3, the effect of ARF6 on tumor-promoting was weakened, which demonstrated ARF6 functioned through STAT3 signaling in HCC. CONCLUSIONS: Our results indicate that ARF6 promotes HCC proliferation through activating STAT3 signaling, suggesting that ARF6 may serve as potential prognostic and therapeutic targets for HCC patients.

Global, regional, and national burden of kidney dysfunction from 1990 to 2019: a systematic analysis from the global burden of disease study 2019.

OBJECTIVE: We aim to explore the prevalence and temporal trends of the burden of kidney dysfunction (KD) in global, regional and national level, since a lack of related studies. DESIGN: Cross-sectional study. MATERIALS: The data of this research was obtained from Global Burden of Diseases Study 2019. The estimation of the prevalence, which was measured by the summary exposure value (SEV), and attributable burden of KD was performed by DisMod-MR 2.1, a Bayesian meta-regression tool. The Spearman rank order correlation method was adopted to perform correlation analysis. The temporal trends were represented by the estimated annual percentage change (EAPC). RESULTS: In 2019, there were total 3.16 million deaths and 76.5 million disability-adjusted life years (DALYs) attributable to KD, increased by 101.1% and 81.7% compared with that in 1990, respectively. From 1990 to 2019, the prevalence of KD has increased in worldwide, but decreased in High-income Asia Pacific. Nearly 48.5% of countries globally, such as South Africa, Egypt and Mexico had increased mortality rates of KD from 1990 to 2019 while 44.6% for disability rate. Countries with lower socio-demographic index (SDI) are facing a higher prevalence as well as mortality and disability rate compared with those with higher SDI. Compared with females, the prevalence of KD was lower in males, however the attributable mortality and disability rate were higher in all years from 1990 to 2019. CONCLUSION: With the progress of senescent, we will face more severe challenges of reducing the prevalence and attributable burden of KD, especially in regions with lower SDI. Effective measures are urgently required to alleviate the prevalence and burden of KD.

Original self-assembled S-scheme BiOBr-(001)/Bi2SiO5/Bi heterojunction photocatalyst with rich oxygen vacancy for boosting CO2 reduction performance.

Photocatalysis CO2 reduction into high-value-added chemical feedstocks is desirable for simultaneously addressing the solar energy storage, CO2 excess and energy shortage issues. In this work, a kind of original S-scheme BiOBr-(001)/Bi2SiO5/Bi (OSB) heterostructure photocatalyst with rich oxygen vacancies is in-situ synthesized, which significantly promotes the photocatalytic CO2 reduction performance. Interestingly, the lower formation energy of oxygen vacancy exhibits the easy feasibility on the BiOBr-(001) surface via the assistant of ultrasound. There exists the highest photocatalytic CO2 reduction activity to CO of 234.05 µmol g-1h-1 for OSB-20 sample (ultrasound time: 20 min), higher 3.3 times than OSB-0 sample (without ultrasound). Combined with experimental and calculated results, the significative formation mechanism, widened light-response range, highly-efficient separation/transfer paths and improved redox-reduction abilities of photogenerated electron-hole pairs for S-scheme OSB-20 heterostructure are investigated and proposed. Our findings provide new insights for the construction and synthesis of the S-scheme Bi-based heterojunction photocatalyst system.

Enhanced adsorption and co-adsorption of heavy metals using highly hydrophilicity amine-functionalized magnetic hydrochar supported MIL-53(Fe)-NH2: performance, kinetics, and mechanism studies.

