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Biomedical subjects

K Yamakawa

Publications and source records attributed to K Yamakawa.

At least 19 recordsLinked to original sources

Vitamin B6 deficiency augments endogenous oxalogenesis after intravenous L-hydroxyproline loading in rats.

The effects of an intravenous hydroxyproline load on endogenous oxalogenesis were compared in rats fed a standard diet or a vitamin B6-deficient diet. Twelve male Wistar rats were randomized to two groups and were fed either a standard diet (control group) or a vitamin B6-deficient diet for 3 weeks. Then the animals were intravenously administered 100 mg (762.6 micromol)/ml hydroxyproline. In the control group, infusion of hydroxyproline increased the 5-h urinary oxalate and glycolate excretion above baseline to 0.27% (2.02 +/- 1.11 micromol) and 0.32% (2.43 +/- 1.60 micromol) of the administered dose (mol/mol), while it was respectively 2.01% (15.24 +/- 2.13 micromol) and 0.00% (-0.02 +/- 0.19 micromol) of the dose in the vitamin B6-deficient group. Therefore, vitamin B6 deficiency augmented endogenous synthesis of oxalate from hydroxyproline by 7.56-fold (15.24/2.02) compared with that in the control group. Urinary citrate excretion was significantly lower at baseline and all other times in the vitamin B6-deficient group compared with the control group. In conclusions, L-hydroxyproline loading augmented endogenous oxalogenesis in the vitamin B6-deficient group without causing hyperglycolic aciduria, and also led to significant hypocitraturia. These findings suggest that hydroxyproline is not metabolized to oxalate via glycolate, but rather via the 4-hydroxyglutamate to glyoxylate pathway (usually requiring vitamin B6-dependent enzymes) even in the presence of vitamin B6 deficiency.

Animals↗

Different real-time PCR assays could lead to a different result of detection of varicella-zoster virus in facial palsy.

Real-time PCR is a useful tool for rapid detection of viral genomic DNA. However, there are many types of real-time PCR, and this variation may induce different results. The sensitivity of two different real-time PCR assays was evaluated for the detection of the varicella-zoster virus (VZV) genome: LightCycler PCR and TaqMan PCR. Auricular skin cells and saliva were sampled from 201 patients with facial nerve paralysis. A hundred and seventy-one of these patients were diagnosed clinically with Bell's palsy, and the remaining 30 with Ramsay Hunt syndrome. In 30 specimens obtained from Ramsay Hunt syndrome patients, VZV DNA was detected in 26 skin and 3 saliva specimens using the LightCycler PCR, while 28 skin and 9 saliva specimens were positive using the TaqMan PCR. None of the patients with Bell's palsy were positive for VZV by the LightCycler PCR, whereas five of these patients were positive by the TaqMan PCR. The TaqMan PCR assay has a better sensitivity compared to the LightCycler PCR for the detection of VZV genome from patients with facial palsy. Further study is required to develop a more sensitive real-time PCR.

Chickenpox↗

Multiple laryngeal foreign bodies composed of dried fish vertebral bones: a case report.

We present a rare case of tracheostomy for removal of laryngeal foreign bodies consisting of three connected fish vertebral bones in a 15-month-old girl. Recent endoscopic techniques have made it possible to extract nearly all tracheobronchial foreign bodies with rigid bronchoscopes. However, the three connected foreign bodies in this report could not be extracted entirely by single endoscopy because the glottis as an exit was narrow due to severe oedema. Accordingly, tracheostomy was required to assist ventilation, prevent prolonged post-operative endotracheal intubation, remove the secondary tracheal foreign bodies and to provide a conduit for the introduction of a bronchoscope. This suggests that tracheostomy should be considered to avoid the potential dangers of severe laryngeal oedema and to secure the route for removal of foreign bodies from the trachea when treating patients with multiple laryngeal foreign bodies and laryngeal oedema.

Animals↗

Phenotypes and genotypes in epilepsy with febrile seizures plus.

