An autopsy case of diffuse atypical argyrophilic grain disease (AGD) with presenile onset and three-year course of motor and cognitive impairment.

We report a case of argyrophilic grain disease (AGD) with unique clinical and pathological presentations. A 52-year-old man presented with spastic quadriparesis, bulbar palsy, and mild cognitive decline. His condition deteriorated rapidly and he died of pneumonia three years from onset. Pathologically, neuronal degeneration was involved severely in the amygdala, ambient gyrus, midbrain tegmentum, and reticular formation. The neurons of the temporal lobe, cingulate gyrus, brainstem, and spinal gray matter were also lost moderately. There was diffuse 4-repeat tau-pathology with argyrophilic grains. There were pretangles, globose-type neurofibrillary tangles, and coiled bodies in the cerebral cortices, basal ganglia, thalami, brainstem, and the spinal cord except for the cerebellar cortices. There was no pathologic mutation in MAPT.

Room-Temperature Type-II Multiferroic Phase Induced by Pressure in Cupric Oxide.

According to previous theoretical work, the binary oxide CuO can become a room-temperature multiferroic via tuning of the superexchange interactions by application of pressure. Thus far, however, there has been no experimental evidence for the predicted room-temperature multiferroicity. Here, we show by neutron diffraction that the multiferroic phase in CuO reaches 295 K with the application of 18.5 GPa pressure. We also develop a spin Hamiltonian based on density functional theory and employing superexchange theory for the magnetic interactions, which can reproduce the experimental results. The present Letter provides a stimulus to develop room-temperature multiferroic materials by alternative methods based on existing low temperature compounds, such as epitaxial strain, for tunable multifunctional devices and memory applications.

Humidity Sensitivity of Chemically Synthesized ZnAl2O4/Al.

Humidity sensitivity is evaluated for chemically synthesized ZnAl2O4/Al devices. We succeeded in synthesizing the ZnAl2O4/Al device by applying chemical techniques only. Hydrothermal treatment for the anodized aluminum (AlOx/Al) gives us the device of the ZnAl2O4/Al structure. All fabrication processes were conducted under 400 °C. The key was focusing on ZnAl2O4 as the sensing material instead of MgAl2O4, which is generally investigated as the humidity sensor. The evaluation of this ZnAl2O4/Al device clarified its effectiveness as a sensor. Both electrical capacitance, Cp, and the resistivity, Rp, measured by an LCR meter, obviously responded to the humidity with good sensitivity and appreciable repeatability. Our synthesis technique is possible in principle to improve on the process for the device with a complex structure providing a large surface area. These characteristics are believed to expand the application study of spinel aluminate devices as the sensor.

Synthesis of novel hexamolybdenum cluster-functionalized copper hydroxide nanocomposites and its catalytic activity for organic molecule degradation.

A novel heterogeneous catalytic nanomaterial based on a molybdenum cluster-based halide (MC) and a single-layered copper hydroxynitrate (CHN) was first prepared by colloidal processing under ambient conditions. The results of the elemental composition and crystalline pattern indicated that CHN was comprehensively synthesized with the support of the MC compound. The absorbing characteristic in the ultraviolet and near-infrared regions was promoted by both of the ingredients. The proper chemical interaction between the materials is a crucial reason to modify the structure of the MCs and only a small decrease in the magnetic susceptibility of CHN. The heterogeneous catalytic activity of the obtained MC@CHN material was found to have a high efficiency and excellent reuse when it is activated by hydrogen peroxide (H2O2) for the degrading reaction of the organic pollutant at room temperature. A reasonable catalytic mechanism was proposed to explain the distinct role of the copper compound, Mo6 compound, and H2O2 in the production of the radical hydroxyl ion. This novel nanomaterial will be an environmentally promising candidate for dye removal.

Spodiobacter cordis gen. nov. sp. nov., a member of the family Flavobacteriaceae isolated from patients with infective endocarditis.

