مرکز اطلاعات علمی Scientific Information Database (SID) - Trusted Source for Research and Academic Resources

video

مرکز اطلاعات علمی Scientific Information Database (SID) - Trusted Source for Research and Academic Resources

sound

مرکز اطلاعات علمی Scientific Information Database (SID) - Trusted Source for Research and Academic Resources

Persian Version

مرکز اطلاعات علمی Scientific Information Database (SID) - Trusted Source for Research and Academic Resources

View:

433
مرکز اطلاعات علمی Scientific Information Database (SID) - Trusted Source for Research and Academic Resources

Download:

82
مرکز اطلاعات علمی Scientific Information Database (SID) - Trusted Source for Research and Academic Resources

Cites:

Information Journal Paper

Title

SELECTIVE INHIBITION OF α-GALACTOSIDASE A WITH ANTISENSE OLIGODEOXYNUCLEOTIDE IN MESANGIAL CELLS: A RENAL CELLULAR MODEL FOR FABRY DISEASE

Pages

  397-400

Abstract

 Background and Aims: FABRY DISEASE is an X-linked lysosomal storage disease resulting from deficient activity of the enzyme a-galacotsidase (a-Gal) A. Accumulation of glycosphingolipids, especially GLOBOTRIAOSYLCERAMIDE, leads to various organ damage in FABRY DISEASE. Recently, replacement with recombinant a-Gal A has become available for the treatment of this disease. However, the pathogenic mechanism of this disease, which is the accumulation of glycosphingolipids, is still unknown. Understanding the pathogenesis of FABRY DISEASE may allow more efficient treatments. We examined whether the selective inhibition of a-Gal A with phosphorothioate antisense oligonucleotides could be used as a renal cellular model for FABRY DISEASE.Methods: Phosphorothioate antisense oligonucleotides designed to hyrbridize to sites on the human a-Gal A mRNA were tested for inhibition of a-Gal A expression in human mesangial cells. a-Gal A activity was measured using an artificial substrate, 4-methylumbelliferyl-a-D-galactoside.Results: Two antisense oligonucleotides selectively inhibited a-Gal A activity to below 20% of the mean control activity. These oligonucleotides did not affect other lysosomal enzyme activities.Conclusions: These data indicate that phosphorothioate oligonucleotides are capable of selectively inhibiting a-Gal A expression. It may be a useful model for renal mesangial cells in FABRY DISEASE.

Cites

  • No record.

    • References

    Cite

    APA: Copy

    UTSUMI, KOUICHI, ITOH, KOHJI, HIRAMA, AKIO, UEDA, KAE, SAKAMAKI, MASANORI, KANEKO, TOMOHIRO, YAMAZAKI, MINEO, KOMABA, YUICHI, KATSURA, KEN ICHIRO, IINO, YASUHIKO, & KATAYAMA, YASUO. (2010). SELECTIVE INHIBITION OF α-GALACTOSIDASE A WITH ANTISENSE OLIGODEOXYNUCLEOTIDE IN MESANGIAL CELLS: A RENAL CELLULAR MODEL FOR FABRY DISEASE. THE JOURNAL OF NEPHRO-UROLOGY MONTHLY, 2(3), 397-400. SID. https://sid.ir/paper/323715/en

    Vancouver: Copy

    UTSUMI KOUICHI, ITOH KOHJI, HIRAMA AKIO, UEDA KAE, SAKAMAKI MASANORI, KANEKO TOMOHIRO, YAMAZAKI MINEO, KOMABA YUICHI, KATSURA KEN ICHIRO, IINO YASUHIKO, KATAYAMA YASUO. SELECTIVE INHIBITION OF α-GALACTOSIDASE A WITH ANTISENSE OLIGODEOXYNUCLEOTIDE IN MESANGIAL CELLS: A RENAL CELLULAR MODEL FOR FABRY DISEASE. THE JOURNAL OF NEPHRO-UROLOGY MONTHLY[Internet]. 2010;2(3):397-400. Available from: https://sid.ir/paper/323715/en

    IEEE: Copy

    KOUICHI UTSUMI, KOHJI ITOH, AKIO HIRAMA, KAE UEDA, MASANORI SAKAMAKI, TOMOHIRO KANEKO, MINEO YAMAZAKI, YUICHI KOMABA, KEN ICHIRO KATSURA, YASUHIKO IINO, and YASUO KATAYAMA, “SELECTIVE INHIBITION OF α-GALACTOSIDASE A WITH ANTISENSE OLIGODEOXYNUCLEOTIDE IN MESANGIAL CELLS: A RENAL CELLULAR MODEL FOR FABRY DISEASE,” THE JOURNAL OF NEPHRO-UROLOGY MONTHLY, vol. 2, no. 3, pp. 397–400, 2010, [Online]. Available: https://sid.ir/paper/323715/en

    Related Journal Papers

  • No record.

    • Related Seminar Papers

  • No record.

    • Related Plans

  • No record.

    • Recommended Workshops






    Move to top
    telegram sharing button
    whatsapp sharing button
    linkedin sharing button
    twitter sharing button
    email sharing button
    email sharing button
    email sharing button
    sharethis sharing button