Hypertrophy of the inferior olivary nucleus in corticobasal degeneration: A neuropathological study.

AIMS: Corticobasal degeneration (CBD) is a rare neurodegenerative disorder. The status of the inferior olivary nucleus (ION) in CBD has been inadequately investigated. In this study, we conducted a pathological investigation of the ION in CBD. MATERIALS AND METHODS: We reviewed the data of Japanese patients with pathologically confirmed CBD who underwent consecutive autopsies between 1985 and 2020 at our institute. We retrospectively examined clinical data from medical records and clinicopathological conferences and semi-quantitatively assessed the ION, central tegmental tract, superior cerebellar peduncle, and dentate nucleus. RESULTS: Of the 32 patients included, 14 (43.8%) had hypertrophy of the ION (HION), of whom 6 showed laterality. In the 14 HION cases, with or without laterality, except in 1 unevaluable case, atrophy/myelin pallor of the central tegmental tract was observed on the same side as the hypertrophy. Ten patients with HION, with or without laterality, had atrophy/myelin pallor of the superior cerebellar peduncle on the contralateral side to the hypertrophy. CONCLUSION: The ION presents with hypertrophic changes in CBD. The lesion is a primary degeneration in CBD and is related to the degeneration of the Guillain-Mollaret triangle. This finding contributes to the elucidation of the specific pathological characteristics of CBD.

Neuropathological hallmarks in autopsied cases with mitochondrial diseases caused by the mitochondrial 3243A>G mutation.

The mitochondrial (m.) 3243A>G mutation is known to be associated with various mitochondrial diseases including mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS). Their clinical symptoms have been estimated to occur with an increased mitochondrial DNA (mtDNA) heteroplasmy and reduced activity of oxidative phosphorylation (OXPHOS) complexes, but their trends in the central nervous system remain unknown. Six autopsied mutant cases and three disease control cases without the mutation were enrolled in this study. The mutant cases had a disease duration of 1-27 years. Five of six mutant cases were compatible with MELAS. In the mutant cases, cortical lesions including a laminar necrosis were frequently observed in the parietal, lateral temporal, and occipital lobes; less frequently in the frontal lobe including precentral gyrus; and not at all in the medial temporal lobe. The mtDNA heteroplasmy in brain tissue samples of the mutant cases was strikingly high, ranging from 53.8% to 85.2%. The medial temporal lobe was preserved despite an inhospitable environment having high levels of mtDNA heteroplasmy and lactic acid. OXPHOS complex I was widely decreased in the mutant cases. The swelling of smooth muscle cells in the vessels on the leptomeninges, with immunoreactivity (IR) against mitochondria antibody, and a decreased nuclear/cytoplasmic ratio of choroidal epithelial cells were observed in all mutant cases but in none without the mutation. Common neuropathological findings such as cortical laminar necrosis and basal ganglia calcification were not always observed in the mutant cases. A high level of mtDNA heteroplasmy was observed throughout the brain in spite of heterogeneous cortical lesions. A lack of medial temporal lesion, mitochondrial vasculopathy in vessels on the leptomeninges, and an increased cytoplasmic size of epithelial cells in the choroid plexus could be neuropathological hallmarks helpful in the diagnosis of mitochondrial diseases.

Single-Domain and Atomically Flat Surface of κ-Ga2O3 Thin Films on FZ-Grown ε-GaFeO3 Substrates via Step-Flow Growth Mode.

Herein, single-domain κ-Ga2O3 thin films were grown on FZ-grown ε-GaFeO3 substrates via a step-flow growth mode. The ε-GaFeO3 possessing the same crystal structure and similar lattice parameters as those of the orthorhombic κ-Ga2O3 facilitated the growth of κ-Ga2O3 thin films, as observed by the X-ray diffraction (XRD) analysis. Furthermore, the surface morphologies of the κ-Ga2O3 thin films exhibited a step-terrace and atomically flat structure. XRD φ-scan and transmission electron microscopy with selected area electron diffraction revealed that there is no occurrence of in-plane rotational domains in the κ-Ga2O3 thin films on ε-GaFeO3 substrates and that the κ-Ga2O3 thin film comprised a single domain. TEM analysis revealed that there were no clear dislocations in the observation area. Moreover, high-resolution TEM observation showed that the atomic arrangements of the film and the substrate were continuous without the presence of an intermediate layer along the growth direction and were well-aligned in the in-plane direction.

