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Biomedical subjects

M Okano

Publications and source records attributed to M Okano.

At least 37 records · Page 2Linked to original sources

Combined immunodeficiency, chromosomal instability, and postnatal growth deficiency in a Japanese girl.

We report on an 11-year-old Japanese girl with combined immunodeficiency and chromosomal instability. She had postnatal growth deficiency and microcephaly, preaxial polydactyly of the left hand, and susceptibility to infections. Immunological studies showed marked lymphocytopenia (around 500/ll), reduced lymphocyte response to various mitogens, and reduced or absent serum IgA, IgG, and IgM. Cell biological studies of her primary skin fibroblasts demonstrated spontaneous chromosome aberrations and radiation hypersensitivity. The combination of immunodeficiency, chromosomal instability, and radiation hypersensitivity as seen in the girl is present in both ataxia-telangiectasia and Nijmegen breakage syndrome. Ataxia-telangiectasia was excluded because of differences in clinical features and laboratory data. Likewise, Nijmegen breakage syndrome is unlikely to be the case because the characteristic face, hyperpigmented spots, and mental retardation present in the syndrome were missing in the girl. Sequence analysis of a Nijmegen breakage syndrome responsible gene, NBS1, revealed no mutations. A normal NBS1 product was also demonstrated by immunoblot analysis using an anti-NBS1 antibody. We propose that the disorder in the girl represents a new combination of combined immunodeficiency and chromosomal instability.

Child↗

Surface conduction on insulating BaTiO3 crystal suggesting an intrinsic surface electron layer.

We report conduction measurements on the clean, free surface of transparent insulating BaTiO3 single crystals in high vacuum. We find that the insulating BaTiO3 crystals exhibit surface conductance that is dependent on the spontaneous polarization, whereas no conduction perpendicular to the surface is observed. The surface conduction shows semimetallic temperature dependence and persists down to at least 100 K. The observations suggest a two-dimensional electron on a clean, free ferroelectric surface that may be regarded as a ferroelectric metal. The results have important implications for understandings of the fundamental properties of ferroelectrics, the size effect, and ferroelectric devices.

Journal Article↗

Biochemical characterization of cholesterol-3-sulfate as the sole effector for the phosphorylation of HMG1 by casein kinase I in vitro.

Phosphorylation of high mobility group protein 1 (HMG1) by casein kinase I (CK-I) and potent effectors (inhibitors and activators) of this phosphorylation were investigated in vitro. We found that (i) CK-I phosphorylates specifically threonine residues on HMG1 when incubated with cholesterol-3-sulfate (CH-3S), but no phosphorylation of HMG1 is detected in the presence of other cholesterol related compounds or their sulfated derivatives; (ii) this phosphorylation is selectively inhibited by heparin, but stimulated significantly by 3',4',7-trihydroxy-isofavone at low doses (0.1-3 microM); and (iii) CH-3S directly induces a drastic conformational change in HMG1. The latter finding provides a mechanism to explain how CH-3S alone can induce the phosphorylation of HMG1 by CK-I in vitro.

Animals↗

Hypophysis surgery with or without endoscopy.

OBJECTIVE: Hardy's operation with microscope has long been the standard method for pituitary adenoma. But a new approach via the nasal cavity using an endoscope has been adopted recently. In this study, the postoperative outcome as well as the preoperative evaluation of endoscopic hypophysectomy and non-endoscopic one were compared at our faculty. METHOD: We performed the non-endoscopic transnasal hypophysectomy on 18 patients and the endoscopic transnasal hypophysectomy on thirteen patients who had a pituitary lesions from February 1996 to October 1999. As to these patients the situations from preoperation through postoperation such as chief complaints, serum hormone level, final diagnosis, tumor size, as well as operating time or blood loss during the operation were discussed precisely. Then the merits and demerits of endoscopic hypophysectomy were discussed. RESULT: Five PRL-producing adenoma, three GH-producing adenoma, nine non-functioning adenoma, and two ACTH or TSH-producing adenoma were included in this discussion as endoscopic group. The age of non-endoscopic group are from 23 to 73 (49.4 in average), and they include ten males and eight females. On the other hand three PRL-producing adenoma, two GH-producing adenoma, two non-functioning adenoma, and one Rathke's cyst were included in this discussion as endoscopic group. The age of endoscopic group are from 19 to 73 (49.1 in average), and they include seven males and six females. As to non-endopscopic group the blood loss during each operation is 568 ml and operating time is 256 min in average. For endoscopic group the blood loss is 296 ml and operating time is 234 min in average. CONCLUSION: By microsurgery in the pituitary operation with endoscopy, the minimal invasive surgery becomes possible by reducing blood loss and shortening operating time. During the operation cooperation between neurosurgeon and ENT surgeon is indispensable in order to perform hypophysectomy smoothly. The development of optical better aids and operation instruments for endonasal hypophysectomy is desired in the future. The navigation system was more useful than X-ray fluoroscopy to obtain the detailed information.

