Human mitochondrial tRNA quality control in health and disease: a channelling mechanism? (Q37976956)

26 August 2017

Human mitochondrial tRNA quality control in health and disease: a channelling mechanism? (English)

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26 August 2017

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Nina Entelis

3

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author name string

Ruth Belostotsky

series ordinal

1

1 reference

PubMed ID

22258151

26 August 2017

Yaacov Frishberg

series ordinal

2

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26 August 2017

1 January 2012

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26 August 2017

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26 August 2017

9

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26 August 2017

33-39

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26 August 2017

1

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Oxidative phosphorylation at the fin de siècle

26 August 2017

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

Forces maintaining organellar genomes: is any as strong as genetic code disparity or hydrophobicity?

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

Mamit-tRNA, a database of mammalian mitochondrial tRNA primary and secondary structures

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

Impact of disease-related mitochondrial mutations on tRNA structure and function

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

Human mitochondrial tRNAs in health and disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

Channeling of aminoacyl-tRNA for protein synthesis in vivo

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

The ins and outs of tRNA transport

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

Proofreading and aminoacylation of tRNAs before export from the nucleus.

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

Role of nuclear pools of aminoacyl-tRNA synthetases in tRNA nuclear export

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

Review: transport of tRNA out of the nucleus-direct channeling to the ribosome?

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

Scyl1 facilitates nuclear tRNA export in mammalian cells by acting at the nuclear pore complex

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

Identification of initiation sites for heavy-strand and light-strand transcription in human mitochondrial DNA

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

Mitochondrial tRNA 3' end metabolism and human disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

tRNA punctuation model of RNA processing in human mitochondria

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

Biogenesis of mitochondria

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

The La protein functions redundantly with tRNA modification enzymes to ensure tRNA structural stability

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

Rapid tRNA decay can result from lack of nonessential modifications.

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

Degradation of several hypomodified mature tRNA species in Saccharomyces cerevisiae is mediated by Met22 and the 5'-3' exonucleases Rat1 and Xrn1.

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

tRNA stabilization by modified nucleotides

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

Nuclear control of cloverleaf structure of human mitochondrial tRNA(Lys).

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

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

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

Missense mutation in pseudouridine synthase 1 (PUS1) causes mitochondrial myopathy and sideroblastic anemia (MLASA).

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

Acute infantile liver failure due to mutations in the TRMU gene.

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

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

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

Modification defect at anticodon wobble nucleotide of mitochondrial tRNAs(Leu)(UUR) with pathogenic mutations of mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

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

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

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

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

Nuclear DNA-encoded tRNAs targeted into mitochondria can rescue a mitochondrial DNA mutation associated with the MERRF syndrome in cultured human cells.

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

Decreased mitochondrial tRNALys steady-state levels and aminoacylation are associated with the pathogenic G8313A mitochondrial DNA mutation

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

The 7472insC mitochondrial DNA mutation impairs the synthesis and extent of aminoacylation of tRNASer(UCN) but not its structure or rate of turnover

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

Overexpression of human mitochondrial valyl tRNA synthetase can partially restore levels of cognate mt-tRNAVal carrying the pathogenic C25U mutation

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

A pathogenic point mutation reduces stability of mitochondrial mutant tRNA(Ile)

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

A disease-associated G5703A mutation in human mitochondrial DNA causes a conformational change and a marked decrease in steady-state levels of mitochondrial tRNA(Asn).

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

The role of aminoacyl-tRNA synthetases in genetic diseases

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

Deleterious mutation in the mitochondrial arginyl-transfer RNA synthetase gene is associated with pontocerebellar hypoplasia

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

Mutations in the mitochondrial seryl-tRNA synthetase cause hyperuricemia, pulmonary hypertension, renal failure in infancy and alkalosis, HUPRA syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

Mitochondrial aspartyl-tRNA synthetase deficiency causes leukoencephalopathy with brain stem and spinal cord involvement and lactate elevation

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

Can yeast be used to study mitochondrial diseases? Biolistic tRNA mutants for the analysis of mechanisms and suppressors

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

The yeast counterparts of human 'MELAS' mutations cause mitochondrial dysfunction that can be rescued by overexpression of the mitochondrial translation factor EF-Tu.

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

Overexpressed mitochondrial leucyl-tRNA synthetase suppresses the A3243G mutation in the mitochondrial tRNA(Leu(UUR)) gene

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

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/10.4161%2FRNA.9.1.18009

Additional copies of the mitochondrial Ef-Tu and aspartyl-tRNA synthetase genes can compensate for a mutation affecting the maturation of the mitochondrial tRNAAsp

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

The A3243G tRNALeu(UUR) MELAS mutation causes amino acid misincorporation and a combined respiratory chain assembly defect partially suppressed by overexpression of EFTu and EFG2.

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

Processivity of translation in the eukaryote cell: role of aminoacyl-tRNA synthetases

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

Polyadenylation and degradation of human mitochondrial RNA: the prokaryotic past leaves its mark

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

Identification of a novel human nuclear-encoded mitochondrial poly(A) polymerase

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

PNPASE regulates RNA import into mitochondria

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

The yeast mitochondrial degradosome. Its composition, interplay between RNA helicase and RNase activities and the role in mitochondrial RNA metabolism

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

Localisation of the human hSuv3p helicase in the mitochondrial matrix and its preferential unwinding of dsDNA

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

tRNA biology charges to the front

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

Deep sequencing of Salmonella RNA associated with heterologous Hfq proteins in vivo reveals small RNAs as a major target class and identifies RNA processing phenotypes

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

Selective charging of tRNA isoacceptors induced by amino-acid starvation

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

Tissue-specific differences in human transfer RNA expression

21 January 2018

1 reference

https://api.crossref.org/works/10.4161%2FRNA.9.1.18009

21 January 2018

Identifiers

DOI

10.4161/RNA.9.1.18009

1 reference

26 August 2017

1 reference

PubMed ID

22258151