Effects of preventing a temperature decrease during winter on reproduction of male small-egged Kajika, Cottus pollux SE.

To investigate the effects of preventing temperature decrease on the reproductive activity of the male cold-water teleost, Cottus pollux SE, testicular development, serum 11-ketotestosterone (11-KT) levels, and physiological responses associated with nesting behavior (i.e., elevation of serum 11-KT levels and accumulation of urine in the urinary bladder) were observed from November to January. Specifically, males were exposed to three different cooling regimes (control, 16 to 6 °C; H1, 16 to 11 °C; H2, 16 to 14 °C), and the results were compared. In addition, the effects of temperature on male reproductive behavior were also clarified. At higher water temperature regimes, the rate of testicular development and serum 11-KT levels were both higher from November to mid-December than from mid-December to January. However, the results showed that high water temperature regimes in the coldest period of winter did not suppress spermatogenesis completely. Conversely, the physiological responses to nesting were affected by high water temperatures, with serum 11-KT levels increasing and urine accumulation in the urinary bladder being suppressed. Furthermore, frequencies of two behaviors associated with nesting, i.e., body undulation and face displays, were also suppressed under high water temperatures (~ 14 °C) compared with normal temperatures (~ 7 °C) during the breeding season. Based on the physiological and behavioral responses to nesting, findings showed that preventing a water temperature decrease during winter suppresses reproductive activity in Cottus pollux SE.

Teleost IL-6 promotes antibody production through STAT3 signaling via IL-6R and gp130.

Teleost IL-6 is upregulated after antigen stimulation; therefore, we hypothesized that fish IL-6 contributes to antibody production during immune responses against infections. To verify this hypothesis, we first cloned IL-6R and gp130 in fugu (Takifugu rubripes) in the present study. The membrane and soluble forms of IL-6R were identified by the identification of cDNA clones of IL-6R homologues. Three STAT3-docking sites were found in the intracellular region of fugu gp130. Expression analysis showed that fugu IL-6R and gp130 were expressed in mIgM(+) B cells, suggesting that fugu B cells are stimulated by IL-6. Recombinant fugu IL-6 (rfIL-6) increased the gene expression of secretory antibodies by mIgM(+) B cells in vitro. The rfIL-6 and soluble form of rfIL-6R activated STAT3 phosphorylation in the B cells and a cultured cell line transfected with fugu gp130. These results indicate that fugu IL-6 enhances antibody production in the B-cell lineage via gp130 and STAT3 signaling.

Glass transition dynamics of stacked thin polymer films.

The glass transition dynamics of stacked thin films of polystyrene and poly(2-chlorostyrene) were investigated using differential scanning calorimetry and dielectric relaxation spectroscopy. The glass transition temperature T(g) of as-stacked thin polystyrene films has a strong depression from that of the bulk samples. However, after annealing at high temperatures above T(g), the stacked thin films exhibit glass transition at a temperature almost equal to the T(g) of the bulk system. The α-process dynamics of stacked thin films of poly(2-chlorostyrene) show a time evolution from single-thin-film-like dynamics to bulk-like dynamics during the isothermal annealing process. The relaxation rate of the α process becomes smaller with increase in the annealing time. The time scale for the evolution of the α dynamics during the annealing process is very long compared with that for the reptation dynamics. At the same time, the temperature dependence of the relaxation time for the α process changes from Arrhenius-like to Vogel-Fulcher-Tammann dependence with increase of the annealing time. The fragility index increases and the distribution of the α-relaxation times becomes smaller with increase in the annealing time for isothermal annealing. The observed change in the α process is discussed with respect to the interfacial interaction between the thin layers of stacked thin polymer films.

Anomalous increase in dielectric susceptibility during isothermal aging of ultrathin polymer films.

The aging dynamics of poly(2-chlorostyrene) (P2CS) ultrathin films with thicknesses less than 10 nm were investigated using dielectric relaxation spectroscopy. The imaginary part of the dielectric susceptibility ϵ″ for P2CS ultrathin films with a thickness of 3.7 nm increased with an increase in isothermal aging time, while this was not the case for P2CS thin films thicker than 9.0 nm. This anomalous increase in ϵ″ for the ultrathin films is strongly correlated with the presence of a mobile liquidlike layer within the thin films.

