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Graphene–oxide–semiconductor (GOS) planar-type electron emission devices with a hexagonal boron nitride (h-BN) protective layer have demonstrated improved oxidation resistance while maintaining their emission performance. The devices with a monolayer or a multilayer (13 nm in thickness) h-BN protective layer can emit electrons even after oxygen plasma exposure (ashing). Remarkably, the device with a monolayer h-BN was able to emit electrons with a maximum efficiency of 11% after a 4-min ashing, showing that a thin h-BN protection layer can provide oxygen tolerance to GOS devices without a significant emission loss. The thicker multilayer h-BN imparted higher oxidation resistance to the device but with decreased emission efficiency compared with the device with monolayer h-BN. Thus, the use of h-BN necessitates a trade-off between the device’s emission performance and its oxidation resistance. In addition, the etching rate of h-BN by the oxygen plasma treatment was found to increase by exposure to air after the first plasma treatment, which indicates that the adherence of H2O to the surface of h-BN is one probable cause of h-BN etching during the ashing process.

A p-type graphene–oxide–semiconductor (GOS) planar-type electron emission device holds promise as a photocathode with a high quantum efficiency (QE) for generating a high-speed modulated electron beam. We report the electron emission properties of a planar-type electron emission device based on a GOS structure using lightly doped p-type silicon as a substrate under laser light irradiation. The electron emission efficiency is 12% with and without laser light irradiation. The emission current shows a four-order of magnitude enhancement in the high field region compared to that in the dark, and a 0.3% QE is obtained without a negative electron affinity surface. This GOS device exhibits advanced photoassisted electron emission characteristics with high photosensitivity on the order of mA/W. The photoemission from the p-type GOS device shows a fast photoresponse with a rise time of less than 0.2 µs, which is the same as that of the current amplifier. By contrast, it shows a slow response of about 2.4 µs at the fall time, which is determined by the diffusion process of the photoexcited electrons in the bulk. The use of a highly doped p-type silicon substrate can be a practical route for further improvement.

Objective Among standard treatments for infantile spasms, adrenocorticotropic hormone (ACTH) is reported as the best treatment, but ACTH is ineffective in one-half of the patients. To establish precision medicine, we examined pharmacoresistance of focal epileptic spasms (ES), generalized ES, and generalized ES combined with focal seizures, diagnosed based on the revised seizure classification of ILAE in 2017. Methods We conducted a retrospective nationwide study in Japan on the long-term seizure outcome of ES. Long-term seizure outcome was evaluated by seizure-free rate, seizure-free period, and Kaplan-Meier curve. Seizure-free was defined as seizure control for longer than two months. Results From the medical history of 501 patients, 325 patients had generalized ES only (GES group) at the start of the first treatment, 125 patients had generalized ES after focal seizure onset (FS-GES group), seven patients had focal ES after focal seizure onset (FS-FES group), and 24 patients had generalized ES combined with focal seizures after focal seizure onset (FS-GES+FS group). Seizure-free period of ES (generalized ES and focal ES) [mean (95% confidence interval)] was 2.7 (0.0-5.4) months in GES group, 1.1 (0.1-2.2) months in FS-GES group, 1.0 (0.2-1.9) months in FS-GES+FS group and 0.1 (-0.2-0.5) months in FS-FES group. Seizure-free rate, seizure-free period and Kaplan-Meier curve of generalized ES were almost the same in GES group and FS-GES group, with characteristics of superior response to ACTH. Mean seizure-free period of generalized ES combined with focal seizures was significantly shorter in FS-GES+FS group than in GES group. Mean seizure-free period of focal ES in FS-FES group was extremely short with exceedingly early relapse. Significance Pharmacoresistance was different in generalized ES, focal ES and generalized ES combined with focal seizures. ES with focal features or with focal seizures may have focal lesions, thus consider surgical options earlier in the course.

