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Examination of the uterus of a dead female white shark (Carcharodon carcharias), which contained the earliest known white shark embryos, revealed that the uterine wall produces lipid-rich secretion (histotroph or "uterine milk") for embryonic nutrition. Uterine tissue was processed for light and electron microscopy, and immunohistochemical techniques to identify its secretory mechanism. Our results indicate that the white shark uterus secretes lipids via holocrine secretion. This type of secretion is characterized by the release of large lipid droplets accumulated in the epithelial cells into the uterine lumen through cell disintegration. The secretory epithelium of the uterus is stratified, and new surface epithelial cells are continuously supplied from deeper epithelial layers to replace the dead secretory cells at the surface. This vertical replacement possibly facilitates the active renewal of the surface epithelium, which is necessary for maintaining holocrine secretory mechanisms. These secretory mechanisms are different from those of myliobatiform stingrays, another elasmobranch taxon that exhibits lipid histotrophy. This may reflect the different origins of lipid histotrophy between these taxa.

We herein report a 65-year-old man with progressive multifocal leukoencephalopathy (PML) after 2-year remission from acute myeloid leukemia who developed recurrent episodes of left hemiparesis with gadolinium enhancement on magnetic resonance imaging. Steroid pulse therapy for each exacerbation induced clinical and radiological improvement, suggesting that exacerbations are an excessive immune response to the JC virus and distinct from immune reconstitution inflammatory syndrome (IRIS). Although glucocorticoids are recommended only for IRIS, steroid pulse therapy should be considered as a therapeutic option in cases of exacerbation of hematologic malignancy-associated PML. Importantly, neuroimaging is not sufficient to differentiate excessive inflammation from a controlled inflammatory response, for which steroids are not recommended.

Purpose The aim of this study was to investigate a structured approach for effective speech therapy (ST) for dysarthria and speech-related quality of life in patients with sporadic spinocerebellar degeneration (SCD), including cerebellar-type multiple-system atrophy and cerebellar cortical atrophy. Method Twenty-two patients with SCD (cerebellar-type multiple system atrophy, 15 patients; cerebellar cortical atrophy, seven patients) who underwent intensive ST were examined. Dysarthria was evaluated using the Scale for Assessment and Rating of Ataxia Speech Dysfunction, Assessment of Motor Speech for Dysarthria Articulation, oral diadochokinesis (OD), and Voice Handicap Index-10 (VHI-10). Respiratory muscle strength (inspiratory and expiratory pressure) and respiratory–phonatory coordination (maximum phonation time) were measured. Cognitive function was evaluated using the Montréal Cognitive Assessment and the word fluency test. Mood was evaluated using the Hospital Anxiety and Depression Scale. The relationships between dysarthria scales (particularly, VHI-10) and clinical data were analyzed using stepwise regression. The differences in outcomes after intensive ST were analyzed using the Wilcoxon signed-rank test. The alpha level ( p ) for statistical significance was set at .0125 by Bonferroni correction. Results For both pre- and post-ST, the patient's OD ( p = .002) and maximum phonation time ( p = .002) significantly improved, except for Speech Dysfunction scores of the Scale for Assessment and Rating of Ataxia ( p = .705) and the VHI-10 ( p = .018). The Assessment of Motor Speech for Dysarthria Articulation, OD, and inspiratory pressure were identified as independent variables of VHI-10 (adjusted R 2 = .820) for speech-related quality of life; no correlations among the Montréal Cognitive Assessment, word fluency test, and Hospital Anxiety and Depression Scale scores were observed. Conclusion OD and VHI-10 showed improvements due to changes in speech function and respiratory–phonatory coordination, justifying intensive ST treatment for dysarthria in patients with SCD.

