Standardization of α-L-iduronidase enzyme assay with Michaelis-Menten kinetics.

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Elements of lentiviral vector design toward gene therapy for treating mucopolysaccharidosis I.RTB lectin-mediated delivery of lysosomal α-l-iduronidase mitigates disease manifestations systemically including the central nervous system.

P2860

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P2860

Standardization of α-L-iduronidase enzyme assay with Michaelis-Menten kinetics.

Item

http://www.wikidata.org/entity/Q38170826

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2013 nî lūn-bûn

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2013年の論文

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2013年学术文章

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2013年学术文章

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2013年学术文章

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2013年学术文章

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2013年学术文章

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2013年學術文章

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2013年學術文章

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2013年學術文章

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name

Standardization of α-L-iduronidase enzyme assay with Michaelis-Menten kinetics.

@en

Standardization of α-L-iduronidase enzyme assay with Michaelis-Menten kinetics.

@en-gb

type

Item

label

Standardization of α-L-iduronidase enzyme assay with Michaelis-Menten kinetics.

@en

Standardization of α-L-iduronidase enzyme assay with Michaelis-Menten kinetics.

@en-gb

prefLabel

Standardization of α-L-iduronidase enzyme assay with Michaelis-Menten kinetics.

@en

Standardization of α-L-iduronidase enzyme assay with Michaelis-Menten kinetics.

@en-gb

P1476

Standardization of α-L-iduronidase enzyme assay with Michaelis-Menten kinetics.

@en

P2093

Chester B Whitley

http://www.w3.org/2001/XMLSchema#string

Li Ou

http://www.w3.org/2001/XMLSchema#string

Tyler L Herzog

http://www.w3.org/2001/XMLSchema#string

P2860

Systemic correction of storage disease in MPS I NOD/SCID mice using the sleeping beauty transposon system

Prolonged expression of a lysosomal enzyme in mouse liver after Sleeping Beauty transposon-mediated gene delivery: implications for non-viral gene therapy of mucopolysaccharidoses

Improved retroviral vector design results in sustained expression after adult gene therapy in mucopolysaccharidosis I mice.

A self-inactivating gamma-retroviral vector reduces manifestations of mucopolysaccharidosis I in mice

Gene therapy augments the efficacy of hematopoietic cell transplantation and fully corrects mucopolysaccharidosis type I phenotype in the mouse model.

Lysosomal enzyme can bypass the blood-brain barrier and reach the CNS following intranasal administration.

Characterization of an immunodeficient mouse model of mucopolysaccharidosis type I suitable for preclinical testing of human stem cell and gene therapy.

Direct gene transfer to the CNS prevents emergence of neurologic disease in a murine model of mucopolysaccharidosis type I.

A nonpathologic allele (IW) for low alpha-L-iduronidase enzyme activity vis-a-vis prenatal diagnosis of Hurler syndrome.

A fluorometric assay using 4-methylumbelliferyl alpha-L-iduronide for the estimation of alpha-L-iduronidase activity and the detection of Hurler and Scheie syndromes.

P304

113-115

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scholarly article

P356

10.1016/J.YMGME.2013.11.009

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P478

111

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Carrie M Wilmot

P577

2013-11-26T00:00:00Z

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24332804

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P932

4014300

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Standardization of α-L-iduronidase enzyme assay with Michaelis-Menten kinetics.

about

P2860

P2860

Standardization of α-L-iduronidase enzyme assay with Michaelis-Menten kinetics.

description

name

type

label

prefLabel

P1433

P1476

P2093

P2860

P304

P31

P356

P433

P478

P50

P577

P698

P932

P356

P1433

P1476

P2093

P2860

P304

P31

P356

P433

P478

P50

P577

P698

P932