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Thermochemical heat storage is a promising energy storage method and has received much attention in recent years. To improve the design and application of such systems, a better understanding of the characteristics of thermochemical heat storage is required. In this study, we analyze the heat release characteristics of thermochemical heat storage considering various operating parameters, such as charging temperature, humidity, and material. Three different thermochemical materials are considered herein, namely, zeolite 13X, zeolite 5A, and silica gel. The temperature profile at the inlet and outlet of the storage chamber, and that inside the chamber packed with thermochemical material, were measured. The pressure drop across the chamber was measured as well. The results indicate that the heat release characteristics change with position and time. Furthermore, the temporal curves of released heat are significantly affected by charging temperature and humidity. Zeolite 13X rapidly released a large amount of heat within a short period of time, whereas silica gel gradually released a small amount of heat, over a longer duration of time. The pressure drop across the chamber was found to vary with the bead size of the thermochemical material. Thus, the operating parameters including charging temperature, humidity, and material, have a significant effect on the characteristics of heat release. The data obtained herein can serve as a reference for the design of thermochemical storage systems.

Holoprosencephaly (HPE) is the most common congenital anomaly affecting the forebrain and face in humans and occurs as frequently as 1:250 conceptions or 1:10,000 livebirths. Sonic Hedgehog signaling molecule is one of the best characterized HPE genes that plays crucial roles in numerous developmental processes including midline neural patterning and craniofacial development. The Frizzled class G-protein coupled receptor Smoothened (SMO), whose signaling activity is tightly regulated, is the sole obligate transducer of Hedgehog-related signals. However, except for previous reports of somatic oncogenic driver mutations in human cancers (or mosaic tumors in rare syndromes), any potential disease-related role of SMO genetic variation in humans is largely unknown. To our knowledge, ours is the first report of a human hypomorphic variant revealed by functional testing of seven distinct nonsynonymous SMO variants derived from HPE molecular and clinical data. Here we describe several zebrafish bioassays developed and guided by a systems biology analysis. This analysis strategy, and detection of hypomorphic variation in human SMO, demonstrates the necessity of integrating the genomic variant findings in HPE probands with other components of the Hedgehog gene regulatory network in overall medical interpretations.

Genetic variation in the highly conserved Sonic Hedgehog (SHH) gene is one of the most common genetic causes for the malformations of the brain and face in humans described as the holoprosencephaly clinical spectrum. However, only a minor fraction of known SHH variants have been experimentally proven to lead to abnormal function. Employing a phenotypic rescue assay with synthetic human messenger RNA variant constructs in shha-/- knockout zebrafish, we evaluated 104 clinically reported in-frame and missense SHH variants. Our data helped us to classify them into loss of function variants (31), hypomorphic variants (33), and nonpathogenic variants (40). We discuss the strengths and weaknesses of currently accepted predictors of variant deleteriousness and the American College of Medical Genetics and Genomics guidelines for variant interpretation in the context of this functional model; furthermore, we demonstrate the robustness of model systems such as zebrafish as a rapid method to resolve variants of uncertain significance.

The utilization of next generation sequencing has been shown to accelerate gene discovery in human disease. However, our confidence in the correct disease-associations of rare variants continues to depend on functional analysis. Here, we employ a sensitive assay of human FGF8 variants in zebrafish to demonstrate that the spectrum of isoforms of FGF8 produced by alternative splicing can provide key insights into the genetic susceptibility to human malformations. In addition, we describe novel mutations in the FGF core structure that have both subtle and profound effects on ligand posttranslational processing and biological activity. Finally, we solve a case of apparent digenic inheritance of novel variants in SHH and FGF8, two genes known to functionally coregulate each other in the developing forebrain, as a simpler case of FGF8 diminished function.

It is necessary to understand the role of surface modification in the oxygen permeation mechanism because the active coating layer on the ceramic membrane is important for dramatically enhancing the oxygen permeation flux. The effect of coating layers on oxygen permeation in a fluorite-rich dual-phase membrane [80 vol% Ce0.9Gd0.1O2−δ (GDC): 20 vol% La0.6Sr0.4Co0.2Fe0.8O3−δ(LSCF)] has been investigated as a function of membrane thickness and temperature to elucidate the extent of oxygen flux improvement due to surface modification. Various perovskite materials such as La0.6Sr0.4CoO3-δ (LSC), La0.6Sr0.4Co0.2Fe0.8O3-δ (LSCF), SrTi0.5Fe0.5O3-δ (STF), La0.7Sr0.3MnO3-δ (LSM), as well as the LSM/Ce0.9Gd0.1O2−δ (GDC)composite, have been adopted as the coating layers to enhance the oxygen permeation flux. The role of surface modification in the dual-phase membrane on the enhancement of oxygen flux has been systematically studied from the point of view of oxygen exchange kinetics and bulk diffusion. The oxygen permeation fluxes increased in the order of LSC > STF ≈ LSCF > LSM/GDC > LSM coating materials. The surface exchange kinetics coefficient (k) of the membrane with various coating materials can be calculated based on the characteristic thickness and bulk diffusion coefficient. The k values of the coated dual-phase membrane follow the k values of the coating materials. This result implies that the remarkable enhancement of oxygen flux in the dual-phase membrane with surface modification is closely related to the surface exchange kinetics of the coating materials.

