Palladium-Catalyzed Synthesis of Nitrones Via Redox Cross-Coupling of Nitro Compounds and Alcohols.

A novel methodology for the synthesis of nitrones via palladium-catalyzed redox cross-coupling of nitro compounds and alcohols is established. The protocol is a mild, convenient, ligand-free, and scalable synthesis method that can be compatible with various nitro compounds and alcohols. Nitrone is a significant multifunctional platform synthon which can be synthesized directly and efficiently via this tactic from commercially available and cheap raw materials.

Polyphyllin VII as a potential medication for targeting epithelial mesenchymal transitionin in thyroid cancer.

The need for novel anti-thyroid cancer (TC) medications is urgent due to the rising incidence and metastatic rates of malignant TC. In this study, we investigated the effect of Polyphyllin VII (PPVII) to TC cells, and explored their potential mechanism. B-CPAP and TPC-1 cells, were used to analyze the antitumor activity of PPVII by quantifying cell growth and metastasis as well as to study the effect on epithelial mesenchymal transition (EMT). The results showed that PPVII dramatically reduced the capacity of B-CPAP and TPC-1 cells to proliferate and migrate in a dose-response manner. Following PPVII treatment of TC cells, the expression levels of E-cadherin progressively increased and were higher than the control group, while the expression levels of EMT-related genes Vimentin, N-cadherin, Slug, Zeb-1, and Foxe1 gradually declined and were lower than the control group. It was proposed that PPVII might prevent TC from undergoing EMT. The Foxe1 gene was shown to be significantly expressed in TC, and a statistically significant variation in Foxe1 expression was observed across clinical stages of the disease, according to a bioinformatics database study. There was a strong link between the expression of the Foxe1 gene and the EMT-related gene. In the meantime, TC cells' expression of Foxe1 can be inhibited by PPVII. In conclusion, our results showed that PPVII may as a potential medication for targeting EMT in thyroid cancer.

Modular DNA-Origami-Based Nanoarrays Enhance Cell Binding Affinity through the "Lock-and-Key" Interaction.

Surface proteins of cells are generally recognized through receptor-ligand interactions (RLIs) in disease diagnosis, but their nonuniform spatial distribution and higher-order structure lead to low binding affinity. Constructing nanotopologies that match the spatial distribution of membrane proteins to improve the binding affinity remains a challenge. Inspired by the multiantigen recognition of immune synapses, we developed modular DNA-origami-based nanoarrays with multivalent aptamers. By adjusting the valency and interspacing of the aptamers, we constructed specific nanotopology to match the spatial distribution of target protein clusters and avoid potential steric hindrance. We found that the nanoarrays significantly enhanced the binding affinity of target cells and synergistically recognized low-affinity antigen-specific cells. In addition, DNA nanoarrays used for the clinical detection of circulating tumor cells successfully verified their precise recognition ability and high-affinity RLIs. Such nanoarrays will further promote the potential application of DNA materials in clinical detection and even cell membrane engineering.

Highly pH-Responsive Sensor Based on a EuIII Metal-Organic Framework with Efficient Recognition of Arginine and Lysine in Living Cells.

A lanthanide-based three-dimensional metal-organic framework with excellent water, acid/base, and solvent stability, namely {[(CH3)2NH2]0.7[Eu2(BTDBA)1.5(lac)0.7(H2O)2]·2H2O·2DMF·2CH3CN}n (JXUST-29, H4BTDBA = 4',4‴-(benzo[c][1,2,5]thiadiazole-4,7-diyl)bis([1,1'-biphenyl]-3,5-dicarboxylic acid), Hlac = lactic acid), has been synthesized and characterized. Since the N atoms of the thiadiazole group will not coordinate with lanthanide ions, JXUST-29 has a free basic N-site accessible to small H+ ions, which allows it to be used as a promising pH fluorescence sensor. Interestingly, the luminescence signal was significantly enhanced, with an approximately 54-fold enhancement in the emission intensity when the pH value was increased from 2 to 5, which is the typical behavior of pH probes. In addition, JXUST-29 can also be used as a luminescence sensor to detect l-arginine (Arg) and l-lysine (Lys) in an aqueous solution through fluorescence enhancement and the blue-shift effect. The detection limits were 0.023 and 0.077 µM, respectively. In addition, JXUST-29-based devices were designed and developed to facilitate detection. Importantly, JXUST-29 is also capable of detecting and sensing Arg and Lys in living cells.

