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Biomedical subjects

M Kato

Publications and source records attributed to M Kato.

At least 469 records · Page 26Linked to original sources

Chromatic sensitive epilepsy: a variant of photosensitive epilepsy.

We herein report 4 Japanese children who suffered epileptic seizures while watching a popular animated TV program. They showed a photoparoxysmal response that is more frequently observed in response to rapid color (blue/red) frame changes than monochromatic ones. These patients were all considered to have photosensitive epilepsy; however, chromatic sensitivity also plays an important role in the generation of such seizures.

Adolescent↗

Diffuse pachygyria with cerebellar hypoplasia: a milder form of microlissencephaly or a new genetic syndrome?

We report on 2 families with diffuse pachygyria and cerebellar hypoplasia, who presented hypotonia, ataxia, seizures, and developmental delay since infancy. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed decreased gyral formation in the cerebral cortex and marked hypoplasia in the cerebellum. Cerebellar hypoplasia is often associated with type 2 lissencephaly; however, our cases showed no polymicrogyria, and their clinical findings were quite mild compared with those of microlissencephaly. Their characteristic phenotype suggested a new genetic syndrome, which was possibly inherited as an autosomal recessive trait.

Cerebellar Diseases↗

A method for evaluating phylogenetic relationship of alpha-satellite DNA suprachromosomal family by nucleotide frequency calculation.

The sequence similarity among chromosome-specific alpha-satellite DNA was quantitatively evaluated by a novel procedure: nucleotide frequency calculation. Tandem-arrayed repetitive DNA segments were aligned with unit length repeat, and the nucleotide frequency at each position was used to estimate the phylogenetic distance between repetitive DNA segments. The calculations for human and chimpanzee X chromosome alpha-satellites showed that the results were consistent with the known relationships of primates, indicating that the nucleotide frequency calculation worked effectively to estimate the distances between satellite arrays. Human chromosome-specific alpha-satellites had been grouped into three suprachromosomal families (I, II, and III), and in the current work the nucleotide frequency analysis has defined the quantitative distances between the chromosome-specific alpha-satellite DNA.

Animals↗

Assessment of the myocardial protective effect of antegrade warm blood cardioplegia by measuring the release of biochemical markers.

Intermittent warm blood cardioplegia has been reported as a valuable alternative for myocardial protection in cardiac surgery; however, conflicting experimental data have been published. To assess the clinical effectiveness of intermittent warm cardioplegia, we measured the release of troponin-T (Tn-T), a highly sensitive and specific marker of myocardial damage, and creatine kinase MB isoenzyme (CK-MB), in 12 patients who underwent elective coronary artery bypass grafting (CABG) with antegrade intermittent warm blood cardioplegia (37 degrees C) being the warm group, in comparison with 16 patients who underwent CABG with antegrade intermittent cold blood cardioplegia (4 degrees C) being the cold group. Blood samples were taken to determine the serum concentrations of CK-MB and Tn-T, at the induction of anesthesia, then 3, 6, 12, and 24h after the termination of cardiopulmonary bypass (CPB). The peak increase in serum CK-MB levels, 3h after CPB, was significantly lower in the warm group than in the cold group, at 27.8+/-7.8 IU/l vs. 40.8+/-12.6 IU/l, respectively (P = 0.0042). The serum Tn-T 12 h after CPB was significantly lower in the warm group than in the cold group, at 1.40+/-0.71 ng/ml vs. 2.06+/-0.95 ng/ml, respectively (P = 0.049). In conclusion, intermittent antegrade warm blood cardioplegia showed effective myocardial protection in elective CABG.

Biomarkers↗

An experimental allergic conjunctivitis induced by topical and repetitive applications of Japanese cedar pollens in guinea pigs.