Contains fulltext : 208539.pdf (Publisher’s version ) (Closed access) Zinc finger protein 462 (ZNF462) is a relatively newly discovered vertebrate specific protein with known critical roles in embryonic development in animal models. Two case reports and a case series study have described the phenotype of 10 individuals with ZNF462 loss of function variants. Herein, we present 14 new individuals with loss of function variants to the previous studies to delineate the syndrome of loss of function in ZNF462. Collectively, these 24 individuals present with recurring phenotypes that define a multiple congenital anomaly syndrome. Most have some form of developmental delay (79%) and a minority has autism spectrum disorder (33%). Characteristic facial features include ptosis (83%), down slanting palpebral fissures (58%), exaggerated Cupid's bow/wide philtrum (54%), and arched eyebrows (50%). Metopic ridging or craniosynostosis was found in a third of study participants and feeding problems in half. Other phenotype characteristics include dysgenesis of the corpus callosum in 25% of individuals, hypotonia in half, and structural heart defects in 21%. Using facial analysis technology, a computer algorithm applying deep learning was able to accurately differentiate individuals with ZNF462 loss of function variants from individuals with Noonan syndrome and healthy controls. In summary, we describe a multiple congenital anomaly syndrome associated with haploinsufficiency of ZNF462 that has distinct clinical characteristics and facial features.

Holoprosencephaly is the incomplete separation of the forebrain during embryogenesis. Both genetic and environmental etiologies have been determined for holoprosencephaly; however, a genetic etiology is not found in most cases. In this report, we present two unrelated individuals with semilobar holoprosencephaly who have the identical de novo missense variant in the gene CCR4-NOT transcription complex, subunit 1 (CNOT1). The variant (c.1603C>T [p.Arg535Cys]) is predicted to be deleterious and is not present in public databases. CNOT1 has not been previously associated with holoprosencephaly or other brain malformations. In situ hybridization analyses of mouse embryos show that Cnot1 is expressed in the prosencephalic neural folds at gestational day 8.25 during the critical period for subsequent forebrain division. Combining human and mouse data, we show that CNOT1 is associated with incomplete forebrain division.

Experimental and numerical studies on pressure drop in Reverse electrodialysis (RED) were performed. In this study, a module with 200 unit cells is considered for the demonstration of bench-scale RED module and two different unit cell configurations are utilized. Pressure drop through the module is measured by varying flow rates. For evaluating the hydrodynamic characteristics in the unit cell, a numerical simulation is also conducted and the simplified method using a porous media model is employed to simulate the channel filled with spacer. Due to the insertion of spacer and narrow channel, great pressure loss occurs along the unit cell. Based on estimated pressure data, high pressure difference between seawater and fresh water channel takes place locally in the unit cell configuration with crossflow direction, leading to a leakage problem through the membrane and finally degradation in the output power. Consequently, it is confirmed that the unit cell configuration is one of the important design parameters in a RED module.

The effect of temperature variation on the behavior of a non-premixed oxy-methane flame was investigated with a lab-scale slot burner at atmospheric pressure ( P ∞ = 1.01325 bar). The characteristics of flame behavior were simulated to analyze the flame structure and were observed by measuring the chemiluminescence from OH and CH radicals with a spectrometer and an intensified charge-coupled device (ICCD) camera. Flammable area was estimated by varying the flow velocity from m F = 0.02–0.16 g/s (i.e., u F = 7–50 m/s) for methane gas and m Ox = 0.24–2.71 g/s (i.e., u Ox = 18–117 m/s) for oxygen gas. The oxygen temperature at the nozzle exit ( T Ox ) was changed from T Ox = 300 to 700 K. Experimental measurements showed that the flammable area broadened as the oxygen temperature increased. The increase in oxygen temperature was associated with a decrease in the flame length. The concept of effective diameter was used to derive an empirical formula for flame length for use in a combustor design.

Mutations in FGFR1 have recently been associated with Hartsfield syndrome, a clinically distinct syndromic form of holoprosencephaly (HPE) with ectrodactly, which frequently includes combinations of craniofacial, limb and brain abnormalities not typical for classical HPE. Unrelated clinical conditions generally without craniofacial or multi-system malformations include Kallmann syndrome and idiopathic hypogonadotropic hypogonadism. FGFR1 is a principal cause for these less severe diseases as well. Here we demonstrate that of the nine FGFR1 mutations recently detected in our screen of over 200 HPE probands by next generation sequencing, only five distinct mutations in the kinase domain behave as dominant-negative mutations in zebrafish over-expression assays. Three FGFR1 mutations seen in HPE probands behave identical to wild-type FGFR1 in rescue assays, including one apparent de novo variation. Interestingly, in one HPE family, a deleterious FGFR1 allele was transmitted from one parent and a loss-of-function allele in FGF8 from the other parent to both affected daughters. This family is one of the clearest examples to date of gene:gene synergistic interactions causing HPE in humans.

