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.

Urinary titin reflects the severity of walking ability, muscle strength, and muscle and cardiac damage in patients with Becker muscular dystrophy.

Background: Becker muscular dystrophy (BMD) is a dystrophinopathy caused by a pathological variant of the DMD gene. Urinary titin, a degradation product of the giant protein titin present in muscle sarcomeres, has been used as a biomarker to reflect muscle degradation in Duchenne muscular dystrophy, a more severe dystrophinopathy. However, the clinical significance of urinary titin levels in BMD remains unclear.

Neuronal regeneration in the area postrema of adult mouse medulla oblongata following glutamate-induced neuronal elimination.

Neural stem cells and/or progenitor cells (NSCs/NPCs) in the subventricular and subgranular zones of the adult mammal forebrain generate new neurons and are involved in partial repair after injury. Recently, NSCs/NPCs were identified in the area postrema (AP) of the medulla oblongata of the hindbrain. In this study, we used the properties of fenestrate capillaries to observe specific neuronal elimination in the AP of adult mice and investigated subsequent neuronal regeneration by neurogenesis.

Magnetic structure of the noncentrosymmetric magnet SrMnSiO through irreducible representation and magnetic space group analyses.

Magnetic structures of the noncentrosymmetric magnet SrMnSiO were examined through neutron diffraction for powder and single-crystalline samples, as well as magnetometry measurements. All allowed magnetic structures for space group P42m with the magnetic wavevector q = (0, 0, ½) were refined via irreducible representation and magnetic space group analyses. The compound was refined to have in-plane magnetic moments within the magnetic space group Cmc2.

High Proton Conduction in the Octahedral Layers of Fully Hydrated Hexagonal Perovskite-Related Oxides.

Proton conductors have potential applications such as fuel cells, electrolysis cells, and sensors. These applications require new materials with high proton conductivity and high chemical stability at intermediate temperatures. Herein we report a series of new hexagonal perovskite-related oxides, BaAlSnO ( = Gd, Dy, Ho, Y, Er, Tm, and Yb).

Prediction of Mismatch Repair Status in Endometrial Cancer from Histological Slide Images Using Various Deep Learning-Based Algorithms.

The application of deep learning algorithms to predict the molecular profiles of various cancers from digital images of hematoxylin and eosin (H&E)-stained slides has been reported in recent years, mainly for gastric and colon cancers. In this study, we investigated the potential use of H&E-stained endometrial cancer slide images to predict the associated mismatch repair (MMR) status. H&E-stained slide images were collected from 127 cases of the primary lesion of endometrial cancer.

Longitudinal data of serum creatine kinase levels and motor, pulmonary, and cardiac functions in 337 patients with Duchenne muscular dystrophy.

Introduction/aims: Duchenne muscular dystrophy (DMD) presents with skeletal muscle weakness, followed by cardiorespiratory involvement. The need for longitudinal data regarding DMD that could serve as a control for determining treatment efficacy in clinical trials has increased notably. The present study examined the longitudinal data of Japanese DMD patients collectively and assessed individual patients with pathogenic variants eligible for exon-skipping therapy.

Brothers with Becker muscular dystrophy show discordance in skeletal muscle computed tomography findings: A case report.

Becker muscular dystrophy is caused by mutations and is characterized by progressive muscle atrophy. The wide variations observed in muscle atrophy progression in Becker muscular dystrophy are considered multifactorial, including differences in mutations and environmental factors. In this case, two brothers, aged 2 and 3 years, had the identical mutation, confirming their Becker muscular dystrophy diagnosis.

Thermal multiferroics in all-inorganic quasi-two-dimensional halide perovskites.

Multiferroic materials, particularly those possessing simultaneous electric and magnetic orders, offer a platform for design technologies and to study modern physics. Despite the substantial progress and evolution of multiferroics, one priority in the field remains to be the discovery of unexplored materials, especially those offering different mechanisms for controlling electric and magnetic orders. Here we demonstrate the simultaneous thermal control of electric and magnetic polarizations in quasi-two-dimensional halides (K,Rb)MnCl, arising from a polar-antipolar transition, as evidenced using both X-ray and neutron powder diffraction data.

Newborn Screening for Spinal Muscular Atrophy: A 2.5-Year Experience in Hyogo Prefecture, Japan.

Newborn screening (NBS) for spinal muscular atrophy (SMA) is necessary, as favorable outcomes can be achieved by treatment with disease-modifying drugs in early infancy. Although SMA-NBS has been initiated in Japan, its clinical results have not been fully reported. We report the findings of the initial 2.

Electrocardiographic R wave amplitude in V6 lead as a predictive marker of cardiac dysfunction in Duchenne muscular dystrophy.

Purpose: Duchenne muscular dystrophy (DMD) is an inherited muscular disease characterized by progressive and fatal muscle weakness. Electrocardiographic (ECG) abnormalities, including abnormal R wave amplitudes are frequently observed in DMD. However, clinical implications of abnormal R wave amplitudes remain unclear.

Entanglement Renyi Entropy of Two Disjoint Intervals for Large Liouville Field Theory.

Entanglement entropy (EE) is a quantitative measure of the effective degrees of freedom and the correlation between the sub-systems of a physical system. Using the replica trick, we can obtain the EE by evaluating the entanglement Renyi entropy (ERE). The ERE is a -analogue of the EE and expressed by the replicated partition function.

Impact after the Change from Voluntary to Universal Oral Rotavirus Vaccination on Consecutive Emergency Department Visits for Acute Gastroenteritis among Children in Kobe City, Japan (2016-2022).