α7 Nicotinic acetylcholine receptor: a key receptor in the cholinergic anti-inflammatory pathway exerting an antidepressant effect.

Depression is a common mental illness, which is related to monoamine neurotransmitters and the dysfunction of the cholinergic, immune, glutamatergic, and neuroendocrine systems. The hypothesis of monoamine neurotransmitters is one of the commonly recognized pathogenic mechanisms of depression; however, the drugs designed based on this hypothesis have not achieved good clinical results. A recent study demonstrated that depression and inflammation were strongly correlated, and the activation of alpha7 nicotinic acetylcholine receptor (α7 nAChR)-mediated cholinergic anti-inflammatory pathway (CAP) in the cholinergic system exhibited good therapeutic effects against depression. Therefore, anti-inflammation might be a potential direction for the treatment of depression. Moreover, it is also necessary to further reveal the key role of inflammation and α7 nAChR in the pathogenesis of depression. This review focused on the correlations between inflammation and depression as well-discussed the crucial role of α7 nAChR in the CAP.

Molecular mechanisms of rapid-acting antidepressants: New perspectives for developing antidepressants.

Major depressive disorder (MDD) is a chronic relapsing psychiatric disorder. Conventional antidepressants usually require several weeks of continuous administration to exert clinically significant therapeutic effects, while about two-thirds of the patients are prone to relapse of symptoms or are completely ineffective in antidepressant treatment. The recent success of the N-methyl-D-aspartic acid (NMDA) receptor antagonist ketamine as a rapid-acting antidepressant has propelled extensive research on the action mechanism of antidepressants, especially in relation to its role in synaptic targets. Studies have revealed that the mechanism of antidepressant action of ketamine is not limited to antagonism of postsynaptic NMDA receptors or GABA interneurons. Ketamine produces powerful and rapid antidepressant effects by affecting α-amino-3-hydroxy-5-methyl-4-isoxazole-propionic acid receptors, adenosine A1 receptors, and the L-type calcium channels, among others in the synapse. More interestingly, the 5-HT2A receptor agonist psilocybin has demonstrated potential for rapid antidepressant effects in depressed mouse models and clinical studies. This article focuses on a review of new pharmacological target studies of emerging rapid-acting antidepressant drugs such as ketamine and hallucinogens (e.g., psilocybin) and briefly discusses the possible strategies for new targets of antidepressants, with a view to shed light on the direction of future antidepressant research.

Mechanochromic and Selective Vapochromic Solid-State Luminescence of a Dinuclear Cuprous Complex.

The unraveling of the stimuli-responsive mechanism is crucial to the design and precise synthesis of stimuli-responsive luminescent materials. We report herein the mechanochromic and selective vapochromic solid-state luminescence properties of a new bimetallic cuprous complex [{Cu(bpmtzH)}2(µ-dppm)2](ClO4)2 (1), and the corresponding response mechanisms are elucidated by investigating its two different solvated polymorphs 1·2CH2Cl2 (1-g) and 1·2CHCl3 (1-c). Green-emissive 1-g and cyan-emissive 1-c can be interconverted upon alternate exposure to CHCl3 and CH2Cl2 vapors, which is principally attributable to a combined alteration of both intermolecular NHbpmtzH···OClO3- hydrogen bonds and intramolecular "triazolyl/phenyl" π···π interactions induced by different solvents. Solid-state luminescence mechanochromism present in 1-g and 1-c is mainly ascribed to the grinding-induced breakage of the NHbpmtzH···OClO3- hydrogen bonds. It is suggested that intramolecular π···π-triazolyl/phenyl interactions are affected by different solvents but not by grinding. The results provide new insights into the design and precise synthesis of multi-stimuli-responsive luminescent materials by the comprehensive use of intermolecular hydrogen bonds and intramolecular π···π interactions.