Spindt-type emitters were fabricated with cavities made of Al/Mo/SiO2 using the triode high power pulsed magnetron sputtering method. We explored the process parameters (gas pressure and voltage of the additional cap electrode) to optimize the sharpness of the emitter shape. We found that the intermediate pressure and voltage were suited to obtain sharp emitters. Further, we elucidated the crucial effect of the cavity dimensions, such as the cavity depth and hole diameter in the cavity ceiling, on the emitter shape. At a cavity depth of 480 nm, the aspect ratio (AR) of the emitter increased monotonously with an increase in the hole diameter. With a large hole diameter (900 nm) and even shallower cavity (380 nm depth), we attempted to reoptimize the process parameters. Consequently, a very sharp emitter cone structure with an AR exceeding 1.3 was obtained. The cap voltage that produced the optimum AR was found to decrease for the larger-hole and shallower-depth cavities. Finally, the applicability of the process for preparing a working emitter is discussed.

We report a case of saccharopinuria with hyperammonemia and hypercitrullinemia in a Japanese woman who presented with elderly-onset epilepsy, progressive cognitive decline, and gait ataxia. Blood amino acid analysis revealed an increase in citrulline, cystine, and lysine levels, and urine amino acid analysis showed increased citrulline and cystine levels. Urine metabolomics revealed an increased saccharopine level, leading to the definitive diagnosis of saccharopinuria. In western blots of liver biopsy samples, normal citrin levels were observed, suggesting that adult-onset citrullinemia type 2 (CTLN2) was not present. In addition, decreased argininosuccinate synthetase (ASS) levels were observed, and ASS1 gene, a causative gene for citrullinemia type 1 (CTLN1), was analyzed, but no gene mutations were found. Because the causes of hypercitrullinemia were not clear, it might be secondary to saccharopinuria. Muscle biopsy findings of the biceps brachii revealed diminished cytochrome c oxidase (COX) activity, mitochondrial abnormalities on electron microscopy and p62- positive structures in immunohistochemical analyses. Saccharopinuria is generally considered a benign metabolic variant, but our case showed elevated lysine and saccharopine levels causing ornithine circuit damage, mitochondrial dysfunction, and autophagy disorders. This may lead to so far unknown neurological disorders.

In this study, we fabricated nano-capillary emitter arrays for stable ion emission of ionic liquid electrospray thrusters, employing the fabrication of field emitter arrays or gated nano electron sources. A nano-capillary emitter was successfully fabricated with a 100–300 nm capillary diameter, which prevented ionic liquid leakage by significantly increasing the fluidic impedance of the ionic liquid compared to a previously proposed emitter. An ion emission experiment was conducted with 1-ethyl-3-methylimidazolium dicyanamide as the propellant. Ion emission started at a low voltage of 61 V owing to a small gap of approximately 1 μm between the emitter and extractor electrode. The maximum current density was 43 mA cm−2 on the positive side and −13 mA cm−2 on the negative side without leakage of the ionic liquid, which was more than 100 times higher than that of conventional electrospray thrusters. Moreover, we obtained continuous ion emission without current intercepted by the extractor.

A graphene-oxide-semiconductor (GOS) planar-type electron source was fabricated by direct synthesis of graphene on an oxide layer via low-pressure chemical vapor deposition. It achieved a maximum electron emission efficiency of 32.1% by suppressing the electron inelastic scattering within the topmost gate electrode using a graphene electrode. In addition, an electron emission current density of 100 mA/cm^2 was observed at an electron emission efficiency of 16.2%. The electron energy spread was well fitted to Maxwell-Boltzmann distribution, which indicates that the emitted electrons are the thermally equilibrium state within the electron source. The full-width at half-maximum energy spread of the emitted electrons was approximately 1.1 eV. The electron emission efficiency did not deteriorate after more than 42 h of direct current operation. Thus, the GOS planar-type electron source has the potential to be an excellent electron gun for electron microscopy., This article may be downloaded for personal use only. Any other use requires prior permission of the author and AIP Publishing. This article appeared in (https://aip.scitation.org/doi/10.1063/1.5091585) and may be found at [https://doi.org/10.1063/1.5091585].

