Secondary storage of dermatan sulfate in Sanfilippo disease.
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The cell biology of disease: lysosomal storage disorders: the cellular impact of lysosomal dysfunctionImpairment of homeostasis in lysosomal storage disordersNeurodevelopmental Changes in Excitatory Synaptic Structure and Function in the Cerebral Cortex of Sanfilippo Syndrome IIIA MiceInsulin receptor antibody-sulfamidase fusion protein penetrates the primate blood-brain barrier and reduces glycosoaminoglycans in Sanfilippo type A cells.Disease-specific non-reducing end carbohydrate biomarkers for mucopolysaccharidoses.A Multiplex Assay for the Diagnosis of Mucopolysaccharidoses and MucolipidosesArylsulfatase G inactivation causes loss of heparan sulfate 3-O-sulfatase activity and mucopolysaccharidosis in mice.A genetic model of substrate reduction therapy for mucopolysaccharidosis.Glycan-based biomarkers for mucopolysaccharidoses.Cardiac disease as the presenting feature of mucopolysaccharidosis type IIIA: A case report.Glycosaminoglycan levels and structure in a mucopolysaccharidosis IIIA mice and the effect of a highly secreted sulfamidase engineered to cross the blood-brain barrier.Impaired bone remodeling and its correction by combination therapy in a mouse model of mucopolysaccharidosis-I.Perturbations in cell signaling elicit early cardiac defects in mucopolysaccharidosis type II.Mental retardation in mucopolysaccharidoses correlates with high molecular weight urinary heparan sulphate derived glucosamine.Evaluation of enzyme dose and dose-frequency in ameliorating substrate accumulation in MPS IIIA Huntaway dog brain.Lysosomal Proteome and Secretome Analysis Identifies Missorted Enzymes and Their Nondegraded Substrates in Mucolipidosis III Mouse Cells
P2860
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P2860
Secondary storage of dermatan sulfate in Sanfilippo 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
Secondary storage of dermatan sulfate in Sanfilippo disease.
@ast
Secondary storage of dermatan sulfate in Sanfilippo disease.
@en
Secondary storage of dermatan sulfate in Sanfilippo disease.
@nl
type
label
Secondary storage of dermatan sulfate in Sanfilippo disease.
@ast
Secondary storage of dermatan sulfate in Sanfilippo disease.
@en
Secondary storage of dermatan sulfate in Sanfilippo disease.
@nl
prefLabel
Secondary storage of dermatan sulfate in Sanfilippo disease.
@ast
Secondary storage of dermatan sulfate in Sanfilippo disease.
@en
Secondary storage of dermatan sulfate in Sanfilippo disease.
@nl
P2093
P2860
P356
P1476
Secondary storage of dermatan sulfate in Sanfilippo disease.
@en
P2093
Jeffrey D Esko
Roger Lawrence
Stéphane Sarrazin
William C Lamanna
P2860
P304
P356
10.1074/JBC.M110.192062
P407
P577
2010-12-30T00:00:00Z