Background/aims:Nutritional status is a factor affecting prognosis in patients with amyotrophic lateral sclerosis (ALS). Here, we aimed to clarify the factors associated with hypermetabolism and the prognosticators of ALS., Methods:Forty-two inpatients (22 men, 20 women) diagnosed with ALS according to the revised El-Escorial criteria were investigated. The following data were retrospectively analyzed: anthropometric measurements, blood biochemistry, disease severity, basal energy expenditure (BEE), resting energy expenditure (REE) measured by indirect calorimetry, spirometry, and bioelectrical impedance analysis. Single and multiple regression analysis was performed to examine factors affecting REE and metabolic changes (defined as the ratio of REE to fat-free mass [FFM]). The Kaplan-Meier method was used to examine factors associated with the occurrence of cumulative events (death or tracheostomy)., Results:Among the 42 inpatients, REE was significantly higher than BEE, indicating hypermetabolism in ALS. Multiple regression analysis revealed that REE/FFM is strongly associated with the skeletal muscle index (-3.746 to -1.532, p < 0.0001) and percent forced vital capacity (%FVC) (-0.172 to -0.021, p = 0.013). Moreover, both the skeletal muscle index and %FVC were significant prognosticators associated with the occurrence of cumulative events., Conclusions:Energy metabolism was elevated in ALS, and respiratory status and muscle mass were associated with the hypermetabolism and poor prognosis. Adequate nutritional support may improve outcomes in ALS by preventing deterioration of respiratory status and reduction in muscle mass.

A 69-year-old man was admitted for persistent fever, arthralgia, and visual impairment. Physical examination demonstrated bilateral uveitis and recurrent aphthous stomatitis. The PCR analysis of the aqueous humor of the anterior chamber was positive for the Varicella-zoster virus (VZV). Although no neurological defect was evident, the cerebrospinal fluid contained elevated monocytes but was negative for VZV-PCR. Brain MRI revealed Gd-enhanced lesions in the subcortical white matter, basal ganglia, and cerebellum. With his positive HLAB51, he was diagnosed with neuro-Behcet's disease (NBD) and was successfully treated with high-dose prednisolone. Although the pathogenesis of Behcet's disease is still unknown, the involvement of viral infection is reported. The present case implies that NBD could be triggered by herpes zoster virus associate-uveitis; the accumulation of such cases would help clarify the pathogenesis of Behcet's disease.

This study aimed to reconstruct temperatures experienced during the larval period by adult Pacific bluefin tuna Thunnus orientalis using high-resolution otolith stable oxygen isotope (δ18O) analysis. A novel otolith sample preparation protocol for secondary ion mass spectrometry (SIMS) analysis developed in this study reduced the background noise of SIMS measurements, enabling analyses of >10 times higher resolution around the otolith core compared to previous studies using conventional isotope ratio mass spectrometry (IRMS). The values obtained from SIMS were compared to those obtained by microvolume δ18Ootolith analysis using micromilling and conventional continuous-flow IRMS (CF-IRMS). There was a systematic offset (average 0.41‰ with SIMS resulting in lower values) most likely caused by matrix effects on SIMS δ18Ootolith values that can be calibrated using a strong linear relationship between SIMS and CF-IRMS measurements (r2 = 0.78, p < 0.001). The core-to-edge δ18Ootolith of 5 Pacific bluefin tuna revealed fine-scale seasonal variations in water temperature agreeing with known migration patterns. In addition, the ambient water temperature experienced during larval stages (about 10-20 d post hatch) estimated from otolith core δ18O ranged from 26.7 to 30.7°C, overlapping with temperatures associated with the occurrence of larval Pacific bluefin tuna. Combining SIMS and microvolume CF-IRMS δ18O otolith analyses offers a microscale examination of fish ecology that is not possible with conventional IRMS techniques. This novel method is particularly useful for understanding the early life history of fish that may be affected by climate change and reconstructing a well-resolved migration history for fish species that have small otoliths and/or narrow growth increments.

