CCL22 Induces the Polarization of Immature Dendritic Cells into Tolerogenic Dendritic Cells in Radiation-Induced Lung Injury through the CCR4-Dectin2-PLC-γ2-NFATC2-Nr4a2-PD-L1 Signaling Pathway.

Recruitment of immune cells to the injury site plays a pivotal role in the pathology of radiation-associated diseases. In this study, we investigated the impact of the chemokine CCL22 released from alveolar type II epithelial (AT2) cells after irradiation on the recruitment and functional changes of dendritic cells (DCs) in the development of radiation-induced lung injury (RILI). By examining changes in CCL22 protein levels in lung tissue of C57BL/6N mice with RILI, we discovered that ionizing radiation increased CCL22 expression in irradiated alveolar AT2 cells, as did MLE-12 cells after irradiation. A transwell migration assay revealed that CCL22 promoted the migration of CCR4-positive DCs to the injury site, which explained the migration of pulmonary CCR4-positive DCs in RILI mice in vivo. Coculture experiments demonstrated that, consistent with the response of regulatory T cells in the lung tissue of RILI mice, exogenous CCL22-induced DCs promoted regulatory T cell proliferation. Mechanistically, we demonstrated that Dectin2 and Nr4a2 are key targets in the CCL22 signaling pathway, which was confirmed in pulmonary DCs of RILI mice. As a result, CCL22 upregulated the expression of PD-L1, IL-6, and IL-10 in DCs. Consequently, we identified a mechanism in which CCL22 induced DC tolerance through the CCR4-Dectin2-PLC-γ2-NFATC2-Nr4a2-PD-L1 pathway. Collectively, these findings demonstrated that ionizing radiation stimulates the expression of CCL22 in AT2 cells to recruit DCs to the injury site and further polarizes them into a tolerant subgroup of CCL22 DCs to regulate lung immunity, ultimately providing potential therapeutic targets for DC-mediated RILI.

Single-Cell Analysis in Blood Reveals Distinct Immune Cell Profiles in Gouty Arthritis.

Gout is a chronic disease caused by monosodium urate crystal deposition. Previous studies have focused on the resident macrophage, infiltrating monocyte, and neutrophil responses to monosodium urate crystal, yet the mechanisms of the potential involvement of other immune cells remain largely unknown. In this study, we enrolled seven gout patients and five age-matched healthy individuals and applied single-cell mass cytometry to study the distribution of immune cell subsets in peripheral blood. To our knowledge, our study reveals the immune cell profiles of gout at different stages for the first time. We identified many immune cell subsets that are dysregulated in gout and promote gouty inflammation, especially those highly expressing CCR4 and OX40 (TNFR superfamily member 4), including CCR4+OX40+ monocytes, CCR4+OX40+CD56high NK cells, CCR4+OX40+CD4+ NK T cells, and CCR4+CD38+CD4+ naïve T cells. Notably, the plasma levels of CCL17 and CCL22, measured by ELISA, increased in the acute phase of gout and declined in the interval. We also found a clue that Th2-type immune responses may participate in gout pathology. Moreover, the subset of granzyme B+ (GZMB+) CD38+ NK cells is positively correlated with serum urea acid level, and another two γδT subsets, GZMB+CD161+ γδT cells and GZMB+CCR5+ γδT cells, are negatively correlated with erythrocyte sedimentation rate. In sum, gouty arthritis is not a disease simply mediated by macrophages; multiple types of immune cell may be involved in the pathogenesis of the disease. Future research needs to shift attention to other immune cell subsets, such as NK cells and T cells, which will facilitate the identification of novel therapeutic targets.

AIB3 IIC2 IIIQ6 VIXVII: A Thioborate Halide Family for Developing Wide Bandgap Infrared Nonlinear Materials by Coupling Planar [BS3] and Polycations.