It is a "kill two birds with one stone" method to convert invasive plants into hydrochar via hydrothermal carbonization as well as coinciding with 3R rules (reduction, recycling, and reuse). In this work, a series of hydrochars (pristine, modified, and composite) derived from invasive plants Alternanthera philoxeroides (AP) were prepared and applied to the adsorption and co-adsorption of heavy metals (HMs) such as Pb(II), Cr(VI), Cu(II), Cd(II), Zn(II), and Ni(II). The results show that MIL-53(Fe)-NH2- magnetic hydrochar composite (M-HBAP) displayed a strong affinity for HMs, which the maximum adsorption capacities for HMs were 153.80 (Pb(II)), 144.77 (Cr(VI)), 80.58 (Cd(II)), 78.62 (Cu(II)), 50.39 (Zn(II)), and 52.83(Ni(II)) mg/g (c0 = 200 mg/L, t = 24 h, T = 25 ℃, pH = 5,2,6,4,6,5). This may be because the doping of MIL-53(Fe)-NH2 enhanced the surface hydrophilicity of hydrochar, which allows hydrochar to disperse in the water within 0.12 s and possessed excellent dispersibility compared with pristine hydrochar (BAP) and amine-functionalized magnetic modified hydrochar (HBAP). Furthermore, the BET surface area of BAP was improved from 5.63 to 64.10 m2/g after doing MIL-53(Fe)-NH2. M-HBAP shows a strong adsorption effect on the single HMs system (52-153 mg/g), while it decreased significantly (17-62 mg/g) in the mixed HMs system due to the competitive adsorption. Cr(VI) can produce strong electrostatic interaction with M-HBAP, Pb(II) can react with CaC2O4 on the surface of M-HBAP for chemical precipitation, and other HMs can react with functional groups on the surface of M-HBAP for complexation and ion exchange. In addition, five adsorption-desorption cycle experiments and vibrating sample magnetometry (VSM) curves also proved the feasibility of the M-HBAP application.

Single atom supported on MXenes for the alkaline hydrogen evolution reaction: species, coordination environment, and action mechanism.

The electrochemical hydrogen evolution reaction (HER) in alkaline media provides an environmentally friendly industrial application approach to replace traditional fossil energy. The search for efficient, low-cost, and durable active electrocatalysts is central to the development of this area. Transition metal carbides (MXenes) have been emerging as a new family of two-dimensional (2D) materials that have great potential in the HER. Herein, density functional theory calculations are performed to systematically explore the structural and electronic properties and alkaline HER performances of Mo-based MXenes, as well as the influence of species and the coordination environment of single atoms on the improvement of the electrocatalytic activity of Mo2Ti2C3O2. The results show that Mo-based MXenes (Mo2CO2, Mo2TiC2O2, and Mo2Ti2C3O2) exhibit excellent H binding ability, while slow water decomposition kinetics hinders their HER performance. Replacing the O-terminal of Mo2Ti2C3O2 with a Ru single-atom (RuS-Mo2Ti2C3O2) could promote the decomposition of water owing to the stronger electron-donating ability of the atomic state Ru. In addition, Ru could also improve the binding ability of the catalyst to H by adjusting the surface electron distribution. As a result, RuS-Mo2Ti2C3O2 exhibits excellent HER performance with a water decomposition potential barrier of 0.292 eV and a H adsorption Gibbs free energy of -0.041 eV. These explorations bring new prospects for single atoms supported on Mo-based MXenes in the alkaline hydrogen evolution reaction.

Collagen I-DDR1 signaling promotes hepatocellular carcinoma cell stemness via Hippo signaling repression.

Cancer stem cells (CSCs) are a minority population of cancer cells with stemness and multiple differentiation potentials, leading to cancer progression and therapeutic resistance. However, the concrete mechanism of CSCs in hepatocellular carcinoma (HCC) remains obscure. We found that in advanced HCC tissues, collagen I was upregulated, which is consistent with the expression of its receptor DDR1. Accordingly, high collagen I levels accompanied by high DDR1 expression are associated with poor prognoses in patients with HCC. Collagen I-induced DDR1 activation enhanced HCC cell stemness in vitro and in vivo. Mechanistically, DDR1 interacts with CD44, which acts as a co-receptor that amplifies collagen I-induced DDR1 signaling, and collagen I-DDR1 signaling antagonized Hippo signaling by facilitating the recruitment of PP2AA to MST1, leading to exaggerated YAP activation. The combined inhibition of DDR1 and YAP synergistically abrogated HCC cell stemness in vitro and tumorigenesis in vivo. A radiomic model based on T2 weighted images can noninvasively predict collagen I expression. These findings reveal the molecular basis of collagen I-DDR1 signaling inhibiting Hippo signaling and highlight the role of CD44/DDR1/YAP axis in promoting cancer cell stemness, suggesting that DDR1 and YAP may serve as novel prognostic biomarkers and therapeutic targets in HCC.

