Two novel nitrogen-rich metal-organic nanotubes: syntheses, structures and selective adsorption toward rare earth ions.

The construction and development of metal-organic nanotubes (MONTs) with nanoscale interior channel diameters for potential applications is of great interest. An angular nitrogen-rich ligand, 3,6-bis(2-ethylimidazole)-2-methylpyrimidine (beim-CH3), was designed to construct MONTs by coupling with the V-shaped carboxylate ligands of benzophenone 4,4'-dicarboxylic acid (H2bpndc) and 4,4'-oxybisbenzoic acid (H2obba). Two new MONTs were synthesized and named NCD-166 ([Zn(bpndc)(beim-CH3)]·H2O) and NCD-167 ([Zn(obba)(beim-CH3)]·H2O), and they were isostructural and have almost identical tube inner diameters of approximately 1.76 nm. Benefiting from the abundantly exposed nitrogen and oxygen atoms in their tube walls and open nanoporous channels, they display superior adsorption capacities for Eu3+ (150.90 mg g-1) and high adsorption selectivity (>96%) in the low-concentration solutions. Additionally, it was revealed that the adsorption effect of ether oxygen on rare earth elements was significantly better than that of carbonyl oxygen. The adsorption isotherm conformed to the Langmuir model and the adsorption kinetics obeyed the pseudo-second-order model. These results clearly indicate that such novel MONTs are favorable sorbents for REEs.

Constructing an efficient electrocatalyst for water oxidation: an Fe-doped CoO/Co catalyst enabled by in situ MOF growth and a solvent-free strategy.

The development of non-precious metal electrocatalysts with high activity for the oxygen evolution reaction (OER) is a crucial and challenging task. In this work, we proposed a solvent-free in situ metal-organic framework (MOF) growth strategy for the fabrication of an Fe-doped CoO/Co electrocatalyst. This approach not only partially granted the MOF's porous structure to the catalyst but also resulted in a tighter combination between the Co metal and CoO, thereby enhancing its electrical conductivity. Furthermore, this method enabled the Fe species to be more uniformly dispersed on CoO/Co, which significantly exposed more active sites for efficient electrocatalysis. The entire synthesis process was solvent-free, except for a small amount of water and ethanol used during catalyst washing. The as-synthesized Fe-CoO/Co electrocatalyst exhibited superior OER activity on a glass carbon electrode, with η = 276 mV at a current density of 10 mA cm-2, even higher than that of the commercial precious IrO2/C catalyst. Additionally, it was also extended to prepare a Ni-doped CoO/Co electrocatalyst by the same procedure with satisfactory OER performance. This work presents a new preparation approach for MOF-derived catalysts with potential applications in energy conversion and beyond.

New findings on choroidal features in healthy people by ultra-widefield swept-source optical coherence tomography angiography.

To evaluate the distribution of choroidal thickness (CT) and its trend with age in healthy people using 120° ultra-wide field swept-source optical coherence tomography angiography (UWF SS-OCTA). In this cross-sectional observational study, healthy volunteers underwent single imaging of the fundus with UWF SS-OCTA at a field of view (FOV) of 120° (24 mm × 20 mm) centered on the macula. The characteristics of CT distribution in different regions and its changes with age were analyzed. A total of 128 volunteers with a mean age of 34.9 ± 20.1 years and 210 eyes were enrolled in the study. The thickest mean choroid thickness (MCT) was located at the macular region and supratemporal region, followed by the nasal side of the optic disc, and thinnest below the optic disc. The maximum MCT was: 213.40 ± 36.65 µm for the group aged 20-29, and the minimum MCT was: 162.11 ± 31.96 µm for the group aged ≥ 60. After the age of 50, MCT was significantly and negatively correlated decreased with age (r = - 0.358, p = 0.002), and the MCT in the macular region decreased more remarkably compared to other regions. The 120° UWF SS-OCTA can observe the distribution of choroidal thickness in the range of 24 mm × 20 mm and its variation with age. It was revealed that MCT decreased more rapidly in the macular region relative to other regions after 50 years old.

Early changes to retinal structure in patients with diabetic retinopathy as determined by ultrawide swept-source optical coherence tomography-angiography.