We have developed a highly sensitive, compact image sensor that comprises a field emitter array (FEA) and a high-gain avalanche-rushing amorphous photoconductor target, with the ultimate aim of developing an ultrahighly sensitive, compact, high-definition television camera. Double-gated FEs have an advantage of having a compact electron beam focusing system; however, image intensities reproduced by the sensor were nonuniform due to low electron beam current. Furthermore, the simulated electron beam current disagreed with the measured current. The electron beam current characteristics of two types of double-gated, Spindt-type FEs (both with improved electron beam current extraction) are discussed for possible use within the sensor: convex-structured and volcano-structured. A highly accurate simulation model of the image sensor using a double-gated, Spindt-type FE has been examined; the simulated electron beam currents extracted from the double-gated, Spindt-type FE are in agreement with the measured electron beam currents when the initial electron velocity is assumed, thus suggesting that the simulated anode current–anode voltage characteristic conforms to the measured one. For example, the electron beam currents extracted from the convex-structured and volcano-structured FEs when the focusing electrode is placed 0.2 $\mu \text{m}$ below the gate electrode opening at a focusing electrode voltage of 15 V are, respectively, 1.8 and 1.9 times larger than that extracted from the previously used FEs when the focusing electrode is stacked 1.5 $\mu \text{m}$ above the gate electrode. The results show potential for reducing the degradation of the uniformity of the reproduced image’s intensity, and show that the highly accurate simulation model of the sensor is valid to design the double-gated FEAs for the sensor.

Methionine adenosyltransferase I/III (MAT I/III) deficiency is a metabolic disorder exhibiting persistent hypermethioninemia and neurological problems such as mental retardation and movement disorders by brain demyelination. Current diagnosis of MAT I/III deficiency completely depends upon newborn mass screening. Recently, correlation between the type of mutation of the MAT1A gene and clinical presentation has been investigated. The most common mutation, heterozygous for the autosomal dominant Arg264His mutation, is a relatively benign phenotype which requires no treatment. In contrast, care must be taken on the Arg292Cis mutation, especially if compounded with mutations at Arg356 (Arg356Pro, Arg356Leu, and Arg356Gln), for association of myelination disorder. Since MAT I/III catalyzes conversion of methionine to produce S-adenosylmethionine (SAM), supplementation of SAM is a therapeutic strategy to improve neurological problems. Hypermethioninemia can be corrected by methionine restriction; however, it may cause depletion of SAM. DNA testing is important for early diagnosis to prevent neurological manifestations.

To improve the thrust density of electrospray thrusters using ionic liquids as the propellant, we have fabricated a high-density emitter array utilizing the fabrication technique of a field emitter array (FEA) or gated nano electron sources. The density of the emitter array can reach approximately 4 million cm−2, which is four orders of magnitude higher than that of conventional electrospray thrusters. Performing deep reactive ion etching from the back side of the same structure as FEAs and removing their Ni cones by dilute nitric acid, we have successfully fabricated sub-μm scale capillary emitters with self-aligned extractor electrodes and ionic liquid reservoirs behind them. Current measurements were also conducted with EMI-BF4 and EMI-DCA as the propellant. Although the ion was not extracted with EMI-BF4 owing to its electrochemical reaction with the electrodes, both positive and negative ion currents were detected using EMI-DCA, where the current density exceeded that of conventional electrospray thrusters.

Electron emission properties of planar type electron emission device based on graphene-oxide-semiconductor (GOS) structure was investigated. The electron emission efficiency defined as the ratio of anode current to cathode current has no dependence on the graphene electrode thickness, which indicates that the electron scattering within the electrode is perfectly suppressed. A high electron emission current density of 1–100 mA/cm2 with high electron emission efficiency of 0.1–1.0 % was found to be achieved in GOS devices.

