Notes

Cardiac asystole subsequent large spinal-cord injury: a case document.
Arg (R)-rich dipeptide repeat proteins (DPRs; poly(PR) Pro-Arg and poly(GR) Gly-Arg), encoded by a hexanucleotide expansion in the C9ORF72 gene, induce neurodegeneration in amyotrophic lateral sclerosis (ALS). Although R-rich DPRs undergo liquid-liquid phase separation (LLPS), which affects multiple biological processes, mechanisms underlying LLPS of DPRs remain elusive. Here, using in silico, in vitro, and in cellulo methods, we determined that the distribution of charged Arg residues regulates the complex coacervation with anionic peptides and nucleic acids. Proteomic analyses revealed that alternate Arg distribution in poly(PR) facilitates entrapment of proteins with acidic motifs via LLPS. Transcription, translation, and diffusion of nucleolar nucleophosmin (NPM1) were impaired by poly(PR) with an alternate charge distribution but not by poly(PR) variants with a consecutive charge distribution. ε-poly-L-lysine in vitro We propose that the pathogenicity of R-rich DPRs is mediated by disturbance of proteins through entrapment in the phase-separated droplets via sequence-controlled multivalent protein-protein interactions.
The past 4 decades have seen substantial changes in allogeneic blood or marrow transplantation (BMT) practice, with the overarching goal of expanding the eligible patient pool while optimizing disease-free survival.

To determine trends in life expectancy and cause-specific late mortality after allogeneic BMT performed over a 40-year period.

A retrospective cohort study of 4741 individuals who lived 2 or more years after allogeneic BMT performed between January 1, 1974, and December 31, 2014, was conducted at City of Hope, University of Minnesota, or University of Alabama at Birmingham. The end of follow-up was March 23, 2020.

Allogeneic BMT performed in 3 eras 1974-1989, 1990-2004, and 2005-2014.

All-cause, recurrence-related, and nonrecurrence-related mortality and projected reduction in life expectancy. Information regarding vital status and cause of death was obtained from the National Death Index Plus and Accurint databases.

Of the 4741 individuals included in the study, 2735 (57.7%) were malerow. Further efforts to mitigate disease recurrence, infections, subsequent neoplasms, cardiovascular disease, and pulmonary disease may be useful in this population.
This cohort study noted that late mortality among recipients of allogeneic BMT has decreased over the past 40 years; however, life expectancy was not restored to expected rates compared with the general US population. Furthermore, the reduction in risk of late mortality appeared to be confined to those who underwent transplantation at a younger age or those who received bone marrow. Further efforts to mitigate disease recurrence, infections, subsequent neoplasms, cardiovascular disease, and pulmonary disease may be useful in this population.
Chronic kidney disease (CKD) and end-stage renal disease (ESRD) are among the important causes of mortality and morbidity in childhood. Early diagnosis and treatment of the underlying primary disease may prevent most of CKD patients from progressing to ESRD. There is no study examining chronic kidney diseases and dialysis modalities in Syrian immigrant children. We aimed to retrospectively research the etiologic, sociodemographic, and clinical factors in CKD among Syrian refugee children, and at the same time, to compare the clinical characteristics of patients with ESRD on peritoneal dialysis and hemodialysis.

Our study included a total of 79 pediatric Syrian patients aged from 2-16 years monitored at Hatay State Hospital pediatric nephrology clinic with diagnosis of various stages of CKD and with ESRD. Physical-demographic features and clinical-laboratory information were retrospectively screened.

The most common cause of CKD was congenital anomalies of the kidneys and urinary tracts (CAKUT) (37.9%). Other causes were urolitiasis (15.1%), nephrotic syndrome (10.1%), spina bifida (8.8%), hemolytic uremic syndrome (7.5%), and glomerulonephritis (7.5%). Twenty-five patients used hemodialysis due to bad living conditions. Only 2 of the patients with peritoneal dialysis were using automatic peritoneal dialysis (APD), with 5 using continuous ambulatory peritoneal dialysis (CAPD). Long-term complications like left ventricle hypertrophy and retinopathy were significantly higher among hemodialysis patients. There was no difference identified between the groups in terms of hypertension and sex.

