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T Nishio

Publications and source records attributed to T Nishio.

At least 19 recordsLinked to original sources
PubMed1997

CAG repeat length and disease duration in Machado-Joseph disease: a new clinical classification.

To evaluate the clinical characteristics of Machado-Joseph disease (MJD) with reference to CAG repeat length and disease duration, we analyzed neurologic findings in 108 patients from 84 families. The majority of MJD patients presented with an ataxic gait as the initial symptom. Dysarthria and nystagmus were observed from an early stage. Bulging eyes, muscle atrophy and bradykinesia developed later. Patients with a shorter CAG repeat length or later onset had more frequent involvement of proprioceptive sensory deficit. Incidence of abnormal reflexes, tones, and proprioceptive sensation was not associated with disease duration, but with CAG repeat length. Based on these results, we propose a new clinical classification: type A (juvenile type), with hyperreflexia and dystonia, but without a proprioceptive sensory deficit; type C (adult type), with hyporeflexia and a proprioceptive sensory deficit, but without dystonia; and type B (intermediate type), the remaining patients with a mixed presentation.

Adult↗
PubMed1997

Striking sequence similarity in inter- and intra-specific comparisons of class I SLG alleles from Brassica oleracea and Brassica campestris: implications for the evolution and recognition mechanism.

Self-incompatibility in Brassica is controlled by a single multi-allelic locus (S locus), which contains at least two highly polymorphic genes expressed in the stigma: an S glycoprotein gene (SLG) and an S receptor kinase gene (SRK). The putative ligand-binding domain of SRK exhibits high homology to the secretory protein SLG, and it is believed that SLG and SRK form an active receptor kinase complex with a self-pollen ligand, which leads to the rejection of self-pollen. Here, we report 31 novel SLG sequences of Brassica oleracea and Brassica campestris. Sequence comparisons of a large number of SLG alleles and SLG-related genes revealed the following points. (i) The striking sequence similarity observed in an inter-specific comparison (95.6% identity between SLG14 of B. oleracea and SLG25 of B. campestris in deduced amino acid sequence) suggests that SLG diversification predates speciation. (ii) A perfect match of the sequences in hypervariable regions, which are thought to determine S specificity in an intra-specific comparison (SLG8 and SLG46 of B. campestris) and the observation that the hypervariable regions of SLG and SRK of the same S haplotype were not necessarily highly similar suggests that SLG and SRK bind different sites of the pollen ligand and that they together determine S specificity. (iii) Comparison of the hypervariable regions of SLG alleles suggests that intragenic recombination, together with point mutations, has contributed to the generation of the high level of sequence variation in SLG alleles. Models for the evolution of SLG/SRK are presented.

Alleles↗
PubMed1997

Technetium-99m-sestamibi scintimammography of benign and malignant phyllodes tumors.

We presented two cases of phyllodes tumor of the breast examined by 99mTc-sestamibi (MIBI) two-phase scintimammography. In the case with malignant phyllodes tumor, 99mTc-MIBI accumulation was recognized on both early and delayed images. In the case with benign phyllodes tumor, however, 99mTc-MIBI accumulation was recognized on only the early image. 99mTc-MIBI delayed imaging may have the potential to distinguish between benign and malignant phyllodes tumors.

Breast Neoplasms↗
PubMed1997

Chronic expanding hematoma in the chest.

We herein report the successful surgical treatment of 2 cases of chronic expanding hematoma in the chest. The first patient, who had undergone thoracoplasty 42 years earlier due to tuberculosis, became aware of a slowly growing mass protruding in the lateral thoracic wall. The second patient, who had tuberculous pleurisy 36 years earlier, was referred to our department because of a slowly expanding intrathoracic mass revealed by a roentgenogram. The tumors, which were encapsulated chronic hematomas, were both surgically resected. These cases are rare because of the development of a very large mass after undergoing treatment for tuberculosis more than 30 years previously.

Aged↗
PubMed1997

Molecular features of the CAG repeats of spinocerebellar ataxia 6 (SCA6).

Spinocerebellar ataxia 6 (SCA6) is an autosomal dominant spinocerebellar degeneration caused by the expansion of the polymorphic CAG repeat in the human alpha1A voltage-dependent calcium channel subunit gene (CACNL1A4 gene). We have analyzed 60 SCA6 individuals from 39 independent SCA6 Japanese families and found that the CAG repeat length is inversely correlated with the age of onset (n = 58, r = -0.51, P < 0.0001). SCA6 chromosomes contained 21-30 repeat units, whereas normal chromosomes displayed 6-17 repeats. There was no overlap between the normal and affected CAG repeat number. The anticipation of the disease was observed clinically in all eight parent-child pairs that we examined; the mean age of onset was significantly lower (P = 0.0042) in children than in parents. However, a parent-child analysis showed the increase in the expansion of CAG repeats only in one pair and no diminution in any affected cases. This result suggests that factors other than CAG repeats may produce the clinical anticipation. A homozygotic case could not demonstrate an unequivocal gene dosage effect on the age of onset.