In Situ Formation ZnIn2 S4 /Mo2 TiC2 Schottky Junction for Accelerating Photocatalytic Hydrogen Evolution Kinetics: Manipulation of Local Coordination and Electronic Structure.

Regulating electronic structures of the active site by manipulating the local coordination is one of the advantageous means to improve photocatalytic hydrogen evolution (PHE) kinetics. Herein, the ZnIn2 S4 /Mo2 TiC2 Schottky junctions are designed to be constructed through the interfacial local coordination of In3+ with the electronegative O terminal group on Mo2 TiC2 based on the different work functions. Kelvin probe force microscopy and charge density difference reveal that an electronic unidirectional transport channel across the Schottky interface from ZnIn2 S4 to Mo2 TiC2 is established by the formed local nucleophilic/electrophilic region. The increased local electron density of Mo2 TiC2 inhibits the backflow of electrons, boosts the charge transfer and separation, and optimizes the hydrogen adsorption energy. Therefore, the ZnIn2 S4 /Mo2 TiC2 photocatalyst exhibits a superior PHE rate of 3.12 mmol g-1 h-1 under visible light, reaching 3.03 times that of the pristine ZnIn2 S4 . This work provides some insights and inspiration for preparing MXene-based Schottky catalysts to accelerate PHE kinetics.

Controllable Synthesis of BiOCl with Z-Scheme (001)/(110) Facet Homojunction and their Photocatalytic Killing Effect on HePG2 Cells in vitro.

In this study, a set of BiOCl with controllable ratios of (001) and (110) facets was prepared by adjusting the content of diethylene glycol (DEG) during the preparation process. The degradation experiment of bisphenol A (BPA) shows that under simulated sunlight, when the ratio of (001) to (110) is 0.61, BiOCl (BOC-2) has the best degradation activity, which can degrade 96.2% BPA within 20 min. After theoretical calculations and experimental characterization, a Z-scheme (001)/(110) facet homojunction is proposed. Then, three typical samples were selected to test the biological toxicity of HepG2 cells and the activity of killing HepG2 cells under ultraviolet light conditions. Studies have found that exposed facets play a more important role in the biotoxicity of BiOCl to cells; with a (001)/(110) ratio of 0.61, BOC-2 exhibits excellent endocytosis and phototoxicity but no obvious dark cytotoxicity, while with a (001)/(110) ratio of 0.15, BiOCl (BOC-4) has poor endocytosis and strong cytotoxicity under dark conditions. Through reactive oxygen species (ROS) and lactate dehydrogenase (LDH) assay detection, the process of photocatalytic killing cells of BOC-2 more looks like an apoptosis mechanism, while BOC-4 mainly causes cell necrosis.

Comprehensive research on the distribution of HLA-DRB1 in Chinese populations.

By presenting antigen peptides, HLA-DRB1 plays an important role in the immune system. However, the allele frequency of HLA-DRB1 exon 2 across China has not been comprehensively studied, especially in minority populations. We sampled 3757 individuals from 59 population. The HLA-DRB1 region from 212 to 463 bp (NM_002124.4 exon 2) in each population was sequenced by Sanger sequencing and genotyped via SBTengine® software, and the allele frequency was calculated by GenAlEx 6.5. Eighty-two DRB1 alleles were identified. The expected heterozygosity of DRB1 was lower in the south than in the north, which was inconsistent with the Y chromosome and mitochondrial DNA results. The Mantel test and nonparametric correlation analysis showed that the correlations of the genetic distance with geographical distance and of DRB1 allele frequencies with latitude weakened after the southern and northern groups were considered separately. Principal coordinate analysis showed that populations speaking the same languages were not codistributed. Compared with other genetic markers, the distribution of DRB1 seems less affected by geographic distance and ethnic origin. Local factors such as gene flow with neighbouring populations, geographic isolation or natural selection are important forces shaping the DRB1 gene pool of local populations.

Effects of nutrient supply on leaf stoichiometry and relative growth rate of three stoloniferous alien plants.

