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Biomedical subjects

T Orii

Publications and source records attributed to T Orii.

At least 343 records · Page 19Linked to original sources
PubMed1986

Carbamyl phosphate synthetase I deficiency with no detectable mRNA activity.

In the autopsied liver of a neonate with carbamyl phosphate synthetase (CPS)-I deficiency, the activity of CPS-I was about 9% of the normal neonatal control. The enzyme protein of CPS-I was hardly detectable by sodium dodecyl sulphate/polyacrylamide gel electrophoresis (SDS/PAGE) and an immunoblotting method using anti-rat liver CPS-I. The level of translatable mRNA for CPS-I was markedly decreased in a cell-free protein synthesis system consisting of rabbit reticulocyte lysate and total RNA extracted from the autopsied liver of the patient. These observations indicate that the enzyme deficiency in this case is probably mainly due to a diminished level of translatable mRNA, which would lead to a decrease in the synthesis of the CPS-I precursor.

Amino Acids↗
PubMed1986

Metabolism of sphingomyelin in cultured skin fibroblasts from patients with different types of Niemann-Pick disease.

The metabolism of [choline-methyl-14C]sphingomyelin in cultured skin fibroblasts from patients with different types of Niemann-Pick disease was measured 1 and 3 days after uptake from the media. The cell lines obtained from type A disease had more than 95% unhydrolyzed sphingomyelin in situ on day 3 while two cell lines obtained from type B had 36.3% and 43.3% unhydrolyzed sphingomyelin on day 3. The cell line derived from one patient with the transitory type disease had 48.1% unhydrolyzed sphingomyelin on day 3, and there was no significant difference in the sphingomyelinase activity measured in vitro or in degradation of sphingomyelin in situ between the type B and transitory type disease. In three cell lines from patients with type C disease, there was 18.5%, 29.6% and 31.1% unhydrolyzed sphingomyelin on day 3, which indicates that this type has a decreased ability to metabolize sphingomyelin. Cell from type E disease metabolized sphingomyelin normally.

Cells, Cultured↗
PubMed1985

Glycosaminoglycan excretion in random samples of urine.

The measurement of glycosaminoglycans (GAGs) on a single random urine specimen in 405 healthy individuals and in 79 patients with mucopolysaccharidoses (MPSs) is described. The uronic acid (UA) content of the precipitated GAGs was estimated by the method of Bitter and Muir. The results were expressed as uronic acid to creatinine (UA/C) ratio. We found that the UA/C ratio decreased with age but had a rather constant low value between the ages of 20 and 33 in healthy individuals. Except for most patients with Morquio syndrome, the UA/C ratio in random urine samples was higher in patients with MPSs than in healthy individuals.

Adolescent↗
PubMed1985

Screening test for urinary glycosaminoglycans and differentiation of various mucopolysaccharidoses.

The Ames MPS paper spot test and the cetylpyridinium chloride (CPC)-citrate turbidity test were used to detect urinary glycosaminoglycans (GAGs) as screening tests for the diagnosis of mucopolysaccharidoses (MPSs). The simplicity and reliability of these two methods were evaluated. The MPS paper spot test correlated more closely with the carbazole test than did the CPC-citrate turbidity test. The monodimensional electrophoresis of Cappelletti et al was also used to screen the subtypes of MPSs using differences in electrophoretic patterns. MPS VII cannot be distinguished from normal by its electrophoretic pattern, but can be detected by the MPS paper spot test. Therefore, the MPS paper spot test combined with monodimensional electrophoresis can detect these subtypes of MPSs (MPS I-II, III, IV, VI, VII). Since only 20 ml of random urine is needed and the result can be obtained with complete reliability in only 7 hours, this screening test appears to be useful for the early diagnosis of MPSs.

Adolescent↗
PubMed1983

Activities of sulfatases for the degradation of acidic glycosaminoglycans in cultured skin fibroblasts from two siblings with multiple sulfatase deficiency.