Adult↗
PubMed1997

Substrate recognition by mitochondrial processing peptidase toward the malate dehydrogenase precursor.

Mitochondrial processing peptidase (MPP) cleaves the extension peptides of precursor proteins newly imported into the mitochondria. Using synthetic oligopeptides modeled on the extension peptide of malate dehydrogenase, the critical elements of the substrate for the processing of MPP were determined [Niidome, T., Kitada, S., Shimokata, K., Ogishima, T., and Ito, A. (1994) J. Biol. Chem. 269, 24719-24722; Ogishima, T., Niidome, T., Shimokata, K., Kitada, S., and Ito, A. (1995) ibid. 270, 30322-30326]. In the present study, we constructed mutant precursors and compared the processing reaction with that of the peptide substrates to confirm the validity of use of peptide substrates. In both cases, the arginine residue presents at a proximal (-2) position relative to the processing site proved to be important for the processing. The distal arginine residue at position 7 was replaceable with alanine with no significant loss in cleavage efficiency if the precursor protein contained two consecutive arginine residues at a proximal position, although the arginine residue at a position 7 was indispensable in the model peptide. The proline residue, lying between the distal and proximal arginine residues, which is assumed to break a continuous alpha-helix region in the extension peptide, was needed for the processing. This peptidase has a preference for aromatic amino acids at the P1' site. These results were essentially the same as those obtained with model peptides except for the role of the distal arginine. We also found that amino acids at P2' and P3' sites had some effects on the processing. Thus we concluded that an effective combination of model peptides with precursor proteins is needed for the studies on MPP responsible substrate-recognition mechanisms.

Amino Acid Sequence↗
PubMed1997

Two cases of MND/ALS developing the syndrome of inappropriate secretion of antidiuretic hormone.

We report 2 autopsy-proven cases of motor neuron disease/amyotrophic lateral sclerosis (MND/ALS), who developed the syndrome of inappropriate secretion of antidiuretic hormone (SIADH) prior to the respiratory failure. With respect to the underlying mechanisms of SIADH in the cases, we considered two factors; 1) dysfunction of the feedback system in ADH secretion via intrathoracic volume receptors caused by changes in intrathoracic circulation, and 2) primarily functional abnormalities in the limbic system which could influence hypothalamic osmoreceptors. It appears that SIADH can be followed by an exacerbation of respiratory dysfunction in MND/ALS.

Amyotrophic Lateral Sclerosis↗
PubMed1997

Detection of Methylobacterium species by 16S rRNA gene-targeted PCR.

We designed PCR primers for specific amplification of the 16S rRNA genes of seven species of the genus Methylobacterium. All of the pairwise species tested were successfully differentiated by PCR detection with a combination of five primer sets, with the exception of M. extorquens and M. rhodesianum. These primers did not cross-react with closely related bacteria in the alpha subclass tested.

Bacterial Typing Techniques↗
PubMed1997

Anti-platelet aggregation activity of some pyrazines.

This report describes the anti-platelet aggregation activity of 48 pyrazines. Among alkyl- and arylpyrazines tested, 2,3-diphenylpyrazines showed the strongest anti-platelet aggregation activity. Then, various substituents were introduced into the phenyl groups, and the 2,3-bis(p-methoxyphenyl)pyrazine derivatives were consequently found to possess considerably strong inhibitory activity.

Animals↗
PubMed1996

Efrapeptins block exocytic but not endocytic trafficking of proteins.

During the course of screening studies to identify inhibitors of intracellular protein trafficking, we isolated efrapeptins as active principles. These compounds arrested syncytium formation (SF) and cytopathic effect (CPE) in Newcastle disease virus (NDV)- and vesicular stomatitis virus (VSV)-infected BHK cells, respectively, without profoundly affecting glycoprotein synthesis. Efrapeptins blocked cell surface expression of NDV-HN and VSV-G glycoproteins, but did not suppress intoxication by ricin or diphtheria toxin even after prolonged pretreatment. Efrapeptins are inhibitors of F-ATPase, or ATP synthase, but their inhibitory effect on SF and CPE was independent of the amount of intracellular ATP.

Adenosine Triphosphate↗
PubMed1996

Self-incompatibility (S) alleles of the Rosaceae encode members of a distinct class of the T2/S ribonuclease superfamily.

Stylar ribonucleases (RNases) are associated with gametophytic self-incompatibility in two plant families, the Solanaceae and the Rosaceae. The self-incompatibility-associated RNases (S-RNases) of both the Solanaceae and the Rosaceae were recently reported to belong to the T2 RNase gene family, based on the presence of two well-conserved sequence motifs. Here, the cloning and characterization of S-RNase genes from two species of Rosaceae, apple (Malus x domestica) and Japanese pear (Pyrus serotina) is described and these sequences are compared with those of other T2-type RNases. The S-RNases of apple specifically accumulated in styles following maturation of the flower bud. Two cDNA clones for S-RNases from apple, and PCR clones encoding a further two apple S-RNases as well as two Japanese pear S-RNases were isolated and sequenced. The deduced amino acid sequences of the rosaceous S-RNases contained two conserved regions characteristic of the T2/S-type RNases. The sequences showed a high degree of diversity, with similarities ranging from 60.4% to 69.2%. Interestingly, some interspecific sequence similarities were higher than those within a species, possibly indicating that diversification of S-RNase alleles predated speciation in the Rosaceae. A phylogenetic tree of members of the T2/S-RNase superfamily in plants was obtained. The rosaceous S-RNases formed a new lineage in the tree that was distinct from those of the solanaceous S-RNases and the S-like RNases. The findings suggested that self-incompatibility mechanisms in Rosaceae and Solanaceae are similar but arose independently in the course of evolution.