Two gram-negative, catalase-negative, oxidase-positive strains (PAGU 1467T and PAGU 1468) isolated from patients with infective endocarditis were investigated to determine their taxonomic status. 16S rRNA gene sequence analysis indicated that the two strains were members of the Bergeyella-Chryseobacterium-Riemerella branch of the family Flavobacteriaceae. Strains PAGU 1467T and PAGU 1468 were highly related to each other (98.8% 16S rRNA gene sequence similarity). Phylogenetically closely-related species to PAGU 1467T comprised Bergeyella zoohelcum (95.0% 16S rRNA gene sequence similarity), Riemerella anatipestifer (94.3%) and Cloacibacterium normanense (94.3%). The major fatty acids of the two isolates were iso-C15:0 , iso-C17:0 3-OH and iso-C15:0 3-OH. The presence of C16:0 3-OH and iso-C15:0 2-OH allowed these isolates to be distinguished from B. zoohelcum. Menaquinone MK-6 was the only respiratory quinone in these organisms; this is a consistent characteristic of the family Flavobacteriaceae. The guanine-plus-cytosine content of the genomic DNA was 42.0%, which is higher than that of other close phylogenetic relatives. On the basis of their phenotypic properties and genetic distinctiveness, isolates PAGU 1467T and PAGU 1468 were classified within the novel genus Spodiobacter, as Spodiobacter cordis gen. nov., sp. nov., which is also the type species. The type strain of S. cordis is PAGU 1467T ( = CCUG 65564T = NBRC 109998T ).

Shape-Controlled Syntheses of Magnetite Microparticles and Their Magnetorheology.

Magnetic microspheres in a concentrated suspension can be self-assembled to form chain structures under a magnetic field, resulting in an enhanced viscosity and elasticity of the suspension (i.e., the magnetorheological (MR) effect). Recently, interest has been raised about the relationship between nonspherical particles, such as octahedral particles and the MR effect. However, experimental studies have not made much progress toward clarifying this issue due to the difficulty associated with synthesizing microparticles with well-defined shapes and sizes. Here, we presented a method for the shape-controlled synthesis of magnetite (Fe3O4) microparticles and investigated the MR effects of two suspensions prepared from the two shape-controlled samples of Fe3O4 microparticles. Our method, which was based on the polyol method, enabled the preparation of spherical and octahedral Fe3O4 microparticles with similar sizes and magnetic properties, through a reduction of α-FeOOH in a mixed solvent of ethylene glycol (a polyol) and water. The water played an important role in both the phase transition (α-FeOOH to Fe3O4) and the shape control. No substantial difference in the MR effect was observed between an octahedral-particle-based suspension and a spherical-particle-based one. Therefore, in this study, the shape of the microparticles did not strongly influence the MR effect, i.e., the properties of the chain structures.

Observation of the First Spin Crossover in an Iron(II) Complex with an S6 Coordination Environment: Tris[bis(N,N-diethylamino)carbeniumdithiocarboxylato]iron(II) Hexafluorophosphate.

For the first time, the spin-crossover (SCO) phenomenon has been observed in an FeII -S6 system in a tris(chelate)-type iron(II) complex with a zwitterionic sulfur donor bidentate, bis(N,N-diethylamino)carbeniumdithiocarboxylate (EtL), [FeII (EtL)3 ](PF6 )2 (1), as synthesized by the reaction of a precursor complex [FeII (CH3 CN)6 ](PF6 )2 with EtL. In the solid state, the high-spin (HS) d6 state at ambient temperature and the low-spin (LS) d6 state at temperatures lower than approximately 240 K were evidenced by magnetic measurements with SQUID and Mössbauer spectra in the temperature range 4-290 K. X-ray analyses of the crystals at various temperatures disclosed that the distorted trigonal prismatic coordination environments essentially do not change; however, contraction of Fe-S distances by approximately 10 % (0.22 Å), ordering of alkyl groups in EtL and PF6 - counteranions, and formation of significant intermolecular S⋅⋅⋅S interactions between adjacent molecules (average distances of 3.59 Å) take place during the transition from the HS to the LS state. A large decrease in the volume of the formula unit (78.1 Å3 ) might be responsible for the large activation barrier, thereby resulting in a slow phase transition upon cooling.

Evidence that glial cells attenuate G47R transthyretin accumulation in the central nervous system.

