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  1. Article: Increased globotriaosylceramide levels in a transgenic mouse expressing human α1,4-galactosyltransferase and a mouse model for treating Fabry disease

    Shiozuka, Chikara / Taguchi, Atsumi / Matsuda, Junichiro / Noguchi, Yoko / Kunieda, Takanori / Uchio-Yamada, Kozue / Yoshioka, Hidekatsu / Hamanaka, Ryoji / Yano, Shinji / Yokoyama, Shigeo / Mannen, Kazuaki / Kulkarni, Ashok B / Furukawa, Koichi / Ishii, Satoshi

    Journal of biochemistry. 2011 Feb., v. 149, no. 2

    2011  

    Abstract: Fabry disease is a lysosomal storage disorder caused by an α-galactosidase A (α-Gal A) deficiency and resulting in the accumulation of glycosphingolipids, predominantly globotriaosylceramide (Gb3). A transgenic mouse expressing the human α-Gal A R301Q ... ...

    Abstract Fabry disease is a lysosomal storage disorder caused by an α-galactosidase A (α-Gal A) deficiency and resulting in the accumulation of glycosphingolipids, predominantly globotriaosylceramide (Gb3). A transgenic mouse expressing the human α-Gal A R301Q mutant in an α-Gal A-knockout background (TgM/KO) should be useful for studying active-site-specific chaperone (ASSC) therapy for Fabry disease. However, the Gb3 content in the heart tissue of this mouse was too low to detect an ASSC-induced effect. To increase the Gb3 levels in mouse organs, we created transgenic mice (TgG3S) expressing human α1,4-galactosyltransferase (Gb3 synthase). High levels of Gb3 were observed in all major organs of the TgG3S mouse. A TgG3S (+/-)M(+/-)/KO mouse was prepared by cross-breeding the TgG3S and TgM/KO mice and the Gb3 content in the heart of the TgG3S(+/-)M(+/-)/KO mouse was 1.4 μg/mg protein, higher than in the TgM(+/-)/KO (<0.1 μg/mg protein). Treatment with an ASSC, 1-deoxygalactonojirimycin, caused a marked induction of α-Gal A activity and a concomitant reduction of the Gb3 content in the TgG3S(+/-) M(+/-)/KO mouse organs. These data indicated that the TgG3S(+/-) M(+/-)/KO mouse was suitable for studying ASSC therapy for Fabry disease, and that the TgG3S mouse would be useful for studying the effect of high Gb3 levels in mouse organs.
    Language English
    Dates of publication 2011-02
    Size p. 161-170.
    Publishing place Japanese Biochemical Society
    Document type Article
    ZDB-ID 218073-x
    ISSN 1756-2651 ; 0021-924X
    ISSN (online) 1756-2651
    ISSN 0021-924X
    Database NAL-Catalogue (AGRICOLA)

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    In stock of ZB MED Bonn / Germany

    Z 80/714: Show issues
    Z 6.2: Show issues

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  2. Article ; Online: Increased globotriaosylceramide levels in a transgenic mouse expressing human alpha1,4-galactosyltransferase and a mouse model for treating Fabry disease.

    Shiozuka, Chikara / Taguchi, Atsumi / Matsuda, Junichiro / Noguchi, Yoko / Kunieda, Takanori / Uchio-Yamada, Kozue / Yoshioka, Hidekatsu / Hamanaka, Ryoji / Yano, Shinji / Yokoyama, Shigeo / Mannen, Kazuaki / Kulkarni, Ashok B / Furukawa, Koichi / Ishii, Satoshi

    Journal of biochemistry

    2010  Volume 149, Issue 2, Page(s) 161–170

    Abstract: Fabry disease is a lysosomal storage disorder caused by an α-galactosidase A (α-Gal A) deficiency and resulting in the accumulation of glycosphingolipids, predominantly globotriaosylceramide (Gb3). A transgenic mouse expressing the human α-Gal A R301Q ... ...

