Production of MPS VII mouse (Gus(tm(hE540A x mE536A)Sly)) doubly tolerant to human and mouse beta-glucuronidase - Wikidata - wikimedia - TriplyDB

Production of MPS VII mouse (Gus(tm(hE540A x mE536A)Sly)) doubly tolerant to human and mouse beta-glucuronidase

Production of MPS VII mouse (Gus(tm(hE540A x mE536A)Sly)) doubly tolerant to human and mouse beta-glucuronidase

http://www.wikidata.org/entity/Q35032587

about

A novel GUSB mutation in Brazilian terriers with severe skeletal abnormalities defines the disease as mucopolysaccharidosis VII Enzyme replacement therapy on hypophosphatasia mouse model.Enhancement of drug delivery: enzyme-replacement therapy for murine Morquio A syndrome.Mutations and polymorphisms in GUSB gene in mucopolysaccharidosis VII (Sly Syndrome).Contribution of the H63D mutation in HFE to murine hereditary hemochromatosis.Corrective GUSB transfer to the canine mucopolysaccharidosis VII cornea using a helper-dependent canine adenovirus vector.Chronic enzyme replacement therapy ameliorates neuropathology in alpha-mannosidosis mice Corrective GUSB transfer to the canine mucopolysaccharidosis VII brain.Induction of tolerance to human arylsulfatase A in a mouse model of metachromatic leukodystrophy.

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P2860

Production of MPS VII mouse (Gus(tm(hE540A x mE536A)Sly)) doubly tolerant to human and mouse beta-glucuronidase

http://www.wikidata.org/entity/Q35032587

description

2003 nî lūn-bûn

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2003 թուականի Մայիսին հրատարակուած գիտական յօդուած

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2003 թվականի մայիսին հրատարակված գիտական հոդված

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2003年の論文

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2003年論文

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2003年論文

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2003年論文

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2003年論文

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2003年論文

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2003年论文

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name

Production of MPS VII mouse (G ...... n and mouse beta-glucuronidase

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Production of MPS VII mouse (G ...... n and mouse beta-glucuronidase

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Production of MPS VII mouse (G ...... n and mouse beta-glucuronidase

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Production of MPS VII mouse (G ...... n and mouse beta-glucuronidase

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Production of MPS VII mouse (G ...... n and mouse beta-glucuronidase

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Human Molecular Genetics

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Production of MPS VII mouse (G ...... n and mouse beta-glucuronidase

@en

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Carole Vogler

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Christopher C Holden

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Jeffrey H Grubb

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Kazuko Sukegawa

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Koji O Orii

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Monica Gutierrez

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Naomi Kondo

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Shunji Tomatsu

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Tadao Orii

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Tatiana Dieter

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P2860

Murine mucopolysaccharidosis type VII. Characterization of a mouse with beta-glucuronidase deficiency

Mouse model of Sanfilippo syndrome type B produced by targeted disruption of the gene encoding alpha-N-acetylglucosaminidase

Mucopolysaccharidosis type VII: characterization of mutations and molecular heterogeneity

Mutational analysis of a patient with mucopolysaccharidosis type VII, and identification of pseudogenes

Molecular analysis of patients with beta-glucuronidase deficiency presenting as hydrops fetalis or as early mucopolysaccharidosis VII

Structure of human beta-glucuronidase reveals candidate lysosomal targeting and active-site motifs

Reversal of pathology in murine mucopolysaccharidosis type VII by somatic cell gene transfer

Molecular basis of mucopolysaccharidosis type VII: replacement of Ala619 in beta-glucuronidase with Val.

Active site residues of human beta-glucuronidase. Evidence for Glu(540) as the nucleophile and Glu(451) as the acid-base residue.

Phenotype correction in retinal pigment epithelium in murine mucopolysaccharidosis VII by adenovirus-mediated gene transfer.

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961-973

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10.1093/HMG/DDG119

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9

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