Forms of embryonic nutrition are highly diverse in cartilaginous fishes, which contain oviparity, yolk-sac viviparity and several types of matrotrophic viviparity (histotrophy, oophagy, and placentotrophy). The molecular mechanisms of embryonic nutrition are poorly understood in these animals as few species are capable of reproducing in captivity. Oviparous cartilaginous fishes solely depend on yolk nutrients for their growth and development. In the present study, we compared the contribution to embryonic nutrition of the embryonic intestine with the yolk sac membrane (YSM). RNA-seq analysis was performed on the embryonic intestine and YSM of the oviparous cloudy catshark Scyliorhinus torazame to identify candidate genes involved in nutrient metabolism to further the understanding of nutrient utilization of developing embryos. RNA-seq discovery was subsequently confirmed by quantitative PCR analysis and we identified increases in several amino acid transporter genes (slc3a1, slc6a19, slc3a2, slc7a7) as well as genes involved in lipid absorption (apob and mtp) in the intestine after ‘pre-hatching’, which is a developmental event marked by an early opening of the egg case about 4 months before hatching. Although a reciprocal decrease in the nutritional role of YSM was expected after the intestine became functional, we observed similar increases in gene expression among amino acid transporters, lipid absorption molecules, and lysosomal cathepsins in the extraembryonic YSM in late developmental stages. Ultrastructure of the endodermal cells of YSM showed that yolk granules were incorporated by endocytosis, and the number of granules increased during development. Furthermore, the digestion of yolk granules in the YSM and nutrient transport through the basolateral membrane of the endodermal cells appeared to be enhanced after pre-hatching. These findings suggest that nutrient digestion and absorption is highly activated in both intestine and YSM after pre-hatching in catshark embryos, which supports the rapid growth at late developmental stages.

To examine whether hypermetabolism could predict the prognosis of early amyotrophic lateral sclerosis (ALS) patients with differing nutritional profiles. This single-center, retrospective study examined the prognosis of ALS patients with hypermetabolism in relation to their nutritional status at hospitalization. The metabolic state was estimated by the ratio of measured resting energy expenditure (mREE) to lean soft tissue mass (LSTM) (mREE/LSTM), wherein patients with ratios ≥ 38 were defined as hypermetabolic. Malnutrition was defined as %ideal body weight p = 0.025). Prognostic prediction by hypermetabolism varies depending on the nutritional status in ALS, and the BMM index is a consistent prognostic factor.

Previous studies have reported that the relative abundances of volatile elements in the silicate Earth are non-chondritic. The abundance and distribution of volatile elements in terrestrial planets would have been predominantly controlled by planetary formation processes, including core-mantle separation, magma ocean crystallization, and volatility-dependent high-temperature fractionation. Thus, the current abundance patterns of volatile elements in the silicate fraction of terrestrial planets are the key to understanding the accretional history of terrestrial volatiles and the chemical differentiation of terrestrial planets. Although the origin of the non-chondritic ratios of volatile elements in terrestrial planets has been previously studied, it is still a matter of debate. In this study, we focused on the super-chondritic F/Cl ratio of the bulk silicate Earth and experimentally investigated the silicate mineral-melt partitioning of fluorine and chlorine at pressures from 18 GPa to 25 GPa. Our experimental results show that fluorine is moderately compatible with mantle minerals, whereas chlorine is highly incompatible. These results support the formation of a solid mantle with high F/Cl ratios, and a residual magma ocean and steam atmosphere with low F/Cl ratios during magma ocean crystallization. Thus, the F/Cl ratio in the residual solid parts of terrestrial planets would have become relatively enriched following escape of volatile elements from the planetary surface into outer space. This model is consistent with the collisional erosion hypothesis of primordial crusts and atmospheres, and current observations on the abundance and distribution of terrestrial fluorine and chlorine.

Homing peptides to the spinal cord were identified and isolated using phage display technology. In vivo biopanning was performed by intravenous systemic injection of a phage library to screen specific peptides targeting the spinal cord of mice. Analyses of the sequences of targeted phages yielded two candidate peptides targeting the spinal cord: SP1 (C-LHQSPHI-C) and SP2 (C-PTNNPRS-C). These peptides were synthesized and intravenously injected into mice to evaluate their tissue specificity and potential as gene delivery carriers. The complexes between SP1 or SP2 peptides and the plasmid vector expressing the reporter gene could induce gene transduction in the spinal cord through systemic injection without gene expression in the brain, liver, and kidney. In addition, intravenous injection of the complex between SP1 and the vectors induced interleukin-4 expression in the spinal cord, resulting in effective suppression of lipopolysaccharide-induced hyperalgesia. Therefore, intravenously administered spinal cord homing peptides complexed with a plasmid vector provided tissue-specific treatment featuring gene delivery to the CNS through systemic circulation. This novel method of gene delivery is feasible and has great potential for clinical application., Graphical Abstract

