Oxalate-derived porous C-doped NiO with amorphous-crystalline heterophase for supercapacitors.

Constructing amorphous/crystalline heterophase structure with high porosity is a promising strategy to effectively tailor the physicochemical properties of electrode materials and further improve the electrochemical performance of supercapacitors. Here, the porous C-doped NiO (C-NiO) with amorphous/crystalline heterophase grown on NF was prepared using NF as Ni source via a self-sacrificial template method. Calcining the self-sacrificial NiC2O4 template at a suitable temperature (400 °C) was beneficial to the formation of porous heterophase structure with abundant cavities and cracks, resulting in high electrical conductivity and rich ion/electron-transport channels. The density functional theory (DFT) calculations further verified that in-situ C-doping could modulate the electronic structure and enhance the OH- adsorption capability. The unique porous amorphous/crystalline heterophase structure greatly accelerated electrons/ions transfer and Faradaic reaction kinetic, which effectively improved the charge storage. The C-NiO calcined at 400 °C (C-NiO(400)) displayed a markedly enhanced specific charge, outstanding rate property and excellent cycling stability. Furthermore, the hybrid supercapacitor assembled by C-NiO(400) and active carbon achieved a high energy density of 49.0 Wh kg-1 at 800 W kg-1 and excellent cycle stability (90.9 % retention at 5 A/g after 10 000 cycles). This work provided a new strategy for designing amorphous/crystalline heterophase electrode materials in high-performance energy storage.

Oxalate modification enabled advanced phosphate removal of nZVI: In Situ formed surface ternary complex and altered multi-stage adsorption process.

Nano zero-valent iron (nZVI) is a promising phosphate adsorbent for advanced phosphate removal. However, the rapid passivation of nZVI and the low activity of adsorption sites seriously limit its phosphate removal performance, accounting for its inapplicability to meet the emission criteria of 0.1 mg P/L phosphate. In this study, we report that the oxalate modification can inhibit the passivation of nZVI and alter the multi-stage phosphate adsorption mechanism by changing the adsorption sites. As expected, the stronger anti-passivation ability of oxalate modified nZVI (OX-nZVI) strongly favored its phosphate adsorption. Interestingly, the oxalate modification endowed the surface Fe(III) sites with the lowest chemisorption energy and the fastest phosphate adsorption ability than the other adsorption sites, by in situ forming a Fe(III)-phosphate-oxalate ternary complex, therefore enabling an advanced phosphate removal process. At an initial phosphate concentration of 1.00 mg P/L, pH of 6.0 and a dosage of 0.3 g/L of adsorbents, OX-nZVI exhibited faster phosphate removal rate (0.11 g/mg/min) and lower residual phosphate level (0.02 mg P/L) than nZVI (0.055 g/mg/min and 0.19 mg P/L). This study sheds light on the importance of site manipulation in the development of high-performance adsorbents, and offers a facile surface modification strategy to prepare superior iron-based materials for advanced phosphate removal.

Expression profiles of urine exosomal tRNA-derived small RNAs and their potential roles in calcium oxalate stone disease.

Background and objective: Exosomes have been confirmed to be implicated in the pathogenesis of calcium oxalate (CaOx) stones. tRNA-derived small RNAs (tsRNAs) are among the oldest small RNAs involved in exosome-mediated intercellular communication, yet their role in kidney stones remains unexplored. This pilot study aimed to identify differentially expressed tsRNAs (DEtsRNAs) in urine exosomes between CaOx stone patients and healthy controls and explore their potential roles in nephrolithiasis. Method: First-morning urine samples were collected from three CaOx stone patients and three healthy controls. Urinary exosomes were isolated and analyzed by high-throughput sequencing to generate the expression profiles of tsRNAs and detect DEtsRNAs. Predicted target genes of DEtsRNAs were subjected to functional enrichment analysis. The authors also combined the public dataset GSE73680 to investigate how DEtsRNAs were related to stone formation. Results: Four DEtsRNAs were significantly upregulated in CaOx stone patients compared to healthy controls. tRF-Lys-TTT-5005c was the most elevated, followed by tRF-Lys-CTT-5006c, tRF-Ala-AGC-5017b, and tRF-Gly-CCC-5004b. Bioinformatics analysis indicated that these four types of DEtsRNAs might serve distinct biological functions. Combined with data mining from the public dataset GSE73680, the authors assumed that exosomes carrying tRF-Lys-TTT-5005c and tRF-Lys-CTT-5006c could inhibit the expression of SMAD6, FBN1, and FZD1, thereby activating the BMP signaling pathway, which might induce an osteogenic-like transformation in target cells, resulting in the formation of Randall's plaques and CaOx stones. Conclusion: The authors' findings shed light on the potential roles of tsRNAs in the pathogenesis of CaOx stone disease, highlighting exosomal DEtsRNAs as promising diagnostic biomarkers and therapeutic targets in nephrolithiasis.