Here, we applied targeted capture to examine 153 genes representative of all the major vertebrate developmental pathways among 333 probands to rank their relative significance as causes for holoprosencephaly (HPE). We now show that comparisons of variant transmission versus nontransmission among 136 HPE Trios indicates some reported genes now lack confirmation, while novel genes are implicated. Furthermore, we demonstrate that variation of modest intrinsic effect can synergize with these driver mutations as gene modifiers.

This paper deals with the development of a regenerator system with oxy-fuel combustion that enables fuel preheating. Such a system would save input energy as its combustor configuration is designed to provide high fuel temperature. In this study, three different combustor configurations were first considered and numerically analyzed. The simulation results revealed that fuel temperature and the distributions of the mixed gases were affected by the configuration of the combustor. The regenerator system with fuel preheating combustor was then set up and demonstrated in a bench-scale test rig. The experimental results indicated that the fuel temperature increased to over 410°C and that the heat recovery ratio increased by 9.6%. Thus, it was shown that the application of fuel preheating could enhance the thermal efficiency in the regenerator system with oxy-fuel combustion.

In this experimental study, a new system combining the oxy-fuel combustion with the concept of regeneration is developed. The performance of the regenerator system, which consists of a pair of oxy-fuel burners and regenerators, is demonstrated. To improve the thermal efficiency of the oxy-fuel combustion-based regenerator system, new methods are considered, such as exhaust gas bypass and CO 2 recirculation. The regeneration characteristics obtained when using oxy-fuel combustion are investigated by applying each method to the oxy-fuel combustion-based regenerator system. The temperature variation in the gas passing through the regenerator is significantly affected by the different operating methods and design parameters such as the switching time, regenerator configuration, and regenerator weight. The reference method, which is the same as air-fuel combustion, shows a low heat recovery ratio in the regenerator, whereas the exhaust gas bypass method has the highest value of the heat recovery ratio, followed by the CO 2 recirculation method, because of an improvement in the enthalpy imbalance between the exhaust gas and regeneration gas. From the study results, it is confirmed that the use of the exhaust gas bypass method or CO 2 recirculation method should be effective in improving the thermal efficiency of the oxy-fuel combustion-based regenerator system.

From the moment that a firing pin triggers the detonator to the moment that a grenade leaves a barrel is called Action Time. Since a loading and percussion of 40mm grenade happens simultaneously, action time should be within a certain time in order to prevent a Jamming malfunction. Previously, unreliable action time device of 40mm grenade made it difficult to improve quality assurance of K4 Grenade Machine Gun. Here, various sensors were compared and a special device was designed to seek an accurate measurement on action time. In this device, the gap between a signal from an optical sensor in Firing Pin and that from Eddy current probe in the barrel was recorded and data were sent to a computer in real time. Confirming if action time is within the criteria, it is expected that action time plays an important role in quality assurance on 40mm grenade.

Regenerative furnaces have been widely used to reduce waste heat, and to achieve constant temperature distribution in a furnace. However, direct application of the regenerative system for an air–fuel combustion furnace to an oxy-fuel combustion furnace is not possible, because of much higher volume flow rate in air–fuel combustion than the volume flow rate in oxy-fuel combustion. We therefore experimentally and numerically study the heat transfer performance of a ceramic honeycomb regenerator in oxy-fuel combustion. The pressures and temperatures in a regenerator are measured, and compared with numerical simulation that is calculated by using the CFD code, FLUENT, resulting in agreement. Numerical simulation shows that bypassing of ∼40% of the exhaust gas is essential, to prevent saturation of the honeycomb regenerator. Analysis of experimental data presents that a longer honeycomb and shorter switching time show better efficiency.

We report the synthesis of carbon-encapsulating carbonate apatite nanowires through vapor–solid growth by heat-treatment of biomass comprising calcium compounds such as CaC2O4 or CaCO3 at 900 °C using both PH3 and C2H2 as the reactants. The thermal decomposition of CaC2O4 or CaCO3 to CaO with increasing temperature (CaC2O4 → CaCO3 + CO → CaO + CO2) is the key to achieving the growth of such core–shell nanowires. First, vapor-phase reactions between the gaseous calcium species generated from the derived CaO and gaseous molecules derived from thermal reactions of the reactants (PH3 and C2H2) lead to the oriented growth of core–shell nanowires along the [001] plane. Second, the CO2 generated during the decomposition of CaCO3 may be primarily responsible for the incorporation of carbonate ions into the apatite structure. Nanowire growth with knots along the growth direction reveals that our approach is very controllable. Additional demonstrations using kenaf fibers further verify that other types of biomass too are usable.