Megasplay fault branching from plate boundaries of subduction zones is thought to be important sources of earthquakes and tsunamis. In this study, we performed structural and geochemical analyses on a fossilized megasplay fault (the Nobeoka Thrust of the Shimanto accretionary complex) to understand fluid-rock interaction and how the splay fault plays a role in fluid flow in the seismogenic zone. As a result of structural observations, we report that the principal slip zone (PSZ) of the Nobeoka Thrust is composed of foliated cataclasite originating from a sandstone-shale mélange and includes a thin (~ 1.5 mm thick) pseudotachylyte layer. Major and trace element composition analysis and EPMA element mapping revealed that the pseudotachylyte is enriched in Li and Cs within the PSZ, as well as in the slip zone of a minor fault in the footwall. Li and Cs enrichment in pseudotachylyte is interpreted as a result of fluid-rock interaction in the postseismic stage, because such an anomaly only results from a large fluid/rock ratio (R > 512–24 at 250–350 °C) under the influence of Li- and Cs-enriched fluids. The amount of fluid that reacted with the pseudotachylyte is estimated to be 1.78 × 100 to 7.61 × 103 m3.

A 16-year-old Japanese girl presented with left hemiparesis and coma, following a 2-day history of headache and fever. She had been previously well and no history of a skin wound. MR scan of the brain showed a 6 cm lesion in the right frontal white matter with mass effect and slight edge enhancement (figure 1). A craniotomy was performed for external decompression and brain biopsy. The intraoperative finding was of white matter necrosis rather than tumour or abscess. An interim biopsy showed prominent neutrophil infiltration and fibrinoid necrosis in small vessels but no evidence of a specific pathogen. We suspected either a central nervous system infection or an autoimmune disease such as acute disseminated encephalomyelitis, and so started antibiotics and pulsed corticosteroids in the intensive care unit. Figure 1 MR scan of brain on admission showing a mass lesion in the right frontal lobe. The fluid-attenuated inversion recovery (FLAIR) image …

Sporadic spinocerebellar degenerative diseases such as multiple system atrophy (cerebellar type) and cortical cerebellar atrophy typically present with cerebellar ataxia. Multiple system atrophy is characterized by ataxia, with parkinsonism, dysautonomia and neuropsychiatric symptoms, resulting in reduced quality of life. Effects of physical rehabilitation focused on motor symptoms with ataxia in nonmultiple system atrophy patients have been reported; however, without addressing concomitant nonmotor symptoms. Here, we examined the motor, nonmotor and quality of life effects of inpatient physical rehabilitation in 15 multiple systems atrophy and nine cortical cerebellar atrophy patients without dementia. Rehabilitation involved a 4-week hospitalization with physical, occupational and speech therapy. The following assessments were conducted at admission and discharge: the scale for the assessment and rating of ataxia for ataxia; Montreal cognitive assessment for cognition, hospital anxiety and depression scale for emotion and medical outcomes study short-form for health-related quality of life. Data were analyzed for statistical significance (P < 0.05) using the Wilcoxon signed-rank test. In patients with multiple system atrophy, rehabilitation significantly improved ataxia, cognition with mild cognitive impairment (73.3%) and health-related quality of life; however, patients with depression (86.7%) showed no improvement in emotional health and quality of life. Similar effects on motor and nonmotor symptoms were observed in patients with cortical cerebellar atrophy. This suggests that inpatient rehabilitation could not only improve motor and nonmotor functions, but also the quality of life in patients with spinocerebellar degenerative disease.

