(Q38062335)

28 August 2017

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title

Role of taurine in the pathologies of MELAS and MERRF. (English)

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28 August 2017

4

Junichi Azuma

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28 August 2017

1

Stephen W Schaffer

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28 August 2017

2

Chian Ju Jong

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28 August 2017

Takashi Ito

3

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28 August 2017

language of work or name

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20 November 2012

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28 August 2017

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28 August 2017

volume

46

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28 August 2017

issue

1

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28 August 2017

page(s)

47-56

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Defective kinetics of cytochrome c oxidase and alteration of mitochondrial membrane potential in fibroblasts and cytoplasmic hybrid cells with the mutation for myoclonus epilepsy with ragged-red fibres ('MERRF') at position 8344 nt.

28 August 2017

cites work

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Single atom modification (O-->S) of tRNA confers ribosome binding.

21 January 2018

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Relationship of genotype to phenotype in fibroblast-derived transmitochondrial cell lines carrying the 3243 mutation associated with the MELAS encephalomyopathy: shift towards mutant genotype and role of mtDNA copy number

21 January 2018

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Human diseases with defects in oxidative phosphorylation. 1. Decreased amounts of assembled oxidative phosphorylation complexes in mitochondrial encephalomyopathies

21 January 2018

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Multiple defects of the mitochondrial respiratory chain in a mitochondrial encephalopathy (MERRF): a clinical, biochemical and molecular study.

21 January 2018

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Decreased aminoacylation of mutant tRNAs in MELAS but not in MERRF patients

21 January 2018

1 reference

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Taurine protected myocardial mitochondria injury induced by hyperhomocysteinemia in rats

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00726-012-1414-8

Taurine protects transformed rat retinal ganglion cells from hypoxia-induced apoptosis by preventing mitochondrial dysfunction

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00726-012-1414-8

Epidemiology and treatment of mitochondrial disorders

21 January 2018

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The myoclonic epilepsy and ragged-red fiber mutation provides new insights into human mitochondrial function and genetics

21 January 2018

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The mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episode syndrome-associated human mitochondrial tRNALeu(UUR) mutation causes aminoacylation deficiency and concomitant reduced association of mRNA with ribosomes.

21 January 2018

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Wobble during decoding: more than third-position promiscuity

21 January 2018

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Detection of known base substitution mutations in human mitochondrial DNA of MERRF and MELAS by biochip technology

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00726-012-1414-8

MtDNA mutation in MERRF syndrome causes defective aminoacylation of tRNA(Lys) and premature translation termination

21 January 2018

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Genetic, pathogenetic, and phenotypic implications of the mitochondrial A3243G tRNALeu(UUR) mutation

21 January 2018

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Inherited mitochondrial disorders

21 January 2018

1 reference

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Pathophysiology of the MELAS 3243 transition mutation

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00726-012-1414-8

Search for differences in post-transcriptional modification patterns of mitochondrial DNA-encoded wild-type and mutant human tRNALys and tRNALeu(UUR).

21 January 2018

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The np 3243 MELAS mutation: damned if you aminoacylate, damned if you don't.

21 January 2018

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Altered mitochondrial function in fibroblasts containing MELAS or MERRF mitochondrial DNA mutations.

21 January 2018

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Codon-specific translational defect caused by a wobble modification deficiency in mutant tRNA from a human mitochondrial disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00726-012-1414-8

Specific correlation between the wobble modification deficiency in mutant tRNAs and the clinical features of a human mitochondrial disease

21 January 2018

1 reference

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Modified uridines with C5-methylene substituents at the first position of the tRNA anticodon stabilize U.G wobble pairing during decoding

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00726-012-1414-8

Human mitochondrial leucyl-tRNA synthetase corrects mitochondrial dysfunctions due to the tRNALeu(UUR) A3243G mutation, associated with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like symptoms and diabetes

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00726-012-1414-8

Inhibitory effect of taurine on veratridine-evoked D-[3H]aspartate release from murine corticostriatal slices: involvement of chloride channels and mitochondria.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00726-012-1414-8

Gestational protein restriction in mice has pronounced effects on gene expression in newborn offspring's liver and skeletal muscle; protective effect of taurine

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00726-012-1414-8

The role of modifications in codon discrimination by tRNA(Lys)UUU.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00726-012-1414-8

Respiratory chain activity in tissues from patients (MELAS) with a point mutation of the mitochondrial genome [tRNALeu(UUR) ]

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00726-012-1414-8

Biochemical analysis of respiratory function in cybrid cell lines harbouring mitochondrial DNA mutations.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00726-012-1414-8

Potentiation of mitochondrial Ca2+ sequestration by taurine

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00726-012-1414-8

Enhanced oxidative damage in human cells harboring A3243G mutation of mitochondrial DNA: implication of oxidative stress in the pathogenesis of mitochondrial diabetes

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00726-012-1414-8

The pathogenic A3243G mutation in human mitochondrial tRNALeu(UUR) decreases the efficiency of aminoacylation

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00726-012-1414-8

Taurine prevents tamoxifen-induced mitochondrial oxidative damage in mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00726-012-1414-8

Enhancing effect of taurine on glucose response in UCP2-overexpressing beta cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00726-012-1414-8

Aminoacylation properties of pathology-related human mitochondrial tRNA(Lys) variants

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00726-012-1414-8

Mitochondrial encephalopathy, lactic acidosis, and strokelike episodes: basic concepts, clinical phenotype, and therapeutic management of MELAS syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00726-012-1414-8

PGC-1alpha/beta induced expression partially compensates for respiratory chain defects in cells from patients with mitochondrial disorders.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00726-012-1414-8

Taurine as a constituent of mitochondrial tRNAs: new insights into the functions of taurine and human mitochondrial diseases

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00726-012-1414-8

Impaired complex I assembly in a Leigh syndrome patient with a novel missense mutation in the ND6 gene

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00726-012-1414-8

Mitochondria-specific RNA-modifying enzymes responsible for the biosynthesis of the wobble base in mitochondrial tRNAs. Implications for the molecular pathogenesis of human mitochondrial diseases

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00726-012-1414-8

Taurine concentrations in plasma, blood cells, and urine of children undergoing long-term total parenteral nutrition

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00726-012-1414-8

Impaired mitochondrial Ca2+ homeostasis in respiratory chain-deficient cells but efficient compensation of energetic disadvantage by enhanced anaerobic glycolysis due to low ATP steady state levels.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00726-012-1414-8

Mechanism for expanding the decoding capacity of transfer RNAs by modification of uridines

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00726-012-1414-8

Wobble modification defect in tRNA disturbs codon-anticodon interaction in a mitochondrial disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00726-012-1414-8

Wobble modification defect suppresses translational activity of tRNAs with MERRF and MELAS mutations

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00726-012-1414-8

Wobble modification deficiency in mutant tRNAs in patients with mitochondrial diseases.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00726-012-1414-8

Complementation of mutant and wild-type human mitochondrial DNAs coexisting since the mutation event and lack of complementation of DNAs introduced separately into a cell within distinct organelles

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00726-012-1414-8

10.1007/S00726-012-1414-8

21 January 2018

Identifiers

DOI

1 reference

28 August 2017

1 reference