NLRP3 inflammasome activation and macrophage distribution in kidney tissues from patients with acute oxalate nephropathy.

Background: The current study was initiated to evaluate renal nucleotide-binding and oligomerization domain-like receptor family pyrin domain-containing 3 (NLRP3) inflammasome pathway activation and macrophage subtype distribution and their clinicopathological significance in a cohort of oxalate-induced acute kidney injury. Methods: Twelve patients with biopsy-proven acute oxalate nephropathy (AON) from January 2016 to October 2022 were retrospectively enrolled, with estimated glomerular filtration rate (eGFR)-matched 24 patients with acute tubulointerstitial nephritis (ATIN) as disease control. Pathological lesions as well as markers of NLRP3 inflammasome pathway and macrophage phenotype detected by immunohistochemistry staining were semi-quantitatively analyzed. Results: Oxalate depositions were found in 5% to 20% of tubules with a positive correlation with Sirius Red staining in AON specimens (rp = 0.668, p = 0.02). Disruption of tubular basement membrane and inflammatory cell reaction was more prominent in specimens of AON (both p < 0.05) as compared with ATIN specimens. The expressions of NLRP3, caspase-1, and gasdermin D were significantly increased in AON specimens as well (all p < 0.05). Patients with M1/M2 macrophage ratio <1 were found with more chronic tubulointerstitial lesions and presented with lower eGFR at the last follow-up (24.8  10.6 mL/min/1.73 m2 vs. 55.1  21.2 mL/min/1.73 m2, p = 0.02) in the AON group. Conclusion: The NLRP3 inflammasome pathway was activated in the kidneys of AON patients, and the ratio of M1 and M2 macrophages was associated with chronicity of pathological changes, which needs further exploration.

Pectolinarigenin alleviates calcium oxalate-induced renal inflammation and oxidative stress by binding to HIF-1α.

Calcium oxalate (CaOx) crystals are the main constituents of renal crystals in humans and induce tubular lumen damage in renal tubules, leading to renal calcium deposition and kidney stone formation. Oxidative stress and inflammation play important roles in regulating calcium oxalate-induced injury. Here, we evaluated the efficacy in inhibiting oxidation and inflammation of pectinolinarigenin, a biologically active natural metabolite, in CaOx nephrocalcinosis and further explored its targets of action. First, we developed cellular and mouse models of calcium oxalate renal nephrocalcinosis and identified the onset of oxidative stress and inflammation according to experimental data. We found that pectolinarigenin inhibited this onset while reducing renal crystal deposition. Network pharmacology was subsequently utilized to screen for hypoxia-inducible factor-1α (HIF-1α), a regulator involved in the body's release and over-oxidation of inflammatory factors. Finally, molecular docking, cellular thermal shift assay, and other experiments to detect HIF-1α expression showed that pectolinarigenin directly combined with HIF-1α and prevented downstream reactive oxygen species activation and release. Our results indicate that pectolinarigenin can target and inhibit HIF-1α-mediated inflammatory responses and oxidative stress damage and be a novel drug for CaOx nephrocalcinosis treatment.

NINJ1-mediated Macrophage Plasma Membrane Rupture and Neutrophil Extracellular Trap Formation Contribute to Oxalate Nephropathy.

