'Severe' Prader-Willi syndrome with a large deletion of chromosome 15 due to an unbalanced t(15,22)(q14;q11.2) translocation.
Explore the source record for details and available documents.
Chromosome Deletion↗
Biomedical subjects
S Kadowaki
Publications and source records attributed to S Kadowaki.
Effectiveness and safety of mutant Escherichia coli heat-labile enterotoxin (LT H44A) as an adjuvant for nasal influenza vaccine.
The effectiveness and safety of mutant Escherichia coli heat-labile enterotoxin, LT H44A (His to Arg substitution at position 44 from the N-terminus of the A1 fragment of the A subunit) as an adjuvant for nasal influenza vaccine were examined. (1) When 0.2 microg of LT H44A, together with 0.2 microg of influenza A/PR/8/34 virus (PR8, H1N1) vaccine, was administered intranasally into BALB/c mice (twice, 4 weeks apart), anti-PR8 hemagglutinin (HA) IgA and IgG antibody (Ab) responses were induced at levels that were sufficient to provide either complete protection against infection with a small volume of PR8 virus suspension or partial protection against infection with a lethal dose of the suspension. The dose of the mutant LT and vaccine used here (0.2 microg/ 20 g doses mouse) corresponded to the estimated dose per person, i.e. 0.1 mg/10 kg body weight. (2) Using these vaccination conditions, no additional total IgE Ab responses were induced. (3) The mutant was confirmed to be less toxic than the native LT when the toxicity was analyzed either using Y1 adrenal cells in vitro (1/483 EC(50)) or by an ileal loop test. (4) One hundred micrograms of the mutant, administered intranasally or intraperitoneally into guinea-pigs (Heartley strain, 0.3-0.4 kg), caused no body-weight changes 7 days after administration, although 100 microg of the native LT administered intraperitoneally caused death in all guinea-pigs due to diarrhea within 2 days. The intranasal administration of 100 microg of the mutant resulted in almost no pathological changes in the nasal mucosa 3 days after administration. These results suggest that LT H44A, which can be produced in high yields in an E. coli culture (about 5 mg/l), could be used as one of the effective and safe adjuvants for nasal influenza vaccine in humans.
Adjuvants, Immunologic↗
Body-force effect on the lateral movement of cellular flames at low Lewis numbers.
The body-force effect on the lateral movement of cellular flames is studied by unsteady calculations of reactive flows at low Lewis numbers. We employ the compressible Navier-Stokes equation including chemical reaction to take account of the hydrodynamic effect caused by thermal expansion. A sinusoidal disturbance with the linearly most unstable wavelength is superimposed on a plane flame to simulate the formation of a cellular flame. The superimposed disturbance grows initially with time, and then the flame front changes from a sinusoidal to a cellular shape. After the cell formation, the cellular flame moves laterally at Lewis numbers lower than unity. The reason is that the diffusive-thermal effect, and the nonlinear effect of the flame front, play a primary role in the appearance of the lateral movement of cells. The body-force effect has a great influence on the lateral velocity of cells. When flames are propagated upward, the lateral velocity decreases as the acceleration increases, even though the body-force effect has a destabilizing influence. When flames are propagated downward, on the other hand, the lateral velocity takes a maximum value at the specific acceleration and decreases with an increase in acceleration. The dependence of lateral velocity on the acceleration is due to the augmentation and diminution in maximum flame temperature and to the broadness and narrowness of a high-temperature region behind a convex flame front.
Journal Article↗
Protection against influenza B virus infection by immunization with DNA vaccines.
Protection against a lethal influenza B virus infection was examined in BALB/c mice immunized with plasmid DNAs encoding hemagglutinin (HA), neuraminidase (NA and NB) and nucleoprotein (NP) from the B/Ibaraki/2/85 virus. Each DNA vaccine was administered twice, 3 weeks apart, at a dose of 1 microg per mouse by particle-mediated DNA transfer to the epidermis (gene gun) or at a dose of 30 microg per mouse by electroporation into the muscle. Three weeks after the second vaccination, the mice were challenged with a lethal dose of homologous virus. HA and NA DNAs conferred complete protection against the lethal viral challenge, whereas NB and NP DNAs failed to provide protection against infection. Furthermore, protection in different strains of mice, BALB/c, B10 and C3H, immunized with HA and NA DNAs was compared. Both HA and NA DNAs conferred complete protection against the lethal challenge in all the tested mouse strains. These results suggest that both the HA and NA molecules can be used as vaccine components to provide effective protection against influenza B virus infection.
