Gene therapy ameliorates bowel dysmotility and enteric neuron degeneration and extends survival in lysosomal storage disorder mouse models.

Children with neurodegenerative disease often have debilitating gastrointestinal symptoms. We hypothesized that this may be due at least in part to underappreciated degeneration of neurons in the enteric nervous system (ENS), the master regulator of bowel function. To test this hypothesis, we evaluated mouse models of neuronal ceroid lipofuscinosis type 1 and 2 (CLN1 and CLN2 disease, respectively), neurodegenerative lysosomal storage disorders caused by deficiencies in palmitoyl protein thioesterase-1 and tripeptidyl peptidase-1, respectively.

Lineage-specific intersection of endothelin and GDNF signaling in enteric nervous system development.

Two major ligand-receptor signaling axes - endothelin Edn3 and its receptor Ednrb, and glial-derived neurotrophic factor (GDNF) and its receptor Ret - are required for migration of enteric nervous system (ENS) progenitors to the hindgut. Mutations in either component cause colonic aganglionosis, also called Hirschsprung disease. Here, we have used Wnt1Cre and Pax2Cre in mice to show that these driver lines label distinct ENS lineages during progenitor migration and in their terminal hindgut fates.

Ion sensors based on organic semiconductors acting as quasi-reference electrodes.

Thin-film devices that transduce the chemical activity of ions into electronic signals are essential components in various applications, including healthcare diagnostics and environmental monitoring. Combinations of organic semiconductors (OSCs) and ion-selective materials have been explored for developing solution-processable ion sensors. However, the necessity of reference electrodes (REs) and operational stability in ion-permeable OSCs have posed questions regarding whether reliable measurements with thin-film components are attainable with OSCs.

A natural loss-of-function deletion of the cytohesin 1 (Cyth1) gene in BALB/cByJ mice does not impact cardiomyocyte polyploidy.

Mammalian cardiomyocytes (CMs) mostly become polyploid shortly after birth. Because this feature may relate to several aspects of heart biology, including regeneration after injury, the mechanisms that cause polyploidy are of interest. BALB/cJ and BALB/cByJ mice are highly related sister strains that diverge substantially in CM ploidy.

Efficacy of Computed Tomography-Based Evaluation of Myocardial Extracellular Volume Combined With Red Flags for Early Screening of Concealed Cardiac Amyloidosis in Patients With Atrial Fibrillation.

Background: The prevalence of transthyretin amyloid cardiomyopathy (ATTR-CM) in atrial fibrillation (AF) patients remains unclear. We explored the efficacy of computed tomography-based myocardial extracellular volume (CT-ECV) combined with red flags for the early screening of concealed ATTR-CM in AF patients undergoing catheter ablation.

Methods and results: Patients referred for AF ablation at Oita University Hospital were prescreened using the red-flag signs defined by echocardiographic or electrocardiographic findings, medical history, symptoms, and blood biochemical findings.

An Unusual Abscess Associated with Gallbladder Perforation Successfully Treated with Percutaneous Transhepatic Gallbladder Drainage and Endoscopic Ultrasound-guided Abscess Drainage.

Abscesses associated with gallbladder perforation are often confined to the peri-gallbladder region. We herein report a rare case of gallbladder perforation in which the abscess cavity extended into the left upper quadrant. A 79-year-old woman developed gallbladder perforation secondary to acalculous cholecystitis.

A novel approach to typical atrioventricular nodal reentrant tachycardia with high-resolution mapping using the CARTO 3 cardiac mapping system.

Background: We hypothesized that high-resolution activation mapping during sinus rhythm (SR) in Koch's triangle (KT) can be used to describe the most delayed atrial potential around the atrioventricular node and evaluated whether ablation targeting of this potential is safe and effective for the treatment of patients with typical atrioventricular nodal reentrant tachycardia (AVNRT).

Methods: We conducted a prospective, non-randomized, observational study using high-resolution activation mapping from the sinus node to KT with a PENTARAY or OCTARAY catheter using the CARTO 3 cardiac mapping system (Biosense Webster) during SR in 62 consecutive patients (22 men; age [mean ± standard deviation] = 55 ± 14 years) treated for typical AVNRT at our institution from August 2021 to March 2023.

Results: In all cases, the most delayed atrial potential was observed near the His potential within KT.

Dual linear lesions for right atrial flutter after mitral valve surgery via the superior transseptal approach.

Background: The superior transseptal approach (STA) for mitral valve surgery is associated with a higher risk of developing macroreentrant incisional atrial flutter (AFL) than the left atrial approach. This study aimed to describe the linear lesions for the complex AFL circuit after the STA and to propose an option for the linear ablation target site.

Methods: Of the 26 patients who underwent radiofrequency catheter ablation for AFL after mitral valve surgery, data from seven patients with STA incisions were retrospectively analyzed.

Air/liquid interfacial formation process of conductive metal-organic framework nanosheets.

The air/liquid interface is a superior platform to create nanosheets of materials by promoting spontaneous two-dimensional growth of components. Metal-organic frameworks (MOFs)-intrinsically porous crystals-with π-conjugated triphenylene-based ligands show high electrical conductivities. Forming nanosheets of such conductive MOFs should enable their use in electronic devices.

Purkinje Cardiomyocytes of the Adult Ventricular Conduction System Are Highly Diploid but Not Uniquely Regenerative.