BACKGROUND AND HYPOTHESIS: Oxalate nephropathy is characterized by calcium oxalate crystals deposition, which triggers necrosis of renal tubular epithelial cells, initiates an inflammatory cascade characterized by neutrophil and macrophage activation within the renal microenvironment. Despite the close association of immune cells with acute oxalate nephropathy, the underlying mechanisms still remain unclear. Nerve injury-induced protein 1 (NINJ1) plays an essential role in the induction of plasma membrane rupture (PMR), leading to damage-associated molecular patterns (DAMPs) release and triggering inflammation. We hypothesize that NINJ1-mediated high mobility group box 1 (HMGB1) release from macrophage PMR and neutrophil extracellular traps (NETs) formation synergistically contribute to the progression of acute oxalate nephropathy. METHODS: Using a murine model of acute oxalate nephropathy, myeloid cell-specific deletion of Ninj1 mice (Ninj1fl/flvavcre) and their wild-type littermate control mice (Ninj1wt/wtvavcre) were administered intraperitoneal injection of 100 mg/kg sodium oxalate followed by drinking water with 3% sodium oxalate. Evaluation was conducted on tubular injury and inflammatory cell infiltration. In vitro studies involved isolation and culture of renal proximal tubular epithelial cells (RTECs), bone marrow-derived macrophages, and neutrophils to investigate NETs formation and HMGB1 release. RESULTS: Targeted deletion of Ninj1 in myeloid cells significantly mitigated oxalate-induced acute kidney injury by suppressing both HMGB1 release and NETs formation in vivo. In vitro investigations demonstrated that HMGB1 release from macrophage PMR and NETs formation in neutrophils mediated by NINJ1 oligomerization, which consequently coordinated to enhance renal tubular epithelial cell death. CONCLUSION: Our findings elucidate the pivotal role of NINJ1-dependent macrophage PMR and NETs formation in the progression of acute oxalate nephropathy, providing novel insights for its prevention and therapeutic targets.

Diet-induced hyperoxaluria: A case based mini-review.

INTRODUCTION: Oxalate nephropathy (ON) is a rare condition involving the precipitation of calcium oxalate crystals within the nephrons. Primary hyperoxaluria involves enzymatic defects in the metabolism of glyoxylate, while secondary hyperoxaluria includes dietary and malabsorption-related etiologies. CASE PRESENTATION: We discuss the case of a White male in his 80s who presented to the hospital with acute kidney injury on chronic kidney disease stage 4 in the setting of a new antibiotic prescription. Creatinine had increased to 4.2 mg/dL from a baseline of 2.2 mg/dL, with no etiology identified on urinalysis or renal ultrasound. Renal biopsy then revealed an acute tubular injury with intraluminal calcium oxalate crystals deposits, confirming a diagnosis of ON. DISCUSSION: A detailed history revealed an excessive dietary intake of oxalate-rich foods, including nuts, and daily ingestion of 2 g of vitamin C. The patient was counselled on adjusting his diet and stopping vitamin C supplementation, which led his creatinine to return close to baseline 2 months post-discharge. CONCLUSION: Thorough history-taking enables early recognition and timely interventions to possibly avoid hyperoxaluria from progressing to end-stage kidney disease (ESRD).

Secondary products and molecular mechanism of calcium oxalate degradation by the strain Azospirillum sp. OX-1.

The oxalate-carbonate pathway (OCP) involves degradation of soil oxalate to carbonate. To exploit and manage this natural mineralization of assimilated atmospheric CO2 into stable carbonates, improved understanding of this complex biotransformation process is needed. A strain of oxalate-degrading bacteria, Azospirillum sp. OX-1, was isolated from soil, and its secondary products of calcium oxalate degradation were analyzed and characterized using SEM, XRD, TG/DTG-DTA and FTIR-spectroscopy. The molecular mechanism of calcium oxalate degradation was also analyzed using proteomics. The results showed, for the first time, that OX-1 could not only degrade calcium oxalate to calcium carbonate, but also that the process was accompanied by synthesis of methane. Proteomic analysis demonstrated that OX-1 has a dual enzyme system for calcium oxalate degradation, using formyl-CoA transferase (FRC) and thiamine pyrophosphate (ThDP)-dependent oxalyl-CoA decarboxylase (OXC) to form calcium carbonate. Up-regulated expression of enzymes related to methane synthesis was also detected during calcium oxalate degradation. Since methane is also a potent greenhouse gas, these new results suggest that the utility of exploiting the OCP to reduce atmospheric CO2 must be re-evaluated and that further studies should be conducted to reveal how widespread the methane producing capacity of strain OX-1 is in other bacteria and soil environments.

Recurrent calcium oxalate calculi: the culprit in disguise.