A 23-year-old woman was admitted for slowly progressive proximal limb muscle weakness from childhood with elevated muscle enzyme levels. Although muscular diseases were suspected, an electromyogram showed remarkable neurogenic changes, and a muscle echogram indicated selective muscle involvement, including dissociation between the soleus and gastrocnemius, which was consistent with previous reports using magnetic resonance imaging (MRI). She was diagnosed with SMA type 3 following genetic testing, and nusinersen was soon initiated. An early diagnosis is mandatory to maximize the benefit of treatment. A muscle echogram may facilitate an early diagnosis in a non-invasive and time-saving manner compared to MRI.

Cartilaginous fish have a comparatively short intestine known as the spiral intestine that consists of a helical spiral of intestinal mucosa. However, morphological and functional development of the spiral intestine has not been fully described. Unlike teleosts, cartilaginous fish are characterized by an extremely long developmental period

Cartilaginous fish have a comparatively short intestine known as the spiral intestine that is comprised of a helical spiral of intestinal mucosa. However, morphological and functional development of the spiral intestine is not well described. Unlike teleosts, cartilaginous fish are characterized by an extremely long developmental period in ovo or in utero for example; in the oviparous cloudy catshark (Schyliorhinus torazame), the developing fish remains inside the egg capsule for up to six months, suggesting that the embryonic intestine may become functional prior to hatch. In the present study, we describe the morphological and functional development of the spiral intestine in the developing catshark embryo. Spiral formation of embryonic intestine was completed at the middle of stage 31, prior to “pre-hatching”, which is a developmental event characterized by the opening of egg case occurring at the end of the first third of development. Within 48 hours after pre-hatching event, egg yolk began to flow from the external yolk sac into the embryonic intestine via the yolk stalk. At the same time, there was a rapid increase in mRNA expression of the peptide transporter pept1 and neutral amino acid transporter slc6a19. Secondary folds in the intestinal mucosa and microvilli on the apical membrane appeared after pre-hatching, further supporting the onset of nutrient absorption in the developing intestine at this time. We demonstrate the acquisition of intestinal nutrient absorption at the pre-hatching stage of an oviparous elasmobranch.

Clarification of whether suspension-feeding infaunal bivalves are able to utilize benthic diatoms as food is essential for understanding coastal food webs. Although many benthic diatoms possess hard thickly siliceous cell walls (frustules), no study has examined if bivalves can effectively break their frustules to digest and assimilate the cell contents. In the present study, feeding rates and digestion efficiencies of the Manila clam fed on three benthic and a planktonic diatom species with different mechanical strengths (hardness) of frustules were compared to determine if the clam can efficiently break benthic diatoms with relatively hard frustules. Fragments of diatom frustules in the gut were observed using a scanning electron microscope . Although digestion efficiencies of the Manila clam were significantly different among the diatom species, the efficiencies were all above 90% for all four diatom species indicating that the Manila clam can efficiently crush hard diatom frustules of benthic species. The mechanism for crushing diatom cells in the gut appears to rely on the density of diatom cells ingested into the clam's stomach. By the rotation of the crystalline style, densely packed diatom cells in the stomach are agitated leading to attrition with each other, and subsequently the frustules are ruptured. The results of the present study indicate that when benthic diatoms are suspended in the water column the Manila clam can effectively utilize benthic diatoms as a major food source irrespective of the hardness of the diatom's frustules.

