Hypomyelinating Leukodystrophy 14 (HLD14)-Related UFC1 p.Arg23Gln Decreases Cell Morphogenesis: A Phenotype Reversable with Hesperetin.

Introduction: In the central nervous system (CNS), proper interaction between neuronal and glial cells is crucial for the development of mature nervous tissue. Hypomyelinating leukodystrophies (HLDs) are a group of genetic CNS disorders characterized by hypomyelination and/or demyelination. In these conditions, genetic mutations disrupt the biological functions of oligodendroglial cells, which are responsible for wrapping neuronal axons with myelin sheaths.

Early radiological reduction of periventricular anastomosis after direct bypass surgery for adult moyamoya disease.

Objective: Periventricular anastomosis (PA), a recently recognized cause of hemorrhage in moyamoya disease, is reducible after bypass surgery. The timing of the reduction, however, remains poorly understood. The objectives of the present study were to demonstrate radiological reduction of PA occurring within 48 hours after surgery and to identify factors associated with reduction.

Ten new resin glycosides, ipoalbins I-X, from Ipomoea alba seeds.

Ipomoea alba L. (Convolvulaceae) is an annual vine native to tropical America that is cultivated primarily for ornamental purposes. Its seeds are used in traditional medicine as a laxative, and young shoots are consumed as food.

Plasma and serum concentrations of VEGF-A121, but not of VEGF-A165, increase post-bevacizumab administration.

Background: VEGF-A concentrations were measured in the blood of bevacizumab-treated cancer patients in previous studies, but a consensus has not formed that would develop VEGF-A into a clinical biomarker. Recently, methods to strictly distinguish between the VEGF-A isoforms have been developed but have not yet been applied to cancer patients undergoing bevacizumab treatment.

Methods: An ELISA that strictly distinguishes between VEGF-A121 and VEGF-A165-the major isoforms of VEGF-A-and a commercially available ELISA for VEGF-A are used to determine the concentration of VEGF-A121, VEGF-A165, and VEGF-A in the blood of 12 patients with advanced colorectal cancer receiving bevacizumab therapy.

The Vascular Endothelial Growth Factor-A121/Vascular Endothelial Growth Factor-A165 Ratio as a Predictor of the Therapeutic Response to Immune Checkpoint Inhibitors in Gastric Cancer.

Background/objectives: The response rate to immune checkpoint inhibitor (ICI) therapy is limited. Further, there is a need to discover biomarkers to predict therapeutic efficacy. The vascular endothelial growth factor (VEGF) is strongly associated with intra-tumoral immunity; however, its utility as a marker remains unknown.

Deterioration of the Defecation Status after Hospitalization in Patients with Type 2 Diabetes is Associated with Albuminuria Rather than Diabetic Neuropathy.

Objective To determine the clinical background factors of patients with type 2 diabetes who showed deterioration of defecation status after hospitalization. Methods The defecation status of 128 patients with type 2 diabetes who were admitted to our department for diabetes education was evaluated for 7 days after hospitalization. New-onset constipation was diagnosed when patients without constipation before hospitalization met the criteria for constipation after hospitalization.

Screening of promising chemotherapeutic candidates from plants against human adult T-cell leukaemia/lymphoma (IX): active principles from .

During the screening of novel chemotherapeutic candidates from plants against adult T-cell leukaemia/lymphoma (ATL), we found that extracts of plants in the Solanaceae, Annonaceae, Apocynaceae, and Rutaceae families showed anti-proliferative activity in the MT-1 and MT-2 cell lines. We have isolated active compounds from these plants in the present research because Cupressaceae plants showed potent anti-proliferative activity in the cell lines. We attempted to isolate the active compounds from the leaves of .

Efficacy of high-resolution vessel wall MRI in the postoperative assessment of intracranial aneurysms following flow diversion treatment.

Objective: Certain patients must undergo frequent postoperative digital subtraction angiography (DSA) after flow diversion (FD) therapy. No imaging modality with an efficacy comparable to that of DSA has been established. This study was conducted to determine the efficacy of contrast-enhanced delay alternating with nutation for tailored excitation (DANTE) T1-sampling perfection with application-optimized contrasts by using different flip angle evolution (SPACE), a high-resolution vessel wall magnetic resonance imaging technique for evaluating the occlusion status of intracranial aneurysms after FD treatment, with DSA serving as the reference standard.

Eleven new glycosidic acid methyl esters from the crude resin glycoside fraction of Ipomoea alba seeds.

Resin glycosides are characteristic of plants of the Convolvulaceae family and are well-known purgative ingredients in crude drugs, such as Rhizoma Jalapae, Orizaba Jalapa Tuber, and Pharbitidis Semen, which are used in traditional medicine and derived from plants belonging to this family. Isolated resin glycosides have demonstrated diverse biological activities, including antibacterial, ionophoric, anti-inflammatory, antiviral, and multidrug-resistance-modulating properties, as well as cytotoxicity against cancer cells. These compounds consist of hydroxyl fatty acid oligoglycosides (glycosidic acids), with portions of the saccharide moieties acylated with some organic acids to form the core structure.

High-risk pathogenic germline variants in blood relatives of BRCA1/2 negative probands.

Background: Tailored, preventive cancer care requires the identification of pathogenic germline variants (PGVs) among potentially at-risk blood relatives (BRs). Cascade testing is carried out for BRs of probands who are positive for PGVs of an inherited cancer but not for negative probands. This study was conducted to examine the prevalence of PGVs for BRs of PGV-negative probands.