Congenital sucrase isomaltase deficiency (CSID) is a rare autosomal recessive monogenic disorder of small intestinal malabsorption and manifests typically in early childhood with chronic osmotic diarrhoea. Though there have been case reports in adults presenting with hypercalcemia and renal calculi in CSID, this is quite rare in children. We hereby report a 6-year-old boy who presented with recurrent episodes of calcium oxalate calculi without any gastrointestinal symptoms and was confirmed as having sucrase isomaltase deficiency by genetic analysis.

Tensile Properties of Cattail Fibres at Various Phenological Development Stages.

Cattails (Typha latifolia L.) are naturally occurring aquatic macrophytes with significant industrial potential because of their abundance, high-quality fibers, and high fiber yields. This study is the first attempt to investigate how phenological development and plant maturity impact the quality of cattail fibers as they relate to composite applications. It was observed that fibers from all five growth stages exhibited a Weibull shape parameter greater than 1.0, with a goodness-of-fit exceeding 0.8. These calculations were performed using both the Least Square Regression (LSR) and Maximum Likelihood Estimation (MLE) methods. Among the estimators, the MLE method provided the most conservative estimation of Weibull parameters. Based on the Weibull parameters obtained with all estimators, cattail fibers from all five growth stages appear suitable for composite applications. The consistency of shape parameters across all five growth stages can be attributed to the morphological and molecular developments of cattail fiber during the vegetative period. These developments were confirmed through the presence of calcium oxalate (CaOx) plates, elemental composition, and specific infrared peaks at 2360 cm-1 contributing to the strength, cellulose peaks at 1635 cm-1, 2920 cm-1, and 3430 cm-1. In conclusion, it was found that the mechanical properties of cattail fiber remain similar when harvested multiple times in a single growing season.

Validation of a LC-MS/MS assay for citric acid, cysteine and oxalic acid determination and its application to explore pre-analytical sample storage.

Objectives: Citrate, oxalate and cystine in 24-h urine are considered to be associated with the incidence and recurrence risk of urinary stone disease (USD). An evaluation of the LC-MS/MS kit for simultaneous quantification of the three analytes was undertaken. Design: & Methods: The analytical performance of the kit was investigated based on FDA, EMA and CLSI guidelines. To promote the standardization of sample storage, this kit has been applied to perform systematic pre-analytical stability study of these analytes in urine. Results: This method was validated with good linearity with accuracy of 93.1%-104 %. Intra-day and inter-day imprecision were ≤5.55 % and 5.34 %, respectively. Recoveries of citrate, oxalate and cystine added to clinical samples were in the range of 92.0-103 %, 94.8-100 % and 99.0-107 % with CV ≤ 5.52 %. It was recommended that urine preserved with hydrochloric acid could be preferable in consideration of both reliable test results and neglected sample heterogeneity. Conclusions: This kit is suitable for measurement of citrate, oxalate and cystine for understanding the etiology of urinary stones, and the proper storage of urine samples is crucial for the correctness of the test results.

S100A9 promotes renal calcium oxalate stone formation via activating the TLR4-p38/MAPK-LCN2 signaling pathway.

OBJECTIVES: To explore the role of S100A9 protein in renal calcium oxalate (CaOx) stone formation. METHODS: CaOx nephrocalcinosis mice were established via intraperitoneal injection of glyoxylate. They were treated with S100A9 deficiency, Paquinimod, or p38 MAPK-IN-1. Vonkossa staining was conducted to observe the deposition of CaOx crystals. Renal expression of inflammation, macrophage polarization, and injury markers was detected using immunohistochemistry and qPCR. Effects of S100A9 on renal tubular epithelial cells (HK-2) were explored by transcriptome sequencing. The mechanism of how S100A9 regulated lipocalin 2 (LCN2) was studied through Western Blot. Flow cytometry was performed to detect the influence of LCN2 on macrophages polarization. RESULTS: S100A9 deficiency inhibited the renal deposition of CaOx crystals in nephrocalcinosis mice. S100A9 upregulated the expression of LCN2 in HK-2 cells via activating the TLR4-p38/MAPK pathway. LCN2 promoted the migration and M1 polarization of macrophages. S100A9 deficiency downregulated the renal expression of LCN2, IL1-ß, Kim-1, F4/80, and CD80 in nephrocalcinosis mice. Paquinimod and p38 MAPK-IN-1 both inhibited the renal deposition of CaOx crystals and downregulated the expression of LCN2, IL1-ß, Kim-1, F4/80, iNOS, and CD68 in nephrocalcinosis mice. CONCLUSIONS: S100A9 promotes renal inflammatory injury by activating the TLR4-p38/MAPK-LCN2 pathway and then contributes to CaOx stone formation.

