Inhibition of Glycosaminoglycan Synthesis Using Rhodamine B in a Mouse Model of Mucopolysaccharidosis Type IIIA

No abstract is available for this paper.

• Publication year

  2006

• Venue

  Pediatric Research

• Publication date

  2006-09-01

• Fields of study

  Biology, Medicine

• Identifiers
  DOI 10.1203/01.pdr.0000233037.00707.daPMID 16857766
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

• Intra-Articular Enzyme Administration for Joint Disease in Feline Mucopolysaccharidosis VI: Enzyme Dose and Interval

  2006cited by this paper
• Studies on Glucosaminidase

  2005cited by this paper
• Imino sugar inhibitors for treating the lysosomal glycosphingolipidoses.

  2005cited by this paper
• Transgenic expression of mammalian heparanase uncovers physiological functions of heparan sulfate in tissue morphogenesis, vascularization, and feeding behavior

  2004cited by this paper
• Intrathecal enzyme replacement therapy reduces lysosomal storage in the brain and meninges of the canine model of MPS I.

  2004cited by this paper
• Enzyme-Replacement Therapy from Birth Delays the Development of Behavior and Learning Problems in Mucopolysaccharidosis Type IIIA Mice

  2004cited by this paper
• Enzyme replacement therapy for mucopolysaccharidosis I: a randomized, double-blinded, placebo-controlled, multinational study of recombinant human alpha-L-iduronidase (laronidase).

  2004cited by this paper
• Lipids in Health and Disease

  2004cited by this paper
• Gene therapy for lysosomal storage diseases: the lessons and promise of animal models

  2004cited by this paper
• Enzyme replacement therapy in mucopolysaccharidosis VI (Maroteaux-Lamy syndrome).

  2004cited by this paper
• Gaucher disease and the clinical experience with substrate reduction therapy.

  2003influential reference
• The effects of genistein on the synthesis and distribution of glycosaminoglycans/proteoglycans by two osteosarcoma cell lines depends on tyrosine kinase and the estrogen receptor density.

  2003cited by this paper
• Substrate reduction therapy: clinical evaluation in type 1 Gaucher disease.

  2003influential reference
• Enzyme replacement therapy in mucopolysaccharidosis type II (Hunter syndrome): a preliminary report

  2002cited by this paper
• Stem cell bone marrow transplantation in patients with metabolic storage diseases.

  2002cited by this paper
• A novel missense mutation in lysosomal sulfamidase is the basis of MPS III A in a spontaneous mouse mutant.

  2001cited by this paper
• Enhanced survival in Sandhoff disease mice receiving a combination of substrate deprivation therapy and bone marrow transplantation.

  2001cited by this paper
• Transgenic expression of syndecan-1 uncovers a physiological control of feeding behavior by syndecan-3.

  2001cited by this paper
• The Metabolic and Molecular Bases of Inherited Disease

  2001influential reference
• Synthesis of 4-deoxy-4-fluoro analogues of 2-acetamido-2-deoxy-D-glucose and 2-acetamido-2-deoxy-D-galactose and their effects on cellular glycosaminoglycan biosynthesis.

  2000cited by this paper
• Stemming the tide: glycosphingolipid synthesis inhibitors as therapy for storage diseases.

  2000cited by this paper
• Synthesis and sorting of proteoglycans.

  2000cited by this paper
• Novel oral treatment of Gaucher's disease with N-butyldeoxynojirimycin (OGT 918) to decrease substrate biosynthesis.

  2000cited by this paper
• Cloning of mammalian heparanase, an important enzyme in tumor invasion and metastasis

  1999cited by this paper
• Delayed symptom onset and increased life expectancy in Sandhoff disease mice treated with N-butyldeoxynojirimycin.

  1999cited by this paper
• Glycosaminoglycan accumulation and excretion in the mucopolysaccharidoses: characterization and basis of a diagnostic test for MPS.

  1998cited by this paper
• CNS Drug Design Based on Principles of Blood‐Brain Barrier Transport

  1998cited by this paper
• Hurler syndrome: II. Outcome of HLA-genotypically identical sibling and HLA-haploidentical related donor bone marrow transplantation in fifty-four children. The Storage Disease Collaborative Study Group.

  1998influential reference
• Recombinant caprine 3H-[N-Acetylglucosamine-6-Sulfatase] and human 3H-[N-Acetylgalactosamine-4-Sulfatase]

  1998cited by this paper
• Genotype-phenotype correlations in mucopolysaccharidosis type I using enzyme kinetics, immunoquantification and in vitro turnover studies.