Different nutrient supply brings about changes in leaf stoichiometry, which may affect growth rate and primary production of plants. Invasion of alien plants is a severe threat to biodiversity and ecosystem worldwide. A pot experiment was conducted by using three stoloniferous alien plants Wedelia trilobata, Alternanther philoxeroides and Hydrocotyle vulgaris to investigate effects of nutrient supply on their leaf stoichiometry and relative growth rate. Different nitrogen or phosphorus supply was applied in the experiment (N1:1 mmol L-1, N2:4 mmol L-1, and N3:8 mmol L-1, P1:0.15 mmol L-1, P2:0.6 mmol L-1 and P3:1.2 mmol L-1). Nitrogen and phosphorus concentrations in leaves of the three alien plants significantly increased with increase of nitrogen supply. With increase of phosphorus supply, nitrogen or phosphorus concentration of leaf was complex among the three alien plants. N:P ratio in leaf of the three alien plants subjected to different levels of nutrient supply was various. A positive correlation between relative growth rate and N:P ratio of the leaf is observed in W. trilobata and A. philoxeroides suffering from N-limitation. A similar pattern was not observed in Hydrocotyle vulgaris. We tentatively concluded that correlations between relative growth rate and N: P ratio of the leaf could be affected by species as well as nutrient supply. It is suggested that human activities, invasive history, local abundance of species et al maybe play an important role in the invasion of alien plants as well as relative growth rate.

A novel splice site variant in the POPDC3 causes autosomal recessive limb-girdle muscular dystrophy type 26.

Limb-Girdle muscular dystrophy (LGMD) is a group of muscle disorders with highly heterogeneous genetic patterns and clinical phenotypes, and this group includes multiple subtypes. Different LGMD subtypes have similar phenotypes and clinical overlaps, these subtypes are difficult to distinguish by clinical symptoms alone and can only be accurately diagnosed by analysis in combination with definitive genetic test results. Here, we report a female presenting features of LGMD. After analysis of whole-exome sequencing data, a novel homozygous POPDC3 variant c.486-1G>A (rs113419658) located in the acceptor splice site of intron 2 was identified in the proband. The variant effect on splicing were analyzed by genetic analysis based on cDNA synthesized by the patient's RNA. cDNA analysis indicated that the novel homozygous POPDC3 splice variant disrupted original acceptor splice site, which can cause a frameshift in the mRNA of the POPDC3 gene, thereby producing a truncated POPDC3 protein and ultimately affecting its normal function. POPDC3 variant was recently associated with recessive limb-girdle muscular dystrophy type 26 (LGMDR26). Based on the above results, we hypothesize that this variant is probably a pathogenic variant, and expand the gene variant spectrum of POPDC3.

Amine-functionalized magnetic biochars derived from invasive plants Alternanthera philoxeroides for enhanced efficient removal of Cr(VI): performance, kinetics and mechanism studies.

In this study, novel magnetic biochars derived from Alternanthera philoxeroides and modified by different amines (hexanediamine, melamine, and L-glutathione) were successfully prepared by hydrothermal carbonization and employed as an efficient adsorbent for Cr(VI). When pH = 2.0, T = 25 °C, c0 = 100 mg/L, and the dosage of biochars is 0.05 g, the maximum adsorption capacity of Cr(VI) by pristine biochar (BAP) was 42.47 mg/g and modified biochars (MFBAP, MEBAP, LBAP) was 80.58, 62.26, and 55.66 mg/g, respectively. It was found that hexanediamine and melamine could enhance the SBET of biochars, while L-glutathione could reduce its SBET, which could be supported by BET measurement and SEM images. Adsorption kinetics and isotherm studies showed that the Cr(VI) adsorption process of MFBAP followed Elovich kinetic model and Langmuir isotherm, respectively, which means that it was mainly a chemical adsorption process. The characterization results proved that -NH2 derived from amines plays a significant role in removing Cr(VI), which is mainly degraded by complexation reaction, electrostatic interaction, and reduction. In sum, the biochar modified by amines has excellent Cr(VI) adsorption performance, highly enhanced SBET, and excellent recyclability, which is a promising candidate for solving the problem of invasive plants and wastewater treatment.