Chediak-Higashi syndrome: report of a case with an ovarian tumor.
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Biomedical subjects
Publications and source records attributed to F Motoyoshi.
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Asparagine-linked sugar chains obtained from the plasma membranes of human acute lymphocytic leukemic cells, human B cells, Epstein-Barr virus (EBV)-infected B cell lines, and T cells were quantitatively liberated from the polypeptide portions by hydrazinolysis followed by N-acetylation and NaB3H4 reduction. The radioactive oligosaccharides were fractionated by high-voltage paper electrophoresis. Their structures were studied by column chromatography and sequential exoglycosidase digestion. The neutral oligosaccharides were of a high mannose type. The acidic oligosaccharides were bi-, tri-, and tetraantennary complex-type sugar chains with Man alpha----(Man alpha----) Man beta----GlcNAc beta----(+/- Fuc alpha----)GlcNAc as their cores. A comparative study of the oligosaccharides of these cells revealed that the biantennary complex-type sugar chain with bisecting N-acetylglucosamine residues was found only in B cells, B lymphoblasts, and B cell lines. High molecular weight oligosaccharides decreased during the differentiation stage of lymphocytes.
The proliferative responses of peripheral blood mononuclear cells (PBMCs) to ovalbumin or bovine serum albumin in children with atopic dermatitis (AD) who are sensitive to hen's egg or cow's milk were significantly higher than responses of PBMCs of healthy children and hen's egg- or cow's milk-sensitive children with immediate symptoms. However, the percentages of positive RAST for hen's egg or cow's milk in the patients with AD were lower than percentages in the patients with immediate symptoms. In the patients with AD, there were no significant correlations between the proliferative responses of PBMCs and the RAST values. There were no significant differences of RAST scores among groups of patients having different degrees of severity of AD. The proliferative responses of PBMCs to ovalbumin or bovine serum albumin in patients with severe AD or moderate AD who were sensitive to hen's egg or cow's milk tended to be higher than responses of patients with mild AD, respectively, but there were no significant differences in those results. Taken together, the combination of RAST and the detection of proliferative responses of PBMCs to each food antigen is very useful in the diagnosis of hypersensitivity in children with AD who are sensitive to food allergens.
DNA complementary to the ninth largest (S9) of the 12 genome segments of rice gall dwarf virus (RGDV) was cloned and its sequence was determined. It is 1202 nucleotides in length and contains one open reading frame which extends for 969 nucleotides from nucleotide 26. It encodes a polypeptide of 323 amino acids with an Mr of 35,560. The dinucleotide sequence at the 5' end and the trinucleotide sequence at the 3' end of the plus strand, 5' GG--GAU 3', which are present in the RNA of both wound tumour virus (WTV) and rice dwarf virus (RDV), were also found in RGDV genome segment S9. The nucleotide sequences in the noncoding region at the 5' terminus and in the 15 nucleotides at the 3' terminus, which form an imperfect inverted repeat of 10 bp together with the 5' terminus, are approximately 70% homologous with those of the WTV genome segment S9, but only 30% and 50% homologous with the respective termini of RDV S9.
Five heterogeneous IgA-immunodeficient patients were analysed for expression of the alpha-chain gene. The number of surface IgA-bearing B cells was low in four patients. Southern blot analysis indicated no deletion of immunoglobulin structural genes coding for C alpha or alpha switching-region genes. The number of surface IgM and IgA double-bearing B cells increased in some patients. Addition of recombinant interleukin 4 (rIL-4), rIL-5, and rIL-6 to the normal B cells enhanced IgA production. However, B cells of the patients showed no or one-third lower IgA production in response to these lymphokines, even though there was proliferation. rIL-4, rIL-5, and rIL-6 induced low or no expression of alpha mRNA of the patients' B cells. These results suggested that the patients lacked B cells able to produce transcripts for the IgA heavy chain, and that some patients' B cells might be defective at the switch-recombination process from mu to alpha or from mu and alpha to alpha.