  1998cited by this paper
• The syndecan family of proteoglycans. Novel receptors mediating internalization of atherogenic lipoproteins in vitro.

  1997cited by this paper
• Prevention of lysosomal storage in Tay-Sachs mice treated with N-butyldeoxynojirimycin.

  1997cited by this paper
• Genistein selectively inhibits platelet-derived growth factor-stimulated versican biosynthesis in monkey arterial smooth muscle cells.

  1997cited by this paper
• Segments in the C-terminal Folding Domain of Lipoprotein Lipase Important for Binding to the Low Density Lipoprotein Receptor-related Protein and to Heparan Sulfate Proteoglycans*

  1997cited by this paper
• Rhodamine B inhibition of glycosaminoglycan production by cultured human lip fibroblasts.

  1991cited by this paper
• Influence of chlorate on proteoglycan biosynthesis by cultured human fibroblasts.

  1988cited by this paper
• Acute exposure to rhodamine B.

  1987cited by this paper
• A rapid and sensitive method for the quantitation of microgram quantities of protein utilizing the principle of protein-dye binding.

  1976cited by this paper
• New method for quantitative determination of uronic acids.

  1973cited by this paper
• Recovery of inter-block information when block sizes are unequal

  1971influential reference
• Studies on glucosaminidase. 4. The fluorimetric assay of N-acetyl-beta-glucosaminidase.

  1961cited by this paper

• mRNA Degradation as a Therapeutic Solution for Mucopolysaccharidosis Type IIIC: Use of Antisense Oligonucleotides to Promote Downregulation of Heparan Sulfate Synthesis

  2025cites this paper
• Substrate reduction using a glucosamine analogue in Drosophila melanogaster and mouse models of Sanfilippo syndrome.

  2025cites this paper
• Substrate reduction therapy in a Drosophila melanogaster model of Sanfilippo syndrome

  2023cites this paper
• Ocular perspective of mucopolysaccharidosis

  2023cites this paper
• Mucopolysaccharidosis

  2023cites this paper
• Mucopolysaccharidosis: A broad review

  2022influential citation
• Mucopolysaccharidoses: Cellular Consequences of Glycosaminoglycans Accumulation and Potential Targets

  2022cites this paper
• Cell Surface Glycosaminoglycans As Receptors for Adhesion of Candida Spp. To Corneal Cells

  2022cites this paper
• Differences in gene expression patterns, revealed by RNA-seq analysis, between various Sanfilippo and Morquio disease subtypes.

  2021cites this paper
• MPS I: Early diagnosis, bone disease and treatment, where are we now?

  2021influential citation
• Management of Corneal Clouding in Patients with Mucopolysaccharidosis

  2021cites this paper
• Failures of Endochondral Ossification in the Mucopolysaccharidoses

  2020cites this paper
• Comparative analysis of brain pathology in heparan sulphate storing mucopolysaccharidoses.

  2020cites this paper
• Novel therapies for mucopolysaccharidosis type III

  2020cites this paper
• Hurdles in treating Hurler disease: potential routes to achieve a "real" cure.

  2020cites this paper
• metabolites

  2020cites this paper
• Improving diagnosis and treatment outcomes in mucopolysaccharidoses

  2019cites this paper
• Current and Emerging Therapies for Mucopolysaccharidoses.

  2019cites this paper
• Therapeutic Options for Mucopolysaccharidoses: Current and Emerging Treatments

  2019cites this paper
• The Neurogenic potential of stem cells is altered in Mucopolysaccharidosis type IIIA

  2019cites this paper
• Comparative Quantification Method for Glycosylated Products Elongated on β-Xylosides Using a Stable Isotope-Labeled Saccharide Primer.

  2018cites this paper
• Innovative Treatments for Mucopolysaccharidoses

  2018cites this paper
• Emerging Therapies For the Treatment of Mucopolysaccharidosis Type III

  2018cites this paper
• Mise au point et validation de méthode du dosage de la N-acétylGlucosaminidase (NAGLU) sur leucocytes et sur goutte de sang séchée : diagnostic de la maladie de Sanfilippo type B

  2018influential citation
• Prevention of Neurocognitive Deficiency in Mucopolysaccharidosis Type II Mice by Central Nervous System–Directed, AAV9-Mediated Iduronate Sulfatase Gene Transfer

  2017cites this paper
• Substrate Deprivation Therapy to Reduce Glycosaminoglycan Synthesis Improves Aspects of Neurological and Skeletal Pathology in MPS I Mice

  2017cites this paper
• Treatment of brain disease in the mucopolysaccharidoses.

