Titin fragment is a sensitive biomarker in Duchenne muscular dystrophy model mice carrying full-length human dystrophin gene on human artificial chromosome.

Duchenne muscular dystrophy (DMD) is an X-linked recessive disorder caused by mutations of the dystrophin gene, which spans 2.4 Mb on the X chromosome. Creatine kinase (CK) activity in blood and titin fragment levels in urine have been identified as biomarkers in DMD to monitor disease progression and evaluate therapeutic intervention.

Impact of Fc-gamma receptor IIIA polymorphism on late-onset neutropenia and clinical outcomes in kidney transplant recipients following rituximab induction therapy.

Background: This study aimed to investigate the association between the Fc-gamma receptor IIIA (FCGR3A) 158 polymorphism and clinical outcomes in kidney transplantation (KTx) patients. Specifically, we focused on late-onset neutropenia (LON) in ABO-incompatible (ABOi) or HLA-incompatible (HLAi) KTx recipients who underwent rituximab (RTx) desensitization therapy.

Methods: FCGR3A 158F/V polymorphisms were identified in 85 ABOi or HLAi KTx recipients who underwent RTx desensitization at our institution between April 2008 and October 2021.

Impact of depth of body cavity at the upper-right portion of the abdomen on open and laparoscopic liver resection of segment 7.

Purpose: The impact of body-cavity depth on open (OLR) and laparoscopic liver resection (LLR) of segment 7 remains unclear. Therefore, we investigated the influence of body-cavity depth at the upper-right portion of the abdomen on LLR and OLR of segment 7.

Methods: In total, 101 patients who underwent segment-7 liver resection over 2010-2023 were included.

Corticosteroid therapy and long-term outcomes in patients with cardiac sarcoidosis stratified by left ventricular ejection fraction.

Aims: This study aimed to investigate the relationship between corticosteroid therapy and long-term outcomes in patients with cardiac sarcoidosis, stratified by left ventricular ejection fraction (LVEF) at diagnosis.

Methods And Results: This study conducted a analysis of the ILLUstration of the Management and prognosIs of JapaNese PATiEnts with Cardiac Sarcoidosis, a retrospective multicentre registry. Cardiac sarcoidosis was diagnosed based on the 2016 Japanese Circulation Society and 2014 Heart Rhythm Society criteria.

Characteristics of upper lumbar spondylolysis in children.

Background: Pediatric lumbar spondylolysis (LS) is common in junior and senior high school athletes. Lower LS (L4-L5 level) is more common in children, and upper LS (L1-L3 level) is relatively rare; therefore, the pathogenesis of upper LS remains unclear.

Purpose: To elucidate the mechanisms of upper LS by identifying and comparing characteristics between upper and lower LS cases.

A facile assay for zDHHC palmitoyl transferase activation elucidates effects of mutation and modification.

At least 10% of proteins constituting the human proteome are subject to S-acylation by a long-chain fatty acid, thioesterified to a Cys thiol side chain. Fatty S-acylation (prototypically, S-palmitoylation) operates across eukaryotic phylogeny and cell type. S-palmitoylation is carried out in mammalian cells by a family of 23-24 dedicated zDHHC palmitoyl transferase enzymes, and mutation of zDHHCs is associated with a number of human pathophysiologies.

Proline enhances the hepatic induction of lipogenic gene expression in male hepatic fasn reporter mice.

Hepatic de novo lipogenesis (DNL) is increased by both carbohydrate intake and protein consumption. In hepatic fat synthesis, a key role is played by the induction of the hepatic expression of lipogenic genes, including Fasn, Scd1, and Srebf1. Regarding carbohydrate intake, increased blood glucose and insulin levels promote the expression of hepatic lipogenic genes.

A Native LH1-RC-HiPIP Supercomplex from an Extremophilic Phototroph.

Halorhodospira (Hlr.) halophila strain BN9622 is an extremely halophilic and alkaliphilic purple phototrophic bacterium and has been widely used as a model for exploring the osmoadaptive and photosynthetic strategies employed by phototrophic extreme halophiles that enable them to thrive in hypersaline environments. Here we present the cryo-EM structures of (1) a unique native Hlr.

