Attenuated plasticity in neurons and astrocytes in the mouse model of Sanfilippo syndrome type B.
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Early neurodegeneration progresses independently of microglial activation by heparan sulfate in the brain of mucopolysaccharidosis IIIB miceDevelopment of sensory, motor and behavioral deficits in the murine model of Sanfilippo syndrome type BA rapid and sensitive method for measuring N-acetylglucosaminidase activity in cultured cellsMucopolysaccharidosis IIIB, a lysosomal storage disease, triggers a pathogenic CNS autoimmune responseSanfilippo syndrome type B, a lysosomal storage disease, is also a tauopathyBlood-brain barrier impairment in an animal model of MPS III BTherapeutic efficacy of bone marrow transplant, intracranial AAV-mediated gene therapy, or both in the mouse model of MPS IIIBDefects in the medial entorhinal cortex and dentate gyrus in the mouse model of Sanfilippo syndrome type B.Differential distribution of heparan sulfate glycoforms and elevated expression of heparan sulfate biosynthetic enzyme genes in the brain of mucopolysaccharidosis IIIB mice.Activated microglia in cortex of mouse models of mucopolysaccharidoses I and IIIBAmyloidosis, synucleinopathy, and prion encephalopathy in a neuropathic lysosomal storage disease: the CNS-biomarker potential of peripheral blood.Electrophysiological and Histological Characterization of Rod-Cone Retinal Degeneration and Microglia Activation in a Mouse Model of Mucopolysaccharidosis Type IIIB.A genetic model of substrate reduction therapy for mucopolysaccharidosis.A GLP-Compliant Toxicology and Biodistribution Study: Systemic Delivery of an rAAV9 Vector for the Treatment of Mucopolysaccharidosis IIIB.Mucopolysaccharidosis type I: current knowledge on its pathophysiological mechanisms.Activation of stress kinases in the brain of mucopolysaccharidosis IIIB mice.Serum global metabolomics profiling reveals profound metabolic impairments in patients with MPS IIIA and MPS IIIB.Near-Complete Correction of Profound Metabolomic Impairments Corresponding to Functional Benefit in MPS IIIB Mice after IV rAAV9-hNAGLU Gene Delivery.Repeated administrations of human umbilical cord blood cells improve disease outcomes in a mouse model of Sanfilippo syndrome type III B.Mannitol-facilitated CNS entry of rAAV2 vector significantly delayed the neurological disease progression in MPS IIIB mice.Correction of neurological disease of mucopolysaccharidosis IIIB in adult mice by rAAV9 trans-blood-brain barrier gene delivery.Functional abnormalities of heparan sulfate in mucopolysaccharidosis-I are associated with defective biologic activity of FGF-2 on human multipotent progenitor cellsFeasibility and safety of systemic rAAV9-hNAGLU delivery for treating mucopolysaccharidosis IIIB: toxicology, biodistribution, and immunological assessments in primates.Abnormal polyamine metabolism is unique to the neuropathic forms of MPS: potential for biomarker development and insight into pathogenesis.Intravenous administration of human umbilical cord blood cells in an animal model of MPS III B.Low-dose, continuous enzyme replacement therapy ameliorates brain pathology in the neurodegenerative lysosomal disorder mucopolysaccharidosis type IIIA.GAP43 overexpression and enhanced neurite outgrowth in mucopolysaccharidosis type IIIB cortical neuron cultures.Intracerebroventricular transplantation of human bone marrow-derived multipotent progenitor cells in an immunodeficient mouse model of mucopolysaccharidosis type I (MPS-I).Unfolded protein response is not activated in the mucopolysaccharidoses but protein disulfide isomerase 5 is deregulated.Canine fucosidosis: a neuroprogressive disorder.
P2860
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P2860
Attenuated plasticity in neurons and astrocytes in the mouse model of Sanfilippo syndrome type B.
description
2002 nî lūn-bûn
@nan
2002年の論文
@ja
2002年学术文章
@wuu
2002年学术文章
@zh-cn
2002年学术文章
@zh-hans
2002年学术文章
@zh-my
2002年学术文章
@zh-sg
2002年學術文章
@yue
2002年學術文章
@zh
2002年學術文章
@zh-hant
name
Attenuated plasticity in neuro ...... of Sanfilippo syndrome type B.
@en
Attenuated plasticity in neuro ...... of Sanfilippo syndrome type B.
@nl
type
label
Attenuated plasticity in neuro ...... of Sanfilippo syndrome type B.
@en
Attenuated plasticity in neuro ...... of Sanfilippo syndrome type B.
@nl
prefLabel
Attenuated plasticity in neuro ...... of Sanfilippo syndrome type B.
@en
Attenuated plasticity in neuro ...... of Sanfilippo syndrome type B.
@nl
P2093
P2860
P356
P1476
Attenuated plasticity in neuro ...... of Sanfilippo syndrome type B.
@en
P2093
Fernando Gómez-Pinilla
Hong Hua Li
Hui-Zhi Zhao
P2860
P356
10.1002/JNR.10278
P577
2002-07-01T00:00:00Z