Developing high-performance infrared (IR) nonlinear optical (NLO) materials is urgent but challenging due to the competition between NLO coefficient and bandgap in one compound. Herein, by coupling NLO-active [BS3] planar units and halide-centered polycations, six new metal thioborate halides ABa3B2S6X (A = Rb, Cs; X = Cl, Br, I) composed of zero-dimensional [XBamRbn/Csn] polycations and [BS3] units, belonging to a new A I B 3 II C 2 III Q 6 VI X VII ${\mathrm{A}}^{\mathrm{I}}{\mathrm{B}}_{3}^{\mathrm{II}}{\mathrm{C}}_{2}^{\mathrm{III}}{\mathrm{Q}}_{6}^{\mathrm{VI}}{\mathrm{X}}^{\mathrm{VII}}$ family, are rationally designed and fabricated. The compounds show an interesting structural transition from Pbcn (ABa3B2S6Cl) to Cmc21 (ABa3B2S6Br and ABa3B2S6I) driven by the clamping effect of polycationic frameworks. ABa3B2S6Br and ABa3B2S6I are the first series metal thioborate halide IR NLO materials, and the introduction of [BS3] unit effectively widens the bandgap of planar unit-constructed chalcogenides. ABa3B2S6Br and ABa3B2S6I, exhibiting wide bandgaps (3.55-3.60 eV), high laser-induced damage thresholds (≈ 6 × AgGaS2), and strong SHG effects (0.5-0.6 × AgGaS2) with phase-matching behaviors, are the promising IR NLO candidates for high-power laser applications. The results enrich the chemical and structural diversity of boron chemistry and give some insights into the design of new IR NLO materials with planar units.

Two Hydroxyfluorooxoborates Achieving Deep-Ultraviolet Cutoff Edges and Moderate Birefringence by Assembling Multi-Anionic Groups.

Assembling multi-anionic groups is conducive to utilizing respective advantage to achieve the enhancement of optical performance. Two new hydroxyfluorooxoborates, Ama2-Rb2B3O3F4(OH) and K8Cs2B15O14(OH)7F20 ⋅ H2O with [B3O3F4(OH)] six-membered rings were synthesized for the first time. The title compounds exhibit short ultraviolet cutoff edges (<200 nm) and K8Cs2B15O14(OH)7F20 ⋅ H2O possesses a moderate experimental refractive index difference of 0.051@546 nm.

Cd8(BO3)4SiO4: Metal Cation Inducing the Formation of Isolated [BO3] and [SiO4] Units in Borate Silicate.

The first compound of cadmium-borate silicate Cd8(BO3)4SiO4, crystallizing in space group P42/n (no. 86), has been successfully synthesized by the conventional high-temperature solution method and melts congruently. The zero-dimensional anionic groups of Cd8(BO3)4SiO4 are isolated [BO3] triangles and isolated [SiO4] tetrahedra which are filled in the framework formed by [CdO6] polyhedra. It has a moderate birefringence (Δn = 0.053 at 546 nm), which is measured by experiment and evaluated by first-principles calculations; meanwhile, the source of birefringence is revealed through the response electronic distribution anisotropy method. The UV-vis-NIR diffuse reflectance spectrum indicates that Cd8(BO3)4SiO4 possesses a wide optical transparency range, with a UV cutoff edge at about 254 nm. This work enriches the structure chemistry of borate silicates, and we discussed the possible methods for the exploration and synthesis of novel optical crystals possessing zero-dimensional anionic groups in the borate silicate system.

(C(NH2)3)2(I2O5F)(IO3)(H2O) and C(NH2)3IO2F2: Two Guanidine Fluorooxoiodates with Wide Band Gap and Large Birefringence.

Enhancing anisotropy through an effective synergistic arrangement of anionic and cationic groups is crucial for improving the birefringence optical properties of materials. In this work, by transforming I-O into I-F through the fluorination strategy, two metal-free guanidine fluorooxoiodates (C(NH2)3)2(I2O5F)(IO3)(H2O) and C(NH2)3IO2F2 and one guanidine iodate C(NH2)3IO3 were successfully synthesized using the hydrothermal method. An unprecedented dimer [I2O5F] formed by [IO3F] and [IO3] in (C(NH2)3)2(I2O5F)(IO3)(H2O) was found, which greatly enriches the structural diversity of fluorooxoiodates. All three compounds feature a relatively large birefringence (Δn = 0.068, 0.110 and 0.075 at 546 nm) and a short ultraviolet cutoff edge. The theoretical calculation was carried out to understand the electronic structures and linear optical properties.

