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T Orii

Publications and source records attributed to T Orii.

At least 235 records · Page 13Linked to original sources
PubMed1992

Accelerated expression of secreted alpha-chain gene in anaphylactoid purpura.

The mechanisms of the elevation of serum IgA levels in anaphylactoid purpura were investigated. Serum IgA levels were significantly elevated within 2 weeks (5 to 14 days for all 12 patients) after onset in patients with anaphylactoid purpura. Serum IgM and IgG were not elevated. Although the percentages of surface IgA-bearing cells were not increased in the patients, the numbers of IgA-secreting cells within 2 weeks after onset in the patients with anaphylactoid purpura were significantly higher than those of controls. In northern blot analysis on lymphocytes, the secreted alpha (alpha s)-chain gene was well expressed compared with the membrane-bound alpha (alpha m)-chain gene, within 2 weeks after the onset of anaphylactoid purpura. Therefore, stimulation by a certain agent or a certain immune response may accelerate expression of the alpha s-chain gene in anaphylactoid purpura.

Blotting, Northern↗
PubMed1992

Interstitial deletion of the long arm of chromosome 11: report of a case and review of the literature.

A 12-month-old female infant with developmental delay, growth retardation, and dysmorphic features including dolichocephaly, telecanthus, ptosis, flat nasal bridge, anteverted nares, high-arched palate, carp-shaped mouth, micro-retrognathia, and low-set and posteriorly rotated ears was found to have an interstitial deletion of chromosome 11 involving bands q14-q22. Immunoblot analysis of her fibroblasts revealed a normal amount of mitochondrial acetoacetyl-coenzyme A thiolase, of which gene locus has been assigned to chromosome 11q22.3-q23.1. This result suggested that the region around the boundary of 11q22.3-q23.1 was intact in this patient.

Abnormalities, Multiple↗
PubMed1992

Asparagine-linked sugar chains of erythrocyte membrane glycoproteins from Wiskott-Aldrich syndrome are not impaired.

In both healthy controls and patients with Wiskott-Aldrich syndrome, the main oligosaccharide in asparagine-linked sugar chains of the membrane glycoproteins of erythrocytes was biantennary sugar chain with bisected GlcNAc (Gal2-GlcNAc2-Man3-GlcNAc-GlcNAc-Fuc-GlcNAcOT). Biantennary sugar chain with an alpha-fucosyl residue linked at the proximal GlcNAc was seen but biantennary sugar chain without an alpha-fucosyl residue at the proximal GlcNAc was not detected in each subject. There was no difference in quality and quantity of asparagine-linked sugar chains of erythrocyte membrane glycoproteins between healthy controls and the patients. These results suggest that asparagine-linked sugar chains in membrane glycoproteins of hematopoietic cells may not be impaired in Wiskott-Aldrich syndrome.

Adolescent↗
PubMed1992

Asparagine-linked sugar chains of plasma membrane glycoproteins from healthy and common variable immunodeficiency B lymphoblastoid cell lines.

Asparagine-linked sugar chains of plasma membrane glycoproteins, which are formed by glycosylation during B cell maturation, were examined with B lymphoblastoid cell lines (LCLs) transformed by Epstein-Barr virus derived from healthy controls and patients with common variable immunodeficiency (CVI). Both two patients with CVI showed hypogammaglobulinemia and impaired B cell functions. LCLs from healthy controls and the patients showed CD19+ and HLA/DR+ in the cell surface and secreted IgM. In both healthy controls and the patients, the main oligosaccharide in asparagine-linked sugar chains of the membrane glycoproteins of LCLs was biantennary sugar chain with bisected GlcNAc (Gal2-GlcNAc2-Man3-GlcNAc-GlcNAc-Fuc-GlcNAcOT). Biantennary sugar chain with an alpha-fucosyl residue linked at the proximal GlcNAc was seen but biantennary sugar chain without an alpha-fucosyl residue at the proximal GlcNAc was little detected in each LCL. There was no difference in quality and quantity of asparagine-linked sugar chains between healthy controls and the patients. These results suggest that glycosylation during B cell maturation may not be impaired in patients with CVI.

Asparagine↗
PubMed1992

Lymphocyte responses to food antigens in food sensitive patients with allergic tension-fatigue syndrome.

