ArticleMetabolic brain disease2011
Effects of flavonoids on glycosaminoglycan synthesis: implications for substrate reduction therapy in Sanfilippo disease and other mucopolysaccharidoses.
Article in Metabolic brain disease, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers.
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Who cites it
25 citing papers in PubMed, 46 citations in OpenAlex.
- Genistein supplementation in patients affected by Sanfilippo disease.Journal of inherited metabolic disease · 2011Trial
- Advances in Therapies for Mucopolysaccharidoses.Current issues in molecular biology · 2026Review
- Efficacy of a Combination Therapy with Laronidase and Genistein in Treating Mucopolysaccharidosis Type I in a Mouse Model.International journal of molecular sciences · 2024Article
- Murine Fibroblasts and Primary Hepatocytes as Tools When Studying the Efficacy of Potential Therapies for Mucopolysaccharidosis Type I.International journal of molecular sciences · 2022Article
- The Effects of Genistein at Different Concentrations onInternational journal of molecular sciences · 2022Article
- Mucopolysaccharidosis: A broad review.Indian journal of ophthalmology · 2022Review
- Can genistein be a potential agent against skin side effects associated with the treatment of breast cancer?Postepy dermatologii i alergologii · 2022Review
- Cellular and Gene Expression Response to the Combination of Genistein and Kaempferol in the Treatment of Mucopolysaccharidosis Type I.International journal of molecular sciences · 2022Article
- Improved Pharmacokinetics and Tissue Uptake of Complexed Daidzein in Rats.Pharmaceutics · 2020Article
- Proteasome Composition and Activity Changes in Cultured Fibroblasts Derived From Mucopolysaccharidoses Patients and Their Modulation by Genistein.Frontiers in cell and developmental biology · 2020Article
- The Role of Dimethyl Sulfoxide (DMSO) in Gene Expression Modulation and Glycosaminoglycan Metabolism in Lysosomal Storage Disorders on an Example of Mucopolysaccharidosis.International journal of molecular sciences · 2019Article
- New treatments for the mucopolysaccharidoses: from pathophysiology to therapy.Italian journal of pediatrics · 2018Review
- Article
- Dietary Management of Skin Health: The Role of Genistein.Nutrients · 2017Review
- Small molecules as therapeutic agents for inborn errors of metabolism.Journal of inherited metabolic disease · 2017Review
- Nonsteroidal anti-inflammatory drugs modulate cellular glycosaminoglycan synthesis by affecting EGFR and PI3K signaling pathways.Scientific reports · 2017Article
- New therapeutic approaches for Krabbe disease: The potential of pharmacological chaperones.Journal of neuroscience research · 2016Review
- Activities of genes controlling sphingolipid metabolism in human fibroblasts treated with flavonoids.Metabolic brain disease · 2015Article
- Effects of flavonoids on expression of genes involved in cell cycle regulation and DNA replication in human fibroblasts.Molecular and cellular biochemistry · 2015Article
- Modulation of expression of genes involved in glycosaminoglycan metabolism and lysosome biogenesis by flavonoids.Scientific reports · 2015Article
Corrections and comments
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Authors and funding
5 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Sanfilippo disease (mucopolysaccharidosis type III, MPS III) is a severe metabolic disorder caused by accumulation of heparan sulfate (HS), one of glycosaminoglycans (GAGs), due to a genetic defect resulting in a deficiency of GAG hydrolysis. This disorder is characterized as the most severe neurological form of MPS, revealing rapid deterioration of brain functions. Among therapeutic approaches for MPS III, one of the most promising appears to be the substrate reduction therapy (SRT). Genistein (5, 7-dihydroxy-3- (4-hydroxyphenyl)-4H-1-benzopyran-4-one) is an isoflavone that has been used in SRT for MPS III. In this report, we tested effects of other flavonoids (apigenin, daidzein, kaempferol and naringenin) on GAG synthesis. Their cytotoxicity and anti-proliferation features were also tested. We found that daidzein and kaempferol inhibited GAG synthesis significantly. Moreover, these compounds were able to reduce lysosomal storage in MPS IIIA fibroblasts. Interestingly, although genistein is believed to inhibit GAG synthesis by blocking the tyrosine kinase activity of the epidermal growth factor receptor, we found that effects of other flavonoids were not due to this mechanism. In fact, combinations of various flavonoids resulted in significantly more effective inhibition of GAG synthesis than the use of any of these compounds alone. These results, together with results published recently by others, suggest that combination of flavonoids can be considered as a method for improvement of efficiency of SRT for MPS III.
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