(Q37129457)

18 August 2017

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

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18 August 2017

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5

Leonard A Herzenberg

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18 August 2017

6

Leonore A Herzenberg

1 reference

18 August 2017

Kondala R Atkuri

1

1 reference

18 August 2017

Tina M Cowan

2

1 reference

18 August 2017

Tony Kwan

3

1 reference

18 August 2017

Angelina Ng

4

1 reference

18 August 2017

Gregory M Enns

7

1 reference

18 August 2017

language of work or name

English

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publication date

17 February 2009

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Proceedings of the National Academy of Sciences of the United States of America

18 August 2017

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18 August 2017

106

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18 August 2017

10

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18 August 2017

3941-3945

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Mitochondrial dysfunction in mut methylmalonic acidemia

18 August 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2656184

Importance of culturing primary lymphocytes at physiological oxygen levels

14 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2656184

High-dose oral N-acetylcysteine, a glutathione prodrug, modulates inflammation in cystic fibrosis

14 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2656184

Biomarkers of oxidative damage in human disease.

14 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2656184

The contribution of mitochondria to common disorders

14 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2656184

Analysis of glutathione: implication in redox and detoxification

14 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2656184

Lymphocyte surface thiol levels

14 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2656184

Eleven-color flow cytometry. A powerful tool for elucidation of the complex immune system.

14 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2656184

Epidemiology and treatment of mitochondrial disorders

14 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2656184

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

14 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2656184

Mitochondrial diseases in man and mouse

14 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2656184

The neurogenic weakness, ataxia and retinitis pigmentosa (NARP) syndrome mtDNA mutation (T8993G) triggers muscle ATPase deficiency and hypocitrullinaemia

14 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2656184

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

14 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2656184

Glutathione depletion in PC12 results in selective inhibition of mitochondrial complex I activity. Implications for Parkinson's disease.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2656184

Superoxide production by the mitochondrial respiratory chain

27 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2656184

Methylmalonic acidaemia leads to increased production of reactive oxygen species and induction of apoptosis through the mitochondrial/caspase pathway

27 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2656184

Clinical, pathological, and biochemical studies in a patient with propionic acidemia and fatal cardiomyopathy

3 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2656184

Hypocitrullinemia in patients with MELAS: an insight into the "MELAS paradox".

3 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2656184

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

3 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2656184

Diagnostic criteria for respiratory chain disorders in adults and children.

3 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2656184

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

3 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2656184

Glutathione in blood of patients with Friedreich's ataxia.

3 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2656184

Propionic and L-methylmalonic acids induce oxidative stress in brain of young rats.

3 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2656184

3 September 2018

1 reference

12 December 2020

Comparison of cytosolic and mitochondrial enzyme alterations in the livers of propionic or methylmalonic acidemia: a reduction of cytochrome oxidase activity

1 reference

12 December 2020

Acute extrapyramidal syndrome in methylmalonic acidemia: “Metabolic stroke” involving the globus pallidus

1 reference

12 December 2020

Glutathione deficiency as a complication of methylmalonic acidemia: Response to high doses of ascorbate

1 reference

12 December 2020

Increased oxidative damage is correlated to altered mitochondrial function in heterozygous manganese superoxide dismutase knockout mice

1 reference

12 December 2020

Automatic recording apparatus for use in the chromatography of amino acids

1 reference

12 December 2020

gamma-glutamyltranspeptidase-deficient knockout mice as a model to study the relationship between glutathione status, mitochondrial function, and cellular function

1 reference

12 December 2020

Diagnosis of mitochondrial disorders: clinical and biochemical approach

1 reference

12 December 2020

Identifiers

DOI

10.1073/PNAS.0813409106

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18 August 2017

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1 reference

18 August 2017

PubMed publication ID

19223582

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