Bloom's syndrome (BS) is a rare autosomal recessive genetic disorder in which diabetes mellitus unusually frequently develops as a complication. We report on a 21-year-old Japanese male patient with BS who exhibited impaired glucose tolerance (IGT) in the initial oral glucose tolerance test (OGTT) and had developed patterns of diabetes mellitus by the second OGTT at the 2-years-and-2-months follow-up. German and Passarge reported that the onset of diabetes in patients with BS was in late adolescence or early adulthood. Our results support the findings of German and Passarge. Therefore, when a person with BS reaches late adolescence or early adulthood, an OGTT is necessary to ascertain whether the patient has IGT or diabetes mellitus as a complication, regardless of whether or not diabetic signs such as glucosuria are present.
A resistance-breaking strain of tobacco mosaic virus (TMV), Ltb1, is able to multiply in tomatoes with the Tm-2 gene, unlike its parent strain, L. Nucleotide sequence analysis of Ltb1 RNA revealed two amino acid changes in the 30-kD protein: from Cys68 to Phe and from Glu133 to Lys (from L to Ltb1). Strains with these two changes generated in vitro multiplied in tomatoes with the Tm-2 gene and induced essentially the same symptoms as those caused by Ltb1. Strains with either one of the two changes did not overcome the resistance as efficiently as Ltb1, although increased levels of multiplication were observed compared with the L strain. Results showed that both mutations are involved in the resistance-breaking property of Ltb1. Sequence analysis indicated that another resistance-breaking strain and its parent strain had two amino acid changes in the 30-kD protein: from Glu52 to Lys and from Glu133 to Lys. The fact that the amino acid changes occurred in or near the well conserved regions in the 30-kD protein suggests that the mechanism of Tm-2 resistance may be closely related to the fundamental function of the 30-kD protein, presumably in cell-to-cell movement.
Platonin is one of the photosensitive dyes of trithiazole pentamethine cyanine. It is used as an effective medicine for rheumatoid arthritis. In our study, platonin suppressed the immunoglobulin (Ig) production of human peripheral blood lymphocytes (PBL) stimulated by Staphylococcus aureus Cowan I (SAC) or pokeweed mitogen (PWM). It also suppressed the PWM induced Ig production of B cells when T cells and/or B cells were pretreated with platonin, respectively. The percentage of CD8+ T cells was increased by platonin. Our results suggest that platonin suppresses Ig production through suppressing B cells and enhancing CD8+ (suppressor/cytotoxic) T cells.
A resistance-breaking strain of tobacco mosaic virus (TMV), Ltal, is able to multiply in tomatoes with the Tm-1 gene, unlike its parent strain, L. Comparison of the genomic sequences of L and Lta1 revealed two base substitutions resulting in amino acid changes in the 130 and 180 kd proteins: Gln-979 --> Glu and His-984 --> Tyr. To clarify their involvement in the resistance-breaking property of Lta1, the two substitions were introduced into L by an in vitro transcription system to generate a mutant strain, T1. T1 multiplied in Tm-1/Tm-1 tomatoes with symptoms as did Lta1. Two additional mutant strains were constructed, each of which had one base substitution which caused a His-984 --> Tyr change (T2) or a Gln-979 --> Glu change (T3). T3 multiplied in tomato plants and protoplasts with the Tm-1 gene, indicating that the single base substitution is sufficient to overcome the resistance. T2 also multiplied, but its multiplication was greatly decreased. Although no sequence changes were detected in any progeny viruses recovered from plants without the Tm-1 gene, progeny viruses recovered from T2- or T3- inoculated Tm-1/Tm-1 tomatoes contained in most cases viruses with additional second base substitutions. They caused amino acid changes near the mutagenized residues, suggesting that the ability of T3 to overcome the resistance is not the same as that of Lta1. Sequencing of the genomic RNAs of other independently isolated resistance-breaking strains revealed the same two base substitutions found in the Lta1 RNA. These observations suggest that the two concomitant base substitutions, and possibly also the resulting amino acid changes, guarantee successful replication of these TMV strains in tomatoes containing the Tm-1 gene. A strong correlation was found between the ability to overcome the resistance and a decrease in local net charge, suggesting the involvement of an electrostatic interaction between the viral 130 and 180 kd proteins and a putative host resistance factor.