Amyloidogenic protein forms amyloid aggregations at membranes leading to dysfunction of amyloid clearance and amyloidosis. Glial cells function in the clearance and degradation of amyloid ß (Aß) in the brain. This study aimed to clarify the reason why amyloid transthyretin (ATTR) rarely accumulates in the CNS. We pathologically analyzed the relationship between amyloid deposition with basement membranes or glial cells in a rare case of ATTR leptomeningeal amyloidosis. In addition, we compared the cytotoxicity of ATTR G47R, the amyloidosis-causing mutation in the case studied (n = 1), and Aß in brains from patients with cerebral amyloid angiopathy (n = 6). In the subarachnoid space of the ATTR G47R case, most amyloids accumulated at the components of basement membranes. On the CNS surface, ATTR accumulations were retained by astrocytic end feet. In areas where glial end feet enveloped ATTR, ubiquitination and micro-vacuolation of ATTR was evident. The colocalization of GFAP and ubiquitin was also evident. The accumulation of ATTR G47R in the CNS was negatively correlated with the prevalence of astrocytes. Quantitatively, amyloid deposits along the vessels were mostly partial in cerebral Aß angiopathy cases and nearly complete along the basement membrane in the ATTR G47R case. The vascular expressions of type IV collagen and smooth muscle actin were severely reduced in areas with ATTR G47R deposition, but not in areas with Aß deposition. The vascular protein level recovered in the ATTR G47R case when vessels entered into areas of parenchyma that were rich in astrocytes. In addition, the strong interactions between the transthyretin variant and basement membranes may have led to dysfunction of transthyretin clearance and leptomeningeal amyloidosis. The present study was the first to show that glial cells may attenuate G47R transthyretin accumulation in the CNS.

Porphyromonas bronchialis sp. nov. Isolated from Intraoperative Bronchial Fluids of a Patient with Non-Small Cell Lung Cancer.

Porphyromonas strains, including Porphyromonas-like strains, have been isolated from oral and various other systemic infections. The characterization of such strains is a crucial issue, because such information contributes to both the taxonomy of anaerobic bacteria and the clinical aspects of infectious diseases. We previously isolated four Porphyromonas-like strains from intraoperative bronchial fluids of a patient with non-small cell lung cancer. This study aimed to characterize the genetic, biochemical and chemotaxonomic aspects of these isolates. Each strain only grew under anaerobic conditions and their colony morphology was convex, 0.1-1.0 mm in diameter, light gray, and slightly glistening colony, with no black or brown pigmentation on blood agar plates after five-day incubation. The pigmentation was helpful to differentiate the isolates from other Porphyromonas, as most of Porphyromonas species show the pigmentation. In the 16S rRNA gene phylogenetic analysis (98% sequence identity of isolates indicates the same species), the four isolates were closely related to one another (99.7-100.0%), but not related to Porphyromonas (P.) catoniae, the closest species (96.9%). In addition, the DNA-DNA hybridization data revealed less than 16% similarity values between a representative isolate and the P. catoniae, indicating that the strains were genetically independent. Biochemically, the isolates could be differentiated from closely related species, i.e., P. catoniae, P. gingivalis, P. gulae, and P. pogonae, with trypsin activity (negative only in the isolates) and leucine arylamidase activity (positive only in the isolates). We therefore propose a new species to include these isolates: Porphyromonas bronchialis sp. nov.

Bacterial sphingophospholipids containing non-hydroxy fatty acid activate murine macrophages via Toll-like receptor 4 and stimulate bacterial clearance.

Sphingobacterium spiritivorum has five unusual sphingophospholipids (SPLs). Our previous study determined the complete chemical structures of these SPLs. The compositions of the long-chain bases/fatty acids in the ceramide portion, isoheptadecasphingosine/isopentadecanoate or isoheptadecasphingosine/2-hydroxy isopentadecanoate, are characteristic. The immune response against bacterial lipid components is considered to play important roles in microbial infections. It is reported that several bacterial sphingolipids composed of ceramide are recognized by CD1-restricted T and NKT cells and that a non-peptide antigen is recognized by γδ T cells. In this study, we demonstrated that these bacterial SPLs activated murine bone marrow macrophages (BMMs) via Toll-like receptor (TLR) 4 but not TLR2, although they slightly activated CD1d-restricted NKT and γδT cells. Interestingly, this TLR 4-recognition pathway of bacterial SPLs involves the fatty acid composition of ceramide in addition to the sugar moiety. A non-hydroxy fatty acid composed of ceramide was necessary to activate murine BMMs. The bacterial survival was significantly higher in TLR4-KO mice than in TLR2-KO and wild-type mice. The results indicate that activation of the TLR4-dependent pathway of BMMs by SPLs induced an innate immune response and contributed to bacterial clearance.