Purpose: To investigate retinal vascular changes in patients with diabetic retinopathy (DR) using the newly developed ultrawide rapid scanning swept-source optical coherence tomography angiography (SS-OCTA) device. Methods: This cross-sectional, observational study enrolled 24 patients (47 eyes) with DR, 45 patients (87 eyes) with diabetes mellitus (DM) without DR, and 36 control subjects (71 eyes). All subjects underwent 24 × 20 mm SS-OCTA examination. Vascular density (VD) and the thickness of the central macula (CM; 1 mm diameter) and temporal fan-shaped areas of 1-3 mm (T3), 3-6 mm (T6), 6-11 mm (T11), 11-16 mm (T16), and 16-21 mm (T21) were compared among groups. The VD and the thicknesses of the superficial vascular complex (SVC) and deep vascular complex (DVC) were analyzed separately. The predictive values of VD and thickness changes in DM and DR patients were evaluated by receiver operating characteristic (ROC) curve analysis. Results: The average VDs of the SVC in the CM and the T3, T6, T11, T16, and T21 areas were significantly lower in the DR than in the control group, whereas only the average VD of the SVC in the T21 area was significantly lower in the DM group. The average VD of the DVC in the CM was significantly increased in the DR group, whereas the average VDs of the DVC in the CM and T21 area were significantly decreased in the DM group. Evaluation of the DR group showed significant increases in the thicknesses of SVC-nourishing segments in the CM and T3, T6, and T11 areas and significant increases in the thicknesses of DVC-nourishing segments in the CM and T3 and T6 areas. In contrast, none of these parameters showed significant changes in the DM group. ROC curve analysis showed that the average VD of the SVC in the CM, T3, and T21 had better ability to predict DR, with areas under the ROC curves (AUCs) of 0.8608, 0.8505, and 0.8353, respectively. The average VD of the DVC in the CM was also predictive of DR, with an AUC of 0.8407. Conclusions: The newly developed ultrawide SS-OCTA device was better able to reveal early peripheral retinal vascular changes than traditional devices.

Ultra-widefield color fundus photography combined with high-speed ultra-widefield swept-source optical coherence tomography angiography for non-invasive detection of lesions in diabetic retinopathy.

Purpose: To compare the detection rate of diabetic retinopathy (DR) lesions and the agreement of DR severity grading using the ultra-widefield color fundus photography (UWF CFP) combined with high-speed ultra-widefield swept-source optical coherence tomography angiography (UWF SS-OCTA) or fluorescein angiography (FFA). Methods: This prospective, observational study recruited diabetic patients who had already taken the FFA examination from November 2021 to June 2022. These patients had either no DR or any stage of DR. All participants were imaged with a 200° UWF CFP and UWF SS-OCTA using a 24 × 20 mm scan model. Images were independently evaluated for the presence or absence of DR lesions including microaneurysms (MAs), intraretinal hemorrhage (IRH), non-perfusion areas (NPAs), intraretinal microvascular abnormalities (IRMAs), venous beading (VB), neovascularization elsewhere (NVE), neovascularization of the optic disc (NVD), and vitreous or preretinal hemorrhage (VH/PRH). Agreement of DR severity grading based on UWF CFP plus UWF SS-OCTA and UWF CFP plus FFA was compared. All statistical analyses were performed using SPSS V.26.0. Results: One hundred and fifty-three eyes of 86 participants were enrolled in the study. The combination of UWF CFP with UWF SS-OCTA showed a similar detection rate compared with UWF CFP plus FFA for all the characteristic DR lesions (p>0.05), except NPAs (p = 0.039). Good agreement was shown for the identification of VB (κ = 0.635), and very good agreement for rest of the DR lesions between the two combination methods (κ-value ranged from 0.858 to 0.974). When comparing the grading of DR severity, very good agreement was achieved between UWF CFP plus UWF SS-OCTA and UWF CFP plusr FFA (κ = 0.869). Conclusion: UWF CFP plus UWF SS-OCTA had a very good agreement in detecting DR lesions and determining the severity of DR compared with UWF CFP plus FFA. This modality has the potential to be used as a fast, reliable, and non-invasive method for DR screening and monitoring in the future.

Green and Large-Scale Preparation of Chiral Metal-Organic Frameworks via Mechanochemistry.

