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11.
Matsuda J Kido M Tadano-Aritomi K Ishizuka I Tominaga K Toida K Takeda E Suzuki K Kuroda Y 《Human molecular genetics》2004,13(21):2709-2723
The sphingolipid activator proteins (saposins A, B, C and D) are small homologous glycoproteins that are encoded by a single gene in tandem within a large precursor protein (prosaposin) and are required for in vivo degradation of some sphingolipids with relatively short carbohydrate chains. Human patients with prosaposin or specific saposin B or C deficiency are known, and prosaposin- and saposin A-deficient mouse lines have been generated. Experimental evidence suggests that saposin D may be a lysosomal acid ceramidase activator. However, no specific saposin D deficiency state is known in any mammalian species. We have generated a specific saposin D(-/-) mouse by introducing a mutation (C509S) into the saposin D domain of the mouse prosaposin gene. Saposin D(-/-) mice developed progressive polyuria at around 2 months and ataxia at around 4 months. Pathologically, the kidney of saposin D(-/-) mice showed renal tubular degeneration and eventual hydronephrosis. In the nervous system, progressive and selective loss of the cerebellar Purkinje cells in a striped pattern was conspicuous, and almost all Purkinje cells disappeared by 12 months. Biochemically, ceramides, particularly those containing hydroxy fatty acids accumulated in the kidney and the brain, most prominently in the cerebellum. These results not only indicate the role of saposin D in in vivo ceramide metabolism, but also suggest possible cytotoxicity of ceramide underlying the cerebellar Purkinje cell and renal tubular cell degeneration. 相似文献
12.
Okamoto T Hashimoto M Samejima H Mori A Wakabayashi M Takeda A Nakamura H Naruse H Bouike Y Araki N 《Immunopharmacology and immunotoxicology》2004,26(4):645-652
Immediate hemolytic transfusion reactions (IHTR) occurred in the course of delayed hemolytic transfusion reactions (DHTR). An 84-year-old man had received a blood transfusion 20 years ago. Progressive anemia developed, because of continuous bleeding from a bladder tumor. He was transfused with concentrated red blood cells (CRC) which were Rh-E antigen negative, because he had anti-E antibodies (day 0). He received CRC on day 3, and underwent resection of bladder tumor on day 6. Although crossmatch-compatible CRCs were prepared for the operation, those were not required and were kept in a refrigerator in the ward. On day 9, when a CRC kept in the ward was transfused, he suddenly had a IHTR. In order to analyze a mechanism of IHTR, the anti-Jk(b) and anti-Di(b) antibodies, anti-HLA antibodies and the concentrations of inflammatory cytokines were measured in serum samples. The anti-Jk(b) and anti-Di(b) antibodies increased prior to IHTR experienced on day 9. The concentrations of IL-6 and IL-1beta increased from day 2, while the concentration of IL-8 increased from day 7. The anti-HLA class I antibody could be detected 2 days before IHTR. Thus, the anti-Jk(b) and anti-Di(b) antibodies induced the production of inflammatory cytokines and symptoms of DHTR and IHTR. The anti-HLA class I antibody could be produced in spite of using the filer for removing leukocytes, and may take part in the induction of IHTR. Further, blood products should be transfused soon after completing a crossmatch test in patients with anti-RBC alloantibodies. 相似文献
13.
Furuta M Kose S Koike M Shimi T Hiraoka Y Yoneda Y Haraguchi T Imamoto N 《Genes to cells : devoted to molecular & cellular mechanisms》2004,9(5):429-441
Heat-shock induces a strong stress response and modifies all aspects of cellular physiology, which involves dynamic changes in the nucleocytoplasmic distributions of a variety of proteins. Many distinct nucleocytoplasmic transport pathways exist in eukaryotic cells, but how a particular transport pathway is regulated under different cellular conditions remains elusive. The finding of this study indicate that conventional nuclear import, which is mediated by importin alpha/beta, is down-regulated, while the nuclear import of 70 kD heat-shock cognate protein is up-regulated in heat-shock cells. Among the factors involved in the mediation of the conventional nuclear import, significant levels of importin alpha accumulate in the nucleus in response to heat-shock. An analysis of the behaviour of importin alpha with fluorescence recovery after photobleaching and fluorescence loss in photobleaching studies show that nuclear importin alpha becomes less mobile and its nucleocytoplasmic recycling is impaired in heat-shock cells. These data coincided well with biochemical and cytological studies. Our present data show that heat-shock induces the nuclear accumulation, nuclear retention, and recycling inhibition of importin alpha, resulting in the suppression of conventional nuclear import. This suggests a new regulatory mechanism for the adaptation of cells to environmental changes, such as heat-shock. 相似文献
14.