A numerical study on the design of a steam reforming reactor consisting of several reforming tubes and one burner is conducted with respect to various operating and design parameters such as GHSV (gas hourly space velocity), input heat capacity, catalyst layer length, and number of tubes. The calculation of the reforming reaction rate is coupled with a three-dimensional heat and mass transfer calculation. It is shown that a large temperature gradient exists in the reforming reactor, resulting in significant variation of the gas temperature and reaction rate along the reforming tube. The reduction of the catalyst layer length induces a decrease in H2 (hydrogen) concentration as well as pressure loss. An increased number of tubes leads to better system efficiency owing to the enhanced heat transfer to the reforming tube. Consequently, to improve the system efficiency and reduce the pressure loss, an increase in heat transfer area and decrease in catalyst layer length should be essential design considerations.

An experimental study has been conducted for the design of the regenerative oxy-fuel combustion system with ceramic ball. Various design parameters are considered such as ball size, regenerator weight, and combustion load. Regenerative system with a pair of oxygen burners and regenerators is set up and the temperature of oxygen and exhaust gas passing through ball regenerator is measured. It is shown that the temperature distributions with time are affected by ball diameter and regenerator weight, and the significant temperature change is observed by combustion load. As the ball size decreases and the regenerator weight increases, the regenerating temperature efficiency increases. It is found that the heat recovery ratio is low despites of high regeneration temperature efficiency.

An experimental study of a honeycomb regenerator for oxy-fuel combustion has been performed. A laboratory-scale test rig is set up and various experimental parameters such as cell density and length of the honeycomb regenerator, switching time, and bypass effect are investigated. The typical temperature trend of the heating and regenerating process is obtained for the oxy-fuel combustion. The regenerative characteristics for various combinations of these parameters are shown. It is found that part of the exhaust gas should be bypassed to use waste heat more efficiently and to optimize the efficiency of the honeycomb regenerator system for oxy-fuel combustion.

Background Genetic factors predispose individuals to attention-deficit/hyperactivity disorder (ADHD). Previous studies have reported linkage and association to ADHD of gene variants within ADGRL3 . In this study, we functionally analyzed noncoding variants in this gene as likely pathological contributors. Methods In silico, in vitro, and in vivo approaches were used to identify and characterize evolutionary conserved elements within the ADGRL3 linkage region (~207 Kb). Family-based genetic analyses of 838 individuals (372 affected and 466 unaffected patients) identified ADHD-associated single nucleotide polymorphisms harbored in some of these conserved elements. Luciferase assays and zebrafish green fluorescent protein transgenesis tested conserved elements for transcriptional enhancer activity. Electromobility shift assays were used to verify transcription factor–binding disruption by ADHD risk alleles. Results An ultraconserved element was discovered (evolutionary conserved region 47) that functions as a transcriptional enhancer. A three-variant ADHD risk haplotype in evolutionary conserved region 47, formed by rs17226398, rs56038622, and rs2271338, reduced enhancer activity by 40% in neuroblastoma and astrocytoma cells ( p Bonferroni ADGRL3 expression. The rs2271338 risk allele disrupts binding of YY1 transcription factor, an important factor in the development and function of the central nervous system. Expression quantitative trait loci analysis of postmortem human brain tissues revealed an association between rs2271338 and reduced ADGRL3 expression in the thalamus. Conclusions These results uncover the first functional evidence of common noncoding variants with potential implications for the pathology of ADHD.

Exome sequencing offers an efficient and affordable method to interrogate genetic factors involved in human disease. Performing exome sequencing of monozygotic twins discordant for VACTERL (Vertebral anomalies, Anal atresia, Cardiac malformations, Tracheo-Esophageal fistula, Renal anomalies, and Limb abnormalities) association-type congenital malformations was hypothesized to potentially reveal discordant variants that could demonstrate disease cause(s). After demonstrating monozygosity, we applied high-density microarrays and exome sequencing to 2 twin pairs in which 1 twin had features of VACTERL association while the other was phenotypically normal (demonstrated through comprehensive clinical and radiological evaluation). No obvious discordant genotypic results were found that would explain phenotypic discordance. We conclude that VACTERL association is a complex disease, and while performing microarray analysis and exome sequencing on phenotypically discordant monozygotic twins may hypothetically reveal genetic causes of disorders, challenges remain in applying these methods in this circumstance.

A 1 kW solid oxide fuel cell (SOFC) balance of power (BOP) system is analyzed using experiments and simulations to optimize the design and operating conditions. The system consists of an autothermal reformer, combined heat exchanger and afterburner, and dummy stack. Thermolib program is used for the simulations. The system exhibits stable operation during a two cycle on/off experiment. The simulated results obtained by zero-dimensional modeling are similar to the experimental results although a discrepancy appears early in operation owing to the simplified modeling. The system's characteristics are also investigated in terms of the fuel utilization, presence or absence of internal reforming and external heat transfer coefficient.