Scores of radioallergosorbent test (RAST) for cow's milk or buckwheat flour and proliferative responses of peripheral blood mononuclear cells (PBMCs) to bovine serum albumin and beta-lactoglobulin or buckwheat flour were measured in cow's milk or buckwheat flour sensitive patients with allergic tension-fatigue syndrome. In all 3 cow's milk sensitive patients with allergic tension-fatigue syndrome, RAST scores for cow's milk were negative or slightly positive, but PBMCs well responded to bovine serum albumin and beta-lactoglobulin, but not to ovalbumin. In a buckwheat flour sensitive patient with allergic tension-fatigue syndrome, RAST scores for buckwheat flour were negative, but PBMCs well responded to buckwheat flour, but not to ovalbumin, bovine serum albumin and beta-lactoglobulin. Conversely, in cow's milk or buckwheat flour sensitive patients with immediate allergic symptoms, RAST scores for offending foods were positive although PBMCs did not respond to offending food antigens. These results suggest that proliferative responses of PBMCs to food antigens are very useful for detection of offending foods in allergic tension-fatigue syndrome.

Adolescent↗
PubMed1992

Transformation and characterization of mutant human fibroblasts defective in peroxisome assembly.

Human skin fibroblasts deficient in peroxisome biogenesis were transformed by transfecting SV40 ori- DNA with the use of an electroporator, and the biochemical, immunocytochemical, and cytogenetic properties of the transformants were analyzed. Cells (1 x 10(6)) from a patient with Zellweger syndrome and one with neonatal adrenoleukodystrophy were suspended with 2 micrograms of SV40 ori- DNA in PBS; then a high-voltage pulse (2000 V, 30 microseconds) was generated two times. Several colonies expressing large T-antigen were picked up 4 weeks after transfection. Doubling time of the transformants was about half of that and the saturation density was 5 to 10 times greater than that of the parental cells. Biochemical abnormalities including defective lignoceric acid oxidation, dihydroxyacetone phosphate acyltransferase deficiency, and disturbed biosynthesis of peroxisomal beta-oxidation enzymes were preserved in the transformants. Peroxisomes were defective in all colonies, as determined by immunofluorescence staining using anti-catalase IgG. Cell fusion studies confirmed that the transformants belong to the same complementation groups as those of the parental cells. These transformed mutant cell lines are expected to be useful tools for investigating the pathogenesis of inherited diseases related to defects in peroxisome biogenesis.

Acyltransferases↗
PubMed1992

Improvement of clinical and MRI findings in a boy with adrenoleukodystrophy by dietary erucic acid therapy.

A 5-year-old boy with adrenoleukodystrophy, with clinical symptoms of visual, mental and motor disturbances which progressed rapidly, was treated with Lorenzo's oil consisting 1 volume of glyceryl trierucate and 4 volumes of glyceryl trioleate. Five months after initiation of this therapy, ability to swallow was enhanced and T2-weighted magnetic resonance imaging of the brain revealed regression of high intensity area of the parieto-occipital white matter.

Adrenoleukodystrophy↗
PubMed1992

Assessment of liver graft function after cold preservation using 31P and 23Na magnetic resonance spectroscopy.

We investigated the functional damages of the cytoplasm and the cell membrane of liver grafts in male Wister Kyoto rats after 24-hr and 48-hr cold preservations using the University of Wisconsin solution in vitro. Fructose (10 mM) or NH4Cl (50 mM) was added to the perfusate, and synthesis of fructose-1-phosphate (F-1-P) and Na- and H-ion transports through the cell membrane were evaluated by magnetic resonance spectroscopy (MRS), 31P-MRS and 23Na-MRS. After 30 min of reperfusion, beta-ATP/(inorganic phosphate: Pi) of the 48-hr preserved group was significantly lower than the control group and the 24-hr preserved group. The changes of F-1-P in the control group and the 24-hr preserved group were almost the same, but F-1-P synthesis was lower in the 48-hr preserved liver than those of the other groups. Intracellular pH began to drop after the cessation of NH4Cl loading, and then it recovered to the preloading level. At the same time Nain+ was increased in the control group. However, in the other two groups, the increasing rates of Nain+ were lower, and the recoveries of Nain+ were less. In conclusion, the function of cell membrane was more fragile than that of mitochondria and cytoplasmic sugar metabolism in the liver graft.

Adenosine Triphosphate↗
PubMed1992

Inhibition of proliferative responses of lymphocytes to food antigens by an anti-allergic drug, N(3',4'-dimethoxycinnamoyl) anthranilic acid (Tranilast) in children with atopic dermatitis.

Experimental studies have shown that N(3',4'-dimethoxycinnamoyl) anthranilic acid (Tranilast) inhibits reaginic antibody-mediated hypersensitivity reactions, and it has been demonstrated to be an effective drug for patients with bronchial asthma. On the other hand, from the nature of the cellular infiltrate seen in eczematous lesions, it appears that some form of cell-mediated immunity may be involved in addition to IgE-mediated immunity in the pathogenesis of atopic dermatitis (AD). Moreover, we have previously reported that the proliferative responses of peripheral blood mononuclear cells (PBMCs) to ovalbumin (OA) or bovine serum albumin (BSA) in children with AD who are sensitive to hen's egg or cow's milk were significantly higher than those of healthy children and hen's egg or cow's milk sensitive children with immediate symptoms. In this study, we have showed that the proliferative responses of PBMCs to OA were dose-dependently inhibited by Tranilast on patients with AD. The responding cells to OA were shown, through separation experiments, to be T cells, and the proliferative responses of T cells to OA were also dose-dependently inhibited by Tranilast. Moreover, the inhibition was thought to occur at the initial stage of the proliferative reactions. These results suggest that Tranilast can be clinically applied to patients with AD.