Adenoma↗

Epstein-Barr virus in patients with immunodeficiency disorders.

The Epstein-Barr virus (EBV), one of eight known human herpesviruses, causes a wide spectrum of diseases under certain conditions. In particular, in the setting of immunodeficiency, which includes primary or secondary/acquired immunodeficiencies, they have been increasingly reported. The major clinical phenotype is the EBV genome-positive lymphoproliferative disorder, which ranges from benign lymphoproliferation to malignant lymphoma with cytogenetic alterations. Severe or fatal infectious mononucleosis may develop in some patients with immunodeficiencies such as X-linked lymphoproliferative disease.

Acquired Immunodeficiency Syndrome↗

Dantaxusins A and B, two new taxoids from Taxus yunnanensis.

Two new taxane diterpenes, dantaxusin A [5 alpha-cinnamoyloxy-2 alpha,7 beta,13 alpha-triacetoxy-2(3-->20)abeo-taxa-4(20),11-diene-9,10-dione (1)] and dantaxusin B [5 alpha-cinnamoyloxy-9 alpha-hydroxy-10 beta,13 alpha-diacetoxytaxa-4(20),11-diene (2)], were isolated from an ethanol extract of the aerial parts of Taxus yunnanensis along with taxuspine B, 2-deacetoxytaxinine J, taxuyunnanine C, taxinine B, taxuspine C, and taxinine NN-4. The structures of 1 and 2 were established on the basis of 1D and 2D NMR and HRMS spectroscopic methods.

China↗

Sex-related differences in the initiation of allergic rhinitis in mice.

BACKGROUND: Several clinical and epidemiologic studies have investigated sex differences in the prevalence of allergic rhinitis. At present, however, no reports have demonstrated such differences in experimental models with local, but not parenteral, sensitization with antigens that may reflect natural exposure to allergens. We have recently developed murine models of allergic rhinitis after repeated intranasal sensitization with antigens in the absence of adjuvants. In this study, we investigated the role of sex in the initiation of the disease in vivo. METHODS: Male and female CBA/J and BALB/c mice were sensitized intranasally with phospholipase A2 (PLA2) and Schistosoma mansoni egg antigen (SEA), respectively, in the absence of adjuvants. After the repeated sensitization, serum Ab titers against the sensitizing antigen and nasal eosinophilia were determined. In addition, the involvement of androgen in IgE synthesis was investigated in castrated CBA/J male mice with or without testosterone administration. RESULTS: Females produced significantly higher levels of PLA2-specific IgE than males in CBA/J mice sensitized with PLA2. On the other hand, both titers of PLA2-specific IgG1 and nasal eosinophilia did not significantly differ between the two groups. Castrated male mice produced significantly higher amounts of PLA2-specific IgE than sham-treated male mice. In addition, PLA2-specific IgE production decreased in castrated mice treated with testosterone. Sexual differences in the production of Ag-specific IgE were not seen in BALB/c mice after the sensitization with SEA. CONCLUSION: These results suggest that sex is responsible for the production of Ag-specific IgE, but not IgG1 or nasal eosinophilia, and that androgen appears to be involved in the in vivo production of specific IgE in male mice.

Animals↗

Expression of costimulatory CD80/CD86-CD28/CD152 molecules in nasal mucosa of patients with perennial allergic rhinitis.