The goal of paper is to investigate the flow and scalar distribution through the HP Nozzle Guide Vane (NGV) passage. Flow and scalar distribution measurement are conducted by using 5-hole pressure probe and tracing technique, respectively. Three different experimental cases are considered depending on cooling flow condition. The result shows that the vortical secondary flow patterns are observed clearly and these flow characteristics maintain through the NGV passage regardless of cooling flow injection. Compared to center region, the high axial velocity flow is observed near wall region due to cooling flow injection. Without cooling flow, the (scalar) distribution becomes to be uniform quickly due to the strong flow mixing phenomenon. However, in cases of cooling flow, scalar distribution is significantly non-uniform.

This paper describes an investigation of the local heat/mass transfer for rotating impingement/effusion cooling. A study was conducted of parameters such as jet orientation and surface geometry. An experiment using the naphthalene sublimation method provided the local heat/mass transfer coefficients on the effusion plate. The heat/mass transfer distributions for the axial orientation were similar to those for the stationary cases, while the trailing orientation produced different Sherwood number features, with divided high Sh regions and one low Sh region around the stagnation area. The concave surface provided better and more uniform heat/mass transfer than the flat surface.

The objective of this study is to investigate the heat/mass transfer characteristics on various impinging jets under rotating condition. Two cooling schemes related to impingement jet are considered; array impingement jet cooling and impingement/effusion cooling. The test duct rotates at Ro = 0.075 with two different jet orientations and the jet Reynolds number is fixed at 5000. Two H/d configurations of 2.0 and 6.0 are conducted. The detailed heat/mass transfer coefficients on the target plate are measured by a naphthalene sublimation technique. The rotation changes the local heat/mass transfer characteristics due to the jet deflection and spreading phenomenon. For H/d = 6.0, the jet is strongly deflected at the leading orientation, resulting in the significant decrease in heat/mass transfer. At the axial orientation, the momentum of jet core decreases slightly due to jet spreading into the radial direction and consequently, the value of stagnation peak is a little lower than that of the stationary case. However, reduction of heat/mass transfer due to rotation disappears at a low H/d of 2.0. In the averaged Sh, the leading orientation with H/d = 6.0 shows 35% lower value than that of the stationary case whereas the other rotating cases lead to a similar value of the stationary case.

A detailed measurement of the heat/mass transfer coefficients on the ribbed surfaces for the rotating impingement/effusion cooling system has been conducted. Three different jet orientations (front, leading, and trailing) were investigated at the same rotating speed and impinging jet Reynolds number of 3000. A naphthalene sublimation method was used to obtain local heat/mass transfer coefficients. Regardless of rib turbulators, the leading and trailing orientations lead to totally changed heat/mass transfer distributions due to the jet deflection, while the Sh distributions of the front orientation were similar to those of the stationary case. For leading and trailing orientations, the influence of crossflow, which deflected wall jets, decreased due to the blockage effect of the rib turbulators. Therefore, the wall jets spread more widely and the interaction between adjacent wall jets along spanwise direction became stronger, enhancing the heat/mass transfer compared to that on smooth surface.

In order to enhance the heat/mass transfer, a turbulator has been installed at the exit of injection hole for the impingement/effusion cooling system. The local heat/mass transfer coefficients have been obtained by a naphthalene sublimation method. Experiments have been carried out at the fixed jet Reynolds number of 10,000. Two turbulators with different diameter have been used in the current study. The result presents that the turbulator leads to the increase in flow mixing and jet velocity, consequently enhancing the heat/mass transfer at a stagnation region. Further, the stagnation region is divided into four small areas with peak value. In the existence of initial crossflow, the stagnation regions move downstream and low heat/mass transfer regions are formed regardless of the installation of turbulator. However, the increased jet velocity by turbulator reduces the crossflow effect against the jet, resulting in decrease of low heat/mass transfer regions. Compared to the case without turbulator, the installation of turbulator yields augmentation in averaged Sh value.

The objective of this paper is to investigate the heat/mass transfer characteristics on a concave surface for rotating impinging jets. The jet with Reynolds number of 5,000 is applied to the concave surface and the flat surface, respectively. The rotating experiments have been carried out at the rotating speed of 560RPM which is corresponding to Ro number of 0.075. The two jet orientation (front and trailing orientation) are considered. Detailed heat/mass transfer coefficients on the target plate were measured using a naphthalene sublimation method. The result indicates that the rotation leads to change in local heat/mass transfer distributions and the slight increase in the Sh level. The front orientation induces asymmetric Sh distributions, whereas the trailing orientation shows the shifted heat/mass transfer feature due to rotation-induced flow behavior. The crossflow effect on heat/mass transfer is also observed as the streamwise direction increases. Compared to flat surface, the heat/mass transfer on the concave surface is enhanced with increasing the spanwise direction due to the curvature effect, providing the higher averaged Sh value. It is proved that the difference of surface geometry affects somewhat the local and averaged heat/mass transfer regardless of rotation condition.