Antigens↗
PubMed1992

Reduced secreted mu mRNA synthesis in selective IgM deficiency of Bloom's syndrome.

Serum IgM concentrations were low although serum IgG and IgA concentrations were normal in both our patients with Bloom's syndrome. Although the percentages of surface IgM-bearing cells were not reduced, the numbers of IgM-secreting cells were markedly reduced. The membrane-bound mu (microns) and secreted mu (microseconds) mRNAs are produced from transcripts of a single immunoglobulin mu gene by alternative RNA processing pathways. The control of microseconds mRNA synthesis depends on the addition of poly(A) to microseconds C-terminal segment. In both patients, mu mRNA was well detected but microseconds C-terminal mRNA was scarcely detected, suggesting that microns mRNA was well transcribed but microseconds mRNA was not. There was, at least, no mutation or deletion in the microseconds C-terminal coding sequence, the RNA splice site (GG/TAAAC) at the 5' end of microseconds C-terminal segment and the AATAAA poly(A) signal sequence in both patients. Our results suggest that selective IgM deficiency in Bloom's syndrome is due to an abnormality in the maturation of surface IgM-bearing B cells into IgM-secreting cells and a failure of microseconds mRNA synthesis. Moreover, reduced microseconds mRNA synthesis may be due to the defect on developmental regulation of the site at which poly(A) is added to transcripts of the mu gene.

Adult↗
PubMed1992

Common variable immunodeficiency with increased surface IgM-positive double-bearing B cells.

We report a case of common variable immunodeficiency (CVI) that shows low levels of IgG and IgA, but a normal quantitative or qualitative level of IgM. T-cell functions were not disturbed. Increased numbers of surface IgM (sIgM) and sIgD, sIgM and sIgG, sIgM and sIgA double-bearing B cells were observed as compared with a control. No IgG and IgA induction upon stimulation with Staphylococcus aureus Cowan I (SAC) and recombinant interleukin-2 (rIL-2), or pokeweed mitogen (PWM) and rIL-4 or rIL-6 was observed, although there was proliferation. Although mu mRNA was expressed as much as in a healthy control, transcription of gamma mRNA and alpha mRNA was very low. Furthermore, no enhanced effects of gamma mRNA and alpha mRNA were recognized upon stimulation with rIL-4 and rIL-6. These results suggest that the patient's B cells might be defective at the switching process from mu, mu and delta, mu and gamma to gamma or mu and alpha to alpha.

Antibody Formation↗
PubMed1992

Neuroradiological findings in glutaric aciduria type I: report of four Japanese patients.

We examined neuroradiological computerized tomography (CT) findings and the clinical course of four Japanese children with glutaric aciduria type I (GA1) whose enzyme activity of glutaryl-CoA dehydrogenase was undetectable. Brain CT in all cases examined showed low density white matter, fluid collection in bilateral frontotemporal regions (particularly surrounding the Sylvian fissures), enlargement of the lateral ventricles and slight atrophy of the basal ganglia. Although these findings seemed to be characteristic for GA1, they were unlikely to be more extended, at least over 2 years after infancy. The low density white matter was observed more evidently in the neonatal or early infantile periods than in later periods. The degree of enlargement of fissures in bilateral frontotemporal regions about the Sylvian fissures appeared to correlate with the severity of symptoms such as dystonia or choreoathetosis. Magnetic resonance images (MRI) in one case showed bilateral linear-shaped low intensity in areas of the external capsules and putamen on a T1-weighted image. These CT and MRI findings, as well as clinical symptoms such as choreoathetosis or dystonia, may suggest that metabolic abnormalities in GA1, such as glutaconate, are toxic to the extrapyramidal tract system in the central nervous system, and that the clinical symptoms of the patients are attributable to atrophy of basal ganglia. Brain CT may be useful in diagnosis and evaluation of the clinical course of GA1 patients.

Brain↗
PubMed1992

Accumulation of lysosphingolipids in tissues from patients with GM1 and GM2 gangliosidoses.

