(Q47594976)

4 February 2018

title

Beta-galactosidase deficiency: an approach to chaperone therapy (English)

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4 February 2018

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Yoshiyuki Suzuki

1

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4 February 2018

publication date

1 April 2006

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4 February 2018

Journal of Inherited Metabolic Disease

published in

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4 February 2018

volume

29

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issue

2-3

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page(s)

471-476

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4 February 2018

https://scigraph.springernature.com/pub.10.1007/s10545-006-0287-y

exact match

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cites work

Amniotic tissue transplantation as a trial of treatment in some lysosomal storage diseases

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12 December 2020

N-octyl-beta-valienamine up-regulates activity of F213I mutant beta-glucosidase in cultured cells: a potential chemical chaperone therapy for Gaucher disease

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12 December 2020

Characterization of beta-galactosidase mutations Asp332-->Asn and Arg148-->Ser, and a polymorphism, Ser532-->Gly, in a case of GM1 gangliosidosis

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12 December 2020

Galactose stabilizes various missense mutants of alpha-galactosidase in Fabry disease

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12 December 2020

Chemical chaperone therapy for brain pathology in G(M1)-gangliosidosis

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12 December 2020

Structural features of normal and mutant human lysosomal glycoside hydrolases deduced from bioinformatics analysis

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12 December 2020

Chemical modification of the beta-glucocerebrosidase inhibitor N-octyl-beta-valienamine: synthesis and biological evaluation of 4-epimeric and 4-O-(beta-D-galactopyranosyl) derivatives

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12 December 2020

Improvement in cardiac function in the cardiac variant of Fabry's disease with galactose-infusion therapy

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12 December 2020

Alpha-galactosidase gene mutations in Fabry disease: heterogeneous expressions of mutant enzyme proteins

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12 December 2020

Development of lysosomal storage in mice with targeted disruption of the beta-galactosidase gene: a model of human G(M1)-gangliosidosis.

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12 December 2020

Chemical modification of beta-glucocerebrosidase inhibitor N-octyl-beta-valienamine: synthesis and biological evaluation of N-alkanoyl and N-alkyl derivatives

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12 December 2020

Convenient synthesis and evaluation of glycosidase inhibitory activity of alpha- and beta-galactose-type valienamines, and some N-alkyl derivatives

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12 December 2020

Beta-galactosidase-deficient mouse as an animal model for GM1-gangliosidosis

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12 December 2020

Accelerated transport and maturation of lysosomal α–galactosidase A in Fabry lymphoblasts by an enzyme inhibitor

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12 December 2020

Substrate reduction reduces gangliosides in postnatal cerebrum-brainstem and cerebellum in GM1 gangliosidosis mice.

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12 December 2020

Galactonojirimycin derivatives restore mutant human beta-galactosidase activities expressed in fibroblasts from enzyme-deficient knockout mouse

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12 December 2020

Human glucocerebrosidase: heterologous expression of active site mutants in murine null cells

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12 December 2020

Aggregation of the inactive form of human alpha-galactosidase in the endoplasmic reticulum

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12 December 2020

N-butyldeoxygalactonojirimycin reduces neonatal brain ganglioside content in a mouse model of GM1 gangliosidosis

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12 December 2020

Bone marrow transplantation in canine GM1 gangliosidosis

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12 December 2020

10.1007/S10545-006-0287-Y

Identifiers

DOI

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4 February 2018

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4 February 2018