In zebrafish, the endoderm originates at the blastula stage from the most marginal blastomeres. Through a series of complex morphogenetic movements and differentiation events, the endodermal germ layer gives rise to the epithelial lining of the digestive tract as well as its associated organs such as the liver, pancreas, and swim bladder. How endodermal cells differentiate into distinct cell types such as hepatocytes or endocrine and exocrine pancreatic cells remains a major question. In a forward genetic screen for genes regulating endodermal organ development, we identified mutations at the shiri locus that cause defects in the development of a number of endodermal organs including the liver and pancreas. Detailed phenotypic analyses indicate that these defects are partially due to a reduction in endodermal expression of the hairy/enhancer of split-related gene, her5, at mid to late gastrulation stages. Using the Tg(0.7her5:EGFP)ne2067 line, we show that her5 is expressed in the endodermal precursors that populate the pharyngeal region as well as the organ-forming region. We also find that knocking down her5 recapitulates some of the endodermal phenotypes of shiri mutants, further revealing the role of her5 in endoderm development. Positional cloning reveals that shiri encodes Med12, a regulatory subunit of the transcriptional Mediator complex recently associated with two human syndromes. Additional studies indicate that Med12 modulates the ability of Casanova/Sox32 to induce sox17 expression. Thus, detailed phenotypic analyses of embryos defective in a component of the Mediator complex have revealed new insights into discrete aspects of vertebrate endoderm development, and provide possible explanations for the craniofacial and digestive system defects observed in humans with mutations in MED12.

An experimental investigation was conducted to investigate the heat/mass transfer for impingement/effusion cooling system with inclined jet. Jets with inclined angle of 60 are applied to impingement/effusion cooling. At the jet Reynolds number of 10,000, the experiments were carried out for blowing ratios ranging from 0.0 to 1.5. The local heat/mass transfer coefficients on the effusion plate are measured using a naphthalene sublimation method. The result indicates that the inclined jet causes the non-uniform and low heat/mass transfer compared to the vertical jet. At stagnation region, the peak position is shifted from the geometrical center of injection hole due to Coanda effect and its level is higher than that of vertical jet due to increase in turbulence intensity by steep velocity gradient near the stagnation region. Further, the secondary peak region disappears because the interaction between adjacent wall jets weakens. When the initial crossflow occurs, the distorted heat/mass transfer pattern appears. As the blowing ratio (crossflow rate) increases, the heat/mass transfer distributions become similar to those of the vertical jet. This is because the effect of crossflow is dominant compared to that of inclined jet under high blowing ratio (M≥1.0). At low blowing ratio (M≤0.5), averaged Sh value is 10% lower than that of vertical jet, whereas its value at high blowing ratio (M≥ 1.0) is similar to that of vertical jet.

The present paper deals with the heat/mass transfer characteristics for the rotating impingement/effusion cooling system. By changing the size and number of injection hole, its effects on heat/mass transfer are investigated and three different injection hole cases are considered such as LH, DH and SH, respectively. Reynolds number based on the effusion hole diameter is fixed to 3,330 and two jet orientations are considered. A naphthalene sublimation method is used to obtain the heat/mass transfer coefficients on the effusion plate. The LH case shows that the local heat/mass transfer is significantly varied by the rotation. Moreover, the low and non-uniform Sh distributions occur because the impinging jet is deflected by Coriolis force. Meanwhile, for DH and SH cases, the local heat/mass transfer coefficients are enhanced significantly compared to LH case and the rotation effect decreases with increasing the jet velocity. The averaged Sh value of DH and SH case rises up to 45%, 85% than that of LH case. However, the uniformity of heat/mass transfer deteriorates due to the steep variation of heat/mass transfer.

Limb body wall complex (LBWC) and amniotic band sequence (ABS) are multiple congenital anomaly conditions with craniofacial, limb, and ventral wall defects. LBWC and ABS are considered separate entities by some, and a continuum of severity of the same condition by others. The etiology of LBWC/ABS remains unknown and multiple hypotheses have been proposed. One individual with features of LBWC and his unaffected parents were whole exome sequenced and Sanger sequenced as confirmation of the mutation. Functional studies were conducted using morpholino knockdown studies followed by human mRNA rescue experiments. Using whole exome sequencing, a de novo heterozygous mutation was found in the gene IQCK: c.667C>G; p.Q223E and confirmed by Sanger sequencing in an individual with LBWC. Morpholino knockdown of iqck mRNA in the zebrafish showed ventral defects including failure of ventral fin to develop and cardiac edema. Human wild-type IQCK mRNA rescued the zebrafish phenotype, whereas human p.Q223E IQCK mRNA did not, but worsened the phenotype of the morpholino knockdown zebrafish. This study supports a genetic etiology for LBWC/ABS, or potentially a new syndrome.