OBJECTIVE AND DESIGN: To develop a model of experimental allergic conjunctivitis, guinea pigs were given repetitive and topical applications of Japanese cedar pollen as an antigen, and the resulting allergic reaction was characterised. SUBJECTS: Male Hartley guinea pigs. TREATMENT: Guinea pigs were sensitised by insertion of small gelatin sponge pieces (3 pieces/eye, both eyes) containing the pollen extracts + Al(OH)3 into the palpebra superior/inferior sulci of both eyes for 8 h/day for 6 days. Then the animals were challenged by dropping pollen suspension in each eye once every week. METHODS: Time-course changes of conjunctivitis intensity score (CIS), which represents oedema and redness, and scratching frequency, and gamma1 and IgE levels in sera were assessed following the respective 1st-17th challenges. Ophthalmic lavage was performed to assay for albumin leakage and migrated leukocytes at each of the odd-numbered challenges. RESULTS: At relatively early stages of the repeated challenge, only a gradual increase of CIS was observed. However, thereafter, considerably higher CIS with the maximum at 30 min after the challenge was provoked at the 12th-17th challenges. Quite interestingly, there were differences in the CIS between the right and left eyes, depending on the individuals. Scratching, and albumin leakage and neutrophil influx into the lavage fluid were also developed during the challenges. Although the anaphylactic antibodies against the antigen were detected in the sera from half of the animals, the levels were not correlated to severity of the symptoms. CONCLUSIONS: The present sensitisation/challenge procedures are unique in terms of topical application of antigen. The lack of any correlation between the levels of the anaphylactic antibodies and severity of anaphylactic symptoms, and the difference of CIS between the left and right eyes of an individual strongly suggest that the anaphylactic antibodies are formed in locally limited tissue surrounding an eye. The present method should be useful for analysing the mechanisms of allergic conjunctivitis.

Administration, Topical↗

Handlebar hernia: case report and review of pediatric cases.

The authors describe a rare case of handlebar hernia in a 9-year-old-boy. All layers of his abdominal wall were disrupted by a fall on a bicycle; however, his skin and intra-abdominal organs were completely intact. Computed tomography demonstrated subcutaneous intestinal loops protruding through the rent. Surgical repair was performed, and his postoperative course was uneventful.

Abdominal Muscles↗

Astrocytic hyaline inclusions contain advanced glycation endproducts in familial amyotrophic lateral sclerosis with superoxide dismutase 1 gene mutation: immunohistochemical and immunoelectron microscopical analyses.

To clarify the neuropathological significance of the deposition of N(epsilon)-carboxymethyl lysine (CML), an advanced glycation endproduct, in astrocytic hyaline inclusions in familial amyotrophic lateral sclerosis (FALS), autopsy specimens from five members of two different families who had the superoxide dismutase 1 (SOD1) gene mutations were analysed. Immunohistochemically, most of the neuronal and astrocytic hyaline inclusions were intensely stained by the antibody against CML. The distributions and intensities of the immunoreactivities for CML and SOD1 were similar in the inclusions in both cell types. Immunoelectron microscopy showed that both inclusions consisted of CML-positive granule-coated fibrils and granular materials. No significant CML or SOD1 immunoreactivity was observed in the neurons and astrocytes of the normal control subjects. Our results suggest that astrocytic hyaline inclusions contain CML and SOD1 in FALS patients with SOD1 gene mutations, and that the formation of CML-modified protein (probably CML-modified SOD1) is related to the cell degeneration.

Adult↗

A novel mutation of the doublecortin gene in Japanese patients with X-linked lissencephaly and subcortical band heterotopia.

The doublecortin (DCX) gene was recently found to be involved in patients with X-linked lissencephaly and subcortical band heterotopia or double cortex syndrome. We have studied the coding regions of the DCX gene in 11 Japanese patients with cortical dysplasia and have identified three different mutations (R186C in exon 3, R272X and R303X in exon 5) in four sporadic female cases. R272X, which has been detected in two unrelated cases, is a novel mutation. Although the number of cases studied remains limited, exon 5 may be a common mutational site in Japanese patients in contrast to many previous reports concerning exons 2 and 3.

Adolescent↗

Prader-Willi syndrome in a child with XYY.

We report a 26-month-old boy with XYY syndrome, with the complication of Prader-Willi syndrome (PWS) due to uniparental maternal disomy of chromosome 15. To our knowledge, this is the first case of XYY syndrome and PWS. Clinical findings were fully compatible with the diagnostic criteria for PWS. Molecular analysis revealed a maternal heterodisomy of chromosome 15, indicating that non-disjunction of chromosome 15 had occurred at maternal meiosis I, and that the non-disjunction of chromosome Y and of chromosome 15 had occurred independently.

