Differential distribution of heparan sulfate glycoforms and elevated expression of heparan sulfate biosynthetic enzyme genes in the brain of mucopolysaccharidosis IIIB mice.
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NDST1 Preferred Promoter Confirmation and Identification of Corresponding Transcriptional Inhibitors as Substrate Reduction Agents for Multiple Mucopolysaccharidosis DisordersSialic acid deposition impairs the utility of AAV9, but not peptide-modified AAVs for brain gene therapy in a mouse model of lysosomal storage diseasePeripheral nervous system neuropathology and progressive sensory impairments in a mouse model of Mucopolysaccharidosis IIIB.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.Biochemical, histological and functional correction of mucopolysaccharidosis type IIIB by intra-cerebrospinal fluid gene therapy.Abnormal polyamine metabolism is unique to the neuropathic forms of MPS: potential for biomarker development and insight into pathogenesis.EGFR activation triggers cellular hypertrophy and lysosomal disease in NAGLU-depleted cardiomyoblasts, mimicking the hallmarks of mucopolysaccharidosis IIIB.
P2860
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P2860
Differential distribution of heparan sulfate glycoforms and elevated expression of heparan sulfate biosynthetic enzyme genes in the brain of mucopolysaccharidosis IIIB mice.
description
2011 nî lūn-bûn
@nan
2011 թուականի Յունուարին հրատարակուած գիտական յօդուած
@hyw
2011 թվականի հունվարին հրատարակված գիտական հոդված
@hy
2011年の論文
@ja
2011年論文
@yue
2011年論文
@zh-hant
2011年論文
@zh-hk
2011年論文
@zh-mo
2011年論文
@zh-tw
2011年论文
@wuu
name
Differential distribution of h ...... copolysaccharidosis IIIB mice.
@ast
Differential distribution of h ...... copolysaccharidosis IIIB mice.
@en
Differential distribution of h ...... copolysaccharidosis IIIB mice.
@nl
type
label
Differential distribution of h ...... copolysaccharidosis IIIB mice.
@ast
Differential distribution of h ...... copolysaccharidosis IIIB mice.
@en
Differential distribution of h ...... copolysaccharidosis IIIB mice.
@nl
prefLabel
Differential distribution of h ...... copolysaccharidosis IIIB mice.
@ast
Differential distribution of h ...... copolysaccharidosis IIIB mice.
@en
Differential distribution of h ...... copolysaccharidosis IIIB mice.
@nl
P2093
P2860
P1476
Differential distribution of h ...... copolysaccharidosis IIIB mice.
@en
P2093
Arie Oosterhof
Douglas M McCarty
Julianne DiRosario
Kadra Gulaid
Paul T Martin
Smruti Killedar
Toin H van Kuppevelt
P2860
P2888
P356
10.1007/S11011-010-9230-X
P577
2011-01-12T00:00:00Z
P5875
P6179
1014538975