BACKGROUND: B7 molecules (CD80, CD86) and their counter-receptors, CD28 and CD152 (CTLA-4), play an important role in T cell-mediated immune responses. We previously demonstrated that B7 molecules are selectively up-regulated not only on B cells but also on T cells from the peripheral blood mononuclear cells of patients with perennial rhinitis cultured with allergen. However, the expression of CD80/CD86 molecules and their counter-receptors in nasal mucosa, the actual inflammatory site of allergic rhinitis, has not yet been clarified. PATIENTS AND METHODS: Inferior turbinates from patients with either allergy to house dust or non-allergic rhinitis were excised and immunohistologically stained. In addition, the inferior turbinates were challenged with paper discs containing extracts of house dust and subsequently excised. Samples were double stained with immunofluorescent-labelled antibody to identify cells bearing CD86. RESULTS: Without the nasal provocation, only the expression of CD86 was increased in nasal mucosa of patients with allergic rhinitis compared with those with non-allergic rhinitis. However, following the nasal provocation with house dust, not only CD86, but also CD80, CD28, and CD152 were significantly expressed in allergic patients. Immunofluorescent double staining revealed CD86 expression in CD19, CD1a, CD14 and CD3 lymphocytes. CONCLUSION: These results indicate that the expression of CD80/CD86 molecules and their counter-receptors is induced in allergic patients following nasal provocation with allergen, suggesting a local amplification of allergen-specific immune responses in perennial rhinitis.

Abatacept↗

Roles of carbohydrates on Cry j 1, the major allergen of Japanese cedar pollen, in specific T-cell responses.

BACKGROUND: Carbohydrates expressed on allergens are known to be important for allergenicity. However, little is known about whether the carbohydrates drive the T(H)2 response. OBJECTIVE: We sought to determine a role for carbohydrates expressed on Cry j 1, which is the major allergen of Cryptomeria japonica pollen and causes the most prevalent pollinosis in Japan, in in vitro cellular responses. METHODS: Carbohydrates on Cry j 1 were destroyed by periodate-oxidation under mild conditions. Proliferative responses and cytokine productions against native, periodate-treated, and mock-treated Cry j 1 were compared in peripheral blood mononuclear cells, Cry j 1-specific T-cell lines, and clones from patients with Japanese cedar pollinosis. RESULTS: We found that peripheral blood mononuclear cells from patients with Japanese cedar pollinosis displayed a significant decrease in proliferation and IL-5 production in response to periodate-treated Cry j 1 in comparison with native and mock-treated Cry j 1. Decreased proliferative responses against periodate-treated Cry j 1 were also seen in polyclonal T-cell lines, and the responses showed a heterogeneity. In addition, Cry j 1-specific CD4+ T-cell clones also displayed a significant decrease in proliferation and IL-4 and IL-5 production-but not IFN-gamma production-in comparison with the control antigens. However, most of the clones showed decreased but positive proliferative responses against periodate-treated Cry j 1. Blockade of the mannose receptor had no effect on cellular responses. CONCLUSION: The results suggest that carbohydrates on Cry j 1 play a major role in promoting Cry j 1-specific T(H)2 response in vitro, though they are not major targets as T-cell epitopes.

Adult↗

Lower cervical origin of the P13-like potential in median SSEPS.

The authors studied the origin of the scalp P13-like potential in median somatosensory evoked potentials, which have been reported to be preserved in patients with cervicomedullary lesions or in brain death. There were five patients with high to middle cervical lesions (C2/3 or C3/4 level). Small P13-like potentials after P11 were identified for all patients with a noncephalic reference but not with an ear reference. Their onset latencies were slightly earlier than the expected latency of the true P13/14 onset. In two patients, delayed true P13/14s followed by N18s were identified with both noncephalic and ear references. The authors argue that the P13-like potential observed in these patients is a different entity from scalp P13 in normal subjects. Because the C3/4 vertebral level corresponds to the C5 cord level, the origin of the P13-like potential must be below C5, contradicting the previous opinion that it is generated at the cervicomedullary junction or at the high cervical dorsal column. The authors named this potential lower cervical P13 (or lcP13), and present an opinion that it is generated by the beginning of the second spinal ascending volley, which has been described by direct-recording studies in humans.

Adult↗

Assessment of the clinical effect of topical tacalcitol on ichthyoses with retentive hyperkeratosis.

