Genistein improves neuropathology and corrects behaviour in a mouse model of neurodegenerative metabolic disease
about
Sanfilippo syndrome: causes, consequences, and treatmentsAtaxia is the major neuropathological finding in arylsulfatase G-deficient mice: similarities and dissimilarities to Sanfilippo disease (mucopolysaccharidosis type III).Female mucopolysaccharidosis IIIA mice exhibit hyperactivity and a reduced sense of danger in the open field testHematopoietic stem cell and gene therapy corrects primary neuropathology and behavior in mucopolysaccharidosis IIIA mice.Myeloid/Microglial driven autologous hematopoietic stem cell gene therapy corrects a neuronopathic lysosomal diseaseThe phytoestrogen genistein modulates lysosomal metabolism and transcription factor EB (TFEB) activationRecommendations on clinical trial design for treatment of Mucopolysaccharidosis Type III.Defects in the medial entorhinal cortex and dentate gyrus in the mouse model of Sanfilippo syndrome type B.Neuropathology in mouse models of mucopolysaccharidosis type I, IIIA and IIIBEffects of flavonoids on glycosaminoglycan synthesis: implications for substrate reduction therapy in Sanfilippo disease and other mucopolysaccharidoses.Cognitive development in patients with Mucopolysaccharidosis type III (Sanfilippo syndrome).Modulation of expression of genes involved in glycosaminoglycan metabolism and lysosome biogenesis by flavonoids.Estrogen receptor agonists for attenuation of neuroinflammation and neurodegeneration.Bilateral single-site intracerebral injection of a nonpathogenic herpes simplex virus-1 vector decreases anxiogenic behavior in MPS VII mice.Adverse Effects of Genistein in a Mucopolysaccharidosis Type I Mouse ModelSynthetic genistein derivatives as modulators of glycosaminoglycan storage.A genetic model of substrate reduction therapy for mucopolysaccharidosis.The use of elevated doses of genistein-rich soy extract in the gene expression-targeted isoflavone therapy for Sanfilippo disease patients.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 patientsMucopolysaccharidosis type III (Sanfilippo syndrome) and misdiagnosis of idiopathic developmental delay, attention deficit/hyperactivity disorder or autism spectrum disorder.Residual N-acetyl-α-glucosaminidase activity in fibroblasts correlates with disease severity in patients with mucopolysaccharidosis type IIIBGlycan-based biomarkers for mucopolysaccharidoses.The natural dietary genistein boosts bacteriophage-mediated cancer cell killing by improving phage-targeted tumor cell transduction.Factors and processes modulating phenotypes in neuronopathic lysosomal storage diseases.Emerging therapies for neurodegenerative lysosomal storage disorders - from concept to reality.Gene expression-targeted isoflavone therapy.Putative biological mechanisms of efficiency of substrate reduction therapies for mucopolysaccharidoses.Treatment options for lysosomal storage disorders: developing insights.Blood-brain barrier structure and function and the challenges for CNS drug delivery.Current and potential therapeutic strategies for mucopolysaccharidoses.Emerging drugs for the treatment of mucopolysaccharidoses.Genistein increases glycosaminoglycan levels in mucopolysaccharidosis type I cell models.Drug-Mediated Regulation of Glycosaminoglycan Biosynthesis.Beta amyloid peptide (25-35) leading to inflammation through Toll-like receptors and the anti-inflammatory effect of genistein in BV-2 cells.Busulfan conditioning enhances engraftment of hematopoietic donor-derived cells in the brain compared with irradiation.Substrate Deprivation Therapy to Reduce Glycosaminoglycan Synthesis Improves Aspects of Neurological and Skeletal Pathology in MPS I Mice.Coutinho et al. Less Is More: Substrate Reduction Therapy for Lysosomal Storage Disorders. Int. J. Mol. Sci. 2016, 17, 1065.Hyperactive behaviour in the mouse model of mucopolysaccharidosis IIIB in the open field and home cage environments.How close are we to therapies for Sanfilippo disease?Macrophage enzyme and reduced inflammation drive brain correction of mucopolysaccharidosis IIIB by stem cell gene therapy.
P2860
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P2860
Genistein improves neuropathology and corrects behaviour in a mouse model of neurodegenerative metabolic disease
description
2010 nî lūn-bûn
@nan
2010 թուականի Դեկտեմբերին հրատարակուած գիտական յօդուած
@hyw
2010 թվականի դեկտեմբերին հրատարակված գիտական հոդված
@hy
2010年の論文
@ja
2010年論文
@yue
2010年論文
@zh-hant
2010年論文
@zh-hk
2010年論文
@zh-mo
2010年論文
@zh-tw
2010年论文
@wuu
name
Genistein improves neuropathol ...... degenerative metabolic disease
@ast
Genistein improves neuropathol ...... degenerative metabolic disease
@en
type
label
Genistein improves neuropathol ...... degenerative metabolic disease
@ast
Genistein improves neuropathol ...... degenerative metabolic disease
@en
prefLabel
Genistein improves neuropathol ...... degenerative metabolic disease
@ast
Genistein improves neuropathol ...... degenerative metabolic disease
@en
P2093
P2860
P50
P1433
P1476
Genistein improves neuropathol ...... degenerative metabolic disease
@en
P2093
Brett E Crawford
Fiona L Wilkinson
Grzegorz Grynkiewicz
J Ed Wraith
Jillian R Brown
Kia J Langford-Smith
Marie T Vanier
Rob F Wynn
P2860
P304
P356
10.1371/JOURNAL.PONE.0014192
P407
P577
2010-12-01T00:00:00Z