Hereditary Hemorrhagic Telangiectasia with Recurrent Epistaxis, Telangiectasia, Hepatic Arteriovenous Malformation, and a Poorly Developed Middle Cerebral Artery in a Patient with a Novel Mutation in the ACVRL1 Gene.

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal-dominant vascular disorder characterized by intractable epistaxis, mucocutaneous telangiectasias, and arteriovenous malformations (AVMs) in multiple organs, including the lungs, liver, gastrointestinal tract, brain, and spinal cord. We herein report a 50-year-old Japanese man with HHT who experienced recurrent epistaxis, telangiectasia in the cornea, apex of the tongue and fingers; hepatic AVM; and a poorly developed main arterial trunk in the right middle cerebral artery. A genetic analysis revealed a novel heterozygous mutation in the activin A receptor-like type 1 gene, with a frameshift mutation in NM_000020.3:c.826_836del (p.Ile276ProfsTer112).

The mannan of Candida albicans lacking ß-1,2-linked oligomannosides increases the production of inflammatory cytokines by dendritic cells.

Mannans are mannose polymers attached to cell wall proteins in all Candida species, including the pathogenic fungus Candida albicans. Mannans are sensed by pattern recognition receptors expressed on innate immune cells. However, the detailed structural patterns affecting immune sensing are not fully understood because mannans have a complex structure that includes α- and ß-mannosyl linkages. In this study, we focused on the ß-1,2-mannosides of N-linked mannan in C. albicans because this moiety is not present in the non-pathogenic yeast Saccharomyces cerevisiae. To investigate the impact of ß-1,2-mannosides on immune sensing, we constructed a C. albicans ∆mnn4/∆bmt1 double deletant. Thin-layer chromatography and nuclear magnetic resonance analyses revealed that the deletant lacked ß-1,2-mannosides in N-linked mannan. Mannans lacking the ß-1,2-mannosides induced the production of higher levels of inflammatory cytokines, including IL-6, IL-12p40 and TNF-α, in mice dendritic cells compared to wild-type mannan. Our data show that ß-1,2-mannosides in N-linked mannan reduce the production of inflammatory cytokines by dendritic cells.

Color-controlled nonstoichiometric spinel-type cobalt gallate nanopigments prepared by supercritical hydrothermal synthesis.

Spinel-type inorganic pigments with intensive color and chemical/thermal stability are showing extensive applications that could be further broadened by color manipulation and improvement of the material properties through nanosizing. In this study, we report the supercritical hydrothermal synthesis of nonstoichiometric spinel-type cobalt gallate nanoparticles (Co-Ga NPs) with controlled color. Without the conventional calcination procedure, NPs with greenish-blue, blue, and yellowish-green colors were synthesized from precursor solutions at pH 7, 9, and 11, respectively, with a low Co/Ga molar ratio of 0.25. X-ray diffraction, scanning/transmission electron microscopy, and inductively coupled plasma-atomic emission spectroscopy methods suggest that the products were spinel-type cobalt gallate NPs with high crystallinity and a nonstoichiometric composition. Based on an X-ray absorption fine structure investigation, the prepared nonstoichiometric Co-Ga NPs were found to have different cationic configurations from stoichiometric CoGa2O4 produced by a solid-state reaction during calcination. Meanwhile, the degrees of distortions at tetrahedral and octahedral sites in the NPs were evaluated by Raman spectroscopy. In particular, nonstoichiometric Co-Ga NPs with a blue color were prepared without calcination for the first time and were found to have lower tetrahedral cobalt occupancy but comparable octahedral cobalt occupancy and larger polyhedral distortions at tetrahedral sites when compared to calcined CoGa2O4. We also discuss strategies that could realize Co-Ga NPs with a more brilliant blue color using the present technique based on an investigation of the growth process.

Serum ferritin level during hospitalization is associated with Brain Fog after COVID-19.