Sulfide deposits with extremely high Au concentrations (up to 275 ppm; avg. 102 ppm, n = 15), high Au/Ag ratios (0.24, n = 15), and low Cu/(Cu + Zn) ratios (0.03, n = 15) were discovered in 2015 in active hydrothermal fields at a water depth of 760 m in a basalt-dominated submarine caldera in the Izu-Ogasawara frontal arc, Japan. Native gold grains occur in massive sulfide fragments, concretions, and metalliferous sediments from a sulfide mound (40 m across and 20 m high) with up to 30-m-high black smoker chimneys. Tiny native gold grains up to 14 μm in diameter are mainly present in sulfide fallouts from chimney orifices and plumes. Larger native gold grains up to 150 μm long occur mostly as discrete particles and/or with amorphous silica and sulfides. The larger gold grains are interpreted to represent direct precipitation from Au-bearing hydrothermal fluids circulating in and/or beneath the unconsolidated sulfide mound deposits. Sulfur isotope compositions from a limited number of sulfide separates (n = 4) range from 4.3 to 5.8‰ δ34S, similar to the quaternary volcanic rocks of the arc. Barite separates have values of 22.2 and 23.1‰, close to modern seawater values, and indicate probable seawater sulfate origin. The Cu, Zn, and Pb concentrations in bulk samples of sulfide-rich rocks are similar to those of volcanogenic massive sulfides formed in continental crustal environments. The gold is interpreted to have formed by low-temperature hydrothermal activity, perhaps genetically different from systems with documented magmatic contributions or from seafloor hydrothermal systems in other island arc settings. Its presence suggests that basalt-dominated submarine calderas situated on relatively thick continental crust in an intraoceanic arc setting such as the Higashi-Aogashima knoll caldera may be perspective for gold mineralization.

Astrocyte- and microglia-targeting peptides were identified and isolated using phage display technology. A series of procedures, including three cycles of both in vivo and in vitro biopanning, was performed separately in astrocytes and in M1 or M2 microglia, yielding 50–58 phage plaques in each cell type. Analyses of the sequences of this collection identified one candidate homing peptide targeting astrocytes (AS1[C-LNSSQPS-C]) and two candidate homing peptides targeting microglia (MG1[C-HHSSSAR-C] and MG2[C-NTGSPYE-C]). To determine peptide specificity for the target cell in vitro , each peptide was synthesized and introduced into the primary cultures of astrocytes or microglia. Those peptides could bind to the target cells and be selectively taken up by the corresponding cell, namely, astrocytes, M1 microglia, or M2 microglia. To confirm cell-specific gene delivery to M1 microglia, the complexes between peptide MG1 and siRNA-interferon regulatory factor 5 were prepared and intrathecally injected into a mouse model of neuropathic pain. The complexes successfully suppressed hyperalgesia with high efficiency in this neuropathic pain model. Here, we describe a novel gene therapy for the treatment neuropathic pain, which has a high potential to be of clinical relevance. This strategy will ensure the targeted delivery of therapeutic genes while minimizing side effects to non-target tissues or cells.

In the bulk silicate Earth, chlorine is more depleted than other elements with similar volatilities; however, the cause of terrestrial chlorine depletion is not well understood. Two major hypotheses have been proposed to explain this depletion: Incorporation into the Earth's metallic core and escape to space. The former hypothesis can be tested by investigating the partitioning of chlorine between iron-rich metallic liquids and silicate melts. In this study, we investigated the experimental partitioning of chlorine between iron-rich metallic liquids and silicate melts at pressures from 4 to 23 GPa and temperatures from 1,650 to 2,400°C using multi-anvil presses. The results demonstrate that chlorine is moderately to highly lithophile under the experimental conditions. In sulfur-free experiments, chlorine becomes slightly more siderophile as temperature increases and less siderophile as pressure increases. For sulfur-bearing experiments, no significant effects of pressure or temperature were observed. Based on these data and thermodynamic considerations, we obtained empirical laws to estimate chlorine partition coefficients between iron-rich metallic liquids and silicate melts. Under the P-T conditions that would have controlled metal-silicate equilibration during core segregation in the Earth, the calculated metal-silicate partition coefficients for chlorine are much lower than unity. This result suggests that terrestrial chlorine that may have been present in the accreting Earth was not partitioned into its core, supporting that escape to space is the more likely hypothesis. If terrestrial chlorine was lost to space, chlorine depletion may have resulted from the loss of the primordial hydrosphere during the formation of the Earth.