Comparison of surgical outcomes of da Vinci surgical systems X and Xi: A single-center study.

Background: The da Vinci surgical systems (X and Xi) are fourth-generation systems marketed by Intuitive Inc. The X system is less expensive than the Xi system. This study compared the surgical outcomes of patients who underwent hysterectomy using the X and Xi systems.

RNA editing enzyme ADAR2 regulates P-glycoprotein expression in murine breast cancer cells through the circRNA-miRNA pathway.

Among the various RNA modifications, adenosine-to-inosine RNA editing, catalyzed by adenosine deaminase acting on RNA (ADAR) family, ADAR1 and ADAR2, is the most common nucleotide conversion in mammalian cells. The pathological relevance of ADAR expression has been highlighted in recent human genetic studies. Low expression of the ADAR2 gene is correlated with a poor prognosis in breast cancer patients, but the underlying mechanism remains enigmatic.

Efficacy of very-low-dose oral food challenge in children with severe hen egg allergy: A retrospective, single-center case series.

Background: To avoid complete elimination of hen eggs (HE) from diet, we introduced a very-low-dose (VLD) oral food challenge (OFC) in patients with severe HE allergy in 2019. Herein, we investigated the efficacy of VLD HE OFC for achieving the full dose OFC.

Methods: Patients with an overt allergic reaction to LD (1/32 HE [≤100 mg]) or less, egg white (EW) protein within 6 months were included.

Effectiveness of dose-dense methotrexate, vinblastine, doxorubicin, and cisplatin as compared to gemcitabine-based regimens as neoadjuvant chemotherapy for oncologic outcomes in muscle-invasive bladder cancer cases-Single-center study in Japan.

Objectives: To compare the efficacy and safety of dose-dense methotrexate, vinblastine, doxorubicin, and cisplatin (dd-MVAC) with gemcitabine-based regimens for neoadjuvant chemotherapy (NAC) in muscle-invasive bladder cancer (MIBC) patients treated in Japan.

Methods: Data for MIBC patients who received NAC-dd-MVAC followed by a radical cystectomy from June 2019 to May 2023 performed at our hospital were analyzed. For comparisons, data for MIBC patients who received NAC gemcitabine and cisplatin (GC) or gemcitabine and carboplatin (GCarbo) therapy between January 2010 and March 2019 were also obtained.

Hepatocyte Intrinsic Innate Antiviral Immunity against Hepatitis Delta Virus Infection: The Voices of Bona Fide Human Hepatocytes.

The pathogenesis of viral infection is attributed to two folds: intrinsic cell death pathway activation due to the viral cytopathic effect, and immune-mediated extrinsic cellular injuries. The immune system, encompassing both innate and adaptive immunity, therefore acts as a double-edged sword in viral infection. Insufficient potency permits pathogens to establish lifelong persistent infection and its consequences, while excessive activation leads to organ damage beyond its mission to control viral pathogens.

Common and distinct risk profiles of asymptomatic extra- and intracranial atherosclerosis in the Nagahama cohort.

Background And Purpose: Atherosclerotic burden increases the risk of both extracranial internal carotid artery stenosis (ICS) and intracranial large artery disease (ICAD). However, the differences in risk profiles have not been thoroughly investigated.

Methods: Participants were recruited from the Nagahama study cohort in Japan.

Autism Spectrum Disorder- and/or Intellectual Disability-Associated Semaphorin-5A Exploits the Mechanism by Which Dock5 Signalosome Molecules Control Cell Shape.

Autism spectrum disorder (ASD) is a neurodevelopmental disorder that includes autism, Asperger's syndrome, and pervasive developmental disorder. Individuals with ASD may exhibit difficulties in social interactions, communication challenges, repetitive behaviors, and restricted interests. While genetic mutations in individuals with ASD can either activate or inactivate the activities of the gene product, impacting neuronal morphogenesis and causing symptoms, the underlying mechanism remains to be fully established.

Characterization of immortalized ovarian epithelial cells with BRCA1/2 mutation.

We aimed to elucidate the mechanism underlying carcinogenesis by comparing normal and BRCA1/2-mutated ovarian epithelial cells established via Sendai virus-based immortalization. Ovarian epithelial cells (normal epithelium: Ovn; with germline BRCA1 mutation: OvBRCA1; with germline BRCA2 mutation: OvBRCA2) were infected with Sendai virus vectors carrying three immortalization genes (Bmi-1, hTERT, and SV40T). The immunoreactivity to anti-epithelial cellular adhesion molecule (EpCAM) antibodies in each cell line and cells after 25 passages was confirmed using flow cytometry.

Outcome of robot-assisted surgery for stage IA endometrial cancer compared to open and laparoscopic surgeries: a retrospective study at a single institution.

Few studies have compared the efficacy of robot-assisted, laparoscopic, and open surgeries for endometrial cancer. When considering the position of robotic surgery in Japan, it was necessary to determine whether it was effective or not. We aimed to compare the efficacy and safety of these three types of surgeries for early-stage endometrial cancer.

Four new resin glycosides from Ipomoea muricata seeds: muricatins XIV-XVII.

Ipomoea muricata (L.) Jacq. seeds (Convolvulaceae) are used as a traditional laxative and carminative medicine.