Alleles↗
PubMed1996

Small increase in triplet repeat length of cerebellum from patients with myotonic dystrophy.

Myotonic dystrophy (DM) is genetically characterized by abnormal expansion of an unstable CTG trinucleotide repeat, located in the 3'-untranslated region of mRNA encoding the family of serine-threonine protein kinases. DNA extracted from various organs of patients with DM was analyzed by the Southern blotting method. We identified differently expanded bands in DNAs from various tissues from patients with DM. In studying the length of the CTG repeat in different regions of the brain, we found a noticeably small increase in repeat length in the cerebellum compared with other tissues. While this phenomenon has been reported in other triplet repeat diseases such as Huntington disease, spinocerebellar ataxia type 1, and dentatorubral-pallidoluysian atrophy, we are the first to describe it in DM. Although the mechanism of expansion of the triplet repeat remains to be defined, the tissue-dependent somatic mosaicism suggests that its occurrence may depend on the differentiated state of each tissue.

Cerebellum↗
PubMed1996

Isolation of Coxiella burnetii from children with influenza-like symptoms in Japan.

The prevalence of Coxiella burnetii antibodies was investigated by indirect immunofluorescence (IF) test in 55 paired sera (acute and convalescent phases) of school children who had influenza-like symptoms. Of the convalescent serum samples examined, 18 (32.7%) sera reacted positively to phase II antigen of C. burnetii. Coxiella-like organism was isolated from the sera of 13 children after injection of the 18 acute phase sera into mice. The organism was identified as C. burnetii by Giemsa staining and the IF antigen test of mouse spleen smears, the polymerase chain reaction (PCR) method, electron microscopic observations of the mouse spleen cells, and the IF antibody test of mouse sera. This is the first report of isolation of C. burnetii from serum specimens of children having influenza-like symptoms. The evidence that C. burnetii was isolated from people indigenous to Japan at a considerably high incidence suggested that C. burnetii may be widespread as a cause of influenza-like symptoms in Japan.

Acute Disease↗
PubMed1996

[The constituents of Taxillus kaempferi and the host, Pinus thunbergii. I. Catechins and flavones of Taxillus kaempferi].

Four flavonoid compounds, avicularin (1), hyperin (2), quercitrin (3), taxillusin (4), were isolated from leaves of Taxillus kaempferi, and three catechin compounds, (+)-catechin (6), procyanidin B-1 (7), procyanidin B-3 (8), showing a reddish purple color on the reaction with Ehrlich reagent, were also isolated from the stems. Each of these seven isolated compounds were identified by comparing the spectral data with those of the references. Compound 8 was examined to substantiate the stereochemical configurations of conformer, a and b in the detailed 1H-nuclear magnetic resonance (1H-NMR) spectrum using the nuclear Overhauser effect (NOE) difference spectroscopy and spin decoupling experiment in the acetone-d6 solution.

Biflavonoids↗
PubMed1996

[Usefulness of continuous arterial infusion chemotherapy for post operative multiple recurrence and residual hepatocellular carcinoma].

Twenty five patients with postoperative multiple recurrence and residual hepatocellular carcinoma received continuous arterial infusion of CDDP and 5-FU via implanted reservoir. For the next five days, 10 mg/body of CDDP and 250 mg/body of 5-FU using arterial infusion were administered. It was discontinued for two days as one course, and 4 courses were basally administered. The efficacy rate was 60%, and there were 7 (28%) CR (complete response) cases. The survival rate was 76.0% for 1 year and 36.5% for 3 years, which is a favorable result considering their advanced stage. Thus, this treatment seemed to be effective for multiple hepatocellular carcinoma.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1996

[Efficacy of implantable injection port for advanced breast cancer patients].

After breast cancer surgery, it is important to avoid drawing blood sample or injecting chemotherapeutic agents from the involved arm, because severe lymphedema and infection sometimes occurs. Adriamycin, one of the most effective drugs for breast cancer, frequently causes peripheral thrombophlebitis. Therefore, it is often difficult for advanced breast cancer patients to secure IV line and to finish the entire course of chemotherapy. For these reasons, an implantable injection port was applied in 26 breast cancer patients. The status of these patients was metastatic (9 patients), locally advanced (7 patients) and bilateral (10 patients). The average dwell time was 535.8 days with a range of 35 to 1,524 days. The complication events occurred at 0.047/100 catheter days. The results of this study indicate that the implantable injection port provides safe and reliable central venous access and improves the QOL of advanced breast cancer patients.

Adult↗