The electron emission properties of planar-type electron emission devices based on a graphene-oxide-semiconductor (GOS) structure before and after vacuum annealing were investigated. The fluctuation of the electron emission current was around 0.07%, which is excellent stability compared to the conventional field emitter array. The GOS devices were operable in very low vacuum of 10 Pa without any deterioration of their electron emission properties. Improvement of the electron emission properties of the GOS devices was achieved by vacuum annealing at 300 °C. The electron emission efficiency of the GOS type electron emission devices reached 2.7% from 0.2% after vacuum annealing. The work function of the graphene electrode was found to decrease 0.26 eV after vacuum annealing by Kelvin force probe microscopy analysis. These results indicated that the improvement of the electron emission efficiency of the GOS devices by vacuum annealing is due to the decrease in the work function of the graphene electrode.

Volcano-structured double-gated field emitter arrays (VDG-FEAs) with different focusing electrode height have been fabricated and the focusing characteristics for each VDG-FEAs were evaluated in detail. The authors experimentally proved that lowering the focusing electrode was very effective to keep a high emission current and successfully removed the electric field relaxation at the tip during the focusing operation. The anode current of about 1.9μA was maintained for the VDG-FEA with the height of the focusing electrode relative to the extraction electrode, hf=−470nm, even in applying 5V to the focusing electrode, and the current was 84.4 times larger, compared with the sample with hf=220nm under the same focusing condition. It was found that the emitted electrons returned back to the extraction electrode by the potential barrier caused by the low focusing potential below 3V in every sample.

We proposed a novel CdTe X-ray image sensor, which was driven by the FEA, to obtain high spatial resolution X-ray images and have demonstrated the principle operation by using the CdTe image sensor with one pixel. We have also fabricated a FEA matrix with 12×12 pixels to obtain X-ray images. For the further improvement of spatial resolution in the CdTe image sensor, we have proposed a novel double-gated FEA with a focusing lens, which was fabricated by using the etch-back method. The double-gated FEA showed a good focusing characteristic without significant decrease of the emission current, when the height of the focus electrode was optimized. The CdTe image sensor driven by the double-gated FEA is promising for an ultra-high-resolution X-ray image sensor.

Technologies for the cathode substrate side of field emission displays (FEDs) are briefly described. In addition to electron emitter technologies, evaluation techniques and ways to improve the emission of field emitters are described in detail because these technologies are important for developing practical applications. FEDs will be commercially available within one or two years, and therefore the next generation FEDs beyond these are discussed. © 2006 Institute of Electrical Engineers of Japan. Published by John Wiley & Sons, Inc.

Electron emissionproperties of Spindt-type platinumfield emissioncathodes were investigated. The current–voltage characteristics together with the current fluctuation during long term operation were evaluated in ultrahigh vacuum. The changes of the emission properties in hydrogen, oxygen or carbon monoxide gas ambient were also investigated. Significant improvement of the emission properties was found when the cathode was operated in carbon monoxide ambient under a certain condition. It was found that the effect of the improvement lasted at least 200 h, and resistance against the oxygen exposure was also improved. The changes of the electron emissionproperties during long term operation in ultrahigh vacuum and in gas ambient were analyzed with the Seppen–Katamuki chart, of which ordinate and abscissa are slope and intercept of Fowler–Nordheim plot. The electron emissionproperties were distributed along a straight line in the Seppen–Katamuki chart, and those of the cathode improved by carbon monoxide exposure were distributed along another line. The deviation of the electron emissionproperty in ultrahigh vacuum was considered to be due to change in the geometry of the emitting surface. The major reason for the modification of electron emissionproperties in carbon monoxide ambient was considered to be deposition of carbon onto the emitting surface, resulting in reduction of the work function. From the deviation of the Fowler–Nordheim characteristics in the Seppen–Katamuki chart, we presented an empirical equation for field emission.