The coronavirus disease 2019 (COVID-19) remains an epidemic worldwide. Most patients suffer residual symptoms, the so-called "Long COVID," which includes respiratory and neuropsychiatric symptoms. Brain Fog, one of the symptoms of Long COVID, is a major public health issue because it can impair patients' quality of life even after recovery from the disease. However, neither the pathogenesis nor the treatment of this condition remains unknown. We focused on serum ferritin levels in this study and collected information on the onset of Brain Fog through questionnaires and found that high ferritin levels during hospitalization were associated with the occurrence of Brain Fog. In addition, we excluded confounders as far as possible using propensity score analyses and found that ferritin was independently associated with Brain Fog in most of the models. We conducted phase analysis and evaluated the interaction of each phase with ferritin levels and Brain Fog. We found a positive correlation between serum ferritin levels during hospitalization and Brain Fog after COVID-19. High ferritin levels in patients with Brain Fog may reflect the contribution of chronic inflammation in the development of Brain Fog. This study provides a novel insight into the pathogenic mechanism of Brain Fog after COVID-19.

Lipid phenotype of two distinct subpopulations of Mycobacterium bovis Bacillus Calmette-Guerin Tokyo 172 substrain.

Bacillus Calmette-Guérin (BCG) Tokyo 172 is a predominant World Health Organization Reference Reagent for the BCG vaccine. Recently, the BCG Tokyo 172 substrain was reported to consist of two subpopulations with different colony morphologies, smooth and rough. Smooth colonies had a characteristic 22-bp deletion in Rv3405c of the region of difference (RD) 16 (type I), and rough colonies were complete in this region (type II). We hypothesized that the morphological difference is related to lipid phenotype and affects their antigenicity. We determined the lipid compositions and biosynthesis of types I and II. Scanning electron microscopy showed that type I was 1.5 times longer than type II. Phenolic glycolipid (PGL) and phthiocerol dimycocerosate (PDIM) were found only in type I. Although it has been reported that the RD16 is involved in the expression of PGL, type II did not possess PGL/PDIM. We examined the ppsA-E gene responsible for PGL/PDIM biosynthesis and found that the existence of PGL/PDIM in types I and II is caused by a ppsA gene mutation not regulated by the RD16. PGL suppressed the host recognition of total lipids via Toll-like receptor 2, and this suggests that PGL is antigenic and involved in host responses, acting as a cell wall component. This is the first report to show the difference between lipid phenotypes of types I and II. It is important to clarify the heterogeneity of BCG vaccine substrains to discuss and evaluate the quality, safety, and efficacy of the BCG vaccine.

Genus Enhydrobacter Staley et al. 1987 should be recognized as a member of the family Rhodospirillaceae within the class Alphaproteobacteria.

The genus Enhydrobacter, first reported as a member of the family Vibrionaceae, has been placed in the family Moraxellaceae, but as a genus incertae sedis in Bergey's Manual of Systematic Bacteriology 2nd edition. During our taxonomic investigation of Enhydrobacter-like organisms, we observed that the 16S rRNA sequences of E. aerosaccus-type strain versions NCIMB 12535(T) , ATCC 27094( T) and CCUG 58314(T) were very different from the accessible data (accession no. AJ550856). Phylogenetic analysis of our 16S rRNA sequence data revealed that these organisms were located within the family Rhodospirillaceae. The genera Inquilinus, Oceanibaculum, Skermanella and Nisaea were closely related (sequence similarities were 88.3~87.0%), but Enhydrobacter could be distinguished from these genera by growth characteristics, fatty acid profiles (C(19:0) cyclo ω8c; 38.4% C(18:1) ω7c; 32.2%, and C(16:0) ; 8.9% were major components), in being non-flagellated, and differing in enzymatic activities, including trypsin and ß-glucosidase. From these data, we conclude that the genus Enhydrobacter should be recognized as an independent genus of the family Rhodospirillaceae within the class Alphaproteobacteria.

The partition representation of enzymatic reaction networks and its application for searching bi-stable reaction systems.