Background Propofol infusion syndrome (PRIS) is a rare but lethal complication of propofol use. It has been suggested that the pathological mechanism of PRIS involves mitochondrial disorder caused by propofol. Case Presentation A 24‐year‐old woman who had been diagnosed with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke‐like episodes was admitted to our hospital with impaired consciousness and myoclonus. To control the non‐convulsive status epilepticus, propofol was administered. Arterial blood gas revealed metabolic acidosis, and creatinine kinase was elevated. The patient was diagnosed with PRIS. We treated her with interruption of propofol. She required mechanical ventilation for 25 days. After rehabilitation, she recovered and was discharged. Conclusion Mitochondrial disorder is a risk factor for PRIS. It is important for clinicians to be aware that mitochondrial disorder is a risk factor for PRIS, especially under conditions of critical illness and status epilepticus., The pathological mechanisms of propofol infusion syndrome (PRIS) are similar to those of mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke‐like episodes. Clinicians should be aware that mitochondrial disorder is a risk factor for PRIS, especially under conditions of critical illness.

In laser treatments, the difference in patient's degree of skin pigmentation is an important factor that can lead to medical accidents. However, the conventional heat transfer model is not available to analyze the effect of degree of pigmentation on the laser treatments. Therefore, we propose a new microscopic radiation heat transfer model that takes into account the point heat sources for the laser treatments. In the proposed model, skin considered as a bulk medium consisting of two layers, namely normal and pigmentation layers with the latter containing a larger number of melanosomes having a specific volume fraction (fv,mel) as the degree of pigmentation. Using the proposed model, it was found that the distribution of the absorbed laser energy and temperature rise of the skin tissue is affected by the degree of pigmentation. When fv,mel is high, the absorbed energy per single melanosome and thus the temperature rise of the pigmentation layer is lower than that in the deeper skin tissue. It was also shown that depending on fv,mel, the distance between melanosomes decreases. When fv,mel is high, the temperature of skin tissue between melanosomes increases.

To clarify hydroxyapatite (HAp) nanocrystal formation based on the interfacial interactions with organic molecules is important for controlling the dispersion states/shapes of HAp and understanding the biomineralization mechanism. In this study, the effects of cetyltrimethylammonium bromide (CTAB) micelles on the HAp nanocrystal formation process were investigated through both the morphosynthesis technique and the molecular orbital calculations. The HAp nanocrystals synthesized in the presence of CTAB exhibited a controlled rod-like shape and subsequently grew up by a thermal treatment to be larger-sized nanocrystals at a well-dispersed state. It was also found that the CTAB micelles strained and disordered at the higher temperature effectively induced heterogeneous nucleation to initiate the nucleation/growth processes. In contrast, HAp nanocrystals synthesized without CTAB exhibited irregular-shaped and aggregated nanocrystals, which are due to dominantly occurring homogeneous nucleation. According to t...

To prevent the self-reduction of the Cu2O photocathode for solar hydrogen production, we developed a compositing process of a highly crystalized TiO2 layer on the Cu2O photocathode using an excimer-laser-assisted metal-organic deposition (ELAMOD) process. The TiO2 layer was successfully crystalized without oxidation of Cu2O to CuO mainly owing to a photothermal effect with nanosecond duration time induced by laser absorption of the TiO2 precursor while the crystallization of the TiO2 layer by usual furnace heating process was accompanied by oxidation of Cu2O which degrade the water reduction ability. On the TiO2/Cu2O photocathode prepared by ELAMOD process, the self-reduction of Cu2O did not occur and then photocurrent due to water reduction was constant with reaction time while on the bare Cu2O photocathode, the photocurrent decreased owing to the occurrence of the self-reduction. This indicated that reaction stability of the photocathode was largely enhanced after compositing of the crystallineTiO2 layer. This ELAMOD process would be applicable for any kinds of chemically unstable photoelectrodes containing non-oxides such as sulfides and phosphides, and therefore any kinds of photoelectrodes would have potentials toward a practical use by improving their chemical stabilities.

