Innate and adaptive immune activation in the brain of MPS IIIB mouse model.
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Neuroinflammation, mitochondrial defects and neurodegeneration in mucopolysaccharidosis III type C mouse model.Genomic expression analyses reveal lysosomal, innate immunity proteins, as disease correlates in murine models of a lysosomal storage disorderImmune-Mediated Inflammation May Contribute to the Pathogenesis of Cardiovascular Disease in Mucopolysaccharidosis Type ICNS-directed gene therapy for the treatment of neurologic and somatic mucopolysaccharidosis type II (Hunter syndrome)Mucopolysaccharidosis IIIB, a lysosomal storage disease, triggers a pathogenic CNS autoimmune responseSanfilippo syndrome type B, a lysosomal storage disease, is also a tauopathyThymic alterations in GM2 gangliosidoses model mice.Brain RNA-Seq Profiling of the Mucopolysaccharidosis Type II Mouse Model.Genistein improves neuropathology and corrects behaviour in a mouse model of neurodegenerative metabolic diseaseTherapeutic efficacy of bone marrow transplant, intracranial AAV-mediated gene therapy, or both in the mouse model of MPS IIIBCommon and uncommon pathogenic cascades in lysosomal storage diseasesDefects in the medial entorhinal cortex and dentate gyrus in the mouse model of Sanfilippo syndrome type B.Dysregulation of gene expression in a lysosomal storage disease varies between brain regions implicating unexpected mechanisms of neuropathology.Neuropathology in mouse models of mucopolysaccharidosis type I, IIIA and IIIBPeripheral nervous system neuropathology and progressive sensory impairments in a mouse model of Mucopolysaccharidosis IIIB.Differential distribution of heparan sulfate glycoforms and elevated expression of heparan sulfate biosynthetic enzyme genes in the brain of mucopolysaccharidosis IIIB mice.Amyloidosis, synucleinopathy, and prion encephalopathy in a neuropathic lysosomal storage disease: the CNS-biomarker potential of peripheral blood.Neuroinflammatory paradigms in lysosomal storage diseases.Diffusion tensor imaging and myelin composition analysis reveal abnormal myelination in corpus callosum of canine mucopolysaccharidosis I.A GLP-Compliant Toxicology and Biodistribution Study: Systemic Delivery of an rAAV9 Vector for the Treatment of Mucopolysaccharidosis IIIB.Hyaluronan, a crucial regulator of inflammation.Clinical neurogenetics: neuropathic lysosomal storage disorders.Mucopolysaccharide diseases: a complex interplay between neuroinflammation, microglial activation and adaptive immunity.Lysosomal storage diseases--the horizon expands.Activation of stress kinases in the brain of mucopolysaccharidosis IIIB mice.Neuroinflammation as modifier of genetically caused neurological disorders of the central nervous system: Understanding pathogenesis and chances for treatment.Serum global metabolomics profiling reveals profound metabolic impairments in patients with MPS IIIA and MPS IIIB.Effective Depletion of Pre-existing Anti-AAV Antibodies Requires Broad Immune TargetingNear-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.Biochemical, histological and functional correction of mucopolysaccharidosis type IIIB by intra-cerebrospinal fluid gene therapy.Correction of murine mucopolysaccharidosis type IIIA central nervous system pathology by intracerebroventricular lentiviral-mediated gene delivery.Feasibility and safety of systemic rAAV9-hNAGLU delivery for treating mucopolysaccharidosis IIIB: toxicology, biodistribution, and immunological assessments in primates.Macrophage enzyme and reduced inflammation drive brain correction of mucopolysaccharidosis IIIB by stem cell gene therapy.Unfolded protein response is not activated in the mucopolysaccharidoses but protein disulfide isomerase 5 is deregulated.Hyperactivity, unexplained speech delay, and coarse facies--is it Sanfilippo syndrome?
P2860
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P2860
Innate and adaptive immune activation in the brain of MPS IIIB mouse model.
description
2009 nî lūn-bûn
@nan
2009年の論文
@ja
2009年論文
@yue
2009年論文
@zh-hant
2009年論文
@zh-hk
2009年論文
@zh-mo
2009年論文
@zh-tw
2009年论文
@wuu
2009年论文
@zh
2009年论文
@zh-cn
name
Innate and adaptive immune activation in the brain of MPS IIIB mouse model.
@en
type
label
Innate and adaptive immune activation in the brain of MPS IIIB mouse model.
@en
prefLabel
Innate and adaptive immune activation in the brain of MPS IIIB mouse model.
@en
P2093
P356
P1476
Innate and adaptive immune activation in the brain of MPS IIIB mouse model
@en
P2093
Alyssa Charrier
Chuansong Wang
David L Newsom
Douglas M McCarty
Erin Divers
Herbert Auer
Jonathan Etter
Julianne DiRosario
Victoria Best
P304
P356
10.1002/JNR.21912
P577
2009-03-01T00:00:00Z