Glutathione: a redox signature in monitoring EPI-743 therapy in children with mitochondrial encephalomyopathies (Q34650435)

3 August 2017

title

Glutathione: a redox signature in monitoring EPI-743 therapy in children with mitochondrial encephalomyopathies (English)

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3 August 2017

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12

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Gianna Di Giovamberardino

3 August 2017

Sara Petrillo

2

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Gianna Di Giovamberardino

7

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Giulia Tozzi

Giulia Tozzi

3

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Anna Pastore

Anna Pastore

1

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Fiorella Piemonte

Fiorella Piemonte

13

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Rosalba Carrozzo

Rosalba Carrozzo

4

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Carlo Dionisi-Vici

Carlo Dionisi-Vici

6

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Diego Martinelli

5

Diego Martinelli

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3 August 2017

10

Guy Miller

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3 August 2017

Ferdinando Ceravolo

8

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3 August 2017

Matthew B Klein

9

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3 August 2017

Gregory M Enns

11

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3 August 2017

publication date

24 March 2013

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Molecular Genetics and Metabolism

3 August 2017

published in

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3 August 2017

volume

109

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3 August 2017

issue

2

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3 August 2017

page(s)

208-214

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Mitochondrial energetics and therapeutics

3 August 2017

cites work

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https://api.crossref.org/works/10.1016%2FJ.YMGME.2013.03.011

Subacute necrotizing encephalomyelopathy in an infant

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2013.03.011

The mutation at nt 8993 of mitochondrial DNA is a common cause of Leigh's syndrome

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2013.03.011

High prevalence of SURF1 c.845_846delCT mutation in Polish Leigh patients.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2013.03.011

SURF1, encoding a factor involved in the biogenesis of cytochrome c oxidase, is mutated in Leigh syndrome

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2013.03.011

X-linked NDUFA1 gene mutations associated with mitochondrial encephalomyopathy.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2013.03.011

Molecular genetic characterization of an X-linked form of Leigh's syndrome

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2013.03.011

X-linked Leigh's syndrome

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2013.03.011

Glutathione deficiency in patients with mitochondrial disease: implications for pathogenesis and treatment

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2013.03.011

Inherited disorders affecting mitochondrial function are associated with glutathione deficiency and hypocitrullinemia

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2013.03.011

Plasma thiol status is altered in children with mitochondrial diseases

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2013.03.011

Redox environment of the cell as viewed through the redox state of the glutathione disulfide/glutathione couple

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2013.03.011

Molecular mechanisms and clinical implications of reversible protein S-glutathionylation

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2013.03.011

S-Glutathionylation signaling in cell biology: progress and prospects

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2013.03.011

Recent trends in glutathione biochemistry--glutathione-protein interactions: a molecular link between oxidative stress and cell proliferation?

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2013.03.011

Glutathione dysregulation and the etiology and progression of human diseases.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2013.03.011

S-glutathionylation: from molecular mechanisms to health outcomes

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2013.03.011

EPI-743 reverses the progression of the pediatric mitochondrial disease--genetically defined Leigh Syndrome.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2013.03.011

Assay for quantitative determination of glutathione and glutathione disulfide levels using enzymatic recycling method

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2013.03.011

Glutathione metabolism and antioxidant enzymes in children with Down syndrome

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2013.03.011

Measurement of protein thiol groups and glutathione in plasma

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2013.03.011

Initial experience in the treatment of inherited mitochondrial disease with EPI-743.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2013.03.011

Nuclear and mitochondrial compartmentation of oxidative stress and redox signaling

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2013.03.011

Radical-free biology of oxidative stress

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2013.03.011

Mitochondrial inhibition and oxidative stress: reciprocating players in neurodegeneration

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2013.03.011

Antioxidant agents have a different expression pattern in muscle fibers of patients with mitochondrial diseases

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2013.03.011

Dysfunctions of cellular oxidative metabolism in patients with mutations in the NDUFS1 and NDUFS4 genes of complex I

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2013.03.011

Biological markers of oxidative stress in mitochondrial myopathies with progressive external ophthalmoplegia

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2013.03.011

Increased expression of manganese-superoxide dismutase in fibroblasts of patients with CPEO syndrome

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2013.03.011

Thiol-disulfide balance: from the concept of oxidative stress to that of redox regulation.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FJ.YMGME.2013.03.011

10.1016/J.YMGME.2013.03.011

7 January 2021

Identifiers

DOI

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3 August 2017

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