Adult hearts are characterized by inefficient regeneration after injury, thus, the features that support or prevent cardiomyocyte (CM) proliferation are important to clarify. Diploid CMs are a candidate cell type that may have unique proliferative and regenerative competence, but no molecular markers are yet known that selectively identify all or subpopulations of diploid CMs. Here, using the conduction system expression marker Cntn2-GFP and the conduction system lineage marker Etv1Cre, we demonstrate that Purkinje CMs that comprise the adult ventricular conduction system are disproportionately diploid (33%, vs.

Prognostic implications of post-discharge hemodynamic congestion assessed by peripheral venous pressure among patients discharged from acute heart failure.

Background: Congestion is a major cause of hospitalization for heart failure (HF). Peripheral venous pressure (PVP) strongly correlates with right atrial pressure. We recently reported that high PVP at discharge portends a poor prognosis in patients hospitalized for HF.

Apposition compound-eye image scanner by glass plate optics.

We propose a design approach for a thin image scanner using the concept of an apposition compound eye comprising many imaging units that take only one pixel image. Although light shielding between adjacent imaging units is always one of the main issues for an artificial compound eye, a simple plane structure using three aperture array layers on two glued glass plates prevents such stray light. Our prototyped scanner, with only 6.

Uniaxially Oriented Electrically Conductive Metal-Organic Framework Nanosheets Assembled at Air/Liquid Interfaces.

Although most metal-organic frameworks (MOFs)─highly porous crystalline metal complex networks with structural and functional varieties─are electrically insulating, high electrical conduction has been recently demonstrated in MOFs while retaining permanent porosity. Usability of electronically active MOFs effectively emerges when they are created in a thin-film state as required in major potential applications such as chemiresistive sensors, supercapacitors, and electrode catalysts. Thin-film morphology including crystallinity, thickness, density, roughness, and orientation sensitively influences device performance.

Electronic excitation spectra of organic semiconductor/ionic liquid interface by electrochemical attenuated total reflectance spectroscopy.

The interface of organic semiconductor films is of particular importance with respect to various electrochemical devices such as transistors and solar cells. In this study, we developed a new spectroscopic system, namely electrochemical attenuated total reflectance ultraviolet (EC-ATR-UV) spectroscopy, which can access the interfacial area. Ionic liquid-gated organic field-effect transistors (IL-gated OFETs) were successfully fabricated on the ATR prism.

Strongly correlated superconductivity in a copper-based metal-organic framework with a perfect kagome lattice.

Metal-organic frameworks (MOFs), which are self-assemblies of metal ions and organic ligands, provide a tunable platform to search a new state of matter. A two-dimensional (2D) perfect kagome lattice, whose geometrical frustration is a key to realizing quantum spin liquids, has been formed in the π - conjugated 2D MOF [Cu(CS)] (Cu-BHT). The recent discovery of its superconductivity with a critical temperature of 0.

Plutonium dioxide particle imaging using a high-resolution alpha imager for radiation protection.

The internal exposure of workers who inhale plutonium dioxide particles in nuclear facilities is a crucial matter for human protection from radiation. To determine the activity median aerodynamic diameter values at the working sites of nuclear facilities in real time, we developed a high-resolution alpha imager using a ZnS(Ag) scintillator sheet, an optical microscope, and an electron-multiplying charge-coupled device camera. Then, we designed and applied a setup to measure a plutonium dioxide particle and identify the locations of the individual alpha particles in real time.

Angiographic evaluation of radial artery injury after transradial approach for percutaneous coronary intervention.

The transradial approach for percutaneous coronary intervention (TRA-PCI) has been increasingly gaining popularity in clinical practice. However, its association with risk for long-term radial artery injury has not been yet thoroughly defined. We retrospectively examined the patients undergoing radial artery angiography (RAG) after TRA-PCI to determine the incidence and risk factors of radial artery injury.

The prevalent I686T human variant and loss-of-function mutations in the cardiomyocyte-specific kinase gene TNNI3K cause adverse contractility and concentric remodeling in mice.

TNNI3K expression worsens disease progression in several mouse heart pathology models. TNNI3K expression also reduces the number of diploid cardiomyocytes, which may be detrimental to adult heart regeneration. However, the gene is evolutionarily conserved, suggesting a beneficial function that has remained obscure.

Damage-free Metal Electrode Transfer to Monolayer Organic Single Crystalline Thin Films.

Solution-processed organic thin film transistors (OTFTs) are an essential building block for next-generation printed electronic devices. Organic semiconductors (OSCs) that can spontaneously form a molecular assembly play a vital role in the fabrication of OTFTs. OTFT fabrication processes consist of sequential deposition of functional layers, which inherently brings significant difficulties in realizing ideal properties because underlayers are likely to be damaged by application of subsequent layers.

High-performance, semiconducting membrane composed of ultrathin, single-crystal organic semiconductors.

Thin film transistors (TFTs) are indispensable building blocks in any electronic device and play vital roles in switching, processing, and transmitting electronic information. TFT fabrication processes inherently require the sequential deposition of metal, semiconductor, and dielectric layers and so on, which makes it difficult to achieve reliable production of highly integrated devices. The integration issues are more apparent in organic TFTs (OTFTs), particularly for solution-processed organic semiconductors due to limits on which underlayers are compatible with the printing technologies.