Efficacy and safety of lumasiran for infants and young children with primary hyperoxaluria type 1: 30-month analysis of the phase 3 ILLUMINATE-B trial.

Background: Primary hyperoxaluria type 1 (PH1) is a genetic disorder resulting in overproduction of hepatic oxalate, potentially leading to recurrent kidney stones, nephrocalcinosis, chronic kidney disease, and kidney failure. Lumasiran, the first RNA interference therapeutic approved for infants and young children, is a liver-directed treatment that reduces hepatic oxalate production. Lumasiran demonstrated sustained efficacy with an acceptable safety profile over 12 months in infants and young children (age <6 years) with PH1 in ILLUMINATE-B (clinicaltrials.gov: NCT03905694), an ongoing, Phase 3, multinational, open-label, single-arm study. Methods: Here, we report interim efficacy and safety findings from ILLUMINATE-B following 30 months of lumasiran treatment. Eligible patients had an estimated glomerular filtration rate (eGFR) >45 ml/min/1.73 m2 if ≥12 months old or normal serum creatinine if <12 months old, and a urinary oxalate to creatinine ratio (UOx:Cr) greater than the upper limit of normal. All 18 patients enrolled in ILLUMINATE-B completed the 6-month primary analysis period, entered an extension period of up to 54 months, and continue to participate in the study. Results: At Month 30, mean percent change from baseline in spot UOx:Cr was -76%, and mean percent change in plasma oxalate was -42%. eGFR remained stable through Month 30. In 14 patients (86%) with nephrocalcinosis at baseline, nephrocalcinosis grade improved at Month 24 in 12; no patient worsened. In the 4 patients without baseline nephrocalcinosis, nephrocalcinosis was absent at Month 24. Kidney stone event rates were ≤0.25 per person-year through Month 30. Mild, transient injection site reactions were the most common lumasiran-related adverse events (17% of patients). Conclusion: In infants and young children with PH1, long-term lumasiran treatment resulted in sustained reductions in urinary and plasma oxalate that were sustained for 30 months, with an acceptable safety profile. Kidney function remained stable, low kidney stone event rates were observed through Month 30, and nephrocalcinosis grade improvements were observed through Month 24. Clinical Trial Registration: https://clinicaltrials.gov, identifier NCT03905694.

Unveiling primary Hyperoxaluria type 1: a fortuitous discovery through bone marrow biopsy.

This paper details a rare case of primary hyperoxaluria type 1 (PH1) identified through a bone marrow biopsy in a 46-year-old female patient with a history of nephrolithiasis and chronic renal failure. Genetic analysis identified the p.Ile244Thr mutation in the AGXT gene, confirming the diagnosis of PH1. The paper aims to highlight this case, focusing on the genetic basis of the disorder, including the identified mutation. It underscores the importance of early diagnosis of infantile and childhood nephrolithiasis, particularly in cases with familial history, to prevent renal loss and systemic oxalosis.

Metabolic diversity analysis and genome wide assessment of oxalate accumulation in the leaves of rice (Oryza sativa) cultivars.

Soluble oxalate accumulates in rice leaves, and it causes mineral deficiency and urinary syndrome in livestock that consume the leaves. In our previous study, we found that the oxalate content was higher in the leaves of Koshihikari (japonica type cultivar) than in those of Takanari (indica type cultivar). This difference was seen even when the two cultivars were grown under a high CO2 concentration, which inhibits oxalate synthesis via photorespiration, suggesting that the difference resulted from genetic factors rather than environmental factors. To clarify whether genetic factors affect the oxalate content of rice leaves, we measured the contents of oxalate and oxalate-related organic acids in the leaves of various rice cultivars the Rice Core Collection (WRC) and Japan Rice Core Collection (JRC) by capillary electrophoresis-mass spectrometry. Results showed that japonica type cultivars tended to accumulate more oxalate than aus or indica type cultivars. Correlation analysis revealed a positive correlation between oxalate accumulation and the citrate content, suggesting that the isocitrate pathway is involved in oxalate accumulation. On the other hand, a genome-wide association study for the oxalate content of the WRC and JRC cultivars did not reveal significant loci directly related to oxalate accumulation. This indicates that the combination of various loci may affect the oxalate contents of rice leaves.