Highly Stable Rare Earth Metal-Organic Frameworks for Fluorescence Recognition of Folic Acid, Proton Conduction, and Magnetic Refrigeration.

Four new isostructural rare earth metal-organic frameworks (RE-MOFs) were synthesized and full characterized, namely, {[(CH)2NH2]3[RE2(BTDBA)2(HCOO)]·5H2O·2DMF}n (H4BTDBA = (4',4'''-(benzo[c][1,2,5]thiadiazole-4,7-diyl)bis([1,1'-biphenyl]-3,5-dicarboxylic acid); RE = Eu (JXUST-34), Gd (JXUST-35), Tb (JXUST-36), and Dy (JXUST-37)). The single-crystal structures analysis shows that JXUST-34-37 are chain-based three-dimensional structures. Importantly, JXUST-34 exhibits excellent water, organic solvents, and acid-base stability, which can be used as a fluorescence sensor for folic acid and Al3+ with detection limits of 0.02 mM and 0.05 µM, respectively. The presence of free [(CH)2NH2]+ cations in the channels can engage the proton carrier during proton conduction. JXUST-34-37 display good proton conductivity, and the conductivities vary with relative humidity and temperatures, among which JXUST-37 has the highest conductivity of 9.66 × 10-3 S·cm-1 at 60 °C and 98% RH. The magnetic studies show that the -ΔSm of JXUST-35 reaches 16.13 J kg-1 K-1 at 2 K and ΔH = 7 T. JXUST-34-37 show multifunctional properties of fluorescence sensing, high proton conductivity, and magnetic refrigeration, which provides a new clue for the development of fluorescent-responsive, magnetic-refrigerant, and proton-conductive RE-MOF materials.

Enhanced Heterogeneous Catalytic Activity of Peroxymonosulfate for Rhodamine B Degradation via a CoII-Based Metal-Organic Framework.

A stable metal-organic framework with the formula {[Co(BBZB)(IPA)]·H2O}n (JXUST-23, BBZB = 4,7-bis(1H-benzimidazole-1-yl)-2,1,3-benzothiadiazole and H2IPA = isophthalic acid) was constructed by incorporating Co2+ ions and two conjugated ligands under solvothermal conditions. JXUST-23 takes a dinuclear cluster-based layer structure with a porosity of 2.7%. In this work, JXUST-23 was used to activate peroxymonosulfate (PMS) to degrade rhodamine B (RhB), a difficult-to-degrade pollutant in water. Compared with pure PMS or JXUST-23, the JXUST-23/PMS system displays the best degradation ability of RhB in neutral solution. When the mass ratio of JXUST-23 to PMS was 2:3, 99.72% of RhB (50 ppm) was removed within 60 min, and the reaction rate was 0.1 min-1. Furthermore, free radical quenching experiments show that SO4•- was the main free radical during the process of RhB degradation. In addition, JXUST-23 exhibits good reusability for the degradation of the organic dye RhB, making it a potential candidate for environmental remediation.

Controllable Synthesis of TbIII Metal-Organic Frameworks with Reversible Luminescence Sensing for Benzaldehyde Vapor.