Child, Preschool↗

Altered purine and glycogen metabolism in skeletal muscle during exercise in patients with heart failure.

Plasma levels of ammonia and hypoxanthine (HX) can be indices of purine nucleotide degradation. The present study determined if patients with heart failure (HF) have altered exercise plasma ammonia and HX levels relative to the peak work rate performed. Blood lactate, plasma ammonia, and plasma HX levels were measured in 59 patients with HF (New York Heart Association [NYHA] classes I:20, II:21, and III:18) and 21 controls at rest and after a maximal cardiopulmonary exercise test. The peak work rate (normal and NYHA I, II, and III, 163+/-11, 152+/-9, 94+/-5, and 69+/-5 W) and peak oxygen uptake ([VO2] 32.3+/-1.7, 25.1+/-0.9, 18.6+/-0.5, and 14.1+/-0.6 mL/min/kg) decreased as the NYHA functional class increased. The increment from rest to peak exercise (delta) for lactate ([(delta)lactate] 6.1+/-0.3, 4.8+/-0.4, 4.6+/-0.3, and 2.9+/-0.3 mmol/L), (delta)ammonia (132+/-14, 119+/-20, 94+/-13, and 32+/-6 microg/dL), and (delta)HX (33.5+/-3.4, 24.9+/-4.7, 20.6+/-3.0, and 9.9+/-1.2 micromol/L) was progressively smaller as HF worsened. The ratio for (delta)lactate to peak work rate (0.037+/-0.003, 0.032+/-0.004, 0.049+/-0.003, and 0.042+/-0.005) was higher in classes II to III HF, while the ratio for (delta)ammonia to peak work rate (0.81+/-0.14, 0.78+/-0.16, 0.99+/-0.11, and 0.47+/-0.11) was significantly lower in class III HF. In summary, patients with HF exhibited a smaller ammonia response with a higher lactate response to exercise when normalized with the peak work rate. These results suggest there may be an altered purine and glycogen metabolism during exercise in skeletal muscle in patients with HF.

Ammonia↗

Behavioral evidence for visual perception of 3-dimensional surface structures in monkeys.

Human subjects perceive two crossing bars, one in front of the other, when shown a cross with disparity added to its horizontal limbs, and they also perceive neon-color spreading when shown a stereoscopic Redies-Spillmann figure. It has thus been hypothesized that the human visual system follows the principle of generic image sampling in reconstructing 3-dimensional (3-D) surface structures. Here we examine whether monkeys also perceive these surface structures. The results indicate that monkeys, like humans, perceive two crossing bars and neon-color spreading and suggest that the principle of generic image sampling may also be applied to visual perception in monkeys.

Animals↗

Specific interaction of fused H protein of bacteriophage phiX174 with receptor lipopolysaccharides.

The DNA fragment encoding the spike H protein of bacteriophage phiX174 was amplified by polymerase chain reaction. The fragment was sub-cloned into pQE-30 to yield pQE-H. The histidine-tagged H protein (HisH) was obtained from the cell extract of Escherichia coli M15 (pREP4) harboring pQE-H and purified by nickel chelating and anion-exchange chromatographies. HisH was shown to bind dose-dependently to the lipopolysaccharides (LPSs) isolated from phiX174-sensitive strains, E. coli C or Salmonella typhimurium TV119 (Ra mutant). In sharp contrast, HisH did not bind to the LPSs from insensitive strains, E. coli F583 (Rd2 mutant) or E. coli O111:B4 (smooth strain). Since the same selectivity was observed in the plaque counting assay for in vitro inactivation of phiX174, the spike H protein was shown to recognize receptor LPS.

Bacteriophage phi X 174↗

Role of Rho small GTP binding protein in the regulation of actin cytoskeleton in hepatic stellate cells.