  2017cites this paper
• Small molecules as therapeutic agents for inborn errors of metabolism

  2017cites this paper
• Drug‐Mediated Regulation of Glycosaminoglycan Biosynthesis

  2017cites this paper
• Glycosaminoglycans are involved in bacterial adherence to lung cells

  2017cites this paper
• Glycosaminoglycans and mucopolysaccharidosis type III.

  2016cites this paper
• Reversal of established bone pathology in MPS VII mice following lentiviral-mediated gene therapy.

  2016cites this paper
• Different Use of Cell Surface Glycosaminoglycans As Adherence Receptors to Corneal Cells by Gram Positive and Gram Negative Pathogens

  2016cites this paper
• Less Is More: Substrate Reduction Therapy for Lysosomal Storage Disorders

  2016cites this paper
• N-butyldeoxynojirimycin treatment restores the innate fear response and improves learning in mucopolysaccharidosis IIIA mice.

  2016cites this paper
• Carbohydrate-Processing Enzymes of the Lysosome: Diseases Caused by Misfolded Mutants and Sugar Mimetics as Correcting Pharmacological Chaperones.

  2016cites this paper
• Characterization of a murine model of mucopolysaccharidosis type IIID: A knockout model

  2016cites this paper
• Drugs affecting glycosaminoglycan metabolism.

  2016cites this paper
• Emerging drugs for the treatment of mucopolysaccharidoses

  2016cites this paper
• Sanfilippo syndrome , is an inherited Glycosaminoglycans and mucopolysaccharidosis type III

  2016cites this paper
• Molecular Therapy and Gene Therapy for Hurler Syndrome A THESIS SUBMITTED TO THE FACULTY OF UNIVERSITY OF MINNESOTA BY Li Ou IN PARTIAL FULFILLMENT OF THE REQUIREMENTS FOR THE DEGREE OF DOCTOR OF PHILOSOPHY

  2015cites this paper
• Nanotechnologies for CNS drug delivery: therapy for the neurological compartment in the Mucopolisaccharidoses

  2015cites this paper
• Hematopoietic Stem Cell Therapy and Novel Approaches for Mucopolysaccharidoses

  2015cites this paper
• Current and potential therapeutic strategies for mucopolysaccharidoses

  2014cites this paper
• Mucopolysaccharidosis III (Sanfilippo Syndrome)- disease presentation and experimental therapies.

  2014cites this paper
• Lentiviral-Mediated Gene Therapy Results in Sustained Expression of β-Glucuronidase for up to 12 Months in the Gus mps/mps and up to 18 Months in the Gus tm(L175F)Sly Mouse Models of Mucopolysaccharidosis Type VII

  2014cites this paper
• [Principles of therapeutic approaches for mucopolysaccharidoses].

  2014cites this paper
• Distribution of Heparan Sulfate Oligosaccharides in Murine Mucopolysaccharidosis Type IIIA

  2014cites this paper
• Molecular, Biochemical and Functional Studies in Genes Determining Missorting of Lysosomal Proteins

  2013cites this paper
• Developing a Potential Substrate Reduction Therapy for Six Mucopolysaccharidoses by Decreasing NDST1 Activity

  2013cites this paper
• Factors and processes modulating phenotypes in neuronopathic lysosomal storage diseases

  2013cites this paper
• New strategies for the treatment of lysosomal storage diseases (review).

  2013cites this paper
• Emerging treatment options for the mucopolysaccharidoses

  2012cites this paper
• Skeletal response to lentiviral mediated gene therapy in a mouse model of MPS VII.

  2012cites this paper
• Synthetic genistein derivatives as modulators of glycosaminoglycan storage

  2012cites this paper
• Gene expression‐targeted isoflavone therapy

  2012cites this paper
• Emerging treatment options for the mucopolysaccharidoses

  2012cites this paper
• Putative Biological Mechanisms of Efficiency of Substrate Reduction Therapies for Mucopolysaccharidoses

  2012cites this paper
• Correction of Pathological Accumulation of Glycosaminoglycans in Central Nervous System and Peripheral Tissues of MPSIIIA Mice Through Systemic AAV9 Gene Transfer

  2012cites this paper
• A Genetic Model of Substrate Reduction Therapy for Mucopolysaccharidosis*

  2012cites this paper
• Rhodamine B and 2-acetamido-1,3,6-tri-O-acetyl-4-deoxy-4-fluoro-D-glucopyranose (F-GlcNAc) inhibit chondroitin/dermatan and keratan sulphate synthesis by different mechanisms in bovine chondrocytes.