Radiological Assessment and Therapeutic Evaluation in Hepatocellular Carcinoma: Differentiation and Treatment Response with Japanese Guidelines.

The liver is supplied by a dual blood flow system consisting of the portal vein and hepatic artery. Imaging techniques for diagnosing hepatocellular carcinoma (HCC) have been developed along with blood flow imaging, which visualizes the amount of arterial and portal blood flow. The diagnosis of HCC differentiation is important for early-stage liver cancer screening and determination of treatment strategies.

Efficient Synthesis of ,-Muconic Acid by Catechol Oxidation of Ozone in the Presence of a Base.

Muconic acid, a crucial precursor in synthesizing materials like PET bottles and nylon, is pivotal for the anticipated growth in the textiles and plastics industries. This study presents a novel chemical synthesis route for ,-muconic acid (ccMA) using catechol. Biochemical methods face scale-up challenges due to microorganism sensitivity and complex extraction processes, while chemical methods involve environmentally harmful substances and have low yields.

Efficacy and safety of indocyanine green-fluorescence imaging guided liver resection: a single-arm prospective cohort study.

Purpose: This study aimed to evaluate the efficacy of indocyanine green (ICG)-fluorescence imaging for the identification of hepatic boundaries during liver resection and its advantages in surgical outcomes over conventional methods.

Methods: This prospective, exploratory, single-arm clinical trial included 47 patients with liver tumors who underwent liver resection using ICG-fluorescence imaging (ICG-LR) between 2019 and 2020. The primary outcome measure was the successful identification of hepatic boundaries during liver resection, from the perspective of both the hepatic surface and intrahepatic boundary, using ICG-fluorescence imaging.

Quadrant, necrosis, and infection criteria for the risk stratification of walled-off necrosis: external validation using multi-institutional data.

Background And Aims: Endoscopic ultrasound-guided peripancreatic fluid drainage (EUS-PFD) with on-demand endoscopic necrosectomy, increasingly utilized to manage walled-off necrosis (WON), is associated with substantial morbidity and mortality. This multicenter study aimed to externally validate recently developed quadrant (an abdominal quadrant distribution), necrosis, and infection (QNI) criteria for risk stratification in this setting.

Methods: Of 423 patients with pancreatic fluid collections treated in a large multi-institutional cohort between 2010 and 2020, 212 with available preprocedural computed tomography images were included.

Divergent evolution of a thermospermine-dependent regulatory pathway in land plants.

Plants adapted to life on land by developing diverse anatomical features across lineages. The molecular basis of these innovations often involves the emergence of new genes or establishing new connections between conserved elements, though evidence for evolutionary genetic circuit rewiring remains scarce. Here, we show that the thermospermine-dependent pathway regulating vascular cell proliferation in Arabidopsis thaliana operates as two distinct modules with different functions in the bryophyte Marchantia polymorpha.

Usefulness of serial in vivo imaging to directly assess the role of inflammation in thrombus resolution and organization.

Deep vein thrombosis (DVT) remains a significant health problem. Although animal models have provided significant insights into the DVT pathophysiology, time-course assessment in a same animal is technically limited. Recently, we reported a novel murine saphenous DVT model for in vivo visualization of spatiotemporal dynamics of inflammatory cells.

Contributions of Both the Eastman Entropy of Transfer and Electric Double Layer to the Electromotive Force of Ionic Thermoelectric Supercapacitors.

Recently, ionic thermoelectric supercapacitors have gained attention because of their high open circuit voltages, even for ions that are redox inactive. As a source of open circuit voltage (electromotive force), an asymmetry in electric double layers developed by the adsorption of ions at the electrode surfaces kept at different temperatures has previously been proposed. As another source, the Eastman entropy of transfer, which is related to the Soret coefficient, has been considered.

Catalytic acceptorless complete dehydrogenation of cycloalkanes.

The advancement of an effective hydrogen liberation technology from liquid organic hydrogen carriers, particularly cycloalkanes such as cyclohexane and methylcyclohexane, holds significance in realizing a hydrogen-centric society. However, the attainment of homogeneous catalytic acceptorless dehydrogenation characterized by elevated selectivity for thorough aromatization under mild conditions remains unrealized. In this study, a catalyst system, facilitated by a double hydrogen atom transfer processes, has been devised for the catalytic acceptorless dehydrogenation of inert cycloalkanes at ambient temperature under visible light irradiation.