In the last several years, mutations of sodium channel genes, SCN1A, SCN2A, and SCN1B, and GABA(A) receptor gene, GABRG2 were identified as causes of some febrile seizures related epilepsies. In 19 unrelated Japanese families whose probands had febrile seizures plus or epilepsy following febrile seizures plus, we identified 2 missense mutations of SCN1A to be responsible for the seizure phenotypes in two FS+ families and another mutation of SCN2A in one family. The combined frequency of SCN1A, SCN2A, SCN1B, SCN2B, and GABRG2 mutations in Japanese patients with FS+ was 15.8%. One family, which had R188W mutation in SCN2A, showed digenic inheritance, and another modifier gene was thought to take part in the seizure phenotype. The phenotypes of probands were FS+ in 5, FS+ and partial epilepsy in 10, FS+ and generalized epilepsy in 3, and FS+ and unclassified epilepsy in 1. We proposed the term epilepsy with febrile seizures plus (EFS+), because autosomal-dominant inheritance in EFS+ might be rare, and most of EFS+ display a complex pattern of inheritance, even when it appears to be an autosomal-dominant inheritance. There is a possibility of simultaneous involvement of multiple genes for seizure phenotypes.

Adolescent↗

Spectroscopic characterization of an ultrashort-pulse-laser-driven Ar cluster target incorporating both Boltzmann and particle-in-cell models.

A model that solves simultaneously both the electron and atomic kinetics was used to generate a synthetic He alpha and satellite x-ray spectra to characterize a high intensity ultrashort laser driven Ar cluster target experiment. In particular, level populations were obtained from a detailed collisional-radiative model where collisional rates were computed from a time varying electron distribution function obtained from the solution of the zero-dimensional Boltzmann equation. In addition, a particle-in-cell simulation was used to model the laser interaction with the cluster target and provided the initial electron energy distribution function (EEDF) for the Boltzmann solver. This study suggests that a high density average, high, of 3.2 x 10(20) cm(-3) was held by the system for a time, delta tau, of 5.7 ps, and during this time the plasma was in a highly nonequilibrium state in both the EEDF and the ion level populations.

Journal Article↗

Electronic structure of charge- and spin-controlled Sr(1-(x+y))La(x+y)Ti(1-x)Cr(x)O3.

We present the electronic structure of Sr(1-(x+y))La(x+y)Ti(1-x)Cr(x)O3 investigated by high-resolution photoemission spectroscopy. In the vicinity of the Fermi level, it was found that the electronic structure was composed of a Cr 3d local state with the t(2g)3 configuration and a Ti 3d itinerant state. The energy levels of these Cr and Ti 3d states are well interpreted by the difference of the charge-transfer energy of both ions. The spectral weight of the Cr 3d state is completely proportional to the spin concentration x irrespective of the carrier concentration y, indicating that the spin density can be controlled by x as desired. In contrast, the spectral weight of the Ti 3d state is not proportional to y, depending on the amount of Cr doping.

Journal Article↗

Novel NHLRC1 mutations and genotype-phenotype correlations in patients with Lafora's progressive myoclonic epilepsy.

BACKGROUND: Lafora's progressive myoclonic epilepsy (Lafora's disease) is an autosomal recessive neurodegenerative disorder characterised by the presence of polyglucosan intracellular inclusions called Lafora bodies. Mutations in two genes, EPM2A and NHLRC1, have been shown to cause the disease. A previous study showed mutations in the EPM2A gene in 14 Lafora's disease families and excluded the involvement of this gene in five other families who were biopsy proven to have the disease. OBJECTIVE: To relate the genetic findings to the clinical course of the disease. METHODS: As part of an ongoing mutational study of the Lafora's disease genes, five new families with the disease were recruited and the genetic analysis was extended to screen the entire coding region of the NHLRC1 gene. Genotype-phenotype correlations were carried out. RESULTS: Seven NHLRC1 mutations were identified, including five novel mutations (E91K, D195N, P218S, F216_D233del, and V359fs32), in eight families with Lafora's disease. On relating the genetic findings to the clinical course of the disease it was shown that patients with NHLRC1 mutations had a slower rate of disease progression (p<0.0001) and thus appeared to live longer than those with EPM2A mutations. A simple DNA based test is described to detect the missense mutation C26S (c.76T-->A) in the NHLRC1 gene, which is prevalent among French Canadians. CONCLUSIONS: Patients with NHLRC1 mutations have a slower rate of disease progression than those with EPM2A mutations.

Adolescent↗

Electron acceleration by a nonlinear wakefield generated by ultrashort (23-fs) high-peak-power laser pulses in plasma.