Animals↗
Plant regeneration from cell suspension-derived protoplasts of Primula malacoides and Primula obconica.
Protoplasts were isolated from cell suspension cultures of Primula malacoides cv. 'Lovely Tokyo' and P. obconica cv. 'Aalsmeer Giant White'. P. obconica protoplasts were embedded in 0.1% (w/v) gellan gum-solidified discs comprising MS medium supplemented with 3 mg/l of 2,4-D or picloram, 0.1 mg/l of zeatin, 0.2 M glucose and 0.2 M mannitol, and surrounded by a liquid medium of the same composition except for the addition of 0.1% (w/v) activated charcoal. The protoplasts formed visible colonies, which were transferred to the regeneration medium containing 30 g/l of sucrose, 0.1 mg/l of picloram and 2 mg/l of zeatin for shoot induction. P. malacoides protoplasts formed visible colonies when cultured in disc culture using 0.1% (w/v) gellan gum-solidified MS medium containing 5 mg/l of 2,4-D, 1 mg/l of NAA, 0.1 mg/l of zeatin and 0.4 M glucose. Small calli were transferred to MS medium supplemented with 5 mg/l of zeatin for shoot regeneration. The shoots of both species readily rooted on plant growth regulator-free 1/2 MS medium and successfully acclimatized to greenhouse conditions. The protoplast-derived plants showed some alterations in morphological characteristics from those of the in-vitro-germinated control plants.
Journal Article↗
Aggressive neoplastic plasma cell growth with MLL gene rearrangement after high-dose therapy with autologous stem cell support for multiple myeloma.
We report a case of a patient with IgA kappa multiple myeloma (MM) mobilized with etoposide and subsequently receiving high-dose melphalan (HDM) with stem cell support. She relapsed rapidly post transplantation. Southern blot and fluorescent in situ hybridization analysis showed MLL gene rearrangement in the myeloma cells, which was not detected in the sample at diagnosis or in the PBSC harvested with etoposide plus G-CSF. These observations suggest that clonal rearrangement of the MLL gene is caused by etoposide. Patients with MM undergoing HDM with stem cell rescue may be at an increased risk of not only secondary leukemia, but also secondary genetic abnormalities in myeloma cells, especially those receiving priming with etoposide for peripheral blood stem cell collection.
Antineoplastic Agents, Phytogenic↗
Cross-protection against a lethal influenza virus infection by DNA vaccine to neuraminidase.
Cross-protection against a lethal influenza virus infection was examined in BALB/c mice immunized with plasmid DNAs encoding the neuraminidase (NA) from different subtype A viruses. Each NA-DNA was administered twice, 3 weeks apart, at the dose of 1 microg per mouse by particle-mediated DNA transfer to the epidermis (gene gun) or at a dose of 30 microg per mouse by electroporation into the muscle. Three weeks after the second vaccination, the mice were challenged with lethal doses of homologous or heterologous viruses and the ability of each NA-DNA to protect the mice from influenza was evaluated by determining the lung virus titers, body weight and survival rates. The H3N2 virus NA-DNA conferred cross-protection against lethal challenge with antigenic variants within the same subtype, but failed to provide protection against infection by a different subtype virus (H1N1). The degree of cross-protection against infection was related to titers of the cross-reacting antibodies. These results suggest that NA-DNA can be used as a vaccine component to provide effective protection against infection not only with homologous virus but also with drift viruses.
Amino Acid Sequence↗
Protection against influenza virus infection in mice immunized by administration of hemagglutinin-expressing DNAs with electroporation.