An experimental investigation was conducted to enhance the heat/mass transfer for impingement/effusion cooling system when the initial crossflow was formed. For the improvement of heat transfer, the circular guide is installed on the injection hole. At the fixed jet Reynolds number of 10,000, the measurements were carried out for blowing ratios ranging from 0.5 to 1.5. The local heat/mass transfer coefficients on the effusion plate are measured using a naphthalene sublimation method. The result presents that the circular guide protects the injected jet from the initial crossflow, increasing the heat/mass transfer. The heat transfer of stagnation region is hardly changed regardless of the blowing ratio. The secondary peak is obviously formed by flow transition to turbulent flow. At high blowing ratio of 1.5, the circular guide produces augmentation on the averaged heat/mass transfer while the case without circular guide leads to the low and non-uniform heat/mass transfer. With the increased heat/mass transfer, the installation of circular guide is accompanied by the increase of pressure loss in the channel. However, the pressure drop caused by the circular guide is lower than that for other cooling technique with the circular pin fin.

The purpose of the present study is to investigate the local heat/mass transfer characteristics with circular pin fins in the impingement/effusion cooling with an initial crossflow. The results are compared with those for the cases without pin fins and with rib turbulators. The pin fins are installed between two perforated plates and the crossflow passes between these two plates. A blowing ratio is changed from 0.5 to 1.5 for the fixed jet Reynolds number of 10,000. The local heat/mass transfer coefficients on the effusion plate are measured using a naphthalene sublimation method. When circular pin fins are installed in the impingement/effusion system, heat/mass transfer is augmented due to the generation of vortex and wake. Especially, locally low heat/mass transfer regions in front of the effusion hole are reduced. Because pin fins divert the crossflow passing in the injection hole region, the wall jet is less swept away than the case without pin fins, which results in the increase of heat/mass transfer. As the blowing ratio increases, the pin fins lead to higher heat/mass transfer than the case without pin fins. Compared with rib turbulators, the pin fins yield lower average Sh value at M = 1.0 but higher average Sh value at other blowing ratios.

Mutation of the gene encoding the Mediator component thyroid hormone receptor-associated protein (TRAP)230/MED12 affects the development of multiple systems in zebrafish embryogenesis. We isolated two ethylnitrosourea-induced alleles in the gene encoding this protein and named the locus kohtalo ( kto ) after the homologous locus in Drosophila . Homozygous kto mutant zebrafish embryos show defects in brain, neural crest, and kidney development and die at ≈6 days postfertilization. In the affected tissues, differentiation is initiated and many cell type-specific genes are expressed, but there is a failure of morphogenesis and failure to complete differentiation. These results suggest that critical targets of TRAP230 function may include proteins important for cell mobility, cell sorting, and tissue assembly.

The present study has been performed to investigate the influences of rectangular fins on heat transfer in an impingement/effusion cooling system with crossflow. To simulate the impingement/effusion cooling system with initial crossflow, two perforated plates are placed in parallel and staggered arrangements with a gap distance of 2 times of the hole diameter. The crossflow passes between the plates, and various rectangular fins are installed on the plates. Reynolds number based on the hole diameter is fixed to 10,000 and the flow rate of crossflow is changed from 0.5 to 1.5 times of that of the impinging jet. A naphthalene sublimation method is used to obtain the heat/mass transfer coefficients on the effusion plate. Also to analyze the flow characteristics, a numerical calculation is performed. When rectangular fins are installed, the flow and heat transfer pattern is changed greatly from the case without fins. In the injection hole region, the jet impinges on effusion plate without deflection and wall jet spreads symmetrically. In the effusion region, the crossflow accelerates due to the decrease of cross-sectional area in the channel. Local heat/mass transfer coefficients are enhanced significantly compared to the case without fins. As the blowing ratio increases, the effect of rectangular fins against the crossflow becomes more significant and then the higher average heat/mass transfer coefficients are obtained than the case without fins. However, the increase of blockage effect gives more pressure loss in the channel.

An experimental study has been conducted to investigate the flow and heat/mass transfer characteristics within a rectangular film cooling hole of normal injection angle for various blowing ratios and Reynolds numbers. The results are compared with those for the square hole. The experiments have been performed using a naphthalene sublimation method and the flow field has been analyzed by numerical calculation using a commercial code (FLUENT). The heat/mass transfer around the hole entrance region is enhanced considerably due to the reattachment of separated flow and the vortices generated within the hole. At the hole exit region, the heat/mass transfer increases because the main flow induces a secondary vortex. It is observed that the overall heat/mass transfer characteristics are similar to those for the square hole. However, the different heat/mass transfer patterns come out due to increased aspect ratio. Unlike the square hole, the heat/mass transfer on the trailing edge side of hole entrance region has two peak regions due to split flow reattachment, and heat/mass transfer on the hole exit region is less sensitive to the blowing ratios than the square hole.