Chiaki Watanabe Makiko Kuwagata Shinsuke Yoshimura Jiro Azegami Kouichi Kojima Hiroshi Ono Tetsuji Nagao 《Clinical genetics》2003,43(3):177-179
ABSTRACT The technique for gavage administration to rat nurslings was improved to allow determination of the direct effects of chemical substances in the nurslings. Rat neonates were treated with distilled water from postnatal day 1 through 20 using this technique. The viability of neonates during the administration period was comparable to that of untreated neonates. No adverse effects of this technique on the development of neonates were found, and no histological alterations of the esophagus or pharynx. Therefore, we conclude that use of our improved gavage administration method will contribute to ensuring successful neonatal development and thus allowing accurate assessment of the toxicological effects of test compounds on rat nurslings. 相似文献
15.
Makiko Itami Toshinao Takenouchi Junichi Tamaru Kenichi Harigaya Atsuo Mikata 《Pathology international》1991,41(4):277-285
It is well known that non Hodgkin's lymphoma (NHL) cells express various antigens which are normally involved in a variety of functions. In addition, NHL is diverse in its proliferative capacity. To investigate the relation between these factors and the clinical picture, 45 cases of NHL were studied by immunohistochemistry using snap-frozen materials obtained before therapy. Reactivities with 27 monoclonal antibodies were examined and the results were correlated with clinical findings. The expression of surface μ and CAM-1 in B-NHLs and CD25 in T-NHLs were significantly associated with bone marrow involvement. B-NHLs without expression of CD21(B2) and T-NHLs with CD25 were seen more frequently in cases with a LDH value of over 500 units/ml. The positivity rate of Ki-67 on B-NHLs was correlated with serum LDH value, NHL histologic classification, and overall survival. These data indicate that immunophenotyping and determination of the proliferative capacity of NHL are of value not only for confirmation of the histopathologic classification of the tumor but also for assessment of clinical behavior. 相似文献
16.
Atsuko Koizumi Makiko Fujii Masuo Kondoh Yoshiteru Watanabe 《European journal of pharmaceutics and biopharmaceutics》2004,57(3):473-478
N-Methyl-2-pyrrolidone (NMP) increased the skin permeation of estradiol (E2) in Yucatan micropig epidermis using a modified Franz-type diffusion cell. The addition of NMP significantly increased the fluxes of E2 from water and soybean oil. The flux and skin concentration of E2 were higher from soybean oil than from water and increased with increasing NMP concentrations in soybean oil. Correlation was observed with E2 flux and skin concentration (R(2) = 0.804) NMP enhanced E2 skin permeation because NMP made E2 skin concentration higher. Thus, NMP (10%) was added to the oily gel made by isocetyl isostearate and hydrogenated phospholipid. E2 permeation from the gel without NMP was the same as that from soybean oil suspension. The flux of E2 from the gel with NMP was 0.6 microg/h per cm(2) and might be sufficient for estrogen replacement therapy. 相似文献
17.
18.
Makiko Nakamura Teruhiko Imamura Takuya Fukui Naoya Kataoka Koichiro Kinugawa 《Internal medicine (Tokyo, Japan)》2022,61(20):3063
Patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) classically present with ventricular arrhythmias and less commonly heart failure. ARVC is an inherited cardiomyopathy and generally based on a variant of desmosomal genes. Recently, the association between myocardial inflammation and ARVC has been a matter of great concern. We encountered a patient with ARVC who had a desmoglein-2 mutation with advanced right ventricular failure accompanying a preserved left ventricular function. Concomitant right ventricular myocarditis was detected four years after the diagnosis of ARVC. ARVC and myocarditis might have a deep pathophysiological association, at least in some cases. 相似文献
19.
Yuko Sawada Noritsugu Honda Makiko Narumiya Hiroshi Mizumoto 《Journal of Physical Therapy Science》2022,34(10):697
[Purpose] This study aimed to examine, using the Interaction Rating Scale, the effect of social skills at 18 months of life on the subsequent development of low birthweight infants. [Participants and Methods] The study participants were made up of a total of 23 infants who were admitted to the neonatal intensive care unit of Hospital A and whose developmental indexes were followed up at the outpatient clinic for up to 3 years of age. The survey was conducted twice in each infant, at a corrected age of 18 months and at 36 full months of age. Social skills and developmental indexes were assessed at the corrected age of 18 months, meanwhile only developmental indexes were assessed at 36 full months, to examine associations. The Interaction Rating Scale was used to assess social skills. This scale measures various aspects of social development by observing caregiver-child interactions in situations wherein children are engaged in tasks more difficult for their age. [Results] The results demonstrated that social skills at 18 months were associated with the developmental indexes at 18 and 36 months, whereas more items were associated with the developmental index at 36 months. [Conclusion] The results indicate the need for early prediction of developmental delay and timely intervention, by assessing social skills in low birthweight infants. 相似文献
20.
Inhibition of autophagy by chloroquine makes chemotherapy in nasopharyngeal carcinoma more efficient
Tomomi Aga Kazuhira Endo Akira Tsuji Mitsuharu Aga Makiko Moriyama-Kita Takayoshi Ueno Yosuke Nakanishi Miyako Hatano Satoru Kondo Hisashi Sugimoto Naohiro Wakisaka Tomokazu Yoshizaki 《Auris, nasus, larynx》2019,46(3):443-450