AYSO4F2 (A = K, Rb): [YO4F4] Polyhedra Enhancement of Birefringence in Non-π-Conjugated Sulfate Systems.

A mild hydrothermal method was employed to successfully synthesize two new sulfate fluorides, namely, AYSO4F2 (A = K, Rb). They are isomorphic, and both contain [YO4F4] polyhedra and [SO4] tetrahedra in the structure. Theoretical calculations and experimental tests show that AYSO4F2 (A = K, Rb) have large band gaps (7.79 and 7.82 eV) and moderate birefringence (0.015 and 0.02 @ 546.1 nm), with significantly enhanced birefringence and band gaps as compared to that of the single alkali metal sulfates A2SO4 (A = K, Rb). Furthermore, theoretical calculations show that [YO4F4] polyhedra are the main reason for the band gap and birefringence enhancement. This work contributes to the advancement of structural chemistry in the field of rare-earth sulfates, offering a novel approach for the design of sulfates characterized by large birefringence.

GGC repeat expansion in NOTCH2NLC induces dysfunction in ribosome biogenesis and translation.

GGC repeat expansion in the 5' untranslated region (UTR) of NOTCH2NLC is associated with a broad spectrum of neurological disorders, especially neuronal intranuclear inclusion disease (NIID). Studies have found that GGC repeat expansion in NOTCH2NLC induces the formation of polyglycine (polyG)-containing protein, which is involved in the formation of neuronal intranuclear inclusions. However, the mechanism of neurotoxicity induced by NOTCH2NLC GGC repeats is unclear. Here, we used NIID patient-specific induced pluripotent stem cell (iPSC)-derived 3D cerebral organoids (3DCOs) and cellular models to investigate the pathophysiological mechanisms of NOTCH2NLC GGC repeat expansion. IPSC-derived 3DCOs and cellular models showed the deposition of polyG-containing intranuclear inclusions. The NOTCH2NLC GGC repeats could induce the upregulation of autophagic flux, enhance integrated stress response and activate EIF2α phosphorylation. Bulk RNA sequencing for iPSC-derived neurons and single-cell RNA sequencing (scRNA-seq) for iPSC-derived 3DCOs revealed that NOTCH2NLC GGC repeats may be associated with dysfunctions in ribosome biogenesis and translation. Moreover, NOTCH2NLC GGC repeats could induce the NPM1 nucleoplasm translocation, increase nucleolar stress, impair ribosome biogenesis and induce ribosomal RNA sequestration, suggesting dysfunction of membraneless organelles in the NIID cellular model. Dysfunctions in ribosome biogenesis and phosphorylated EIF2α and the resulting increase in the formation of G3BP1-positive stress granules may together lead to whole-cell translational inhibition, which may eventually cause cell death. Interestingly, scRNA-seq revealed that NOTCH2NLC GGC repeats may be associated with a significantly decreased proportion of immature neurons while 3DCOs were developing. Together, our results underscore the value of patient-specific iPSC-derived 3DCOs in investigating the mechanisms of polyG diseases, especially those caused by repeats in human-specific genes.

Folic acid-mediated hollow Mn 3 O 4 nanocomposites for in vivo MRI/FLI monitoring the metastasis of gastric cancer.

BACKGROUND: Metastasis is one of the main factors leading to the high mortality rate of gastric cancer. The current monitoring methods are not able to accurately monitor gastric cancer metastasis. METHODS: In this paper, we constructed a new type of hollow Mn 3 O 4 nanocomposites, Mn 3 O 4 @HMSN-Cy7.5-FA, which had a size distribution of approximately 100 nm and showed good stability in different liquid environments. The in vitro magnetic resonance imaging (MRI) results show that the nanocomposite has good response effects to the acidic microenvironment of tumors. The acidic environment can significantly enhance the contrast of T 1 -weighted MRI. The cellular uptake and endocytosis results show that the nanocomposite has good targeting capabilities and exhibits good biosafety, both in vivo and in vitro. In a gastric cancer nude mouse orthotopic metastatic tumor model, with bioluminescence imaging's tumor location information, we realized in vivo MRI/fluorescence imaging (FLI) guided precise monitoring of the gastric cancer orthotopic and metastatic tumors with this nanocomposite. RESULTS: This report demonstrates that Mn 3 O 4 @HMSN-Cy7.5-FA nanocomposites is a promising nano-diagnostic platform for the precision diagnosis and therapy of gastric cancer metastasis in the future. CONCLUSIONS: In vivo MRI/FLI imaging results show that the nanocomposites can achieve accurate monitoring of gastric cancer tumors in situ and metastases. BLI's tumor location information further supports the good accuracy of MRI/FLI dual-modality imaging. The above results show that the MHCF NPs can serve as a good nano-diagnostic platform for precise in vivo monitoring of tumor metastasis. This nanocomposite provides more possibilities for the diagnosis and therapy of gastric cancer metastases.