We study experimentally the interaction of the shortest at present (23-fs) , relativistically intense (20-TW), tightly focused laser pulses with underdense plasma. MeV electrons constitute a two-temperature distribution due to different plasma wave-breaking processes at a plasma density of 10(20) cm(-3). These two groups of electrons are shown numerically to constitute bunches with very distinctive time durations.

Journal Article↗

Measurement of 2l-nl' x-ray transitions from approximately 1 microm Kr clusters irradiated by high-intensity femtosecond laser pulses.

X-ray line emission from 2l-nl' transitions in Ne-like Kr and nearby ions has been observed from approximately 1 microm Kr clusters irradiated by fs-scale laser pulses at the JAERI facility in Kyoto, Japan. The incident laser intensity reached 10(19) W/cm2, with pulse energies from 50 to 300 mJ and pulse durations from 30 to 500 fs. The dependence of the x-ray spectral features and intensity on the incident laser intensity is rather weak, indicating that the 1-2 ps cluster lifetimes limit the number of ions beyond Ne-like Kr that can be produced by collisional ionization. Lines from F- to Al-like Kr emitted from the cluster plasmas have been identified using data from the relativistic multiconfiguration flexible atomic code. A collisional-radiative model based on these data has been constructed and used to determine that the cluster plasma has electron densities near 10(22) cm(-3), temperatures of a few hundred eV, and hot electron fractions of a few percent.

Journal Article↗

Optimal control of ultrafast selection.

Optimal laser control for ultrafast selection of closely lying excited states whose energy separation is smaller than the laser bandwidth is reported on the two-photon transition of atomic cesium; Cs(6S-->7D(J), J=5/2 and 3/2). Selective excitation was carried out by pulse shaping of ultrashort laser pulses which were adaptively modulated in a closed-loop learning system handling eight parameters representing the electric field. Two-color fluorescence from the respective excited states was monitored to measure the selectivity. The fitness used in the learning algorithm was evaluated from the ratio of the fluorescence yields. After fifty generations, a pair of nearly transform-limited pulses were obtained as an optimal pulse shape, proving the effectiveness of the "Ramsey fringes" mechanism. The contrast of the selection ratio was improved by approximately 30% from the simple "Ramsey fringes" experiment.

Journal Article↗

Many-electron dynamics of a Xe atom in strong and superstrong laser fields.

We report on detailed investigations of ionization dynamics of a Xe atom exposed to intense 800-nm pulses of 20-fs duration in the extensive intensity range from 10(13)-10(18) W/cm(2). Ion yields of Xe+-Xe20+ were observed as a function of laser intensity and compared with the results from a single active electron based Ammosov-Delone-Krainov model. Unexpected ionization probabilities for lower charge states and no interplay between the inner and outer shells by screening are inferred. Suppression of nonsequential ionization towards higher intensity and few optical cycle regimes is also proved.

Journal Article↗

Relationship between ambulation and obesity in older persons with and without low back pain.

CONTEXT: For obese older persons, ambulation is both functionally important and a means of weight control. The relationship between weight and ambulation is not known in this population. Also, the extent to which pain interferes with ambulation is not studied. OBJECTIVE: To examine the relationship between obesity and ambulation, and to determine the effect of pain and body mass index (BMI) on ambulation in older persons. DESIGN, SETTING, AND PARTICIPANTS: Retrospective study of 82 older persons, ages 55-79 y, some with no back pain recruited from the community, others with back pain or spinal stenosis recruited from a magnetic resonance imaging (MRI) scanner as part of a larger university study of spinal stenosis. OUTCOME MEASURES: Age, Visual Analog Scales for pain, BMI, patient diagnosis (no pain, mechanical back pain, and spinal stenosis), walking velocity and stride length on a 15-min laboratory ambulation test, and 1-week community ambulation measured with a pedometer (steps, distance, and energy expenditure). RESULTS: BMI had a significant inverse relationship with ambulatory measurements in terms of the distance walked, steps taken, and walking velocity. Pain severity and pain category also had a significant inverse relationship with these measures. A negative correlation was observed between pain and obesity, although the relationship was statistically nonsignificant. DISCUSSION: Obese older people walked less than the nonobese older people. Pain was associated with decreased ambulation. Clinicians who intend to encourage increased ambulation in older obese persons should consider possible barriers posed by musculoskeletal pain.

