Low-dimensional organic-inorganic hybrid materials (OIHs) with charming luminescence behavior and excellent solution-processability have attracted much attention in the field of solid-state luminescent (SSL) materials, but were rarely reported. Herein, a novel 1D hybrid lead(II) bromide, [(C8N4H26)(Pb2Br8)center dot(H2O)2]n (1) was synthesized. On account of the large molecular size of organic ammonium, the 1D lead bromide double-chains in 1 was fully isolated to form the strong structural quantum confinement. Besides, the highly distortion of [PbBr6]4octahedral units could further enhance the electron-phonon coupling in this system. These two effects endow 1 with intrinsic dual centered emission property (weak FE at 390 nm and strong STE at 557 nm). As temperature variation from 300 to 80 K, owing to the dramatical enhancement of STE emission center (69.20-fold), 1 exhibits a fully reversible emission color tunability from yellow to orange, suggesting the potential of 1 in the field of luminescent thermometer. Besides, benefiting from the excellent solution-processability, 1 and BAM (BaMgAl10O7:Eu2+) can be dissolved in UV-curable resin to form a white-light-emitting device with ideal CIE coordinates (0.316, 0.338), moderate correlated color temperature (6268 K), high color-rendering index (87) and high luminance (9323 cd center dot m- 2). These results demonstrate the potential of the stable and solution processable low dimensional OIHs for application in solid-state luminescent materials.

Recently, supramolecular isomers obtained by single-crystal-to-single-crystal (SCSC) transformations have emerged as a significant research focus but are rarely reported. To address this, a lon topological complex, [H2DABCO][FeII2(C2O4)(HCOO)2Cl2]·H2O (1), was synthesized with a cation dynamics nature, in which the [H2DABCO]2+ underwent an order-disorder transition on heating at about 210 K, accompanied by significant dielectric anomalies. Upon further heating above 413 K, 1 undergoes an intriguing SCSC transformation to the anhydrous phase [H2DABCO][FeII2(C2O4)(HCOO)2Cl2] (2), retaining the original coordination geometry of the metal ions and ligands but revealing the zeolite crb net. This supramolecular network isomerization is extremely rare during the single-crystal-to-single-crystal transformation. Besides, the isomerization of the topological network influences the structural phase transitions and magnetic properties of 1 and 2. In 2, the organic ammonium ions remain disordered below 100 K without further structural phase transitions. Magnetic studies further reveal that 1 is an antiferromagnet with an ordering temperature of 25 K and exhibits canting at 15 K, while in 2, no significant spin canting was observed.

Scintillators with the capacity to convert X-rays into visible photons have attracted considerable attention from industrial inspection and medical diagnostics. Organic-inorganic hybrid (OIH) materials with programmable structures and tunable luminescence have emerged as ideal platforms for fabricating novel scintillators. However, it remains challenging to simultaneously achieve an exceptional light yield (LY) and high stability. Herein, by incorporating a suitable amount of Mn2+ ions into a 2D Dion-Jacobson (DJ)-phase OIH [(H2dapo)(PbCl4)]n (H2dapo2+ = 1,3-diaminium-2-hydroxypropane) (1), a novel Pb-Mn heterometallic OIH-based scintillator [(H2dapo)(PbCl4)]n:1.4%Mn (2) is synthesized. The synergistic effect between multihydrogen bond regulation and Mn2+ ions doping endows 2 with excellent environmental/thermal stability and bright orange-red emission at 605 nm with a high photoluminescence quantum yield (PLQY) of 46.85%, originating from the 4T1 → 6A1 transition of Mn2+ centers. Remarkably, 2 exhibits high radiation resistance and outstanding radioluminescence performance with a high light yield of 44347 photons/MeV and a linear response to X-ray dose rate with low detection limits (2.69 μGy·s-1). Moreover, a large-area flexible scintillation film is fabricated by combining 2 with poly(dimethylsiloxane) (PDMS), achieving a high spatial resolution of 11 lp·mm-1. These findings provide an efficient strategy for designing novel OIH-based scintillators with high stability and exceptional RL performance.

By employing oxalate to modify the connectivity of formate perovskites, novel perovskite-like cai topological 3D antiferromagnets [A]2[FeIIIFe2II(HCOO)7(C2O4)] were synthesized. The reduction of π-conjugated systems in organic ammonium increases their structural freedom, adjusting the phase transition of these complexes.