Fish scales are a potential source of collagen for fabricating scaffolds for cells during tissue engineering because fish collagen has a low risk of zoonosis. Since the assembly of collagen fibrils has a significant impact on the functionality of the scaffold, the ability to replicate the fibril assembly of human tissues is critical. To determine the mechanism of fish collagen fibril assembly, we first identified non-collagenous proteins (NCPs), the potential regulators of fibril assembly in vivo, and then used tandem mass spectrometry to analyze the NCPs contained in the basal plates of goldfish Carassius auratus scales, a collagenous plate which is characterized by a plywood-like assembly of collagen fibrils similar to that found in the cornea. We identified a 19-kDa acidic protein as dermatopontin, the NCP which is a possible regulator of fibril assembly in the mammalian cornea. We cloned a goldfish dermatopontin cDNA of 1,074 bp containing an open reading frame encoding 196 amino acids. Reverse transcription-PCR revealed that dermatopontin mRNA was expressed in a wide range of tissues, including scale, skin, fin, eye, and skeletal muscle. In situ hybridization revealed that dermatopontin mRNA was expressed primarily in the basal plate-producing hyposquamal scleroblasts of the scales, suggesting that the dermatopontin is linked to the collagen fibril assembly of the basal plate.

Charcot-Marie-Tooth disease (CMT) is the most common inherited neuropathy characterized by clinical and genetic heterogeneity. Although more than 30 loci harboring CMT-causing mutations have been identified, many other genes still remain to be discovered for many affected individuals. For two consanguineous families with CMT (axonal and mixed phenotypes), a parametric linkage analysis using genome-wide SNP chip identified a 4.3 Mb region on 12q24 showing a maximum multipoint LOD score of 4.23. Subsequent whole-genome sequencing study in one of the probands, followed by mutation screening in the two families, revealed a disease-specific 5 bp deletion (c.247−10_247−6delCACTC) in a splicing element (pyrimidine tract) of intron 2 adjacent to the third exon of cytochrome c oxidase subunit VIa polypeptide 1 (COX6A1), which is a component of mitochondrial respiratory complex IV (cytochrome c oxidase [COX]), within the autozygous linkage region. Functional analysis showed that expression of COX6A1 in peripheral white blood cells from the affected individuals and COX activity in their EB-virus-transformed lymphoblastoid cell lines were significantly reduced. In addition, Cox6a1-null mice showed significantly reduced COX activity and neurogenic muscular atrophy leading to a difficulty in walking. Those data indicated that COX6A1 mutation causes the autosomal-recessive axonal or mixed CMT.

The mechanochemical milling was utilized to control the nanostructures of plate-like graphite (Gp) microparticles in an argon atmosphere and at room temperature. The aggregated Gp nanoparticles were prepared by the simple solid-state process without organic solvent. Electron microscope observations, X-ray diffraction patterns and Raman spectra revealed the fractured plate-like Gp microparticles as well as the Gp microparticles agglomerated to each other through the dissociative edge surfaces by the milling process to finally generate the Gp nanoparticle aggregates. In the process, the ratio of the edge plane surfaces with the dangling bondings to layered internal surfaces clearly increased with the milling. Therefore, the low environmental burden process for preparing the Gp nanoparticle aggregation was achieved.

Importance The regulatory factors explaining the wide spectrum of clinical phenotypes for mitochondrial 3243A>G mutation are not known. Crosstalk between nuclear genes and mitochondrial DNA might be one factor. Observations In this case series, we compared 2 pairs of male twins with the mitochondrial 3243 A>G mutation and mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes syndrome with a female control patient. One pair of monozygotic twins presented with diabetes and deafness in their 30s, stroke-like episodes in their 40s, and cardiac events and death in their 50s. Another pair of twins presented with deafness and stroke-like episodes in their 20s. The degree of heteroplasmy of 3243A>G mutation in the various tissues and organs was similar in the first pair of twins compared with the control patient. Conclusions and Relevance The clinical phenotype and segregation of mitochondrial 3243A>G mutation was similar in monozygotic twins. The onset age and distribution of the symptoms might be regulated by nuclear genes. Our findings might help to predict the clinical course of the surviving twins and afford an opportunity for therapy before the onset of mitochondrial disease, especially for monozygotic twins caused by nuclear transfer with a small amount of nuclear-donor mitochondrial DNA.