We have isolated a basic helix-loop-helix (bHLH) gene homologous to the Drosophila proneural gene atonal, termed zath3, from zebrafish. zath3 is expressed in neurons of the central nervous system and in subsets of cranial ganglia. Zebrafish mindbomb (mib) mutants have a higher density of zath3 expressing cells and narrowminded (nrd) mutants lack zath3 expression in a domain corresponding to primary sensory neurons showing that the expression of zath3 is regulated by both mib and nrd. Injection of synthetic zath3 RNA into zebrafish embryos expands the neural plate size, promotes ectopic expression of neuronal markers, and partially rescues the deficit of sensory neurons seen in nrd mutants. Interfering with zath3 function using antisense morpholino oligonucleotides (MO) has no significant effect on early neurogenesis. However, a double knock down of zath3 and neurogenin1 (ngn1), another atonal homologue, with morpholinos (MOs) leads to more severe defects in neurogenesis than are seen with ngn1 MO alone: a subtle reduction of motor and inter-neurons, and an almost complete loss all cranial ganglia. This study suggests that zath3 and ngn1 have partially overlapping roles in early neurogenesis.

A complete cDNA of a novel zebrafish gene named onecut has been isolated; this gene encodes a protein of 446 amino acids with a Cut domain (73 amino acid residues) and a homeodomain. The Cut domain of zebrafish Onecut is highly similar to those in mammalian hepatocyte nuclear factor-6 and Drosophila Onecut, sharing 90 and 88% amino acid identity, respectively. The expression of zebrafish onecut is restricted to neuronal cells, being first detected in trigeminal ganglia neurons at the end of gastrulation. By the 1-somite stage, onecut expression has begun in primary neurons of the lateral stripes in the neural plate, and appeared in neuronal cells of the medial stripes at the 2-somite stage. By the 4-somite stage, onecut expression expanded to the intermediate stripes and to subsets of neuronal cells in the midbrain and hindbrain. Subsequently, onecut expression intensified in the lateral region of midbrain and hindbrain, yet no onecut-positive cells were seen in the telencephalon. By 24 hpf, onecut transcripts remained abundant in the spinal cord but were no longer detectable in differentiated Rohon–Beard sensory neurons. The expression of onecut was greatly increased in the neural mutant mindbomb, while being decreased in narrowminded.

The aim of this paper is to perform the experiment and the numerical simulation for investigating the heat transfer in a regenerator system with ceramic honeycomb and to suggest a useful correlation for optimization of the regenerator system. For achieving this, the effects of some parameters were investigated, e. g., switching time, cell size and length of honeycomb on the mean temperature efficiency. The measured temperatures by R-type thermocouples were compared with the predictions by means of the commercial package, STAR-CD. A useful correlation for thermal efficiency was newly proposed as a function of the normalized switching time, defined in terms of switching time, cell size and length of honeycomb. The results showed that the thermal efficiency is above 90% and the normalized heat exchange rate is higher than 80% when the normalized switching time is less than 1000.

We have isolated and characterized two complete cDNA clones, Zfz8a and Zfz8b, which encode zebrafish Frizzled (Fz) homologues. The predicted protein sequences, spanning 579 and 576 amino acid residues for ZFz8a and ZFz8b, respectively, were highly homologous (78%) to each other and contained an extracellular cysteine-rich domain and seven transmembrane domains that are well conserved in Fz receptor protein members. In comparison with other Fz family members, ZFz8a and ZFz8b showed the highest homology with mouse Fz8 (MFz8), sharing 84 and 76% amino acid identity, respectively. The presence of Zfz8a and Zfz8b transcripts was detected by in situ hybridization in zebrafish embryos from the 512 cell stage, and their appearance in the future dorsal region could be observed before embryos reached the 30% epiboly stage. At shield stage, Zfz8a transcripts were expressed in both epiblast and shield whereas expression of Zfz8b was only detected in the embryonic shield. During gastrula stages, both Zfz8a and Zfz8b transcripts were found in anterior dorsal regions of the involuting mesendoderm (future prechordal plate). By the 2- to 3-somite stage, expression of both Zfz8a and Zfz8b was restricted to the prechordal plate and prospective anterior neurectoderm, although expression of the Zfz8a gene was no longer present in the most anterior portion of the prechordal plate, the polster. In one-eyed pinhead mutant embryos, which lack prechordal plate, both Zfz8a and Zfz8b transcripts were reduced, confirming the prechordal plate specificity of Zfz8a and Zfz8b gene expression. These results provide an additional evidence supporting the role of Wnt signaling in organizer-mediated axial patterning.