Electroporation for the transfer of plasmid DNA encoding influenza virus hemagglutinin (HA) into muscle or nasal mucosa was tried in BALB/c mice to examine the efficacy of this method for inducing anti-HA immune responses and providing protection against homologous A/PR/8/34 (PR8) virus infection. Mice were immunized by two injections, 3 weeks apart, of HA-DNA with electroporation into the muscle wherein a pair of electrode needles was inserted to deliver the electric pulses. One or 3 weeks after the immunization, the mice were infected with a lethal dose of the PR8 virus. Ten micrograms or more of HA-DNA/dose induced strong serum anti-HA IgG antibody (Ab) responses, in which both IgG1 and IgG2a were predominant, and weak cytotoxic T lymphocyte responses. These immune responses were sufficient to provide efficient protection against the lethal infection. In addition, mice were immunized by dropping HA-DNA (12 microg) three times, 2 weeks between each dose into nostrils where each of two electrode needles was placed on the right nostril or the palate. One week after the immunization, the mice were infected with a sublethal dose of the PR8 virus. The DNA immunization by electroporation provided reduced nasal virus titers, in parallel with a relatively high levels of serum anti-HA IgG Ab and a slight nasal anti-HA IgA Ab production. The intranasal administration of cholera toxin before HA-DNA immunization by electroporation enhanced the nasal IgA Ab production together with enhancement of the efficiency of protection. These results suggest that electroporation can be used as one of the efficient gene delivery systems for the transfer of influenza DNA-vaccine into muscle or nasal mucosa to provide protection against influenza virus infection.
Administration, Intranasal↗
Protection against influenza virus infection by nasal vaccination in advance of sublethal irradiation.
The effect of sublethal gamma-ray irradiation on the protection conferred by a nasal influenza vaccine was investigated in BALB/c mice. A radiation dose of 7 Gy was selected as the sublethal dose as this caused exacerbation of the influenza but was not lethal in the mouse model. Mice were irradiated 7 days before, on the same day as, and 7 days after, administration of a nasal influenza vaccine, and were then infected with a lethal dose of the virus 4 weeks after vaccination. Almost all mice irradiated 7 days before or on the same day as vaccination died from viral pneumonia around 7 days after the challenge infection, whereas all mice irradiated 7 days after vaccination survived with no sign of infection. In mice irradiated 7 days after vaccination, both local anti-viral IgA and systemic IgG antibodies were produced in parallel with a marked reduction in lung viral titer, although no antibody production and no reduction in lung viral titer were detected in mice irradiated 7 days before, or on the same day as, vaccination. These results clearly demonstrate that vaccination with influenza virus before irradiation can protect mice from subsequent infection. This may be applicable to patients due to receive sublethal irradiation for bone marrow transplantation.
Administration, Intranasal↗
Low-dose continuous indomethacin in early days of age reduce the incidence of symptomatic patent ductus arteriosus without adverse effects.
We conducted a comparative study to evaluate whether the low-dose continuous indomethacin therapy in the early days of age would reduce incidence of the symptomatic patent ductus arteriosus (PDA) without the adverse effects. Thirty-seven infants were in the historical comparison group, and 39 infants were given low-dose indomethacin continuously (0.004 mg/kg/h) from 6-12 postnatal hours until the recognition of closing PDA. Low-dose continuous indomethacin significantly decreased the incidence of symptomatic PDA at 5 days of age (p < 0.01) as compared with the historical comparison group. There was no episode of decreasing urinary output and necrotizing enterocolitis in the indomethacin group. We conclude that the low-dose continuous indomethacin therapy results in decreasing the incidence of symptomatic PDA without significant adverse reactions.
Anti-Inflammatory Agents, Non-Steroidal↗
Spatiotemporal dynamics of inositol 1,4,5-trisphosphate that underlies complex Ca2+ mobilization patterns.
Inositol 1,4,5-trisphosphate (IP3) is a second messenger that elicits complex spatiotemporal patterns of calcium ion (Ca2+) mobilization and has essential roles in the regulation of many cellular functions. In Madin-Darby canine kidney epithelial cells, green fluorescent protein-tagged pleckstrin homology domain translocated from the plasma membrane to the cytoplasm in response to increased concentration of IP3. The detection of translocation enabled monitoring of IP3 concentration changes within single cells and revealed spatiotemporal dynamics in the concentration of IP3 synchronous with Ca2+ oscillations and intracellular and intercellular IP3 waves that accompanied Ca2+ waves. Such changes in IP3 concentration may be fundamental to Ca2+ signaling.