High-temperature PTT/CDT coordination nanoplatform realizing exacerbated hypoxia for enhancing hypoxia-activated chemotherapy to overcome tumor drug resistance.

BACKGROUND: Hypoxia-activated prodrugs present new opportunities for safe and effective tumor drug resistance therapy due to their high selectivity for hypoxic cells. However, the uneven distribution of oxygen in solid tumor and insufficient hypoxia in the tumor microenvironment greatly limit its therapeutic efficacy. RESULTS: In this paper, a novel AQ4N-Mn(II)@PDA coordination nanoplatform was designed and functionalized with GMBP1 to target drug-resistant tumor cells. Its excellent photothermal conversion efficiency could achieve local high-temperature photothermal therapy in tumors, which could not only effectively exacerbate tumor hypoxia and thus improve the efficacy of hypoxia-activated chemotherapy of AQ4N but also significantly accelerate Mn2+-mediated Fenton-like activity to enhance chemodynamic therapy. Moreover, real-time monitoring of blood oxygen saturation through photoacoustic imaging could reflect the hypoxic status of tumors during treatment. Furthermore, synergistic treatment effectively inhibited tumor growth and improved the survival rate of mice bearing orthotopic drug-resistant tumors. CONCLUSIONS: This study not only provided a new idea for PTT combined with hypoxia-activated chemotherapy and CDT for drug-resistant tumors but also explored a vital theory for real-time monitoring of hypoxia during treatment.

The impact of comorbid premenstrual syndrome or premenstrual dysphoric disorder on the clinical characteristics of bipolar disorder among Han Chinese women.

Bipolar disorder (BD) is commonly comorbid with premenstrual syndrome (PMS) or premenstrual dysphoric disorder (PMDD). However, little is known about their relationship. This study aimed to assess the impact of comorbid PMS or PMDD on the clinical characteristics of BD. A cross-sectional study was conducted on 262 women with BD. PMS and PMDD were screened with the Premenstrual Symptoms Screening Tool (PSST). Symptomatic features were assessed with Hamilton Depression Scale (HAMD), Young Mania Rating Scale (YMRS), and atypical features by the depressive episode section of SCID-I/P. The rates of PMS and PMDD among BD were 57.6% and 20.6% according to PSST. No significant difference in the rates of PMS and PMDD was found between BD I, BD II, and BD-NOS. Compared to BD patients without PMS or PMDD, patients with comorbid BD and PMS or PMDD were younger, more educated, had a higher risk of OCD, had an earlier age of onset, scored higher on HAMD-17 and its sub-scale of anxiety/somatization, cognitive deficit, psychomotor retardation, and were more likely to have increased appetite and leaden paralysis. In addition, patients with comorbid BD and PMDD were less likely to experience traumatic life events, more likely to have family history of mental disorders and have inflammatory or autoimmune disease, scored higher on HMAD-17, particularly in its sub-scale of anxiety/somatization, cognitive deficit, psychomotor retardation, and sleep disturbance. Compared with BD without PMS or PMDD, BD with PMS or PMDD might be a specific subtype of BD characterized with earlier onset age, heavier genetic load, increased symptom severity, and atypical features.

Potential mechanism of Qinggong Shoutao pill alleviating age-associated memory decline based on integration strategy.