Rotavirus (RV) is the leading cause of acute gastroenteritis (AGE), particularly in infants. In 2006, the high efficacy of oral RV vaccines (RVVs, RotarixTM and RotaTeqTM) was demonstrated. Voluntary RVV started in Japan in 2011, and in October 2020 were launched as universal oral RVVs in Japan.

Pennelliiside D, a New Acyl Glucose from and Chemical Synthesis of Pennelliisides.

Acyl glucoses are a group of specialized metabolites produced by Solanaceae. , a wild-type tomato plant, produces acyl glucoses in its hair-like epidermal structures known as trichomes. These compounds have been found to be herbicides, microbial growth inhibitors, or allelopathic compounds.

Direct production of polyhydroxybutyrate and alginate from crude glycerol by Azotobacter vinelandii using atmospheric nitrogen.

While biodiesel is drawing attention as an eco-friendly fuel, the use of crude glycerol, a byproduct of the fuel production process, has increasingly become a concern to be addressed. Here we show the development of a low-cost fermentation technology using an atmospheric nitrogen-fixing bacterium to recycle crude glycerol into functional biopolymers. Azotobacter vinelandii showed substantial growth on tap water-diluted crude glycerol without any pretreatment.

SrVFeO: A Vacancy-Ordered Fe-Based Perovskite Exhibiting Room-Temperature Magnetoresistance.

We report room-temperature (RT) magnetoresistance (MR) in a novel Fe-based perovskite, SrVFeO. This compound contains ordered oxygen vacancies in every fifth primitive perovskite (111) plane, leading to a layered structure consisting of triple-octahedral and double-tetrahedral layers. Along with the oxygen vacancies, the transition-metal ions are also ordered: the octahedral sites are occupied by 100% of Fe ions, while the tetrahedral sites are occupied by 25% of Fe ions and 75% of V ions.

Synthesis of Hydride-Doped Perovskite Stannate with Visible Light Absorption Capability.

Narrow-gap semiconductors with visible light absorption capability have attracted attention as photofunctional materials. H-doped BaSnYO containing Sn(II) species was recently reported to absorb visible light up to 600 nm, which represents the first demonstration of oxyhydride-based visible-light-absorbers. In the present study, a more detailed investigation was made to obtain information on the synthesis and properties of H-doped perovskite-type stannate with respect to the A-site cation of the material and the preparation conditions.

A sandwich ELISA kit reveals marked elevation of titin N-terminal fragment levels in the urine of mdx mice.

The mdx mouse is a model of Duchenne muscular dystrophy (DMD), a fatal progressive muscle wasting disease caused by dystrophin deficiency, and is used most widely in preclinical studies. Mice with dystrophin deficiency, however, show milder muscle strength phenotypes than humans. In human, the introduction of a sandwich enzyme-linked immunosorbent assay (ELISA) kit revealed a more than 700-fold increase in titin N-terminal fragment levels in the urine of pediatric patients with DMD.

Serum cardiac troponin I is a candidate biomarker for cardiomyopathy in Duchenne and Becker muscular dystrophies.

Introduction/aims: Serum cardiac troponin I (cTnI), its relation to cardiomyopathy, and the contribution of the ACTN3 genotype to serum levels of cTnI in Duchenne and Becker muscular dystrophy (DMD and BMD, respectively) remain unknown. In this study we aimed to reveal the characteristics of cTnI, assess whether cTnI is a biomarker for cardiomyopathy in these dystrophinopathies, and evaluate the contribution of the ACTN3 genotype to the serum levels of cTnI in DMD patients.

Methods: Serum cTnI values obtained from 127 DMD and 47 BMD patients were analyzed retrospectively.

Effects of Soaking Tempe in Vinegar on Metabolome and Sensory Profiles.

Tempe is a fermented soybean food that is globally renowned for its high protein content. Methods of preparing tempe vary worldwide, and include soaking in vinegar before fermentation. This study aimed to determine the effects of soaking in vinegar by metabolome analysis, gas chromatography/mass spectrometry, and sensory attribute evaluation.

A screening assistance system for cervical cytology of squamous cell atypia based on a two-step combined CNN algorithm with label smoothing.

Background: Although many cervical cytology diagnostic support systems have been developed, it is challenging to classify overlapping cell clusters with a variety of patterns in the same way that humans do. In this study, we developed a fast and accurate system for the detection and classification of atypical cell clusters by using a two-step algorithm based on two different deep learning algorithms.

Methods: We created 919 cell images from liquid-based cervical cytological samples collected at Sapporo Medical University and annotated them based on the Bethesda system as a dataset for machine learning.

Pretreatment of extruded Napier grass byhydrothermal process with dilute sulfuric acid and fermentation using a cellulose-hydrolyzing and xylose-assimilating yeast for ethanol production.

One of the potential bioresources for bioethanol production is Napier grass, considering its high cellulose and hemicellulose content. However, the cost of pretreatment hinders the bioethanol produced from being economical. This study examines the effect of hydrothermal process with dilute acid on extruded Napier grass, followed by enzymatic saccharification prior to simultaneous saccharification and co-fermentation (SScF).

Triplon current generation in solids.

A triplon refers to a fictitious particle that carries angular momentum S=1 corresponding to the elementary excitation in a broad class of quantum dimerized spin systems. Such systems without magnetic order have long been studied as a testing ground for quantum properties of spins. Although triplons have been found to play a central role in thermal and magnetic properties in dimerized magnets with singlet correlation, a spin angular momentum flow carried by triplons, a triplon current, has not been detected yet.