Tm-1 is a gene which confers resistance to infection, in tomatoes, by tobacco mosaic virus (TMV). To investigate the biochemical mechanism of the resistance, we have established cell suspensions of three lines of tomatoes, i.e., +/+ (susceptible, wild-type, no Tm-1 gene), Tm-1/+ (heterozygous for the Tm-1 gene), and Tm-1/Tm-1 (homozygous for the Tm-1 gene). Protoplasts isolated from these cells were inoculated with RNA of the tomato strain L and Lta1 (a resistance-breaking strain which was recently isolated spontaneously from L) of TMV by means of electrophoration. The syntheses of all viral-coded proteins and TMV-specific RNAs could be detected in L-inoculated +/+ and Lta1-inoculated +/+, Tm-1/+, Tm-1/Tm-1 protoplasts, while their production was markedly reduced in L-inoculated Tm-1/+ protoplasts. L strain could multiply in Tm-1/+ protoplasts to a greater extent with less delay when a large amount of inoculum RNA was used. However, viral production was completely blocked in Tm-1/Tm-1 protoplasts even when a large amount of L-RNA was used for inoculation.
We have established an in vitro transcription system to produce infectious tobacco mosaic virus (TMV) RNA from a cloned cDNA copy. Using this system, several TMV mutants were transcribed in vitro from cDNA clones mutagenized at or near the leaky amber termination codon of the 130K protein gene, and their infectivity was assayed on tobacco plants. Three (two frame-shift and one non-sense) mutants with an intact 130K but a defective 180K protein gene were not infectious, while two mutants with a one-amino-acid insertion in the 180K protein gene were infectious. When the amber codon of the 130K protein gene was deleted, infectivity was lost. However, when the amber termination codon was replaced with ochre or tyrosine codon, infectivity was retained. Sequence analyses revealed that introduced mutations were retained in progeny viral sequences except in the progeny of the amber-to-tyrosine mutant, which was a mixture of the parental mutagenized virus and a pseudo-revertant with ochre codon.
We have cloned full-length double-stranded cDNAs of tobacco mosaic virus (TMV) (tomato strain L) RNA into a transcription vector, pPM1, which facilitates the correct transcription initiation from the first nucleotide of the inserted double-stranded cDNA, corresponding to the 5' end of TMV RNA. When plasmid DNA is linearized at a unique restriction site (Mlu I) introduced just downstream of the double-stranded cDNA insert and used as a template for in vitro transcription by Escherichia coli RNA polymerase in the presence of m(7)GpppG, the transcribed RNAs are infectious for tobacco plants. A simple reconstitution procedure increases the infectivity >100 times. Unexpectedly, both the uncapped transcript and the transcript from the uncut plasmid DNA are also infectious, although their infectivities are very low. The progeny viruses multiplying in tobacco plants accurately reflect the cloned sequence. By the same method, we succeeded in the in vitro transcription of infectious RNA of attenuated strain L(11)A, which is phenotypically distinguishable from wild-type TMV on both tobacco and tomato plants.
When tris-HCL was used as the buffer for inoculation of tomato protoplasts with tobacco mosaic virus, a greater proportion became infected than when phosphate was used. Using 0-05 M-tris-HCl buffer, pH 8-0, in the presence of poly-L-ornithine or poly-D-lysine, 50 to 80 % infection was obtained.
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Five patients with common variable immunodeficiency treated in our hospital between December 1979 and December 1990 were given six kinds of intravenous immunoglobulin preparations (pepsin treated, S-sulfonated, polyethylene glycol treated, pH4 treated, alkylated, and pH4.25 formulation preparation) for replacement therapy. Duration of the therapy ranged from 7.6 to 11 years. Incidences of fever and acute infections were variable among patients, but no significant differences were seen in the incidences among periods given each preparation. Three cases revealed abnormal pulmonary functions in tests. Adverse reactions were rarely seen in our study periods, and no severe reactions were observed. No significant differences were seen in incidences of adverse reactions. Postinfusion levels of serum complement slightly decreased from preinfusion levels. However, the decrease in complement was not related to any adverse reaction. No long-term complications such as transmission of hepatitis have been observed. Our data suggest that no obvious differences exist between the efficacy and safety of each IVIG preparation. Differences of efficacy of IVIG replacement therapy may be due to the variable pathophysiology of each patient.