The regulation of ligand conformation through crystal transformation processes under external stimuli to control the photoluminescence behavior of complexes demonstrates potential applications in the fields of fluorescent displays and encryption. In this work, water immersion-induced irreversible phase transitions and hydration/ dehydration-triggered reversible phase transitions have been discovered in four tetrakis(4-carboxyphenyl) ethylene (TCPE) based complexes [Cd2(TCPE)center dot(DMF)center dot(H2O)3]n(1), [Cd2(TCPE)center dot(H2O)4]n(2), [Cd2(TCPE)center dot (H2O)2]n(3) and [Cd2(TCPE)]n(4). As 1 was immersed in water, the complex undergoes an irreversible framework dissolution-recrystallization to form complex 2, accompanied by a fluorescence color change from blue to green. After that, complex 2 and 3/4 can be reversibly interconverted through heating-induced dehydration and water absorption, accompanied by a fluorescence color switch between blue and green. Through structural comparison before and after the crystal transformation, it is found that the conformational changes of the ligands lead to the alteration in the fluorescence color of the complexes. The stimuli-responsive luminescence tuning behavior arising from the structural transformations of complexes enables this material to serve as a promising candidate for developing advanced fluorescence anti-counterfeiting technologies.

A simple one-step pyrolysis of the ZnO@KDS precursor constructs a novel N,S-doped defective carbon catalyst with superior ORR activity ( E 1/2 = 0.903 V) and excellent performance in flexible Zn–air batteries.

As a new generation of crystalline materials, coordination polymers (CPs) with modular structures have been widely investigated as precatalysts for the oxygen evolution reaction (OER). However, controlling the structural transformation of CPs to form metal oxyhydroxide phases with high catalytic activity remains a challenge. Herein, two similar anionic metal oxalate chains, {[(DEAPA][Co(C2O4)2]}n (Co-DEAPA) (DEAPA = N,N-diethylpropane-1,3-diaminium) and {[CyNH3]2[Co(C2O4)2]·H2O}n (Co-CyNH3) (CyNH3 = cyclopentylaminium), with different organic ammonium cations, are synthesized. By a simple dissolution-recrystallization method, these two complexes can be easily loaded onto the surface of nickel foam (NF) to form electrodes. The NF-supported Co-CyNH3 electrode exhibited enhanced OER activity with a lower overpotential of 290 mV compared to the Co-DEAPA counterpart (343 mV) at a current density of 100 mA cm-2. On this basis, by doping Fe into the Co-CyNH3 system, a novel NF-supported FeCo-CyNH3 electrode was constructed and exhibited significantly enhanced OER performance, with the overpotential reduced to 255 mV at 100 mA cm-2. The CoOOH and FeOOH generated in situ from the CP precursors during the OER electrochemical process serve as active sites for the enhanced OER catalytic activity. This methodology establishes a straightforward approach for constructing high-performance, nonprecious-metal-based electrocatalytic systems.

Abnormal trinucleotide CAG repeat expansions in exon 1 of the Androgen Receptor (AR) gene has been identified as the cause of Kennedy disease (KD). We generated and characterized a human induced pluripotent stem cell (iPSC) line from peripheral blood mononuclear cells (PBMC) of a patient with genetically confirmed KD. The pluripotency of these iPSCs was verified by the expression of several pluripotency markers at both RNA and protein levels, as well as their capability to differentiate into all three germ layers.

The in situ reactions in solvothermal processes have attracted explosive attention owing to their unique potential for fabricating organic ligands which were hard to achieve by the conventional organic synthesis method and for revealing the generation mechanisms directly by capturing the key intermediate through crystallization. In this work, using formic acid and 4-pyridinemethaneamine (L 0 ) as raw materials, the linear ligand 2,5-di(pyridin-4-yl)pyrazine (L) has been generalized in situ in assembling the 2D coordination polymers [Fe2(C2O4)2L2]n(0.5L)n(0.5H2O)n (1) and [Zn(C2O4)(L)1.5]n (2). After adjusting the reaction time, the intermediate N-(pyridin-4-ylmethyl)formamide (L 1 ) was captured by crystallization of the chain complexes [Fe(L1)2(C2O4)]n (3) and [Zn(L1)2(C2O4)]n (4). The possible in situ reaction mechanism has been revealed to be the cascade reaction between the amidation, nucleophilic addition-elimination reaction, and the oxidative dehydrogenation reaction through the crystal data, mass spectrometry, and 1H NMR. Magnetic studies of 1 illustrate that the spin-canted iron oxalate chains are coupled antiferromagnetically, and the antiferromagnetic interactions between the chain could be overcome by the external magnetic field and leads to metamagnetism. 2 exhibits orange fluorescence emission owing to the formation of a spatial conjugation system in the network. This work proposes an insightful synthetic route to prepare functional coordination polymers (CPs) through an in situ reaction.