Vacuum microelectronics is potential technology of building novel electronic devices that cannot be realized by using solid-state electronics. This paper reviews the current status of this technology. Micron-size cold cathodes using field electron emission have been developed for the electron sources of novel vacuum electron devices. Recently field emission displays are getting into the flat panel display market.

Field emission from individual tips in n- and p-type silicon field emitter arrays (FEAs) has been directly evaluated by using an electrostatic lens projector. The number of the emission spots in the p-type FEA increased with increasing total emission current, and the p-type FEA exhibited better uniformity of the emission than the n-type FEA. In order to improve the emission uniformity, the operation of the FEAs under an atmosphere of ambient gas was executed. The emission uniformity was improved dramatically by exposing C2H4 gas. Also, surface modification of Si field emitters was investigated for damageless vacuum sealing. The vacuum sealing process reduced the emission currents from the nontreated Si FEA by a factor of 10. On the contrary, the emission current from the CHF3-plasma-treated FEA did not change before and after the vacuum sealing process.

In this study, an array of Mo cones for volcano-structured Spindt-type microelectron emitters were fabricated. A recently developed triode high power pulsed magnetron sputtering system was used to control the positive plasma potential and efficiently accelerate ion species. By applying a proper positive voltage to the additional electrode, the authors obtained good cone shapes with high aspect ratios in a water-cooled microcavity structure made of two resist layers, which was previously impossible by conventional vacuum evaporation techniques. The effects of ion acceleration on the alignment of ions along the normal direction, as well as on the stress in the deposited film, are discussed. The former is important for the formation of sharp cones, while the latter is crucial for achieving stable fabrication.

We have fabricated a gated niobium nitride field emitter by the transfer mold method. A silicon mold was fabricated by anisotropical etching, followed by oxidation of the mold and deposition of niobium nitride. Niobium nitride thin films were prepared by ion-beam-assisted deposition. After attaching the sample to a glass substrate, the silicon mold was removed by mechanical and wet etching. A molybdenum thin film was then deposited by electron-beam evaporation. Formation of a gate aperture and an insulating silicon dioxide layer were performed by wet etching of only the apex, with other regions being protected by a photoresist layer. An electron emission test of the fabricated emitter array was performed in a high vacuum. We confirmed electron emission of up to 0.1 μA at the emitter–gate voltage of 30 V.

The electric field required to emit electrons for boron-doped diamond together with the emission site was estimated using measurement systems that can precisely control the emitter–collector gap and lateral position. Three different types of field emitters were prepared by microwave plasma chemical vapor deposition. Type A was a typical diamond film on a flat silicon substrate, type B was a heteroepitaxial diamond film that was deposited on a (111) oriented platinum substrate, and type C was a pyramidal diamond film fabricated by the transfer mold method. For all the emitters, the voltage required to extract 0.2 nA was measured as a function of the emitter–collector gap, where the device of the emitter–collector configuration is approximated by a pair of parallel plates. The results showed that a strong electric field was applied in front of the emitter. For type B, the emission current distribution was measured by a device in which the collector was an etched needle. It was found that the emission curren...

Electron emission characteristics of niobium nitride field emitter arrays were investigated as a function of nitrogen composition of niobium nitride. Field emitter arrays with 1024 tips were fabricated by depositing niobium nitride onto the silicon field emitter array, which was fabricated by photolithography and wet etching technique. The electron emission characteristics such as current-voltage characteristics and current fluctuation were measured in ultra high vacuum. The results showed that the field emitter array with mononitride showed lower effective work function and lower current fluctuation. The present results indicated that stoichiometric niobium nitride is preferred composition for field emission cathodes of vacuum microelectronics.