It is a significant challenge to construct chiral metal-organic frameworks (CMOFs) by developing a facile and green preparation strategy. In this work, CMOFs were first synthesized via a mechanochemical process by combining a truncated mixed ligand strategy and defect engineering theory. The simple, green, and rapid construction strategy could solvent-freely harvest gram-scale CMOFs with a hierarchical micro/mesoporous structure. The as-synthesized CMOFs were evaluated by Aldol asymmetric catalysis and exhibited excellent catalytic performance (conversion was up to 97.1%, the ee value was 44.3%, and the activity was still good after 5 cycles).

Hyperactivated Wnt-ß-catenin signaling in the absence of sFRP1 and sFRP5 disrupts trophoblast differentiation through repression of Ascl2.

BACKGROUND: Wnt signaling is a critical determinant for the maintenance and differentiation of stem/progenitor cells, including trophoblast stem cells during placental development. Hyperactivation of Wnt signaling has been shown to be associated with human trophoblast diseases. However, little is known about the impact and underlying mechanisms of excessive Wnt signaling during placental trophoblast development. RESULTS: In the present work, we observed that two inhibitors of Wnt signaling, secreted frizzled-related proteins 1 and 5 (Sfrp1 and Sfrp5), are highly expressed in the extraembryonic trophoblast suggesting possible roles in early placental development. Sfrp1 and Sfrp5 double knockout mice exhibited disturbed trophoblast differentiation in the placental ectoplacental cone (EPC), which contains the precursors of trophoblast giant cells (TGCs) and spongiotrophoblast cells. In addition, we employed mouse models expressing a truncated ß-catenin with exon 3 deletion globally and trophoblast-specifically, as well as trophoblast stem cell lines, and unraveled that hyperactivation of canonical Wnt pathway exhausted the trophoblast precursor cells in the EPC, resulting in the overabundance of giant cells at the expense of spongiotrophoblast cells. Further examination uncovered that hyperactivation of canonical Wnt pathway disturbed trophoblast differentiation in the EPC via repressing Ascl2 expression. CONCLUSIONS: Our investigations provide new insights that the homeostasis of canonical Wnt-ß-catenin signaling is essential for EPC trophoblast differentiation during placental development, which is of high clinical relevance, since aberrant Wnt signaling is often associated with trophoblast-related diseases.

Retrospective Comparison of 27-Gauge and 25-Gauge Microincision Vitrectomy Surgery with Silicone Oil for the Treatment of Primary Rhegmatogenous Retinal Detachment.

AIM: To retrospectively compare the safety and effectiveness of 27-gauge (27G) microincision vitrectomy surgery (MIVS) with 25-guage (25G) MIVS for the treatment of primary rhegmatogenous retinal detachment (RRD) with silicone oil tamponade. METHODS: Ninety-two patients with RRD who underwent MIVS from May 1, 2015, to June 30, 2017, were included in this study. Fifty-eight eyes underwent 25G MIVS and 34 eyes underwent 27G MIVS. We analyzed the characteristics of the patients, surgical time, main clinical outcomes, and rate of complications. RESULTS: The mean surgical time was 56.7 ± 35.9 min for the 25G MIVS and 55.7 ± 36.1 min for the 27G MIVS, and there was no significant difference (P=0.894) between the two groups. The primary anatomical success rate after a single operation was 94.8% for 25G MIVS and 91.2% for 27G MIVS (P=0.666). Baseline and final visit best-corrected visual acuity (BCVA) were 1.9 ± 1.1 and 1.0 ± 0.8 in the 25G group, and 1.7 ± 1.0 and 1.1 ± 0.8 in the 27G group. Last visit BCVA increased significantly in both groups (P < 0.001). However, there were no significant differences in terms of visual improvement ratio (>0.2 logMAR) between the two groups (P=0.173). No severe intraoperative complication was observed. Iatrogenic retinal breaks occurred in 2 eyes (3.4%) in the 25G group and 1 eye (2.9%) in the 27G group during the peripheral vitreous base shaving. The transient ocular hypertension (>25 mmHg) within postoperative week 1 was 25.9% in the 25G group and 11.8% in the 27G group (P=0.120). CONCLUSIONS: This study found no significant anatomical or functional difference between 27G and 25G MIVS in the treatment of primary RRD. Therefore, 27G vitrectomy appears to be a safe and effective surgery for the treatment of primary RRD.