CONTEXT: Qinggong Shoutao Wan (QGSTW) is a pill used as a traditional medicine to treat age-associated memory decline (AAMI). However, its potential mechanisms are unclear. OBJECTIVE: This study elucidates the possible mechanisms of QGSTW in treating AAMI. MATERIALS AND METHODS: Network pharmacology and molecular docking approaches were utilized to identify the potential pathway by which QGSTW alleviates AAMI. C57BL/6J mice were divided randomly into control, model, and QGSTW groups. A mouse model of AAMI was established by d-galactose, and the pathways that QGSTW acts on to ameliorate AAMI were determined by ELISA, immunofluorescence staining and Western blotting after treatment with d-gal (100 mg/kg) and QGSTW (20 mL/kg) for 12 weeks. RESULTS: Network pharmacology demonstrated that the targets of the active components were significantly enriched in the cAMP signaling pathway. AKT1, FOS, GRIN2B, and GRIN1 were the core target proteins. QGSTW treatment increased the discrimination index from -16.92 ± 7.06 to 23.88 ± 15.94% in the novel location test and from -19.54 ± 5.71 to 17.55 ± 6.73% in the novel object recognition test. ELISA showed that QGSTW could increase the levels of cAMP. Western blot analysis revealed that QGSTW could upregulate the expression of PKA, CREB, c-Fos, GluN1, GluA1, CaMKII-α, and SYN. Immunostaining revealed that the expression of SYN was decreased in the CA1 and DG. DISCUSSION AND CONCLUSIONS: This study not only provides new insights into the mechanism of QGSTW in the treatment of AAMI but also provides important information and new research ideas for the discovery of traditional Chinese medicine compounds that can treat AAMI.

Fluorination Strategy Towards Symmetry Breaking of Boron-centered Tetrahedron for Poly-fluorinated Optical Crystals.

Borate crystals can be chemically and functionally modified by the fluorination strategy, which encourages the identification of emerging fluorooxoborates with a structure and set of characteristics not seen in any other oxide parents. However, the bulk of fluorooxoborates have been found accidentally, rational methods of synthesis are required, particularly for the infrequently occurring poly-fluorinated components. Herein, we reported the use of bifluoride salts as a potent source of fluorine to prepare fluorooxoborates that contain rarely tri-fluorinated [BF3 X] (X=O and CH3 ) tetrahedra and eleven compounds were found. We identified the optical properties of the organofluorinated group [CH3 BF3 ] and their potential for nonlinear optics for the first time. Among these, two non-centrosymmetric components hold potential for the production of 266 nm harmonic coherent light for nonlinear optics, and more crucially, have the benefit of growing large size single crystals. Our study establishes experimental conditions for the coexistence of the diverse functional groups, enabling the production of poly-fluorinated optical crystals.

Breaking the Inherent Interarrangement of [B3O6] Clusters for Nonlinear Optics with Orbital Hybridization Enhancement.

The [B3O6] group as a prime functional unit provides borates with intrinsic properties that are modified by coordination to cations. Inherent [B3O6] cluster structures in borates exclusively made of them have a near-plane configuration, with more than 90% of them having a maximum dihedral angle of zero and the remaining ones being less than 13°. Although such an inherent configuration can produce considerable birefringence for good phase-matching ability, this is not conducive to obtaining high conversion efficiency and beam quality due to the walk-off effects in the nonlinear optical process. In this article, two new borate halides Ca2B3O6X (X = Cl and Br) were reported, in which the confinement effects of distorted halogen-centered secondary building blocks compress the existence space of [B3O6] primitives, resulting in the nonparallel arrangement between [B3O6] clusters in this series. Both compounds show large second harmonic generation effects, and more importantly, the broken inherent interarrangement of [B3O6] clusters makes them a moderate birefringence and small walk-off angle. Their moderate birefringence is due to the large angular alignment between [B3O6] clusters, resulting from the orbital hybridization between the Ca s and the O p orbitals of the terminal O atoms on [B3O6] clusters. Our model supports this viewpoint and offers guidelines for rearranging [B3O6] clusters' arrangements in borates.

NaRb6(B4O5(OH)4)3(BO2) Featuring Noncentrosymmetry, Chirality, and the Linear Anionic Group BO2.