    Abstract Fabry disease is a lysosomal storage disorder caused by an α-galactosidase A (α-Gal A) deficiency and resulting in the accumulation of glycosphingolipids, predominantly globotriaosylceramide (Gb3). A transgenic mouse expressing the human α-Gal A R301Q mutant in an α-Gal A-knockout background (TgM/KO) should be useful for studying active-site-specific chaperone (ASSC) therapy for Fabry disease. However, the Gb3 content in the heart tissue of this mouse was too low to detect an ASSC-induced effect. To increase the Gb3 levels in mouse organs, we created transgenic mice (TgG3S) expressing human α1,4-galactosyltransferase (Gb3 synthase). High levels of Gb3 were observed in all major organs of the TgG3S mouse. A TgG3S (+/-)M(+/-)/KO mouse was prepared by cross-breeding the TgG3S and TgM/KO mice and the Gb3 content in the heart of the TgG3S(+/-)M(+/-)/KO mouse was 1.4 µg/mg protein, higher than in the TgM(+/-)/KO (<0.1 µg/mg protein). Treatment with an ASSC, 1-deoxygalactonojirimycin, caused a marked induction of α-Gal A activity and a concomitant reduction of the Gb3 content in the TgG3S(+/-) M(+/-)/KO mouse organs. These data indicated that the TgG3S(+/-) M(+/-)/KO mouse was suitable for studying ASSC therapy for Fabry disease, and that the TgG3S mouse would be useful for studying the effect of high Gb3 levels in mouse organs.
    MeSH term(s) 1-Deoxynojirimycin/analogs & derivatives ; 1-Deoxynojirimycin/pharmacology ; Animals ; Crosses, Genetic ; Disease Models, Animal ; Enzyme Activation/drug effects ; Fabry Disease/drug therapy ; Fabry Disease/enzymology ; Fabry Disease/genetics ; Female ; Galactosyltransferases/genetics ; Galactosyltransferases/metabolism ; Humans ; Kidney/chemistry ; Liver/chemistry ; Mice ; Mice, Knockout ; Mice, Transgenic ; Molecular Chaperones/pharmacology ; Spleen/chemistry ; Trihexosylceramides/metabolism ; Up-Regulation/drug effects ; alpha-Galactosidase/genetics ; alpha-Galactosidase/metabolism
    Chemical Substances Molecular Chaperones ; Trihexosylceramides ; 1-Deoxynojirimycin (19130-96-2) ; globotriaosylceramide (71965-57-6) ; migalastat (C4XNY919FW) ; Galactosyltransferases (EC 2.4.1.-) ; UDP-galactose-lactosylceramide alpha 1-4-galactosyltransferase (EC 2.4.1.-) ; alpha-Galactosidase (EC 3.2.1.22)
    Language English
    Publishing date 2010-10-19
    Publishing country England
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 218073-x
    ISSN 1756-2651 ; 0021-924X
    ISSN (online) 1756-2651
    ISSN 0021-924X
    DOI 10.1093/jb/mvq125
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Uc I Zs.202: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 2021: Bestellungen von Artikeln über das Online-Bestellformular
    ab Jg. 2022: Lesesaal (EG)

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  3. Article ; Online: Anti-inflammatory effect of buckwheat sprouts in lipopolysaccharide-activated human colon cancer cells and mice.

    Ishii, Satoshi / Katsumura, Takafumi / Shiozuka, Chikara / Ooyauchi, Keisuke / Kawasaki, Kunito / Takigawa, Shigenobu / Fukushima, Tatsunobu / Tokuji, Yoshihiko / Kinoshita, Mikio / Ohnishi, Masao / Kawahara, Mika / Ohba, Kiyoshi

    Bioscience, biotechnology, and biochemistry

    2008  Volume 72, Issue 12, Page(s) 3148–3157

    Abstract: In conducting an in vitro screening of ethanol extracts from various natural foods using a human colon cancer cell line (CoLoTC cells), an extract of buckwheat sprouts (ExtBS) was found to express significant anti-inflammatory activity. The anti- ... ...

