Pharmacological enhancement of α-glucosidase by the allosteric chaperone N-acetylcysteine (Q41912890)

14 October 2017

Pharmacological enhancement of α-glucosidase by the allosteric chaperone N-acetylcysteine (English)

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14 October 2017

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8

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14 October 2017

Massimiliano Meli

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Beatrice Cobucci-Ponzano

object named as

Beatrice Cobucci-Ponzano

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Marco Moracci

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Marco Moracci

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author name string

Caterina Porto

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14 October 2017

Maria C Ferrara

2

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14 October 2017

Emma Acampora

4

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14 October 2017

Valeria Avolio

5

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14 October 2017

Margherita Rosa

6

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14 October 2017

Generoso Andria

10

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14 October 2017

Giancarlo Parenti

11

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14 October 2017

18 September 2012

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14 October 2017

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14 October 2017

2201-2211

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describes a project that uses

14 October 2017

ImageJ

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11 June 2022

https://www.ebi.ac.uk/europepmc/webservices/rest/PMC3519985/fullTextXML

inferred from PubMed Central ID database lookup

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5 June 2018

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5 June 2018

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5 June 2018

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Enzyme replacement therapy with alglucosidase alfa in 44 patients with late-onset glycogen storage disease type 2: 12-month results of an observational clinical trial.

5 June 2018

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Early treatment with alglucosidase alpha prolongs long-term survival of infants with Pompe disease

5 June 2018

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The pharmacological chaperone N-butyldeoxynojirimycin enhances enzyme replacement therapy in Pompe disease fibroblasts

5 June 2018

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5 June 2018

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When more is less: excess and deficiency of autophagy coexist in skeletal muscle in Pompe disease

5 June 2018

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5 June 2018

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Isofagomine increases lysosomal delivery of exogenous glucocerebrosidase

5 June 2018

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A counterintuitive approach to treat enzyme deficiencies: use of enzyme inhibitors for restoring mutant enzyme activity

5 June 2018

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5 June 2018

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5 June 2018

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Pharmacological enhancement of mutated alpha-glucosidase activity in fibroblasts from patients with Pompe disease

5 June 2018

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Structure of acid beta-glucosidase with pharmacological chaperone provides insight into Gaucher disease

5 June 2018

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Recombinant human acid [alpha]-glucosidase: major clinical benefits in infantile-onset Pompe disease

5 June 2018

1 reference

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Autophagy and mistargeting of therapeutic enzyme in skeletal muscle in Pompe disease

5 June 2018

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Long-term intravenous treatment of Pompe disease with recombinant human alpha-glucosidase from milk

5 June 2018

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Targeted disruption of the acid alpha-glucosidase gene in mice causes an illness with critical features of both infantile and adult human glycogen storage disease type II

5 June 2018

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Structural and functional changes of lysosomal acid alpha-glucosidase during intracellular transport and maturation

5 June 2018

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5 June 2018

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Pompe disease: from new views on pathophysiology to innovative therapeutic strategies

5 June 2018

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The pharmacological chaperone 1-deoxynojirimycin increases the activity and lysosomal trafficking of multiple mutant forms of acid alpha-glucosidase

18 August 2018

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Abnormal mannose-6-phosphate receptor trafficking impairs recombinant alpha-glucosidase uptake in Pompe disease fibroblasts

18 August 2018

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18 August 2018

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N-Acetylcysteine enhances UV-mediated caspase-3 activation, fragmentation of E2F-4, and apoptosis in human C8161 melanoma: inhibition by ectopic Bcl-2 expression

18 August 2018

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Recombinant human alpha-glucosidase from rabbit milk in Pompe patients.

18 August 2018

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The effect of N-acetylcysteine on oxygen transport and uptake in patients with fulminant hepatic failure.

18 August 2018

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Turnover and distribution of intravenously administered mannose-terminated human acid beta-glucosidase in murine and human tissues.

18 August 2018

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Synergy between the pharmacological chaperone 1-deoxygalactonojirimycin and the human recombinant alpha-galactosidase A in cultured fibroblasts from patients with Fabry disease

18 August 2018

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/22990675

12 December 2020

inferred from PubMed ID database lookup

Structural and biochemical studies on Pompe disease and a "pseudodeficiency of acid alpha-glucosidase"

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/22990675

12 December 2020

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7 January 2021

Identifiers

DOI

10.1038/MT.2012.152

1 reference

14 October 2017

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14 October 2017

1 reference