The authors have developed a highly sensitive, compact image sensor comprising a field emitter array (FEA) and a high-gain avalanche rushing amorphous photoconductor (HARP) target with the ultimate aim of developing an ultrahigh sensitivity, compact, high-definition television camera. Double-gated field emitters have the advantage of a compact electron beam focusing system; however, image intensities reproduced by a sensor with the double-gated, Spindt-type field emitter array with the focusing electrode stacked 1.5 μm above the gate electrode were nonuniform owing to low electron beam current. The minimum required electron beam current extracted from the double-gated field emitter array is considered for possible use with the sensor. Furthermore, a suitable field emitter array pitch to balance the electron beam current and the electrostatic-focusing lens strength was simulated. For the sensor's design guidelines, a field emitter array pitch of approximately 3 μm would be reasonable in the case of employing the volcano-structured, double-gated, Spindt-type field emitter array with the focusing electrode placed 0.2 μm below the gate electrode hole. Based on simulation results, an image sensor with the volcano-structured, Spindt-type, 3.1-μm-pitch FEA with the focusing electrode placed 0.2 μm below the gate electrode hole was fabricated. It was confirmed that the minimum electron beam current extracted from the volcano-structured FEA was approximately 2 μA/pix, and the sensor could obtain images with improved image intensity uniformity by utilizing the electrostatic-focusing effect. These results indicate that the volcano-structured, double-gated, Spindt-type FEA is potentially suitable for high-definition television FEA-HARP image sensors.

Graphene was used as the topmost electrode for a metal-oxide-semiconductor planar-type electron emission device. With several various layers, graphene as a gate electrode on the thin oxide layer was directly deposited by gallium vapor-assisted chemical vapor deposition. The maximum efficiency of the electron emission, defined as the ratio of anode current to cathode current, showed no dependency on electrode thickness in the range from 1.8 nm to 7.0 nm, indicating that electron scattering on the inside of the graphene electrode is practically suppressed. In addition, a high emission current density of 1–100 mA/cm2 was obtained while maintaining a relatively high electron emission efficiency of 0.1%–1.0%. The graphene-oxide-semiconductor planar-type electron emission device has great potential to achieve both high electron emission efficiency and high electron emission current density in practical applications.

A 985A--G transition is the single prevalent mutation representing 89% of all variant medium-chain acyl-CoA dehydrogenase (MCAD) alleles that causes MCAD deficiency. We and others previously devised a molecular method for the detection of the 985G allele that involves PCR coupled with NcoI digestion. The method has been widely used. However, when used for the analysis of dried blood samples, it sometimes produced ambiguous results with weak target bands in the presence of numerous artifact bands. An improved version of the method has been developed here, involving two stages of amplification using two different sets of primers. In the first stage, the entire exon-11 was amplified. A small aliquot (5 microliters) of the first PCR products were directly used as a template for the second PCR amplification. The second PCR is similar to the original method, but utilizes a pair of primers encompassing a smaller section within exon-11. The upstream primer incorporates a substitution at 981, so that a NcoI site involving 985G is introduced in the copies of the variant allele, as in the original PCR/NcoI method. The improved 2-stage method produces an intense target band with a very high sensitivity, yet devoid of artifacts, providing clean, unequivocal results.

We have developed a multi-gated field emitter (FE) such as a quadruple-gated FE with a three-stacked electrode lens and a quintuple-gated FE with a four-stacked electrode lens. Both the FEs can focus the electron beam. However, the quintuple-gated FE has a stronger electron convergence than the quadruple-gated FE, and a beam crossover is clearly observed for the quintuple-gated FE.

Argininosuccinate lyase (ASL) deficiency (McKusick 207900) is a rare autosomal recessive disorder affecting the urea cycle. The cardinal symptom in the neonatal form is progressive hyperammonemia, which is often life-threatening. However, clinical symptoms in the late onset form are quite heterogeneous. As well as measurement of ASL activity, analysis of the ASL gene is necessary to clarify the genetic basis of various phenotypes. We report a patient with late onset argininosuccinate lyase deficiency (ASLD) who had hepatomegaly and mildly increased level of ammonia. By mutation analysis of the mRNA and genomic DNA from the patient's leukocytes, we identified a novel missense mutation 1395G>C in the homozygous state, which results in the exchange of a stop codon to tyrosine at amino acid position 465 (X465Y). This unique mutation causes an elongation of fifty amino acids in the C-terminal region of the ASL protein, and is likely related to a milder phenotype compared with previously reported mutations. In addition, this is the first report on mutation analysis in a Japanese ASLD patient.