    Abstract In conducting an in vitro screening of ethanol extracts from various natural foods using a human colon cancer cell line (CoLoTC cells), an extract of buckwheat sprouts (ExtBS) was found to express significant anti-inflammatory activity. The anti-inflammatory activity of ExtBS was confirmed by oral administration of lipopolysaccharide (LPS) to mice. Inflammatory cytokines (interleukin 6 and tumor necrosis factor alpha) were markedly up-regulated in the spleen and liver from LPS-administrated mice, and combinatory treatment with LPS and ExtBS decreased up-regulation of them in both cytokines. Both serum cytokine levels corresponded to their gene expressions in tissues, but no anti-inflammatry effect in mice was observed when ExtBS was treated intraperitoneally. ExtBS oral administration also showed protective activity as to hepatic injury induced by galactosamine/LPS treatment. Based on these data, we suggest that ExtBS contains anti-inflammatory compounds.
    MeSH term(s) Animals ; Anti-Inflammatory Agents/administration & dosage ; Anti-Inflammatory Agents/pharmacology ; Cell Line, Tumor ; Cell Survival/drug effects ; Colonic Neoplasms/pathology ; Fagopyrum/chemistry ; Flavonoids/pharmacology ; Galactosamine/toxicity ; Gene Expression Regulation, Neoplastic/drug effects ; Humans ; Inflammation/chemically induced ; Inflammation/metabolism ; Interleukin-8/genetics ; Lipopolysaccharides/administration & dosage ; Lipopolysaccharides/toxicity ; Liver/drug effects ; Liver/injuries ; Mice ; Oligonucleotide Array Sequence Analysis ; Plant Extracts/administration & dosage ; Plant Extracts/pharmacology ; Plant Shoots/chemistry ; Reference Standards ; Up-Regulation/drug effects
    Chemical Substances Anti-Inflammatory Agents ; Flavonoids ; Interleukin-8 ; Lipopolysaccharides ; Plant Extracts ; Galactosamine (7535-00-4)
    Language English
    Publishing date 2008-12
    Publishing country England
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 1106450-x
    ISSN 1347-6947 ; 0916-8451
    ISSN (online) 1347-6947
    ISSN 0916-8451
    DOI 10.1271/bbb.80324
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 4647: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
    Jg. 1995 - 2021: Lesesall (2.OG)
    ab Jg. 2022: Lesesaal (EG)

    Order via subito

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  4. Article: Anti-Inflammatory Effect of Buckwheat Sprouts in Lipopolysaccharide-Activated Human Colon Cancer Cells and Mice

    ISHII, Satoshi / KATSUMURA, Takafumi / SHIOZUKA, Chikara / OOYAUCHI, Keisuke / KAWASAKI, Kunito / TAKIGAWA, Shigenobu / FUKUSHIMA, Tatsunobu / TOKUJI, Yoshihiko / KINOSHITA, Mikio / OHNISHI, Masao / KAWAHARA, Mika / OHBA, Kiyoshi

    Bioscience, biotechnology, and biochemistry. 2008 Dec. 23, v. 72, no. 12

    2008  

    Abstract: In conducting an in vitro screening of ethanol extracts from various natural foods using a human colon cancer cell line (CoLoTC cells), an extract of buckwheat sprouts (ExtBS) was found to express significant anti-inflammatory activity. The anti- ... ...

    Abstract In conducting an in vitro screening of ethanol extracts from various natural foods using a human colon cancer cell line (CoLoTC cells), an extract of buckwheat sprouts (ExtBS) was found to express significant anti-inflammatory activity. The anti-inflammatory activity of ExtBS was confirmed by oral administration of lipopolysaccharide (LPS) to mice. Inflammatory cytokines (interleukin 6 and tumor necrosis factor alpha) were markedly up-regulated in the spleen and liver from LPS-administrated mice, and combinatory treatment with LPS and ExtBS decreased up-regulation of them in both cytokines. Both serum cytokine levels corresponded to their gene expressions in tissues, but no anti-inflammatry effect in mice was observed when ExtBS was treated intraperitoneally. ExtBS oral administration also showed protective activity as to hepatic injury induced by galactosamine/LPS treatment. Based on these data, we suggest that ExtBS contains anti-inflammatory compounds.
    Keywords anti-inflammatory activity ; biotechnology ; blood serum ; buckwheat ; colorectal neoplasms ; ethanol ; galactosamine ; genes ; humans ; interleukin-6 ; lipopolysaccharides ; liver ; neoplasm cells ; oral administration ; spleen ; tumor necrosis factor-alpha
    Language English
    Dates of publication 2008-1223
    Size p. 3148-3157.
    Publishing place Japan Society for Bioscience, Biotechnology, and Agrochemistry
    Document type Article
    Note NAL-AP-2-clean
    ZDB-ID 1106450-x
    ISSN 1347-6947 ; 0916-8451
    ISSN (online) 1347-6947
    ISSN 0916-8451
    DOI 10.1271/bbb.80324
    Database NAL-Catalogue (AGRICOLA)

    More links

    Kategorien

    In stock of ZB MED Bonn / Germany

    Z 1783: Show issues

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