Current Trends and Technological Advancements in the Use of Oxalate-Degrading Bacteria as Starters in Fermented Foods-A Review.

Nephrolithiasis is a medical condition characterized by the existence or development of calculi, commonly referred to as stones within the renal system, and poses significant health challenges. Calcium phosphate and calcium oxalate are the predominant constituents of renal calculi and are introduced into the human body primarily via dietary sources. The presence of oxalates can become particularly problematic when the delicate balance of the normal flora residing within the gastrointestinal tract is disrupted. Within the human gut, species of Oxalobacter, Lactobacillus, and Bifidobacterium coexist in a symbiotic relationship. They play a pivotal role in mitigating the risk of stone formation by modulating certain biochemical pathways and producing specific enzymes that can facilitate the breakdown and degradation of oxalate salts. The probiotic potential exhibited by these bacteria is noteworthy, as it underscores their possible utility in the prevention of nephrolithiasis. Investigating the mechanisms by which these beneficial microorganisms exert their effects could lead to novel therapeutic strategies aimed at reducing the incidence of kidney stones. The implications of utilizing probiotics as a preventive measure against kidney stone formation represent an intriguing frontier in both nephrology and microbiome research, meriting further investigation to unlock their full potential.

Kukoamine A alleviates nephrolithiasis by inhibiting endogenous oxalate synthesis via the IL-6/JAK/STAT3/DAO signaling pathway.

BACKGROUND: The recurrent nature and socioeconomic burden of nephrolithiasis demand effective treatments. Delineating the crosstalk between inflammatory processes and the endogenous oxalate metabolism pathway, which underpins nephrolithiasis pathogenesis, is essential for advancing treatment strategies. PURPOSE: We aim to screen therapeutic Chinese herbal remedies and their bioactive constituents for kidney stone treatment using a fruit fly model, followed by efficacy and mechanism validation in a rodent nephrolithiasis model as well as in vitro human cell culture model. STUDY DESIGN AND METHODS: We developed a fruit fly model to screen for efficient traditional Chinese medicine herbs and their active compounds for kidney stone treatment. Candidate active compounds from efficient herbs were separated and identified by solid-phase chromatography coupled with LC-MS analysis. Fruit fly genetic tools were employed to manipulate the expression of related genes to explore the therapeutic mechanisms of the Lycii Cortex and kukoamine A (KuA). To confirm the therapeutic effects and mechanisms of KuA for mammalian nephrolithiasis, mouse model of glyoxylate-induced kidney stone and human renal tubular cells were used. The therapeutic role of kukoamine A in nephrolithiasis was evaluated by assessing tubular injury, crystal deposition, and adhesion. The level of expression and phosphorylation in cells and mice was assessed using RT-qPCR and western blot. RESULTS: Our findings indicate that Lycii Cortex potently inhibits calcium oxalate stone formation via activation of the JNK/Upd3/JAK/STAT signaling cascade, resulting in diminished endogenous oxalate synthesis by downregulating D-amino acid oxidase (DAO) gene expression, predominantly in fruit fly Malpighian tube stellate cells. KuA was identified as the principal bioactive constituent mediating these effects. Both mouse models and human cell assays confirmed KuA's efficacy in preventing calcium oxalate nephrolithiasis in mammals, through hepatic JAK/STAT3 pathway activation and upregulation of IL-6, culminating in reduced urinary crystal deposition. CONCLUSION: Our research underscores the potential of kukoamine A as a lead compound in treating nephrolithiasis and reveals the interplay between the IL-6/JAK/STAT3 inflammatory pathway and endogenous oxalate metabolism in nephrolithiasis pathogenesis. Additionally, it highlights the utility of the fruit fly model as a powerful tool for deciphering the therapeutic mechanisms of traditional Chinese herbs.

Dual metal-organic-frameworks mediated resonance energy transfer for ultrasensitive electrochemiluminescence immunosensing.