Abnormal trinucleotide CAG repeat expansions in exon 10 of the ataxin-3 (ATXN3) gene has been identified as the cause of Spinocerebellar Ataxia Type 3 (SCA3). We generated and characterized a human induced pluripotent stem cell (iPSC) line from skin fibroblasts of a patient with genetically confirmed SCA3. The pluripotency of these iPSCs was verified by the expression of several undifferentiated hPSCs markers at both RNA and protein levels, as well as their capability to differentiate into all three germ layers.

We used a non-integrated reprogramming approach to establish a human induced pluripotent stem cell (hiPSC) line (INNDSUi011-A) from the skin fibroblasts of a 38-year-old female individual with autosomal dominant spastic paraplegia 9A due to ALDH18A1 mutation. The cells obtained demonstrate key characteristics of embryonic stem cells, including the expression of specific pluripotency markers and the capacity to differentiate into the three germ layers in vitro. This iPSC cell line retains the patient’s genetic information, making it a valuable model for studying disease mechanisms and developing novel therapies.

The construction of chiral organic-inorganic hybrid materials (COIHs) based on achiral building blocks remains a huge challenge. Herein, a novel COIH [HNEt3]2n[Ag5I7]n (1) induced by achiral ammonium (HNEt3 = (CH3CH2)3NH) was synthesized, which exhibited intriguing second harmonic generation (SHG) activity and an obvious piezoelectric coefficient d33 of 5.2 pC N-1 along the polar axis.

Three isotopological (stb) mixed-valence iron-formate frameworks, [A][FeIII(FeII)2(HCOO)9] (1-3), were constructed and exhibit ferroelastic phase transition with spontaneous strain of 0.16. 1-3 are spin-canted antiferromagnets with distinct TN from 26 K to 36 K and display exchange bias (EB) performance induced by the pinning of the spins at distinct sublattices.

To satisfy the rising requirement for luminescence anticounterfeiting technology, the development of luminescent materials with multiple modes of emission and adjustable luminescence properties is essential. On account of the great structural programmability of luminescent metal-organic frameworks (LMOFs), the multiple emitting components can be integrated into the matrices of LMOFs and form the multicentered synergistic emission system; thus, LMOFs can be an ideal platform for fabricating luminescent anticounterfeiting materials. Herein, based on the pore space partition (PSP) strategy, a novel pacs-MOF Mg-TPA-bdc with the formula of {(NH2Me2)[Mg3(μ3-OH)(bdc)3(TPA)]·12H2O}n (TPA = Tri(pyridin-4-yl)amine; bdc = terephthalic acid) was constructed as a host matrix to encapsulate the acriflavine during the self-assembly process to construct a new dual-centered emission material Mg-TPA-bdc⊇AF, which exhibited blue and green emission under 365 and 460 nm excitation, respectively. Intriguingly, upon external pressure or temperature variation, both of these two materials exhibit reversible stimuli-responsive luminescence-tuning behavior, which can be observed by the naked eye. Considering the charming dual-centered emission features of Mg-TPA-bdc⊇AF, a novel binary-coded microarray data storage matrix with high security reliability, real-time response, and great reversibility was constructed and applied for information encryption and luminescence anticounterfeiting.

Carbon-supported atomic clusters (CACs) have been demonstrated to have significant potential for heterogeneous catalysis; however, their synthesis remains a "black box" process due to the complexities associated with metal migration, aggregation, and carbon structural reconstruction behaviors. Herein, the general formation mechanism of metal clusters is uncovered in defective graphene oxide (DGO) during pyrolysis. First, multiple coordination modes of platinum (Pt) atoms on DGO provide opportunities for their temperature-dependent release, where the highly thermally stable sites (Pt & horbar;C2 on edge defect) serve as migration termini and nucleation centers. Notably, the size (from 1.5 to 0.99 nm) of PtCACs can be precisely manipulated by increasing the defect densities of DGO. Moreover, the dynamic evolution process is monitored using multiple in situ technologies, revealing a more intense structural reconstitution of DGO compared to that of graphene oxide, which, in turn, limits the aggregation of metal clusters. Electrocatalytic results revealed a negative relationship between the size of PtCACs and their hydrogen evolution performance, where the PtCACs (size: 0.99 nm) exhibit a high mass activity in HER that is 32 times higher than that of the commercial 20% Pt/C catalyst. This work provides a paradigm for establishing the underlying architecture of CAC synthesis methodology.