Two novel lanthanide metal-organic frameworks (MOFs) with the formulas [Tb(bidc)(Hbidc)(H2O)]n (JXUST-20) and {[Tb3(bidc)4(HCOO)(DMF)]·solvents}n (JXUST-21) were synthesized based on 2,1,3-benzothiadiazole-4,7-dicarboxylic acid (H2BTDC) under solvothermal conditions. Interestingly, benzimidazole-4,7-dicarboxylic acid (H2bidc) was formed in situ using H2BTDC as the starting material. The self-assembly process of the targeted MOFs with different topological structures can be controlled by the solvents and concentration of the reactants. Luminescence experiments show that JXUST-20 and JXUST-21 exhibit strong yellow-green emission. JXUST-20 and JXUST-21 can selectively sense benzaldehyde (BzH) via a luminescence quenching effect with detection limits of 15.3 and 1.44 ppm, respectively. In order to expand the practical application of MOF materials, mixed-matrix membranes (MMMs) have been constructed by mixing targeted MOFs and poly(methyl methacrylate) in a N,N-dimethylformamide (DMF) solution, which can also be used for BzH vapor sensing. Therefore, the first case of MMMs derived from TbIII MOFs has been developed for the reversible detection of BzH vapor, providing a simple and efficient platform for the future detection of volatile organic compounds.

Characteristics variations of size-fractionated anammox granules and identification of the potential effects on these evolutions.

Anaerobic ammonium oxidation (anammox) granulation which contributed to system stabilization and performance improvement has great potential in the field of wastewater nitrogen removal. The researchers fractionated anammox granules into small-size (0.5-0.9 mm), medium-size (1.8-2.2 mm), and large-size (2.8-3.5 mm) categories to examine their properties and mechanisms. Various analyses, including high-throughput sequencing, determination of inorganic elements and extracellular polymeric substances (EPS), and microbial function prediction, were conducted to characterize these granules and understand their impact. The results revealed distinct characteristics among the different-sized granules. Medium-size granules exhibited the highest sphericity, EPS content, and anammox abundance. In contrast, large-size granules had the highest specific surface area, heme c content, specific anammox activity, biodiversity, and abundance of filamentous bacteria. Furthermore, the precipitates within the granules were identified as CaCO3 and MgCO3, with the highest inorganic element content found in the large-size granules. Microbial community and function annotation also varied with granule size. Based on systematic analysis, the researchers concluded that cell growth, chemical precipitation, EPS secretion, and interspecies interaction all played a role in granulation. Small-size granules were primarily formed through cell growth and biofilm formation. As granule size increased, EPS secretion and chemical precipitation became more influential in the granulation process. In the large-size granules, chemical precipitation and interspecies interaction, including synergistic effects with nitrifying, denitrifying, and filamentous bacteria, as well as metabolic cross-feeding, played significant roles in aggregation. This interplay ultimately contributed to higher anammox activity in the large-size granules. By fully understanding the mechanisms involved in granulation, this study provides valuable insights for the acclimation of anammox granules with optimal sizes under different operational conditions.

The application of extracellular vesicles in colorectal cancer metastasis and drug resistance: recent advances and trends.

Colorectal cancer (CRC) has high incidence and mortality rates and is one of the most common cancers of the digestive tract worldwide. Metastasis and drug resistance are the main causes of cancer treatment failure. Studies have recently suggested extracellular vesicles (EVs) as a novel mechanism for intercellular communication. They are vesicular particles, which are secreted and released into biological fluids, such as blood, urine, milk, etc., by a variety of cells and carry numerous biologically active molecules, including proteins, nucleic acids, lipids, metabolites, etc. EVs play a crucial part in the metastasis and drug resistance of CRC by delivering cargo to recipient cells and modulating their behavior. An in-depth exploration of EVs might facilitate a comprehensive understanding of the biological behavior of CRC metastasis and drug resistance, which might provide a basis for developing therapeutic strategies. Therefore, considering the specific biological properties of EVs, researchers have attempted to explore their potential as next-generation delivery systems. On the other hand, EVs have also been demonstrated as biomarkers for the prediction, diagnosis, and presumed prognosis of CRC. This review focuses on the role of EVs in regulating the metastasis and chemoresistance of CRC. Moreover, the clinical applications of EVs are also discussed.

Development and psychometric evaluation of a self-management behaviours scale in rheumatoid arthritis patients (RA-SMBS).