Noncentrosymmetric (NCS) structures are of particular interest owing to their symmetry-dependent physical properties, e.g., pyroelectricity, ferroelectricity, piezoelectricity, and nonlinear optical (NLO) behavior. Among them, chiral materials exhibit polarization rotation and host topological properties. Borates often contribute to NCS and chiral structures via their triangular [BO3] and tetrahedral [BO4] units and their numerous superstructure motifs. However, no chiral compound with the linear [BO2] unit has been reported to date. Herein, an NCS and chiral mixed-alkali-metal borate, NaRb6(B4O5(OH)4)3(BO2), with a linear BO2- unit in the structure was synthesized and characterized. The structure features a combination of three types of basic building units (BBUs), [BO2], [BO3], and [BO4] with sp-, sp2-, and sp3-hybridization of boron atoms, respectively. It crystallizes in the trigonal space group R32 (No. 155), one of the 65 Sohncke space groups. Two enantiomers of NaRb6(B4O5(OH)4)3(BO2) were found, and their crystallographic relationships are discussed. These results not only expand the small family of NCS structures with the rare linear BO2- unit but also prompt recognition to the fact that NLO materials have generally overlooked the existence of two enantiomers in achiral Sohncke space groups.

Inokosterone activates the BMP2 to promote the osteogenic differentiation of bone marrow mesenchymal stem cells and improve bone loss in ovariectomized rats.

Evidence suggests that enhancing the osteogenic ability of bone marrow-derived mesenchymal stem cells (BMSCs) may be beneficial in the fight against osteoporosis (OP) effects. Inokosterone (IS) is a major active constituent of Achyranthis bidentatae radix (ABR), which stimulates osteogenic differentiation of mouse embryonic osteoblasts. This study aims to investigate effect of IS on OP using osteogenic differentiated BMSCs and ovariectomy (OVX)-induced OP rats. The BMSCs were treated with 50, 100, or 200 mg/L IS and OP rats were given 2 or 4 mg/kg of IS by gavage. Cell viability, the osteogenic differentiation marker protein expression level, and mineralization were observed. This study proved that IS improved cell viability, osteogenic differentiation, and cellular mineralization in BMSCs and raised expression levels of bone morphogenetic protein-2 (BMP2), Smad1, runt-related transcription factor 2 (RUNX2), collagen I, ALP, and OCN. By BMP2 knockdown/overexpression, this study also proved the BMP2 signaling pathway activation is a potential biological mechanism of IS to improve osteogenic differentiation and mineralization in osteogenic differentiated BMSCs. In OVX-induced OP rats, IS was observed to antagonize bone loss, improve osteogenic differentiation marker protein expression levels, and activate BMP-2, smad1, and RUNX2. These findings provide scientific support for further investigation of the biological mechanisms of IS in ameliorating OP.

Golgi damage caused by dysfunction of PiT-2 in primary familial brain calcification.

The Golgi apparatus is vital for protein modification and molecular trafficking. It is essential for nerve development and activity, and damage thereof is implicated in many neurological diseases. Primary familial brain calcification (PFBC) is a rare inherited neurodegenerative disease characterized by multiple brain calcifications. SLC20A2, which encodes the inorganic phosphate transporter 2 (PiT-2) protein, is the main pathogenic gene in PFBC. The PiT-2 protein is a sodium-dependent phosphate type III transporter, and dysfunction leads to a deficit in the cellular intake of inorganic phosphate (Pi) and calcium deposits. Whether the impaired Golgi apparatus is involved in the PFBC procession requires elucidation. In this study, we constructed induced pluripotent stem cells (iPSCs) derived from two PFBC patients with different SLC20A2 gene mutations (c.613G > A or del exon10) and two healthy volunteers as dependable cell models for research on pathogenic mechanism. To study the mechanism, we differentiated iPSCs into neurons and astrocytes in vitro. Our study found disruptive Golgi structure and damaged autophagy in PFBC neurons with increased activity of mTOR. We also found damaged mitochondria and increased apoptosis in the PFBC dopaminergic neurons and astrocytes. In this study, we prove that dysfunctional PiT-2 leads to an imbalance of cellular Pi, which may disrupt the Golgi apparatus with impaired autophagy, mitochondria and apoptosis in PFBC. Our study provides a new avenue for understanding nerve damage and pathogenic mechanism in brain calcifications.

Zn2 HgP2 S8 : A Wide Bandgap Hg-Based Infrared Nonlinear Optical Material with Large Second-Harmonic Generation Response.