Exchange bias (EB) materials, whose magnetization curve can shift along the field axis after field cooling, have attracted tremendous attention and play a crucial role in the development of fundamental physics as well as practical applications of magnetization storage. In this work, the N-, P-, and Q-type ferrimagnets of Néel’s notation were realized in mixed valence metal formates [CH3NH2CH3]n[CrIII1−xFeIIIxFeII(HCO2)6]n by altering x, respectively. The positive and negative EB was found in N- and P-type ferrimagnets. The exchange anisotropy originates from the antiferromagnetic exchange interaction between the uncompensated spin of the host ferrimagnetic lattice and the pinned compensated spin of the antiferromagnetic clusters as a guest, which is rooted in the valence disorder of the iron ions.

ABSTRACTBackgroundProgressive external ophthalmoplegia (PEO) is a classic manifestation of mitochondrial disease. However, the link between its genetic characteristics and clinical presentations remains poorly investigated.MethodsWe analysed the clinical, pathological and genetic characteristics of a large cohort of patients with PEO, based on the type of their mtDNA variations. Eighty‐two PEO patients were enrolled and grouped into three categories: mtDNA single large‐scale deletions (SLDs), multiple deletions (MulDs) and the m.3243A > G point variant. Patients in the SLD category were further divided into ‘common deletion’ and ‘noncommon deletion’ groups based on the presence or absence of a 4977‐bp deletion. The mutational load of deleted mtDNA of these patients was comprehensively detected by real‐time polymerase chain reaction (RT‐PCR).ResultsSLD Patients showed the highest proportion of cytochrome C oxidase‐negative (COX‐n) fibres on muscle biopsy. The mutational load of deleted mtDNA exhibited an inverse relationship with deletion length and a direct relationship with the COX‐n fibre ratio. Compared with patients having noncommon deletions, those with common deletions tend to have other muscle involvement, lower body mass index (BMI) scores (17 ± 3 vs. 22 ± 4 kg/m2), higher mutational load in muscle (63% ± 22% vs. 46% ± 24%), more COX‐n fibres (26% vs. 9%, interquartile range [IQR]: 15%–32% vs. 6%–26%) and higher growth and differentiation factor 15 (GDF15) levels (2583 vs. 1472, IQR: 1746–4081 vs. 924–2155 pg/mL). MulDs patients displayed milder symptoms, especially compared to patients with m.3243A > G variant, as indicated by their later age of onset (31 vs. 13, IQR: 27–49 vs. 6–29 years), higher BMI scores (24.0 ± 4 vs. 16.5 ± 3.4 kg/m2), lower lactate (1.6 ± 1.1 vs. 6.3 ± 6.0 mmol/L) levels and lower proportion of ragged‐blue fibres (RBFs) (3 vs. 16, IQR: 1%–9% vs. 7%–27%).ConclusionThe m.3243A > G variant group exhibits more severe symptoms compared to other subgroups, particularly MulDs patients. In the SLD group, those with common deletions experience more severe clinical and pathological manifestations. These findings enhance our understanding of PEO, facilitating its diagnosis, prognosis and genetic counselling.

The implications of the early phases of human telencephalic development, involving neural stem cells (NSCs), in the etiology of cortical disorders remain elusive. Here, we explore the expression dynamics of cortical and neuropsychiatric disorder-associated genes in datasets generated from human NSCs across telencephalic fate transitions in vitro and in vivo. We identify risk genes expressed in brain organizers and sequential gene regulatory networks throughout corticogenesis, revealing disease-specific critical phases when NSCs may be more vulnerable to gene dysfunction and converging signaling across multiple diseases. Further, we simulate the impact of risk transcription factor (TF) depletions on neural cell trajectories traversing human corticogenesis and observe a spatiotemporal-dependent effect for each perturbation. Finally, single-cell transcriptomics of autism-affected patient-derived NSCs in vitro reveals recurrent expression alteration of TFs orchestrating brain patterning and NSC lineage commitment. This work opens perspectives to explore human brain dysfunction at early phases of development.

Amyotrophic lateral sclerosis (ALS) is a fatal neurological disorder characterized by progressive degeneration of nerve cells in the spinal cord and brain. We generated and characterized a human induced pluripotent stem cell (iPSC) line from skin fibroblasts of a patient with ALS due to SOD1 Mutation. The pluripotency of these iPSCs was verified by the expression of several pluripotency markers at both RNA and protein levels, as well as their capability to differentiate into all three germ layers.