BACKGROUND: Self-management behaviours can be crucial to improving disease symptoms and health outcomes in rheumatoid arthritis (RA) patients. Currently, the tools available for measuring self-management behaviours in RA patients are either generalized for patients with chronic diseases, which lack specificity or have poor reliability in the only specific scale-self-care behaviours scale (SCBS). The aim of this study was to develop a self-management behaviours scale for RA patients and evaluate its psychometric properties. METHODS: The study included two steps: scale development and the psychometric evaluation. The items were developed from a literature review, in-depth individual interviews, nominal group technique, Delphi expert consultation, and a pilot test. For the psychometric evaluation, a sample of 561 patients with RA was recruited. Item analysis, content validity, exploratory and confirmatory factor analysis, convergent and discriminant validity, and internal consistency reliability were conducted to examine the psychometric properties of the RA-SMBS. RESULTS: The final scale consists of 23 items with 4 dimensions, including medication management, exercise and joint protection, resource utilization and emotional management, and symptom management. The content validity index was 0.78. Exploratory factor analysis explained 61.89% of the total item variance. Confirmatory factor analysis indicated that the RA-SMBS fit well. Good internal consistency reliability was demonstrated (Cronbach's alpha = 0.908), and the test-retest reliability was found to be acceptable (ICC = 0.628, r = 0.780). CONCLUSIONS: The scale has good content validity, construct validity, and internal consistency reliability. It can be used to assess the level of self-management behaviours in RA patients.

A pH-Stable TbIII-Based Metal-Organic Framework as a Turn-On and Blue-Shift Fluorescence Sensor toward Benzaldehyde and Salicylaldehyde in Aqueous Solution.

A new polydentate tetracarboxylic acid with a benzothiadiazole unit (4',4'''-(benzo[c][1,2,5]thiadiazole-4,7-diyl)bis([1,1'-biphenyl]-3,5-dicarboxylic acid), H4BTDBA) has been used to prepare a pH-stable three-dimensional TbIII-based metal-organic framework (MOF) with the formula {[(CH3)2NH2]0.7[Tb2(BTDBA)1.5(lac)0.7(H2O)2]·solvents}n (Hlac = lactic acid, JXUST-19). JXUST-19 exhibits a new (4,4,12)-connected topology based on tetranuclear [Tb4] clusters. JXUST-19 can remain stable when soaked in water for at least 1 week and in aqueous solutions with various pH values (2-12) for 24 h. Fluorescence study indicates JXUST-19 can be employed as a rare turn-on and blue-shift MOF sensor toward benzaldehyde (BZ) and salicylaldehyde (SA). To date, JXUST-19 represents the first TbIII-based turn-on MOF sensor toward salicylaldehyde in aqueous solution, and the fluorescence enhancement and naked-eye detection of BZ have been rarely reported. In addition, JXUST-19 based fluorescent test papers, light-emitting diode lamp beads, and portable composite films were developed to realize naked-eye detection of BZ and SA, which has great potential in practical applications.

Thermo-, Mechano-, and Vapochromic Dinuclear Cuprous-Emissive Complexes with a Switchable CH3CN-Cu Bond.

A thermo-, mechano-, and vapochromic bimetallic cuprous-emissive complex has been reported, and the origin and application of its tri-stimuli-responsive luminescence have been explored. As revealed by single-crystal structure analysis, thermo- and vapochromic luminescence adjusted by heating at 60 °C and CH3CN vapor fuming, accompanied by a crystalline-to-crystalline transition, is due to the breaking and rebuilding of the CH3CN-Cu bond, as supported by 1H nuclear magnetic resonance (NMR), Fourier-transform infrared (FT-IR) spectroscopy, powder X-ray diffraction (PXRD), thermogravimetry (TG), and time-dependent density functional theory (TD-DFT) analyses of the CH3CN-coordinated species [Cu2(µ-dppa)2(µ-η1(N)η2(N,N)-fptz)(CH3CN)](ClO4)·H2O (1) and its CH3CN-removed derivative [Cu2(µ-dppa)2(µ-η1(N)η2(N,N)-fptz)](ClO4)·H2O (2). Luminescence mechanochromism, mixed with a crystalline-to-amorphous transition where the initial crystalline is different for 1 and 2, is mainly assigned as the destruction of the CH3CN-Cu bonding and/or the O···HNdppa and OH···Ntriazolyl hydrogen bonds. It is also suggested that a rational use of switchable coordination such as weak metal-solvent bonding is a feasible approach to develop multi-stimuli-responsive luminescent materials and devices.