Hg-based chalcogenides, as good candidates for the exploration of high-performance infrared (IR) nonlinear optical (NLO) materials, usually exhibit strong NLO effects, but narrow bandgaps. Herein, an unprecedented wide bandgap Hg-based IR NLO material Zn2 HgP2 S8 (ZHPS) with diamond-like structure is rationally designed and fabricated by a tetrahedron re-organization strategy with the aid of structure and property predictions. ZHPS exhibits a wide bandgap of 3.37 eV, which is the largest one among the reported Hg-based chalcogenide IR NLO materials and first breaks the 3.0 eV bandgap "wall" in this system, resulting in a high laser-induced damage threshold (LIDT) of ≈2.2 × AgGaS2 (AGS). Meanwhile, it shows a large NLO response (1.1 × AGS), achieving a good balance between bandgap (≥3.0 eV) and NLO effect (≥1 × AGS) for an excellent IR NLO material. DFT calculations uncover that, compared to normal [HgS4 ]n , highly distorted [HgS4 ]d tetrahedral units are conducive to generating wide bandgap, and the wide bandgap in ZHPS can be attributed to the strong s-p hybridization between Hg─S bonding in distorted [HgS4 ]d , which gives some insights into the design of Hg-based chalcogenides with excellent properties based on distorted [HgS4 ]d tetrahedra.

GIPC1 CGG Repeat Expansion Is Associated with Movement Disorders.

OBJECTIVE: CGG/GGC repeat expansion in FMR1 and NOTCH2NLC is reportedly associated with movement disorders; therefore, we hypothesized that the CGG repeat expansion in LRP12, NUTM2B-AS1, and GIPC1, which was previously identified in myopathy, might also be associated with movement-disorder phenotypes. Here, we investigated whether CGG repeat expansion in LRP12, NUTM2B-AS1, and GIPC1 presents in a cohort of patients with movement disorders. METHODS: We screened for the CGG repeat expansion in LRP12, NUTM2B-AS1, and GIPC1 in 1,346 movement-disorder patients and 1,451 matched healthy controls. RESULTS: No patients or controls harbored expanded CGG repeats in LRP12 or NUTM2B-AS1, whereas 16 patients harbored >40 CGG repeats in GIPC1, with 11 of these patients harboring >60 CGG repeats. One control individual harbored an expanded GIPC1 allele (83 CGG units), suggesting that approximately 1% of patients affected by movement disorders in our population might harbor GIPC1 CGG repeat expansion, with this likely extremely rare in healthy controls (<0.001). The clinical phenotypes of the GIPC1 CGG repeat-positive patients strongly resembled those in patients displaying NOTCH2NLC GGC repeat-positive movement disorders. Additionally, the GIPC1 CGG repeat-positive patients presented white-matter hyperintensities but without typical NOTCH2NLC-related high-intensity signals in the corticomedullary junction. Furthermore, 44% of the GIPC1 CGG repeat-positive patients showed a cognitive deficit, and skin biopsies in 2 patients revealed deposition of intranuclear inclusions. INTERPRETATION: The CGG repeat expansion in GIPC1 might be associated with movement-disorder phenotypes and lead to diseases related to intranuclear inclusions. ANN NEUROL 2022;91:704-715.

Finding a Deep-UV Borate BaZnB4 O8 with Edge-sharing [BO4 ] Tetrahedra and Strong Optical Anisotropy.

The polarization modulation of deep-UV light is an important process that incorporates functionality to selectively respond to light-mater interaction. Typically, optical anisotropy is foremost to the use efficiency of deep-UV birefringent crystals. Herein, a new congruently melting polyborate with extremely large birefringence (Δn(001) =0.14@589.3 nm) and band gap (6.89 eV) is discovered as a high performance birefringent crystal, which breaks the current deadlock of deep-UV polyborates that usually show small birefringence. The rigid tetrahedra, including [ZnO4 ] and edge-sharing [BO4 ] tetrahedra, make all the planar [BO3 ] triangles in the lattice adopt preferential arrangement and thereby lead to an extraordinary large birefringence that is larger than all the deep-UV borates with experimentally measured values. Structural analyses with the additional theoretical calculations were used to study the origin of strong optical anisotropy in BaZnB4 O8 .