Adenosine Triphosphate↗
Trisomy 10 in adult pre-B-cell leukemia.
Explore the source record for details and available documents.
Adult↗
[Elderly dyserythropoietic anemia first diagnosed after presentation of hemorrhagic gastric ulcer].
A 71-year-old man presented with epigastralgia and tarry stool. Laboratory examination showed anemia (Hb 7.1 g/dl) due to hemorrhagic gastric ulcer and positive Coombs' test without features of hemolysis. Initial bone marrow smears disclosed normal granulocytes and megakaryocytes, but only erythroid hyperplasia with multinuclearity and megaloblastosis was identified. Cytogenetic studies revealed normal karyotype. Congenital dyserythropoietic anemia (CDA) type II was initially suspected. Serologically, however, acid hemolysis and anti-I * i tests were negative as were the results of another Coombs' test. The second bone marrow aspiration disclosed a marked decrease in multinucleated erythroblasts together with an increase in those that had internuclear chromatin bridges. Electron microscopy of bone marrow specimens demonstrated morphological features of CDA types I and II, thus yielding a final diagnosis of elderly dyserythropoietic anemia, which is similar to both CDA types. It was suggested that the reactive secretion of erythropoietin due to bleeding played a role in these pathologic changes.
Aged↗
Allosteric regulation by cytoplasmic Ca2+ and IP3 of the gating of IP3 receptors in permeabilized guinea-pig vascular smooth muscle cells.
1. The potentiation by Ca2+ of inositol 1,4,5-trisphosphate (IP3)-induced Ca2+ release was studied by measuring luminal Ca2+ concentrations of the Ca2+ stores using a fluorescent Ca2+ indicator, furaptra, in permeabilized smooth muscle cells. 2. Ca2+ release at 10 microM IP3 was potentiated by an increase in the cytoplasmic Ca2+ concentration in the presence of 10 mM EGTA. This effect was not due to the pharmacological effect of EGTA, because changes in the EGTA concentration at a constant Ca2+ concentration had no effect on the Ca2+ release rate. 3. With an increase in the cytoplasmic Ca2+ concentration from 30 to 630 nM, the Ca2+ release rate at a saturating IP3 concentration increased 110-fold and the EC50 for IP3 increased from 0.07 to 1.0 microM. It was also indicated that the relationship between Ca2+ concentration and Ca2+ release rate was shifted towards higher Ca2+ concentrations at higher IP3 concentrations. 4. These results suggest that IP3 and submicromolar concentrations of Ca2+ allosterically lower the affinity of the IP3 receptor for each other and are both required for IP3 receptor activation. These properties enable the IP3 receptors to detect simultaneous increases in IP3 and Ca2+ concentrations.
Action Potentials↗
Atrial septal defect in cyanotic CL/Fr mice.
OBJECTIVE: It is widely known that some newborn CL/Fr mice with cleft lip and palate (CLP) also have cyanotic symptoms, which have been thought to depend on an atrial septal defect (ASD). In a previous study, we found that cyanotic mice tended to have more severe types of CLP. We hypothesize that the mechanical airway obstruction due to a poorly developed palatal shelf and unmoved tongue in CLP(+) mice might be related to the occurrence of cyanosis. The purpose of this study was to examine the relationships between ASD and cyanosis in CLP(+) newborns. METHOD: The newborn hearts from CLP(-), noncyanotic CLP(+), cyanotic CLP(+), CL/Fr mice and ICR mice were examined histologically, and the incidence and size of ASD was determined on neonatal day (ND) 0. In CLP(-) newborns, similar procedures were performed from ND 1 to ND 4. Furthermore, in CLP(+) newborns, development of the palatal shelf was examined. RESULTS: While all the ICR mice had a well-developed atrial septum, and the incidence of ASD was 0%, about 80% of CL/Fr mice had ASD, irrespective of the presence or absence of CLP and cyanosis. On ND 0, the septum primum was significantly shorter in cyanotic CLP(+) mice than in CLP(-) mice. It also tended to be shorter in CLP(+) mice than in CLP(-) mice. Between the cyanotics and noncyanotics, there were no significant differences in the incidences of ASD and the rate of septal development. In CLP(-) mice, the septum primum developed well later and no ASD was observed on ND 4. Cyanotic newborns had significantly less developed palatal shelves than did noncyanotics. CONCLUSIONS: Cyanosis may not be related to ASD and the rate of septal development, but may be related to the occurrence of CLP in this strain. Furthermore, we confirmed that some relationship exists between the development of the palatal shelf and cyanosis. The present study supports our hypothesis concerning the cause of cyanosis in CL/Fr mice.