A Mechanochromic and Vapochromic Luminescent Cuprous Complex Based on a Switchable Intramolecular π···π Interaction.

An in-depth study on a stimuli-responsive tetranuclear cuprous luminescent complex is reported and gives new insights into the origin and possible use of the observed stimuli-responsive luminescence. Its crystalline polymorphs with two different shapes are obtained by using different crystallization solvents and show distinct emissions, with one being blue emissive and the other being yellow emissive. Upon grinding, only the blue-emitting polymorph has a marked change in the emission color from blue to yellow, and its ground sample exhibits a yellow emission similar to that of the yellow-emitting polymorph. Interestingly, the yellow-emitting polymorph after exposure to acetone vapor can emit a blue emission and display luminescence mechanochromism similar to that of the blue-emitting polymorph. Single-crystal structural analyses of the two different polymorphs reveal the relationship between the mechanochromic luminescence and the geometrical configuration of the {Cu(µ-dppm)2Cu} unit and intramolecular "pyridyl/phenyl" π···π interactions, supported as well by their PXRD, FT-IR, TGA, and PL studies in various states and by TD-DFT analyses. The results demonstrate the different roles of switchable intramolecular π···π interactions and the geometrical configuration of the {Cu(µ-dppm)2Cu} unit in this stimuli-responsive luminescence and potential applications of such stimuli-responsive luminescence in optical sensing and anticounterfeiting encryption technologies and deepen the understanding of such stimuli-responsive luminescence originating from switchable intramolecular π···π interactions. In addition, it is clearly suggested that the rational utilization of switchable intramolecular π···π interactions is a feasible route for developing stimuli-responsive intelligent luminescent materials and devices.

A Benzothiadiazole-Based Eu3+ Metal-Organic Framework as the Turn-On Luminescent Sensor toward Al3+ and Ga3+ with Potential Bioimaging Application.

The design and preparation of novel multifunctional lanthanide metal-organic frameworks (Ln-MOFs) have been arisen widespread attention. In particular, Ln-MOFs have shown great luminescence potential in chemical sensing. Herein, a new benzothiadiazole-based Eu-MOF {[(CH3)2NH2][Eu(BTDB)2]·2H2O}n (JXUST-11) was obtained based on 4,4'-(benzo[c][1,2,5]thiadiazole-4,7-diyl)dibenzoic acid (H2BTDB), which exhibits a chain-based three-dimensional framework. Moreover, JXUST-11 is considered as a photoluminescent sensor to identify Al3+ and Ga3+ ions by fluorescence enhancement with the detection limits of 2.9 and 10.2 ppm, severally. Importantly, Al3+ and Ga3+ can be discerned with the naked eye by color change under a natural lamp. In addition, a portable MOF film based on JXUST-11 was developed for Al3+ and Ga3+ detection. This is the first Ln-MOF that can be employed as a naked-eye fluorescent probe to identify Ga3+. Interestingly, JXUST-11 is also capable of detecting Al3+ and Ga3+ in living cells.

Fabrication and DFT Calculation of Amine-Functionalized Metal-Organic Framework as a Turn-On Fluorescence Sensor for Fe3+ and Al3+ Ions.