Type II citrullinemia (CTLN2) is characterized by a deficiency of argininosuccinate synthetase (ASS) in the liver. Mutation analysis of the SLC25A13 gene, which is responsible for CTLN2, provides a rapid and accurate diagnosis. We describe clinical, biochemical and histologic features of two patients, whose diagnosis was finally made by mutation analysis. They initially presented with symptoms related to hyperammonemia at 16 to 22 years of age. A patient had shown mental retardation and growth failure from early childhood. Laboratory findings including amino acids, were characteristic, such as elevated citrulline, arginine, and lysine concentrations, but definitive diagnosis had not been made. The patients died of liver cirrhosis and hepatoma at 31 and 34 years old, respectively. Fatty change in the hepatocytes was commonly observed in the autopsied specimens. ASS activity was decreased in the liver of both patients, and a concomitant decrease of arginase activity was found in one case. Investigation for the SLC25A13 mutation revealed that one patient was homozygous for IVS11 + 1G>A, and the other was compound heterozygote (851del4/S225X). Comparison of genetic, enzymatic and biochemical data among various cases of CTLN2 will be essential to understand the real nature of the disease.

We analyzed mutant genotypes at the human phenylalanine hydroxylase (PAH) locus among phenylketonuria (PKU) patients in the Far East of Russia. A total of 60 variant alleles from 30 PKU families were analyzed for prevalent Caucasian mutations and restriction fragment length polymorphism/variable number of tandem repeats (RFLP/VNTR) haplotypes. Seventy-eight percent of all variant alleles carried six mutations. The most prevalent mutation was R408W (63%), with a haplotype background of 2.3. It also showed a very high degree of homozygosity (43%). The other five mutations (R158Q, R261Q, R252W, R261X, and IVS12nt-1) accounted for 1.7%–6.7% of all PKU alleles, and a single haplotype was associated with each genotype, except for R261Q. The genetic structure of PKU patients in the Far East of Russia seems to be relatively homogeneous, compared with that in the other Slavic and Oriental populations of surrounding countries. Prediction of a clinical phenotype and carrier detection will be feasible using DNA tests.

We describe a mutation analysis for the phenylalanine hydroxylase gene and the clinical outcome of two Japanese patients with non-phenylketonuria (PKU) hyperphenylalaninemia (serum phenylalanine level below 600 mumol/l under a free diet) detected by a mass-screening program. Single strand conformation polynorphism analysis and direct sequencing of their genomic DNAs revealed that non-PKU hyperphenylalaninemia resulted from compound heterozygosity for a mutation causing classical PKU and a mutation with a milder effect on phenylalanine hydroxylase activity. The mutations were R241C and R243Q in exon 7, and R413P in exon 12. The mutation genotypes of the two patients were R241C/R243Q and R241C/R413P. It has been demonstrated that homozygosity for the R243Q or R413P mutation is associated with a severe phenotype of PKU and low in vitro expression activity. In contrast, the R241C mutation has much less effect on phenylalanine hydroxylase activity. The metabolic consequence of each variant allele was confirmed by a phenylalanine loading test in the patients and their parents. The patients achieved normal results in all intellectual and neurologic tests. Brain magnetic resonance imaging revealed no abnormalities. The dietary restriction was continued until 10 years of age in order to maintain the serum phenylalanine level below 400 mumol/l. The genetic analysis to distinguish non-PKU hyper-phenylalaninemia from classical PKU helps to determine the principles of dietary management in the early infantile period.