Airway Obstruction↗
Characterization of Bacillus sp. endo-beta-N-acetylglucosaminidase and its application to deglycosylation of hen ovomucoid.
A bacterial strain isolated from soil and identified as a Bacillus species produced two endo-beta-N-acetylglucosaminidases in the culture broth when it was cultivated on medium containing only hen ovomucoid. Almost no production of the enzymes occurred when the bacterium was grown on glucose medium. The two endo-beta-N-acetylglucosaminidases, named Endo-BI and Endo-BII, were separated and purified to homogeneity by preparative gel electrophoresis after partial purification by column chromatography on DEAE-resins. Endo-BI hydrolysed oligosaccharides of both hen ovalbumin and ovomucoid. In contrast, Endo-BII could act only on oligosaccharides of hen ovalbumin and showed almost no activity towards those of hen ovomucoid. Deglycosylation of hen ovomucoid was performed with the partly purified endo-beta-N-acetylglucosaminidase preparation, with the aid of contaminating beta-N-acetylhexosaminidase. The deglycosylated ovomucoid exhibited no changes in trypsin inhibitory activity but was very unstable to heat treatment in comparison with native ovomucoid. These results suggest that oligosaccharides of ovomucoid have an important role in the stabilization of the protein against heat.
Animals↗
Strain specific production of a negative regulator of IL-3 (NIL-3): difference in the negative feedback mechanism of hemopoiesis among mouse strains.
The producing cells of the negative regulator of interleukin-3 (NIL-3) were investigated. The 5-fluorouracil-treated bone marrow cells did not produce NIL-3. The bone marrow cells of stem cell-depleted W/WV mouse did not produce the NIL-3, either. The production of NIL-3 was different among mouse strains. Mice of C3H/HeN, A/J and ICR strains produced NIL-3, but the C57BL/6 mice did not produce NIL-3. These results indicate that the negative feedback mechanism of hemopoiesis is different among mouse strains. In the present study, we could not definitely identify the NIL-3 producing cells, although the present results are suggestive that the stem cells in cycle are a NIL-3 producer. Instead, we found that hemopoietic regulatory mechanisms might be different among mouse strains, especially in C57BL/6 mice.
Animals↗
Embryologic features of term fetuses and newborns in CL/Fr mice with special reference to cyanosis.
OBJECTIVE: The objective of this study was to clarify the embryologic features of CL/Fr mice in relation to cyanosis. METHOD: Orofacial examinations were conducted on 18-day-old term fetuses and newborns. Except for open eyelids in a few cases, all of the external abnormalities in this strain were cleft lip and/or palate (CLP). RESULTS: The CLP incidence in term fetuses was 28.4%. The body and placental weights were not different between CLP(+) and CLP(-) fetuses. Several types of CLP were found; the most frequent was bilateral complete CLP (60%) followed by left complete (16%). Bilateral complete CL, which is the most severe CL type, was associated with poorly developed secondary palate and wide cleft. In newborns, 39.4 % of CLP(+) mice showed cyanosis, where no CLP(-) mice had such signs. The body weight of cyanotic mice with CLP was significantly lighter than that of noncyanotic mice with CLP. Cyanotic mice had severe types of CLP and unelevated palatal shelves. CONCLUSION: We suggest that mechanical airway obstruction may be one of the causes of cyanosis in newborn CL/Fr mice.
Airway Obstruction↗