(Q41963195)

English

The isolated carboxy-terminal domain of human mitochondrial leucyl-tRNA synthetase rescues the pathological phenotype of mitochondrial tRNA mutations in human cells

scientific article published on 10 January 2014

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scholarly article

1 reference

Europe PubMed Central

PMC publication ID

14 October 2017

The isolated carboxy-terminal domain of human mitochondrial leucyl-tRNA synthetase rescues the pathological phenotype of mitochondrial tRNA mutations in human cells (English)

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PMC publication ID

14 October 2017

2

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31 May 2020

Arianna Montanari

4

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31 May 2020

Antonio F Campese

5

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31 May 2020

7

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31 May 2020

8

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31 May 2020

Patrizio Di Micco

11

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31 May 2020

14

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31 May 2020

Silvia Francisci

15

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31 May 2020

16

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31 May 2020

Robert William Taylor

13

object named as

Robert W Taylor

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31 May 2020

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Elena Perli

1

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31 May 2020

Annalinda Pisano

3

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31 May 2020

Aurelio Reyes

6

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31 May 2020

Helen A Tuppen

9

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31 May 2020

Maurizia Orlandi

10

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31 May 2020

Elena Poser

12

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31 May 2020

Laura Frontali

17

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31 May 2020

Giulia d'Amati

19

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31 May 2020

Massimo Zeviani

18

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31 May 2020

publication date

10 January 2014

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14 October 2017

EMBO Molecular Medicine

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31 May 2020

6

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31 May 2020

2

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31 May 2020

169-182

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31 May 2020

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1 reference

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Mitochondrial phenylalanyl-tRNA synthetase mutations underlie fatal infantile Alpers encephalopathy

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Structural dynamics of the aminoacylation and proofreading functional cycle of bacterial leucyl-tRNA synthetase

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Mutations in the mitochondrial methionyl-tRNA synthetase cause a neurodegenerative phenotype in flies and a recessive ataxia (ARSAL) in humans

1 reference

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5 June 2018

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Natural history of MELAS associated with mitochondrial DNA m.3243A>G genotype

1 reference

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Human mitochondrial tRNAs: biogenesis, function, structural aspects, and diseases

1 reference

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5 June 2018

Peptides from aminoacyl-tRNA synthetases can cure the defects due to mutations in mt tRNA genes

1 reference

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Exome sequencing identifies mitochondrial alanyl-tRNA synthetase mutations in infantile mitochondrial cardiomyopathy

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Notch3 and canonical NF-kappaB signaling pathways cooperatively regulate Foxp3 transcription.

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Actin and myosin contribute to mammalian mitochondrial DNA maintenance.

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

1 reference

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5 June 2018

A human pathology-related mutation prevents import of an aminoacyl-tRNA synthetase into mitochondria

1 reference

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5 June 2018

Mutation of the mitochondrial tyrosyl-tRNA synthetase gene, YARS2, causes myopathy, lactic acidosis, and sideroblastic anemia--MLASA syndrome

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Identification and characterization of mitochondrial targeting sequence of human apurinic/apyrimidinic endonuclease 1

1 reference

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5 June 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3927953

5 June 2018

The balance between pre- and post-transfer editing in tRNA synthetases

1 reference

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5 June 2018

The background of mitochondrial DNA haplogroup J increases the sensitivity of Leber's hereditary optic neuropathy cells to 2,5-hexanedione toxicity

1 reference

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Mitochondrial DNA background modulates the assembly kinetics of OXPHOS complexes in a cellular model of mitochondrial disease.

1 reference

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5 June 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3927953

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The role of aminoacyl-tRNA synthetases in genetic diseases

1 reference

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

1 reference

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Multiple pathways for sorting mitochondrial precursor proteins

1 reference

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A Flexible peptide tether controls accessibility of a unique C-terminal RNA-binding domain in leucyl-tRNA synthetases

1 reference

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

1 reference

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

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Translocation of proteins into mitochondria

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Structure of the 70S ribosome complexed with mRNA and tRNA

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Aminoacyl-tRNA synthesis

1 reference

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Purification and characterization of the Saccharomyces cerevisiae mitochondrial leucyl-tRNA synthetase

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1 reference

https://pubmed.ncbi.nlm.nih.gov/24413190

12 December 2020

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10.1002/EMMM.201303198

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31 May 2020

release_3rrrbkixybdixg2oe65soemf7m

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31 May 2020

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31 May 2020

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