Aged↗

0.85-PW, 33-fs Ti:sapphire laser.

We have successfully produced a laser pulse with a peak power of 0.85 PW for a pulse duration of 33 fs in a four-stage Ti:sapphire amplifier chain based on chirped-pulse amplification. To our knowledge this result represents the highest peak power pulses yet produced in any Ti:sapphire chirped-pulse amplification system.

Journal Article↗

Enhanced insulin gene expression by reduced intracellular glutathione level in insulin secreting cells MIN6.

Glutathione (GSH) participates in deoxidization and elimination of hydrogen peroxide and other reactive oxygen species, and plays an important part in the antioxidant system. To investigate the effect of GSH content on insulin gene expression, we utilized a stable transfectant, designated as ribo-MIN6 cells, which were stably transfected with the ribozyme of gamma-glutamylcysteine synthetase (gamma-GCS), exhibiting approximately 50% reduction of intracellular GSH content. We transiently transfected a luciferase expression vector driven by human preproinsulin gene promoter spanning from -1998 to +237 (pINS-1998/luc) and several deletion constructs into ribo-MIN6. Furthermore, transient transfection with ribozyme vector and pINS-1998/luc into wild-type MIN6 cells was also carried out. Luciferase activity was about 9-fold higher in ribo-MIN6 cells as compared to wild-type MIN6 cells. In the transient transfection of pINS-1998/luc with gamma-GCS ribozyme vector into wild-type MIN6 cells, the luciferase activity was increased in proportion to the added amounts of ribozyme vector. In transfection with deletion constructs, two major sites were found to be critical for insulin promoter activity. For the wild-type MIN6 cells, regions important for the promoter activity were also located at regions similar to those of ribo-MIN6 cells. Our results suggest that the suppression of intracellular GSH level might, in part, regulate the insulin gene expression.

Animals↗

Frequent mutations of SCN1A in severe myoclonic epilepsy in infancy.

Mutations in the neuronal voltage-gated sodium channel alpha-subunit type I gene (SCN1A) were found responsible for severe myoclonic epilepsy in infancy (SMEI). The authors describe novel mutations of SCN1A in Japanese patients with SMEI. They screened 12 unrelated patients and a pair of monozygotic twins and detected 10 mutations that lead to truncation of the protein.

Diseases in Twins↗

Decrease in the insulin receptor protein level by anti-insulin receptor antibodies: roles of tyrosine kinase activity and receptor internalization.

To investigate the mechanism of severe impairment of insulin action in type B insulin resistance, we extracted IgG from the serum of a patient with type B insulin resistance (B-IgG) and analyzed the inhibiting effect of B-IgG not only on insulin signaling but also on IGF-I signaling in Chinese hamster ovary (CHO) cells expressing human insulin receptor or human IGF-I receptor. Preincubation with 1 mg/ml B-IgG prevented insulin-induced phosphorylation of insulin receptor and insulin receptor substrate-1 (IRS-1) but did not alter the IGF-I-induced phosphorylation of the IGF-I receptor and IRS-1. (125)I-insulin binding was inhibited by 93% after preincubation with B-IgG at 37 degrees C and was recovered up to 50% of the control value by acid washing. However, when cells were preincubated with B-IgG at 4 degrees C, the insulin binding completely recovered the control value by acid washing. (125)I-IGF-I binding was not altered by B-IgG preincubation. Immunoblot study revealed that the protein level of the insulin receptor was strongly decreased by preincubation with 1 mg/ml B-IgG at 37 degrees C, but never at 4 degrees C. The IRS-1 protein level did not change by B-IgG preincubation. In order to know the role of the insulin receptor internalization in the inhibiting effect of B-IgG, we employed CHO cells expressing mutant insulin receptors which do not undergo internalization (CHO-K1018R). B-IgG incubation of CHO-K1018R at 37 degrees C failed to decrease the protein level of the insulin receptor. The present data indicate that IgG from the diabetic patient with type B insulin resistance decreased insulin receptor protein level, probably due to the enhanced degradation rate of the insulin receptor, in which insulin receptor tyrosine kinase activity and internalization are required for this process. This effect of B-IgG was specific for the insulin receptor with no effect on either IGF-I receptor or IRS-1, as reflected by the IGF-I effectiveness on glycemic control in this patient.

Animals↗