The signal transduction system, which is known as a regulatory mechanism for biochemical reaction systems in the cell, has been the subject of intensive research in recent years, and its design methods have become necessary from the viewpoint of synthetic biology. We proposed the partition representation of enzymatic reaction networks consisting of post-translational modification reactions such as phosphorylation, which is an important basic component of signal transduction systems, and attempted to find enzymatic reaction networks with bistability to demonstrate the effectiveness of the proposed representation method. The partition modifiers can be naturally introduced into the partition representation of enzymatic reaction networks when applied to search. By randomly applying the partition modifiers as appropriate, we searched for bistable and resettable enzymatic reaction networks consisting of four post-translational modification reactions. The proposed search algorithm worked well and we were able to find various bistable enzymatic reaction networks, including a typical bistable enzymatic reaction network with positive auto-feedbacks and mutually negative regulations. Since the search algorithm is divided into an evaluation function specific to the characteristics of the enzymatic reaction network to be searched and an independent algorithm part, it may be applied to search for dynamic properties such as biochemical adaptation, the ability to reset the biochemical state after responding to a stimulus, by replacing the evaluation function with one for other characteristics.

Ferromagnetism and exchange bias in compressed ilmenite-hematite solid solution as a source of planetary magnetic anomalies.

We propose a compressed ilmenite-hematite solid solution as a new potential source of Earth's magnetic anomalies. The 0.5FeTiO3·0.5Fe2O3 solid solution compressed by collision synthesis with super-high-energy ball milling showed a decrease in molar volume of approximately 1.8%. Consequently, the sample showed a saturation magnetization of 1.5 ampere square meter per kilogram (Am2/kg) at 300 kelvin, a Curie temperature of 990 kelvin, and a magnetic exchange bias below 100 kelvin, e.g., 1.7 × 105 ampere per meter at 60 kelvin. Ilmenite-hematite solid solutions are common mineral systems in most mafic igneous and metamorphic rocks, and the compressive force in the rocks is generated by the high pressure in the upper mantle or by shock events with high pressure such as the collision of these rocks with meteorites. Therefore, we consider that the compressed ilmenite-hematite solid solution is an additional candidate source of other planetary magnetic anomalies including those in the Moon and Earth.

[Rippling muscle disease with myasthenia gravis].

In February 2020, a 51-year-old woman experienced leg myalgia and noticed calf muscle movements that resembled a rippling wave while crouching down. In June 2020, she complained of bilateral arm myalgia. In August 2020, she developed left ptosis, had difficulty raising her bilateral arms, and developed diplopia and was admitted to our hospital. Anti-acetylcholine receptor antibodies turned out to be positive. We made a diagnosis of myasthenia gravis and acquired rippling muscle disease (RMD). Her myasthenia gravis symptoms and myalgia decreased with oral prednisolone. Contrast-enhanced computed tomography revealed thymoma. She underwent extended thymectomy and was discharged from the hospital. Her myalgia worsened, but it was responsive to methylprednisolone pulse therapy. CAV3 gene mutations are recognized as causes of congenial RMD whereas acquired RMD is associated with myasthenia gravis. Acquired RMD is rarely reported in Japan, but should be kept in mind as a condition treatable with immunotherapy.

Bottom-up synthesis of 2D layered high-entropy transition metal hydroxides.

Low-dimensional high-entropy materials, such as nanoparticles and two-dimensional (2D) layers, have great potential for catalysis and energy applications. However, it is still challenging to synthesize 2D layered high-entropy materials through a bottom-up soft chemistry method, due to the difficulty of mixing and assembling multiple elements in 2D layers. Here, we report a simple polyol process for the synthesis of a series of 2D layered high-entropy transition metal (Co, Cr, Fe, Mn, Ni, and Zn) hydroxides (HEHs), involving the hydrolysis and inorganic polymerization of metal-containing species in ethylene glycol media. The as-synthesized HEHs demonstrate 2D layered structures with interlayer distances ranging from 0.860 to 0.987 nm and homogeneous elemental distribution of designed equimolar stoichiometry in the layers. These 2D HEHs exhibit a low overpotential of 275 mV at 10 mA cm-2 in a 0.1 M KOH electrolyte for the oxygen evolution reaction. Superparamagnetic spinel-type high-entropy nanoparticles can also be obtained by annealing these HEHs. Our polyol approach creates opportunities for synthesizing low-dimensional high-entropy materials with promising properties and applications.