Outcomes of transconjunctival sutureless 27-gauge vitrectomy for vitreoretinal diseases.

AIM: To evaluate the safety and efficacy profile of 27-gauge (27G) pars plana vitrectomy (PPV) for the treatment of various vitreoretinal diseases. METHODS: The clinical outcomes of 61 eyes (58 patients) with various vitreoretinal diseases following 27G PPV were retrospectively reviewed. RESULTS: Surgical indications included rhegmatogenous retinal detachment (n=24), full-thickness macular hole (n=12), diabetic retinopathy (n=11), vitreous hemorrhage (n=6), Eales disease (n=4), pathological myopia-related vitreous floater (n=2), and macular epiretinal membrane (n=2). The mean follow-up was 166.4±61.3d (range 98-339d). The mean logMAR best-corrected visual acuity (BCVA) improved from 1.7±1.1 [0.02 decimal visual acuity (VA) equivalent] preoperatively to 1.2±1.0 (0.06 decimal VA equivalent) at the last postoperative visit (P<0.001). The mean operative time was 49.9min. With the exception of complicated cataract in one eye, no intraoperative complications were encountered. No case required conversion to conventional 20-, 23- or 25G instrumentation in all surgical maneuvers except for silicone oil infusion, which required a 25G oil injection syringe. Postoperative complications included transient ocular hypertension, vitreous hemorrhage, persistent intraocular pressure elevation, subconjunctival oil leakage, and recurrent retinal detachment. No cases of hypotony, endophthalmitis, and sclerotomy-related tears were observed. CONCLUSION: The current results suggest that 27G PPV system is a safe and effective treatment for various vitreoretinal diseases. When learning to perform 27G PPV, surgeons may encounter a learning curve and should gradually expand surgical indications from easy to pathologically complicated cases.

Whole-exome sequencing identifies a novel homozygous frameshift mutation in the PROM1 gene as a causative mutation in two patients with sporadic retinitis pigmentosa.

Retinitis pigmentosa (RP) refers to a heterogeneous group of inherited retinal diseases caused by the loss of photoreceptors. The present study aimed to identify the gene mutations responsible for RP in two patients diagnosed with sporadic RP using next-generation sequencing technology. For this purpose, two patients with sporadic RP and family members (namely parents and siblings) were recruited into this study and underwent a complete ophthalmological assessment. Whole-exome sequencing (WES) was performed on genomic DNA samples isolated from peripheral leukocytes which had been obtained from the two patients diagnosed with sporadic RP. WES data were annotated and filtered against four public databases and one in-house database. Subsequently, Sanger sequencing was performed in order to determine whether any of the candidate variants co-segregated with the disease phenotype in the families. A homozygous frameshift mutation, c.1445dupT (p.F482fs) in exon 12 of the PROM1 gene (MIM: 604365), satisfied a recessive inheritance model and showed complete co-segregation of the mutation with the disease phenotype in the families. The same mutation was not detected in the 200 ethnically-matched control samples by Sanger sequencing. The novel homozygous mutation c.1445dupT (p.F482fs) in the PROM1 gene was identified as a causative mutation for RP. Thus, the identification of this mutation has further expanded the existing spectrum of PROM1 mutations in patients with RP, thereby assisting in the molecular diagnosis of RP and enhancing our understanding of genotype-phenotype correlations in order to provide effective genetic counseling.

A novel transgenic mouse model of Chinese Charcot-Marie-Tooth disease type 2L.