By using a sensitive method, we assayed lysocompounds of gangliosides and asialogangliosides in tissues from four patients with GM2 gangliosidosis (one with Sandhoff disease and three with Tay-Sachs disease) and from three patients with GM1 gangliosidosis [one with infantile type (fetus), one with late-infantile, and one with adult type]. In the brain and spinal cord of all the patients except for an adult GM1 gangliosidosis patient, abnormal accumulation of the lipids was observed, though the concentration in the fetal tissue was low. In GM2 gangliosidosis, the amounts of lyso GM2 ganglioside accumulated in the brain were similar among the patient with Sandhoff disease and the patients with Tay-Sachs disease, whereas the concentration of asialo lyso GM2 ganglioside in the brain was higher in the former patient than in the latter patients. By comparing the sphingoid bases of neutral sphingolipids, gangliosides, and lysosphingolipids, it was suggested that lysosphingolipids in the diseased tissue are synthesized by sequential glycosylation from free sphingoid bases, but not by deacylation of the sphingolipids. Because lysosphingolipids are known to be cytotoxic, the abnormally accumulated lysophingolipids may well be the pathogenetic agent for the neuronal degeneration in gangliosidoses.

Brain↗
PubMed1992

Long-term study of the immunodeficiency of Bloom's syndrome.

The immune state was evaluated over a 10-year period in two individuals with Bloom's syndrome. In both patients, serum concentrations of IgM were markedly low. Mildly decreased serum concentrations of IgG and IgA increased significantly with age, whereas the IgM levels remained low. From assessments of B-cell and T-cell functions in pokeweed mitogen-induced immunoglobulin production, the IgM deficiencies were thought to result from B-cell dysfunction. T-cell function appeared intact. Moreover, although the percentages of surface IgM-bearing cells were not reduced, the numbers of IgM-secreting cells were reduced. These findings suggest that the IgM deficiency is due to an abnormality in the maturation of surface IgM-bearing B cells into IgM-secreting cells.

Adolescent↗
PubMed1992

Proliferative responses of CD4+ T-cell population to ovalbumin in patients with atopic dermatitis who are sensitive to hen eggs.

Proliferative responses of peripheral blood mononuclear cells and T cells with monocytes to ovalbumin were significantly higher than those of B cells with monocytes to ovalbumin in patients with atopic dermatitis (AD) who were sensitive to hen eggs. The CD4+ T-cell/CD8+ T-cell ratios (the values obtained by dividing the maximum stimulation index of CD4+ T cells with monocytes to ovalbumin by the maximum stimulation index of CD8+ T cells with monocytes to ovalbumin) were significantly higher in AD patients sensitive to hen eggs than in non-atopic healthy controls. The proliferative responses of CD4+ T cells with monocytes to ovalbumin were more intensive than those of CD8+ T cells with monocytes in patients with AD sensitive to hen eggs compared with non-atopic healthy controls. These results suggest that the cells responding to ovalbumin are predominantly CD4+ T cells. However, there was no relationship between the stimulation index of proliferative responses of peripheral blood mononuclear cells to ovalbumin and RAST scores for hen eggs. Thus it is possible that the majority of the CD4+ T cells which respond to ovalbumin are not CD4+ helper T cells for IgE production.

Adolescent↗
PubMed1992

Diversity in DNA rearrangements and in RNA expressions of immunoglobulin gene on common variable immunodeficiency.

Six heterogeneous common variable immunodeficiency (CVID) patients were analysed for germ-line DNA, DNA rearrangements, and RNA expressions of immunoglobulin (Ig) gene by Southern or northern blotting using appropriate probes. We detected no polymorphism in neutrophil DNA hybridized to a C mu and a C gamma probe. In three patients, both serum Ig and Ig-bearing cells were scarcely detected, and by northern hybridization methods, neither mu mRNA, gamma mRNA, alpha mRNA nor kappa mRNA was detected. However, one Epstein-Barr virus-transformed B lymphoblastoid cell line (LCL) of these three patients was different from the germ line in the region of JH, C gamma, and C kappa, and expressed mu mRNA at a higher level. The B cell defects of these three patients lay on the B cell maturation stage similar to X-linked agammaglobulinaemia (XLA). In two others among the six CVID patients, serum IgM and IgM-bearing cells were detected to a certain degree, and by northern hybridization, mu mRNA was detected at a lower level, but neither mu mRNA, alpha mRNA, nor kappa mRNA was detected. One LCL of these two patients could express mu mRNA at the normal level. In the last patient, the serum IgM was normal, serum IgG and IgA were somewhat low, Ig-bearing cells were normal, mu mRNA and kappa mRNA were detected at the normal level, and gamma mRNA and alpha mRNA were detected at a lower level. The defect of this patient affected the class switch stage. These results showed that primary B cell defects in CVID occurred at several B cell differentiation stages which could be classified by expression of the Ig gene, and at the degree of clonal diversity in the B cell repertoire. Furthermore, this study provides support for the idea that the CVID defect is related to a more generalized cellular function, such as regulating the proliferation and/or clonal expansion of cells of the B lymphoid lineage.

Antibody Formation↗