Aging dynamics in the polymer glass of poly(2-chlorostyrene): Dielectric susceptibility and volume.

Aging dynamics was investigated in the glassy states of poly(2-chlorostyrene) by measuring the complex electrical capacitance during aging below the glass transition temperature. The variations with time and temperature of the ac dielectric susceptibility and volume could be determined by simply measuring the variation in the complex electrical capacitance. Isothermal aging at a given temperature for several hours after an intermittent stop in constant-rate cooling is stored in the deviations of both the real and imaginary parts of the complex ac dielectric susceptibility and volume. During cooling after isothermal aging, the deviation of the ac dielectric susceptibility from the reference value decreases and almost vanishes at room temperature. By contrast, the deviation in volume induced during isothermal aging remains almost constant during cooling. The simultaneous measurement of ac dielectric susceptibility and volume clearly revealed that the ac dielectric susceptibility exhibits a full rejuvenation effect, whereas the volume does not show any rejuvenation effects. We discuss a plausible model that can reproduce the present experimental results.

Monoclonal antibodies recognising serum immunoglobulins and surface immunoglobulin-positive cells of puffer fish, torafugu (Takifugu rubripes).

Immunoglobulin of the torafugu, Takifugu rubripes, was purified by a combination of precipitation by low ionic strength dialysis and gel filtration. The Ig was used to immunise mice for the production of monoclonal antibody (MAb). Supernatants of hybridoma cultures were screened by enzyme-linked immunosorbent assay using purified-torafugu Ig-coated plates, and two stable hybridomas producing MAbs against torafugu Ig were obtained. Sodium dodecyl sulphate-polyacrylamide gel electrophoresis under reducing conditions and Western blotting indicated that one MAb (16F3) was specific for the deglycosylated heavy chain of torafugu, and the other MAb (4H5) did not bind to the reduced Ig, suggesting that 4H5 recognised the higher-order structure of Ig. Under non-reduced conditions, both MAbs recognised mainly a 750 kDa band and also minor bands of 672, 410 and 205 kDa. MAb 16F3- and 4H5-primed magnetic beads (Dynabeads) adsorbed 84.9+/-3.3% and 63.6+/-4.4% of the torafugu Ig, respectively. The Ig adsorbed by MAb 16F3-primed Dynabeads was reactive to 4H5 on immunoblotting, and vice versa, indicating that the epitopes for both MAbs are held on the same Ig molecule. Both of these MAbs cross-reacted extensively with the Ig of other Takifugu species, but not with other genus. The MAbs were used to identify surface Ig-positive lymphocytes in the spleen, pronephros, peripheral blood and thymocytes of torafugu by flow cytometry. Flow cytometric analysis of the cells in the lymphocyte-enriched fraction revealed that 50.2+/-6.9% in the PBL, 11.8+/-1.7% in the mesonephros, 13.3+/-2.1% in the pronephros, 42.5+/-4.3% in the spleen and 3.2+/-0.6% in thymus were reactive to 4H5 or 16F3.

Maturation-related variations in prostaglandin and fatty acid content of ovary in the kuruma prawn (Marsupenaeus japonicus).

Total lipid, fatty acids and prostaglandins (PGF(2 alpha) and PGE(2)) in the ovary of kuruma prawns (Marsupenaeus japonicus) were measured during ovarian development. The level of ovarian total lipid increased with an increase in the gonad-somatic index (GSI). No significant difference was found in fatty acid composition among different stages of ovarian development. However, the content of arachidonic acid (precursor of PG(2)), but not eicosapentanoic acid (precursor of PG(3)), was significantly lower at stages I and II than at stage V (P<0.01). When ovarian PGF(2 alpha) and PGE(2) levels were plotted against GSI, no correlation was found in either PG. However, in terms of ovarian developmental stages, the level of ovarian PGs was high (approx. 20 pg/mg) at stage I, followed by marked decreases at stages IV and V (PGF(2 alpha), P<0.01) and stage IV (PGE(2), P<0.01). These results suggest that ovarian PGs and arachidonic acid are deeply involved in ovarian maturation in kuruma prawns.