Skip to main content
Article thumbnail
Location of Repository

Genistein-mediated inhibition of glycosaminoglycan synthesis, which corrects storage in cells of patients suffering from mucopolysaccharidoses, acts by influencing an epidermal growth factor-dependent pathway

By Joanna Jakóbkiewicz-Banecka, Ewa Piotrowska, Magdalena Narajczyk, Sylwia Barańska and Grzegorz Węgrzyn
Topics: Research
Publisher: BioMed Central
OAI identifier:
Provided by: PubMed Central
Download PDF:
Sorry, we are unable to provide the full text but you may find it at the following location(s):
  • http://www.pubmedcentral.nih.g... (external link)
  • Suggested articles


    1. (2006). A: A phase II/III clinical study of enzyme replacement therapy with idursulfase in mucopolysaccharidosis II (Hunter syndrome). Genet Med
    2. (1960). Aliphatic ammonium salts in the assay of acidic polysaccharides from tissues. Methods Biochem Anal
    3. (2004). Cox GF: Enzyme replacement therapy for mucopolysaccharidosis I: a randomized, doubleblinded, placebo-controlled, multinational study of recombinant human α-L-iduronidase (Laronidase). J Pediatr
    4. (2007). Cytokines, neurotrophins, and oxidative stress in brain disease from mucopolysaccharidosis IIIB.
    5. (2001). EF: Enzyme-replacement therapy in mucopolysaccharidosis I.
    6. (1987). Epidermal growth factor potentiates the induction of ornithine decarboxylase activity by prostaglandins in embryonic palate mesenchymal cells: effects on cell proliferation and glycosaminoglycan synthesis. Dev Biol
    7. (1987). Fukami Y: Genistein, a specific inhibitor of tyrosinespecific protein kinase.
    8. (2006). G: Genistein-mediated inhibition of glycosaminoglycan synthesis as a basis for gene expression-targeted isoflavone therapy for mucopolysaccharidoses.
    9. (2006). Inhibition of glycosaminoglycan synthesis using rhodamine B in a mouse model of mucopolysaccharidosis type IIIA. Pediatr Res
    10. (2007). Kakkis ED: A follow-up study of MPS I patients treated with laronidase enzyme replacement therapy for 6 years. Mol Genet Metab
    11. (2007). Karelson E: Antioxidative effects of plant polyphenols: from protection of G protein signaling to prevention of age-related pathologies.
    12. (2007). King AA: Genistein genotoxity: critical considerations of in vitro exposure dose. Toxicol Appl Pharmacol
    13. (2007). L: Genistein – a dietary compound inducing hormonal and metabolic changes.
    14. (1998). Mechanisms of action of the soy isoflavone genistein: emerging role for its effects via transforming growth factor beta signaling pathways.
    15. (2005). Meikle PJ: Prediction of neuropathology in mucopolysaccharidosis I patients. Mol Genet Metab
    16. Mucopolysaccharidoses: clinical features and management. In Genetic Metabolic Disorders: Management, Costs and Sociomedical Aspects Edited by: vom
    17. (2007). New therapeutic options for lysosomal storage disorders: enzyme replacement, small molecules and gene therapy. Hum Genet
    18. (2007). S: Improvement in behaviour after substrate deprivation therapy with rhodamine B in a mouse model of MPS IIIA. Mol Genet Metab
    19. (1997). Salustri A: Hyaluronan synthesis by mouse cumulus cells is regulated by interactions between folicle-stimulating hormone (or epidermal growth factor) and a soluble oocyte factor (or transforming growth factor β1).
    20. (2007). Substrate deprivation therapy: A new hope for patients suffering from neuronopathic forms of inherited lysosomal storage diseases.
    21. (2006). SV: Sulforaphane causes autophagy to inhibit release of cytochrome c and apoptosis in human prostate cancer cells. Cancer Res
    22. (2006). TB: A historical perspective of the EGF receptor and related systems. Methods Mol Biol
    23. The mucopolysaccharidoses. In The Metabolic and Molecular Bases of Inherited Disease Volume 3. 8th edition. Edited by: Scriver
    24. (2003). Towle CA: A role for the interleukin-1 receptor in the pathway linking static mechanical compression to decreased proteoglycan synthesis in surface articular cartilage. Arch Biochem Biophys
    25. (2004). Tylki-Szymańska A: A general model for genetic regulation of turnover of glycosaminoglycans suggests a possible procedure for prediction of severity and clinical progress of mucopolysaccharidoses. Med Hypoth
    26. (2009). Węgrzyn G: Correlation between severity of mucopolysaccharidoses and combination of the residual enzyme activity and efficiency of glycosaminoglycan synthesis. Acta Paediatr
    27. (2008). Węgrzyn G: Genistin-rich isoflavone extract in substrate reduction therapy for Sanfilippo syndrome: an open-label, pilot study in 10 pediatric patients. Curr Ther Res Clin Exp

    To submit an update or takedown request for this paper, please submit an Update/Correction/Removal Request.