BACKGROUND/AIMS: In the fibrotic response to liver injury, hepatic stellate cells are activated, leading to the myofibroblastic cell shape, with actin cytoskeletal reorganization and increased extracellular matrix production. The reorganization of actin cytoskeleton suggests that the small GTP binding protein Rho might modulate the process of this myofibroblastic change. The aim of this study was to investigate the role of Rho in the phenotypic changes of hepatic stellate cells. METHODS: The phenotypic changes were investigated by the overexpression of Rho regulator, Rho GDI or dominant negative mutant of Rho in mouse hepatic stellate cell line, GRX cells. In activated rat hepatic stellate cells, the effects of microinjection of Botulinus toxin C3, which is the specific inhibitor for Rho, were analyzed. Furthermore, the effect of C3 on the type I collagen accumulation in hepatic stellate cells was investigated. RESULTS: Overexpression of Rho GDI or the dominant negative mutant of Rho caused the shrinkage cell shape and suppressed stress fiber formation. Microinjection of toxin C3 caused a markedly distorted cell shape and the disappearance of stress fibers in rat stellate cells. In addition, C3 strongly suppressed collagen accumulation in activated stellate cells. CONCLUSIONS: These results suggest that Rho regulates the actin cytoskeletal reorganization, and may be implicated in the collagen accumulation in activated stellate cells. These findings provide evidence for the role of Rho in the myofibroblastic phenotype in hepatic stellate cells.

Actins↗

Mercuric chloride induces increases in both cytoplasmic and nuclear free calcium ions through a protein phosphorylation-linked mechanism.

The mechanism of the lymphocyte stimulatory action of sulfhydryl group-reactive mercuric ions was studied with respect to its potential ability to induce a protein tyrosine phosphorylation-linked signal for mobilization of free Ca2+ into cytoplasm and nucleus of the cell. Exposure of human leukamic T cell line (Jurkat) cells to high (1 mM) and low (0.01 mM) concentrations of HgCl2 induced tyrosine phosphorylation of multiple proteins in a concentration-dependent manner. Confocal microscopy directly visualized the time course localization of Ca2+ inside the cells after exposure to HgCl2. The onset and level of Ca2+ mobilization following HgCl2 exposure were in parallel to those of protein tyrosine phosphorylation. Interestingly, by either concentration of HgCl2, Ca2+ was mobilized in both cytoplasm and nucleus almost simultaneously, and the level of Ca2+ mobilization in the nucleus was more than that in the cytoplasm. All the HgCl2-mediated Ca2+ mobilization was prevented by addition of protein kinase inhibitor staurosporin prior to HgCl2. These results suggest that heavy metal stress triggers a protein tyrosine phosphorylation-linked signal that leads to a nuclear event-dominant Ca2+ mobilization.

Calcium Signaling↗

Differences in evoked potential characteristics between DRPLA patients and patients with progressive myoclonic epilepsy: preliminary findings indicating usefulness for differential diagnosis.

The characteristics of evoked potentials in patients with dentatorubral-pallidoluysian atrophy (DRPLA) were investigated. Twelve patients with DRPLA and three patients with progressive myoclonic epilepsy (PME) attributable to other causes participated in the study. In 11 out of the 12 patients, the diagnosis of DRPLA was genetically confirmed, based on a 56-75 CAG triplet repeat expansion on chromosome 12p; in the remaining patient, the diagnosis was not genetically confirmed but the patient was clinically diagnosed as having DRPLA and was within the same pedigree as one of the 11 genetically confirmed patients. Two out of the three patients with PME, who had been tested for dodecamer repeat expansion in the cystatin B gene, were genetically confirmed as having Unverricht-Lundborg disease (UL); the remaining patient was also clinically diagnosed as having UL, but the patient did not have the aforementioned genetic abnormality. Somatosensory evoked potentials (SEPs) and brainstem auditory evoked responses (BAERs) were recorded. The amplitudes of the SEPs were determined as the peak-to-peak amplitudes between P2 and N2 deflections. The results revealed that high-amplitude SEPs were not evoked in any of the DRPLA patients; on the other hand, high-amplitude SEPs were evoked in all the patients with UL. Moreover, BAERs were absent in seven out of the 12 patients with DRPLA; on the other hand, all UL patients showed BAERs in which all peaks, from I to V, were distinguishable. These results suggest differences in pathophysiology between DRPLA, which predominantly affects the brainstem and subcortical regions, and PME, characterized by cortical hyperexcitability. Thus, evoked potential measurements may be useful to differentiate DRPLA patients from those with progressive myoclonic epilepsy.

Adolescent↗