BACKGROUND: Topical tacalcitol (1alpha,24-dihydroxyvitamin D3) has been demonstrated to produce beneficial clinical effects on epidermo-hyperproliferative disorders such as psoriasis and pityriasis rubra pilaris. However, the efficacy of the agent has not been elucidated in retentive hyperkeratotic disorders. OBJECTIVE: The object of the study was to assess the clinical efficacy of topical tacalcitol against ichthyoses with retentive hyperkeratosis; X-linked ichthyosis (XLI), ichthyosis vulgaris (IV), and acquired ichthyosis (AI). METHODS: Tacalcitol was topically applied on 9 patients with the retention ichthyoses, using a single-blinded, bilaterally paired approach with right-left comparison of tacalcitol and the base. RESULTS: Clinical improvement obtained by topical tacalcitol exclusively was not superior to that of the vehicle alone in any of the ichthyotic patients. CONCLUSION: Topical tacalcitol therapy was ineffective against ichthyoses without epidermal hyperproliferation.

Administration, Topical↗

Inhibitory effect of glycyrrhizin on the phosphorylation and DNA-binding abilities of high mobility group proteins 1 and 2 in vitro.

The physiological correlation between glycyrrhizin (GL) and high mobility group proteins I and 2 (HMG1/2) and the inhibitory effect of GL on their phosphorylation by three protein kinases (CK-I, CK-II and PKC) were investigated biochemically in vitro. It was found that GL binds directly to HMG1/2, because (i) HMG1/2 have a high affinity with a GL-affinity column; and (ii) GL induces the conformational changes in HMG1/2. Both purified HMG1/2 functioned as phosphate acceptors for these two protein kinases (CK-I and PKC), but not phosphorylated by CK-II. Phosphorylation of HMG1/2 by two protein kinases (CK-I and PKC) was completely inhibited by a glycyrrhetinic acid derivative (oGA) at one-tenth the concentration of GL. Also, the DNA-binding abilities of HNG1/2 were reduced by GL in a dose-dependent manner. These results show that the binding of GL to HMG1/2 results in the inhibition of their physiological activities (DNA-binding ability and phosphorylation by PKC or CK-I) in vitro. The GL-induced inhibition of the physiological activities of HMG1/2 may be involved in the anti-inflammatory effect of GL in vivo.

Animals↗

Anti-alpha-fodrin autoantibody is an early diagnostic marker for childhood primary Sjögren's syndrome.

OBJECTIVE: alpha-fodrin is a recently identified autoantigen associated with adult primary Sjögren's syndrome (SS). We tested whether anti-alpha-fodrin antibody could also be used as a diagnostic marker for childhood SS. METHODS: We performed immunoblot analysis of sera from 7 patients with childhood primary SS using glutathione-S-transferase alpha-fodrin fusion protein as an antigen. RESULTS: Anti-alpha-fodrin antibody was detected in sera from all 7 patients with childhood primary SS, 2 of 4 with secondary SS, and one of 7 with systemic lupus erythematosus, but in no other healthy controls. CONCLUSION: The anti-alpha-fodrin autoantibody was detected before anti-SSA or SSB antibody became positive; thus anti-alpha-fodrin antibody could be a useful marker for the early diagnosis of SS.

Adolescent↗

[Expression and pathophysiologic features of orotate phosphoribosyl transferase activity (OPRT) in gastric carcinoma].

Orotate phosphoribosyl transferase (OPRT) is an enzyme that converts the pyrimidine fluoride-class anticancer drug 5-fluorouracil (5-FU) into the active nucleotide form. As such, it can be considered a primary enzyme in the first stage inhibiting DNA and RNA expression. The present study measured OPRT activity both in gastric carcinoma tissue and in surrounding normal tissue, and investigated the correlation between these findings and clinicopathologic characteristics in the patients. The study subjects were 20 patients with gastric carcinoma who were treated by surgical resection in our department. The relationship between OPRT activity in gastric carcinoma and surrounding normal tissue and patient age, sex, tissue type, extent of tumor invasion, extent of metastasis to the lymph nodes, lymphatic invasion and the existence of venous invasion of the gastric wall were investigated. The mean OPRT activity for all patients was 0.039 +/- 0.042 nmol/min/mg-prot in normal tissue and 0.120 +/- 0.099 nmol/min/mg-prot in tumor tissue, and the mean ORPT activity was significantly higher in tumor tissue than in surrounding normal tissue (p < 0.01). The OPRT ratio for tumor tissue/normal tissue (T/N) was significantly decreased as the invasiveness of the tumor increased, and was also significantly lower in patients with lymph node metastasis (p < 0.05) than in patients without lymph node metastasis. A decrease of OPRT activity in tumor tissue is a possible reason for the equivocal results of 5-FU-based chemotherapy in advanced gastric carcinoma.

Aged↗