Cultured skin fibroblasts from two siblings with multiple sulfatase deficiency (MSD) were assayed for the activities of sulfatases known to degrade acidic glycosaminoglycans (AGAG). There were iduronate sulfatase, arylsulfatase B, heparan sulfate (HS) sulfatase, N-acetylgalactosamine-6-sulfate sulfatase, HS-derived N-acetylglucosamine-6-sulfate sulfatase, and two keratan sulfate (KS)-derived N-acetylglucosamine-6-sulfate sulfatases. The activities of sulfatases required for the degradation of HS were reduced to a greater extent than those for the degradation of dermatan sulfate (DS), and those of sulfatases associated with basic defect of Morquio disease type A were moderately decreased or normal. On the other hand, urinary excretion of AGAG in both patients was increased about 10-fold compared to controls, and especially, the excretion of HS and DS was increased about 150-fold and 50-fold, respectively. Keratan sulfate was not detected. The results suggest that in patients with MSD the degradation of HS might be affected to a greater extent than that of DS.

Cells, Cultured↗
PubMed1983

Competence of B cells for T-cell help in pokeweed mitogen-induced immunoglobulin production.

Pokeweed mitogen (PWM)-induced polyclonal immunoglobulin (Ig) production of B cells is highly reproducible and the response has been shown to be dependent on T cells. We investigated whether or not human helper T cells are able to collaborate with B cells in PWM-induced polyclonal Ig production across allogeneic barriers, using T and B cells obtained from healthy individuals and a(hypo)-gammaglobulinemia patients. In results, it has become clear that the B-cell competence is required for T-cell help among healthy individuals and between healthy and a(hypo)-gammaglobulinemic individuals. These phenomena were associated with suppressor or killer T cells rather than HLA-A, -B, -C, and -DR antigens. They also imply that in order to investigate the T-cell helper activities of a(hypo)-gammaglobulinemia patients their T cells should be tested in cocultures not only with B cells of the third party individuals but also with B cells of patient family members.

Agammaglobulinemia↗
PubMed1983

Dimorphism of sister chromatid exchange in Bloom's syndrome B- and T-cell lines transformed with Epstein-Barr and adult T-cell leukemia viruses.

The present study describes the establishment of both B- and T-cell lines from the peripheral blood cells of two Bloom's syndrome (BS) patients and one healthy female by using Epstein-Barr (EBV) and adult T-cell leukemia viruses (ATLV). The cell lines from normal and BS subjects exhibited cell surface markers compatible with B- and T-cell origin; in addition, the BS B- and T-cell lines retained the original cytogenetic characteristics of the syndrome. Even though phytohemagglutinin-stimulated BS lymphocytes from the two BS patients studied all showed high levels of sister chromatid exchange (SCE), the established BS B-lines with EBV yielded two separate lines each, i.e., one with increased SCE and another with normal levels of SCE; also, one of the BS T-lines retained high SCE levels in 100% of the cells, whereas the other BS T-line contained two populations, one with high SCE (70%) and the other with normal SCE levels (30%), at a relatively constant frequency over a period of 6 months. Neither EBV nor ATLV caused a significant increase in chromosome instability in the established lines compared to fresh lymphocytes. Reinfection of the BS B- and T-cell lines with EBV or ATLV did not alter the SCE or karyotypes. These results strongly suggest that BS patients have two populations in vivo, one with high and another with normal levels of SCE, at least in the lymphoid cell system.

Adolescent↗
PubMed1983

Transformation of Bloom syndrome T-lymphocytes by cocultivation with a lethally irradiated human T-cell line carrying type C virus particles.

The present study describes the establishment of T-cell lines from the peripheral blood of two Bloom syndrome (BS) patients and one healthy female by co-cultivation with a lethally irradiated human T-cell line (MT-2) carrying adult T-cell leukemia (ATL) virus (ATLV). The cell lines from normal and BS subjects exhibited cell surface markers compatible with T-cell origin, and BS T-cell lines retained the original cytogenetic characteristics of the syndrome. Even though phytohemagglutinin-stimulated BS lymphocytes from the two BS patients studied all showed high levels of sister chromatid exchange (SCE), one of the BS T-lines retained the high SCE level in 100% of the cells and the other BS T-line contained two populations, one with high SCE (70%) and the other with normal SCE (30%), at a relatively constant frequency over 6 months. Transformation of normal and BS cells by cocultivation with MT-2, which carries ATLV, did not cause karyotypic changes over 6 months. ATLV infection, chromosome instability, karyotypic abnormality and SCE in BS T-lymphocytes are also discussed.

Bloom Syndrome↗