Progression to ESRD due to preventable reasons is very frequent among CKD patients. For more effective use of peritoneal dialysis in pediatric patients, the responsibility of states must be improved.
Progression to ESRD due to preventable reasons is very frequent among CKD patients. For more effective use of peritoneal dialysis in pediatric patients, the responsibility of states must be improved.The first B-ring-extended (to biphenyl) flavonol-based Cys-ratiometric fluorescent probe B-bph-fla-acr (2-([1,1'-biphenyl]-4-yl)-4-oxo-4H-chromen-3-yl acrylate) is developed. B-bph-fla-acr can ratiometrically sense and non-ratiometrically image endogenous and exogenous cysteine (Cys) in living HeLa cells and zebrafish rapidly (45 s), selectively (vs. homocysteine and glutathione), sensitively (detection limit 18.5 nM), and with a large Stokes shift (186 nm). Quantitatively released (from the reaction of B-bph-fla-acr with Cys) fluorophore B-bph-fla-OH (2-([1,1'-biphenyl]-4-yl)-3-hydroxy-4H-chromen-4-one) is designed as a photoCORM (photo-triggered CO releasing molecule). Under O2 and visible light irradiation, the amount of CO released by B-bph-fla-OH can be accurately controlled linearly by adjusting the light irradiation intensity, irradiation time, or photoCORM dose. This process is accompanied by fluorescence quenching; therefore, the location of the photoCORM and the CO release process can be monitored in real time. B-bph-fla-acr and all reaction products exhibit good membrane permeability and low toxicity for living HeLa cells. In living HeLa cells and zebrafish, B-bph-fla-acr can image endogenous and exogenous Cys, and the released B-bph-fla-OH can photo-release CO under O2 at room temperature. This study is the first to combine a B-ring-extended flavonol-based fluorescent probe (for the effective ratiometric sensing and non-ratiometric imaging of endogenous and exogenous Cys in vitro and in vivo) with a photoCORM (Cys-activated, visible light-triggered linear CO release under O2). Our study provides important insights into the biological roles of Cys and CO, as well as a reliable method for safely supplying accurately controlled amounts of CO to living systems, thereby facilitating the development of convenient clinical diagnostic molecular tools and therapeutic prodrugs.Drug-induced acute liver injury (DIALI) is a common liver disease, affecting a number of people worldwide with increasing morbidity each year. Thus, it is vital to develop new tools for intervention and diagnosis. Peroxynitrite (ONOO-), a highly reactive species, plays an important role in the DIALI process. Thus, in situ molecular imaging of endogenous ONOO- levels is considerably significant for detecting ONOO-. In this work, we present two destroyed-type ratiometric fluorescent probes, AHC and AHMC, for ONOO- detection by using a molecular hybridization strategy. The probe AHMC was developed by introducing the ester structure to AHC directly to enhance its membrane penetrability for living cell imaging. Probe AHC exhibited good analytical performance toward ONOO- compared to other reactive species, with a low detection limit (≈1.8 nM) and a strong ratiometric fluorescence response (134-fold). In cell imaging experiments, AHMC showed outstanding selectivity, favourable biocompatibility and mitochondria-targeting ability, which not only was used to detect endogenous and exogenous ONOO- changes, but also enabled noninvasive visualization of ONOO- generation in a different drug-induced DIALI model. We hope that these ratiometric probes can be useful chemical tools for the in-depth research of drug-induced acute hepatotoxicity.Tremendous progress in two-dimensional (2D) nanomaterial chemistry affords abundant opportunities for the sustainable development of membranes and membrane processes. In this review, we propose the concept of mixed dimensional membranes (MDMs), which are fabricated through the integration of 2D materials with nanomaterials of different dimensionality and chemistry. Complementing mixed matrix membranes or hybrid membranes, MDMs stimulate different conceptual thinking about designing advanced membranes from the angle of the dimensions of the building blocks as well as the final structures, including the nanochannels and the bulk structures. In this review, we survey MDMs (denoted nD/2D, where n is 0, 1 or 3) in terms of the dimensions of membrane-forming nanomaterials, as well as their fabrication methods. Subsequently, we highlight three kinds of nanochannels, which are 1D nanochannels within 1D/2D membranes, 2D nanochannels within 0D/2D membranes, and 3D nanochannels within 3D/2D membranes. Strategies to tune the physical and chemical microenvironments of the nanochannels as well as the bulk structures based on the size, type, structure and chemical character of nanomaterials are discussed. Some representative applications of MDMs are illustrated for gas molecular separations, liquid molecular separations, ionic separations and oil/water separation. Finally, current challenges and a future perspective on MDMs are presented.Wearable and highly sensitive pressure sensors are of great importance for robotics, health monitoring and biomedical applications. For simultaneously achieving high sensitivity within a broad working range, fast response time (within a few milliseconds), minimal hysteresis and excellent cycling stability are critical for high performance pressure sensors. However, it remains a major challenge. Herein, we report a conceptual micro-cliff design of a graphene sensor with a record high sensitivity of up to 72 568 kPa-1 in a broad working range of 0-255 kPa, which is one order of magnitude higher than the state-of-the-art reported sensitivity. In addition, the detection limit can be as low as 0.35 Pa and the fast response time is less than 5 ms. The sensor also has a minimal hysteresis and an outstanding cycling stability of 5000 cycles, all of which meet the requirements of an ideal pressure sensor. More interestingly, the micro-cliff graphene sensor is made by the fast and scalable flash reduction of graphene oxide using a single flashlight pulse within 150 ms and has been integrated into a wearable smart insole and an E-glove prototype for demonstration of health monitoring applications. This micro-cliff graphene pressure sensor achieves record-high sensitivity, which brings new possibilities in sensor research and promises broad applications.Invention of DNA origami has transformed the fabrication and application of biological nanomaterials. In this review, we discuss DNA origami nanoassemblies according to their four fundamental mechanical properties in response to external forces elasticity, pliability, plasticity and stability. While elasticity and pliability refer to reversible changes in structures and associated properties, plasticity shows irreversible variation in topologies. The irreversible property is also inherent in the disintegration of DNA nanoassemblies, which is manifested by its mechanical stability. Disparate DNA origami devices in the past decade have exploited the mechanical regimes of pliability, elasticity, and plasticity, among which plasticity has shown its dominating potential in biomechanical and physiochemical applications. On the other hand, the mechanical stability of the DNA origami has been used to understand the mechanics of the assembly and disassembly of DNA nano-devices. At the end of this review, we discuss the challenges and future development of DNA origami nanoassemblies, again, from these fundamental mechanical perspectives.
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