Due to their important role in biological systems, it is urgent to develop a material that can rapidly and sensitively detect the concentration of Fe3+ and Al3+ ions. In this work, a brand-new CdII-based metal-organic framework [Cd(BTBD)2(AIC)]n (JXUST-18, BTBD = 4,7-bis(1H-1,2,4-triazol-1-yl)-2,1,3-benzothiadiazole and H2AIC = 5-aminoisophthalic acid) with a 4-connected sql topology was designed and synthesized. The symmetrical CdII centers are linked by AIC2- ligands with µ3-η1:η1:η1:η1 coordination mode to form a [Cd2(COO)2] secondary building unit (SBU). The contiguous SBUs are further connected by BTBD ligands to form a two-dimensional (2D) layer structure. JXUST-18 can remain stable in aqueous solutions with pH values of 3-12 or in boiling water. Luminescent experiments suggest that JXUST-18 displays more than eightfold fluorescence enhancement in the presence of Fe3+ and Al3+ ions, and the detection limits for Fe3+ and Al3+ ions are 0.196 and 0.184 µM, respectively. Furthermore, the change in luminescence color is uncomplicatedly distinguishable with the naked eye under ultraviolet light at 365 nm. In addition, a series of devices based on JXUST-18 including fluorescence test strips, lamp beads, and composite films were developed to detect metal ions via visual changes in luminescence color. Significantly, JXUST-18 is a rare MOF-based turn-on fluorescence sensor for the detection of Fe3+ ions. The theoretical calculation suggests that the complexation of Fe3+/Al3+ ions and the -NH2 group contributes to fluorescence enhancement.

Clinical and genetic analysis of a case with centronuclear myopathy caused by SPEG gene mutation: a case report and literature review.

BACKGROUND: Centronuclear myopathy (CNM), a subtype of congenital myopathy (CM), is a group of clinical and genetically heterogeneous muscle disorders. Since the discovery of the SPEG gene and disease-causing variants, only a few additional patients have been reported. CASE PRESENTATION: The child, a 13-year-old female, had delayed motor development since childhood, weakness of both lower extremities for 10 years, gait swinging, and a positive Gower sign. Her distal muscle strength of both lower extremities was grade IV. The electromyography showed myogenic damage and electromyographic changes. Her 11-year-old sister had a similar muscle weakness phenotype. Gene sequencing revealed that both sisters had SPEG compound heterozygous mutations, and the mutation sites were c.3715 + 4C > T and c.3588delC, which were derived from their parents. These variant sites have not been reported before. The muscle biopsy showed the nucleic (> 20% of fibers) were located in the center of the cell, the average diameter of type I myofibers was slightly smaller than that of type II myofibers, and the pathology of type I myofibers was dominant, which agreed with the pathological changes of centronuclear myopathy. CONCLUSIONS: The clinical phenotypes of CNM patients caused by mutations at different sites of the SPEG gene are also different. In this case, there was no cardiomyopathy. This study expanded the number of CNM cases and the mutation spectrum of the SPEG gene to provide references for prenatal diagnosis and genetic counseling.

Turn-On Luminescent Sensor toward Fe3+, Cr3+, and Al3+ Based on a Co(II) Metal-Organic Framework with Open Functional Sites.

A novel Co-based metal-organic framework (MOF) with the formula of {[Co3(BIBT)3(BTC)2(H2O)2]·solvents}n (JXUST-2, where JXUST denotes Jiangxi University of Science and Technology, BIBT = 4,7-bi(1H-imidazol-1-yl)benzo-[2,1,3]thiadiazole, and H3BTC = 1,3,5-benzenetricarboxylic acid) has been solvothermally prepared, which takes 3D structure with a rare 3,4,6-c topology and contains intramolecular hydrogen bonds. Interestingly, the sensing investigations suggest that JXUST-2 could be considered as a multifunctional fluorescence sensor toward Fe3+, Cr3+, and Al3+ via a turn-on effect with good reusability and detection limits of 0.13, 0.10, and 0.10 µM, respectively. The turn-on effect of JXUST-2 could be ascribed to an absorbance caused enhancement (ACE) mechanism. Notably, JXUST-2 is the first turn-on MOF fluorescent sensor for Fe3+, Cr3+, and Al3+ simultaneously.