Direct Click Bonding of Dissimilar Solid Materials Based on the Catalyst-Free Huisgen 1,3-Dipolar Cycloaddition.

Here, "direct click bonding" of solid materials is proposed, which is the direct bonding of solid surfaces via the formation of covalent bonds without any adhesive. The present study shows that the Cu-free Huisgen 1,3-dipolar cycloaddition reaction proceeds between solid surfaces displaying cyclooctyne and azide groups, and it achieved the strong bonding of dissimilar solid materials as a macroscopic reaction. The bonding strength obtained is sufficiently high for practical use, and the strength can be controlled by the surface density of the cyclooctyne groups.

Prevalence and Clinical Outcomes of Discordant Lesions Between Fractional Flow Reserve and Nonhyperemic Pressure Ratios in Clinical Practice: The J-PRIDE Registry.

Background: Limited large-scale, real-world data exist on the prevalence and clinical impact of discordance between fractional flow reserve (FFR) and nonhyperemic pressure ratios (NHPRs).

Methods: The J-PRIDE registry (Clinical Outcomes of Japanese Patients With Coronary Artery Disease Assessed by Resting Indices and Fractional Flow Reserve: A Prospective Multicenter Registry) prospectively enrolled 4304 lesions in 3200 patients from 20 Japanese centers. The lesions were classified into FFR+/NHPR-, FFR-/NHPR+, FFR+/NHPR+, or FFR-/NHPR groups according to cutoff values of 0.

Association Between Maximum Lifetime Body Mass Index and Nailfold Capillary Changes in Patients With Type 2 Diabetes Mellitus.

Objective: Microvascular changes, such as crossing nailfold capillaries, could be crucial for linking maximum lifetime body mass index (BMI) and microvascular complications in patients with type 2 diabetes mellitus (T2DM). However, the relationship between maximum lifetime BMI and microvascular changes remains elusive. This study aimed to elucidate the relationship between maximum lifetime BMI and the percentage of crossing nailfold capillaries among patients with T2DM.

Clinical characteristics of individuals stratified by the number of answered items on the 25-question Geriatric Locomotive Function Scale.

The 25-question Geriatric Locomotive Function Scale (GLFS-25) is a tool to identify locomotive syndrome, however, this tool is associated with the problem of a low complete response rate. We conducted this cross-sectional study of 2,474 community-dwelling residents to investigate the clinical characteristics of individuals who are prone to provide incomplete responses to the GLFS-25 questionnaire. The participants were divided into the following four groups based on the number of the GLFS-25 items they answered: 0 (n=279), 1-21 (n=36), 22-24 (n=273), and 25 (n=1,886).

Intravenous branched-chain amino acid administration for the acute treatment of hepatic encephalopathy: a systematic review and meta-analysis.

Background: Hepatic encephalopathy (HE) is a severe complication of acute hepatic failure requiring urgent critical care management. Branched-chain amino acids (BCAAs) such as leucine, isoleucine, and valine have been investigated as potential treatments to improve outcomes in patients with acute HE. However, the effectiveness of BCAA administration during the acute phase remains unclear.

Epidemiology and treatment trends for acute encephalopathy under the impact of SARS-CoV-2 pandemic based on a prospective multicenter consecutive case registry.

Background: Acute encephalopathy is a severe condition predominantly affecting children with viral infections. The purpose of this study was to elucidate the epidemiology, treatment, and management of acute encephalopathy. The study also aimed to understand how the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic has affected epidemiological trends.

The N-Terminal Fragment of Urine Titin Is Not a Product of Degradation by Calpain 3.

Introduction: A 20 kDa fragment at the N-terminus of titin is highly excreted in the urine of patients with Duchenne muscular dystrophy (DMD), making urine titin a prominent biomarker for muscle breakdown. This N-terminal fragment is presumed to be a product of degradation by a protein-degrading enzyme, calpain 3; however, whether calpain 3 is required remains unclear. We aimed to determine whether urine titin elevation occurs in the absence of calpain 3.