We previously found that the K141N mutation in heat shock protein B8 (HSPB8) was responsible for Charcot-Marie-Tooth disease type 2L in a large Chinese family. The objective of the present study was to generate a transgenic mouse model bearing the K141N mutation in the human HSPB8 gene, and to determine whether this (K141N)HSPB8 transgenic mouse model would manifest the clinical phenotype of Charcot-Marie-Tooth disease type 2L, and consequently be suitable for use in studies of disease pathogenesis. Transgenic mice overexpressing (K141N)HSPB8 were generated using K141N mutant HSPB8 cDNA cloned into a pCAGGS plasmid driven by a human cytomegalovirus expression system. PCR and western blot analysis confirmed integration of the (K141N)HSPB8 gene and widespread expression in tissues of the transgenic mice. The (K141N)HSPB8 transgenic mice exhibited decreased muscle strength in the hind limbs and impaired motor coordination, but no obvious sensory disturbance at 6 months of age by behavioral assessment. Electrophysiological analysis showed that the compound motor action potential amplitude in the sciatic nerve was significantly decreased, but motor nerve conduction velocity remained normal at 6 months of age. Pathological analysis of the sciatic nerve showed reduced myelinated fiber density, notable axonal edema and vacuolar degeneration in (K141N)HSPB8 transgenic mice, suggesting axonal involvement in the peripheral nerve damage in these animals. These findings indicate that the (K141N)HSPB8 transgenic mouse successfully models Charcot-Marie-Tooth disease type 2L and can be used to study the pathogenesis of the disease.

[Cellular expression of (R127W)HSPB1 and its co-localization with neurofilament light chain].

OBJECTIVE: To observe the cellular expression of (R127W) HSPB1 and its influence on neurofilament light chain (NFL) self-assembly and co-localization with NFL. METHODS: Eukaryotic expression vectors pEGFPN1-(wt) HSPB1 and pEGFPN1- (R127W) HSPB1 were constructed. Hela cells were transiently transfected with pEGFPN1-(wt) HSPB1 or pEGFPN1- (R127W) HSPB1 and observed under a confocal microscope. Hela cells were also transiently co-transfected with Pcl-NFL and pEGFPN1-(wt)HSPB1, or pCL-NFL and pEGFPN1-(R127W)HSPB1. The self-assembly of NFL was observed and the co-localization study of HSPB1/ (R127W)HSPB1 with NFL was carried out in these two cell models by immunofluorescence technique. RESULTS: The aggregates formed by EGFP-(R127W)HSPB1 predominantly located around the nucleus, and EGFP-(wt)HSPB1 showed diffusion pattern in Hela cells. When co expressed with EGFP-(wt)HSPB1, NFL formed homogeneous structure in cytosol. When co-expressed with EGFP-(R127W)HSPB1, however, NFL had amorphous staining pattern predominantly consisting of NFL aggregates, and NFL co-localized with (R127W)HSPB1 in these aggregates. CONCLUSION: The R127W mutant of HSPB1 may have reduced capacity to serve as a chaperone to prevent aggregate formation, and fail to correctly organize the neurofilament network. Dysfunction of the axon cytoskeleton and axon transport may be the primary mechanism of R127W mutation of HSPB1 in the pathogenesis of Charcot-Marie-Tooth disease.

[Mutation analysis of LITAF, RAB7, LMNA and MTMR2 genes in Chinese Charcot-Marie-Tooth disease.].

The purpose of this study was to understand the mutation features of lipopolysaccharide-induced tumor necrosis factor-alpha factor (LITAF), ras-associated protein RAB7 (RAB7), lamin A/C (LMNA) and myotubularin-related protein 2 (MTMR2) genes in Chinese Charcot-Marie-Tooth disease (CMT) patients. Mutation analysis of LITAF gene was carried out using PCR combined with DNA sequencing, and mutation analysis of RAB7 gene by PCR-single strand conformation polymorphism (PCR-SSCP) combined with DNA sequencing in 33 CMT patients including 6 probands of autosomal domi-nated CMT families and 27 sporadic patients; mutation analysis of LMNA and MTMR2 genes was observed using PCR-SSCP combined with DNA sequencing in 41 CMT patients, including 14 probands of autosomal recessive CMT fami-lies and 27 sporadic patients. Two sequence variations c.269G-->A and c.274A-->G were detected in LITAF gene and two sequence variations c.1243G-->A and c.1910C-->T were detected in LMNA gene. No sequence variation was found in RAB7 and MTMR2 gene. Variations of c.269G-->A in LITAF gene and c.1243G-->A, c.1910C-->T in LMNA gene are newly found SNPs in this study. Variation of c.274A-->G in LITAF gene is known SNP reported in SNP database. Mutations in LITAF, RAB7, LMNA, and MTMR2 genes are rare in Chinese CMT patients.