  2012cites this paper
• Endocrine Disorders Emerging treatment options for the mucopolysaccharidoses

  2012cites this paper
• Developing drugs from sugars: current state of glycosaminoglycan-derived therapeutics

  2012cites this paper
• Differential distribution of heparan sulfate glycoforms and elevated expression of heparan sulfate biosynthetic enzyme genes in the brain of mucopolysaccharidosis IIIB mice

  2011cites this paper
• Glycosaminoglycan Storage Disorders: A Review

  2011cites this paper
• Effects of flavonoids on glycosaminoglycan synthesis: implications for substrate reduction therapy in Sanfilippo disease and other mucopolysaccharidoses

  2011cites this paper
• Therapies for neurological disease in the mucopolysaccharidoses.

  2011cites this paper
• Two-year follow-up of Sanfilippo Disease patients treated with a genistein-rich isoflavone extract: Assessment of effects on cognitive functions and general status of patients

  2011cites this paper
• Recent developments in therapeutic approaches for lysosomal storage diseases.

  2011cites this paper
• Pompe disease: from new views on pathophysiology to innovative therapeutic strategies.

  2011cites this paper
• Mouse mutagenesis and disease models for neuropsychiatric disorders.

  2011cites this paper
• Substrate reduction therapies for mucopolysaccharidoses.

  2011cites this paper
• Mukopolisacharydozy — biochemiczne mechanizmy chorób oraz możliwości terapeutyczne

  2011cites this paper
• Inhibitory Peptides of the Sulfotransferase Domain of the Heparan Sulfate Enzyme, N-Deacetylase-N-sulfotransferase-1

  2010cites this paper
• Impairment of glycosaminoglycan synthesis in mucopolysaccharidosis type IIIA cells by using siRNA: a potential therapeutic approach for Sanfilippo disease

  2010cites this paper
• Gene silencing of EXTL2 and EXTL3 as a substrate deprivation therapy for heparan sulphate storing mucopolysaccharidoses

  2010cites this paper
• Substrate deprivation therapy: a new hope for patients suffering from neuronopathic forms of inherited lysosomal storage diseases

  2010cites this paper
• Correction of mucopolysaccharidosis type IIIA somatic and central nervous system pathology by lentiviral‐mediated gene transfer

  2010cites this paper
• Genistein reduces glycosaminoglycan levels in a mouse model of mucopolysaccharidosis type II

  2010cites this paper
• Genistein as an experimental drug in Sanfilippo disease ( mucopolysaccharidosis type III ) and a potential therapeutic agent for other LSDs ( lysosomal storage diseases )

  2010cites this paper
• Galsulfase: enzyme therapy for mucopolysaccharidosis type VI (Maroteaux-Lamy syndrome)

  2010cites this paper
• Genistein: a natural isoflavone with a potential for treatment of genetic diseases.

  2010cites this paper
• RESEARCH PAPER Genistein reduces glycosaminoglycan levels in a mouse model of mucopolysaccharidosis type II

  2010cites this paper
• Trans-generational exposure to low levels of rhodamine B does not adversely affect litter size or liver function in murine mucopolysaccharidosis type IIIA.

  2010influential citation
• Genistein reduces lysosomal storage in peripheral tissues of mucopolysaccharide IIIB mice.

  2009cites this paper
• 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

  2009cites this paper
• Gastrointestinal pathology in a mouse model of mucopolysaccharidosis type IIIA

  2009cites this paper
• Abnormalities in the hair morphology of patients with some but not all types of mucopolysaccharidoses

  2008cites this paper
• Sanfilippo syndrome: A mini-review

  2008cites this paper
• Genistin-rich soy isoflavone extract in substrate reduction therapy for Sanfilippo syndrome: An open-label, pilot study in 10 pediatric patients.

  2008cites this paper
• Genetic Vaccines and Therapy

  2007cites this paper
• Glycan Antagonists and Inhibitors: A Fount for Drug Discovery

  2007cites this paper
• Improvement in behaviour after substrate deprivation therapy with rhodamine B in a mouse model of MPS IIIA.

  2007cites this paper
• Substrate deprivation as a novel therapy for the mucopolysaccharidoses.

  2007cites this paper
• The Application of Clinical Genetics Dovepress Sanfilippo Syndrome: Causes, Consequences, and Treatments

  year unknowncites this paper