We investigated the proteolipid protein (PLP) gene in two brothers in a Japanese family with a connatal form of Pelizaeus-Merzbacher disease (PMD). Direct sequencing of the PLP gene revealed an A-to-T transition in exon 4, which led to an Asp-to-Val substitution at re-sidue 202. Their mother was confirmed to be heterozygous for the mutation. The mutation was not found in 78 X-chromosomes of normal Japanese individuals. A correlation between the clinical severity of the disease in the brothers and the Asp202-to-Val mutation in the PLP gene was suggested.

We propose a spin-on glass (SOG) film densification technique based on the plasma-treatment technology. We demonstrated the densification of the SOG film at the temperature of lower than 53 degree C without compulsory cooling. We investigate the plasma-densification condition and found that the optimum RF power needed to densify the SOG film. This technique is applicable for production processes in wide range of electronic devices.

A vertical thin film field emitter array (VTF-FEA) was integrated with a thin film transistor (TFT) through a simple fabrication process that is compatible with the existing TFT manufacturing process. Specifically, the authors integrated a poly-Si TFT and a previously reported VTF-FEA. The VTF-FEA was fabricated using the ion-induced bending (IIB) technique. The IIB is a technique that can bend a cantilever by ion-beam irradiation and can be used with a wide range of materials. The emission from the TFT-VTF-FEA was controlled from subnanoampere to microampere levels using the built-in TFT. This work demonstrated that VTF-FEAs can be integrated with poly-Si TFTs and other electronic devices, such as metal-oxide semiconductor field effect transistors.

A field emitter array (FEA) with a five-stacked gate electrode, that is, a quintuple-gated FEA with a four-stacked electrode lens, was fabricated. The four-stacked electrode forms an einzel lens, where the second and third electrodes are connected through a contact hole. Because the electron velocity of the einzel lens in a quintuple-gated FEA is smaller than for the quadruple-gated FEA, the quintuple-gated FEA has a stronger electron convergence. The slit sweeping method confirmed that a beam crossover is formed for the quintuple-gated FEA.

The authors proposed a simple fabrication technique of field emitter array based on a ion-induced bending (IIB) phenomenon. This technique is a very simple two-step process, involving the formation of a cantilever and subsequent ion irradiation. To form a vertical thin film emitter with practical ion dose, however, only a few specific film materials with limited thickness can be used. Therefore, an enhancement method of the IIB phenomenon is necessary. To enhance the IIB phenomenon, the authors proposed the double-layered film method. It was found that the ion-induced bending effect could be extremely enhanced by using a double-layered film method. The required dose could consequently be decreased to less than 5×1015cm−2 in the case of using a heavy element for the cantilever film. This methodology increases the potential of ion-induced bending as a simple fabrication technique for FEA device structures.

The authors describe a novel CdTe x-ray imaging device that is driven by an electron beam from a field-emitter array. This CdTe x-ray imaging device comprises a CdTe Schottky diode with a vertical thin-film field-emitter array (VTF-FEA). The VTF-FEA was fabricated by an ion-induced bending process and an etch-back technique. The output signal from the detector was obtained through the recombination of x-ray-generated holes and electrons emitted from the VTF-FEA. The output signal clearly depended on the intensity of the x-ray beam. The shape of a Cu plate was successfully detected by the VTF-FEA-driven CdTe diode.

The cytotoxicities of sodium benzoate was studied using primary culture of hepatocytes established from adult rat liver by a collagenase perfusion technique and maintained as a monolayer in serum-free culture medium. The activities of ornithine transcarbamylase (as a marker of mitochondria) and tyrosine aminotransferase (as a marker of cytosol) were clearly suppressed by sodium benzoate at concentration in excess of 500 micrograms/ml. Intracellular protein synthesis and DNA synthesis were also suppressed, and the suppression of DNA synthesis was observed even with a lower concentration of benzoate (100 micrograms/ml).