An electrochemiluminescence (ECL) biosensor for carcinoembryonic antigen (CEA) based on electrochemiluminescence resonance energy transfer (ECL-RET) was designed. Tris(2,2'-bipyridine)ruthenium(II) (Ru(bpy)32+) was used as an energy donor for ECL-RET, and an Au nanoparticle-modified MOF framework (AuCoFe MOF) was used as an energy receptor for ECL-RET. The ECL emission spectra of Ru(bpy)32+ were in the range 550 to 680 nm, and a zinc oxalate MOF encapsulating Ru(bpy)32+ (Ru@ Zn oxalate MOF) was prepared. The UV-vis absorption spectrum of AuCoFe MOF ranges from 280 to 700 nm and overlaps with emission spectra of Ru@Zn oxalate MOF, which is critical for RET. The AuCoFe MOF-Ab2 bioconjugate, target CEA antigen, and the Ru@Zn oxalate MOF-Ab1 bioconjugate together form a sandwich structure, resulting in quenching of the ECL signal of Ru@Zn oxalate MOF by AuCoFe MOF. Under the optimized experimental conditions, the ECL-RET sensor exhibited excellent analytical performance in CEA detection with a linear range of 1.0 × 10-13 to 1.0 × 10-8 mg mL-1; the minimum limit of detection is 1.4 × 10-14 mg mL-1 (S/N = 3); and its recoveries of spiked samples ranging from 99.1 to 100.7%. The developed sensor has excellent stability, reproducibility, and specificity and is suitable for the detection of CEA in human serum and has the potential to provide sensitive detection of other biomarkers of diseases.

Primary hyperoxaluria type 3: from infancy to adulthood in a genetically unique cohort.

BACKGROUND: Primary hyperoxaluria type 3 (PH3) is a rare autosomal recessive disorder caused by bi-allelic genetic variants in the 4 hydroxy-2 oxoglutarate aldolase (HOGA-1) gene. We report the natural history of PH3 in a 16-patient cohort, 15 from a unique genetically isolated population. METHODS: This retrospective single-center study followed PH3 patients between 2003 and 2023 with demographic, clinical, radiographic, genetic, and biochemical parameters. Genetic population screening was performed in four villages to determine carrier frequency and identify couples at risk in a genetically isolated population. RESULTS: Sixteen patients with biallelic (or homozygous) pathogenic variants (PV) in HOGA-1 (c.944_946 del, c.119C > A, c.208C > T) were included in the study, 15 Druze and one Jewish, aged 0-63 years at diagnosis (4 adults and 12 pediatric patients). All symptomatic patients had clinical or imaging signs of nephrolithiasis. One developed chronic kidney disease (CKD) stage 5; biopsy showed focal mesangial sclerosis and chronic tubulo-interstitial changes with few oxalate deposits. Two other patients had CKD stage 2 (eGFR 87 and 74 mL/min/1.73 m2) upon their last visit. The remaining cohort showed preserved kidney function until the latest follow-up. Of 1167 healthy individuals screened, 90 carriers were found, a rate of 1:13 in the genetically unique cohort screened. CONCLUSIONS: A high prevalence of PH3 patients was found among a unique cohort, but probably still underdiagnosed due to relatively mild disease course. The carrier rate is high. There is no specific therapy for PH3, but early diagnosis can prevent redundant diagnostic efforts and provide early treatment for kidney stone disease. Even in our homogeneous cohort, kidney stone disease severity and CKD degree were variable, supporting a suspected contribution of yet unknown genetic or environmental factors.

Multipoint Anionic Bridge: Asymmetric Solvation Structure Improves the Stability of Lithium-Ion Batteries.

In this study, a novel concept of multipoint anionic bridge (MAB) is proposed and proved, which utilizes anions with different sites to connect with the asymmetric solvation structure (ASS). Compared to usual solvation structures, this study utilizes the multifunctional groups of difluoro(oxalate)borate anion (ODFB-), which can connect with Li+. By tailoring the concentration, the anion serves as a bridge between different solvated structures. The electrolyte is investigated through in situ techniques and simulations to draw correlations between different solvation structures and reaction pathways. The proposed design demonstrates remarkable high-temperature performance on both the anode and cathode sides, enabling stable cycling of LCO||graphite (0.5 Ah, 1.0 C) pouch cell for over 200 cycles at 80 °C and facilitating Li||MCMB and Li||LFP cells to deliver stable performance for 200 cycles at 100 °C. This work paves the way for the development of high-performance electrolyte systems by designing and using new multipoint anions to construct ASSs.