Mitochondrial diseases: the contribution of organelle stress responses to pathology (Q47974129)

7 February 2018

title

Mitochondrial diseases: the contribution of organelle stress responses to pathology (English)

1 reference

7 February 2018

0 references

Anu Suomalainen

1

1 reference

7 February 2018

Brendan J Battersby

series ordinal

2

1 reference

7 February 2018

publication date

9 August 2017

1 reference

7 February 2018

Nature Reviews Molecular Cell Biology

published in

1 reference

7 February 2018

volume

19

1 reference

7 February 2018

issue

2

1 reference

7 February 2018

page(s)

77-92

1 reference

7 February 2018

Mitochondrial disorders as windows into an ancient organelle

cites work

1 reference

Mitochondria: In Sickness and in Health

21 January 2018

1 reference

Therapy for mitochondrial disorders: Little proof, high research activity, some promise

21 January 2018

1 reference

New treatments for mitochondrial disease-no time to drop our standards

21 January 2018

1 reference

A mitochondrial protein compendium elucidates complex I disease biology

21 January 2018

1 reference

Mechanisms of mitochondrial diseases

21 January 2018

1 reference

Mitochondria: impaired mitochondrial translation in human disease

21 January 2018

1 reference

Molecular basis of infantile reversible cytochrome c oxidase deficiency myopathy

21 January 2018

1 reference

mtDNA Mutagenesis Disrupts Pluripotent Stem Cell Function by Altering Redox Signaling

21 January 2018

1 reference

Mitochondrial reactive oxygen species regulate cellular signaling and dictate biological outcomes

21 January 2018

1 reference

A Mitofusin-2-dependent inactivating cleavage of Opa1 links changes in mitochondria cristae and ER contacts in the postprandial liver.

21 January 2018

1 reference

NAD( )-dependent activation of Sirt1 corrects the phenotype in a mouse model of mitochondrial disease

21 January 2018

1 reference

Ribosome. The complete structure of the 55S mammalian mitochondrial ribosome

21 January 2018

1 reference

Ribosome. The structure of the human mitochondrial ribosome

21 January 2018

1 reference

Maintenance and Expression of Mammalian Mitochondrial DNA.

21 January 2018

1 reference

Organization and Regulation of Mitochondrial Protein Synthesis

21 January 2018

1 reference

Human mitochondrial DNA deletions associated with mutations in the gene encoding Twinkle, a phage T7 gene 4-like protein localized in mitochondria

21 January 2018

1 reference

Reconstitution of a minimal mtDNA replisome in vitro

21 January 2018

1 reference

A novel processive mechanism for DNA synthesis revealed by structure, modeling and mutagenesis of the accessory subunit of human mitochondrial DNA polymerase

21 January 2018

1 reference

Human mitochondrial RNA polymerase primes lagging-strand DNA synthesis in vitro

21 January 2018

1 reference

Mitochondrial transcription factor A is necessary for mtDNA maintenance and embryogenesis in mice

21 January 2018

1 reference

Premature ageing in mice expressing defective mitochondrial DNA polymerase

21 January 2018

1 reference

Mitochondrial DNA mutations, oxidative stress, and apoptosis in mammalian aging

21 January 2018

1 reference

Twinkle helicase is essential for mtDNA maintenance and regulates mtDNA copy number

21 January 2018

1 reference

The mitochondrial transcription factor TFAM coordinates the assembly of multiple DNA molecules into nucleoid-like structures

21 January 2018

1 reference

Mutation of RRM2B, encoding p53-controlled ribonucleotide reductase (p53R2), causes severe mitochondrial DNA depletion

21 January 2018

1 reference

Depletion of muscle mitochondrial DNA in AIDS patients with zidovudine-induced myopathy

21 January 2018

1 reference

Mitochondrial DNA depletion syndromes--many genes, common mechanisms

21 January 2018

1 reference

Mitochondrial DNA deletions in progressive external ophthalmoplegia and Kearns-Sayre syndrome

21 January 2018

1 reference

Mutant mitochondrial thymidine kinase in mitochondrial DNA depletion myopathy

21 January 2018

1 reference

The deoxyguanosine kinase gene is mutated in individuals with depleted hepatocerebral mitochondrial DNA

21 January 2018

1 reference

Multiple deletions of mitochondrial DNA in several tissues of a patient with severe retarded depression and familial progressive external ophthalmoplegia

21 January 2018

1 reference

Mitochondrial form and function

21 January 2018

1 reference

OPA1, encoding a dynamin-related GTPase, is mutated in autosomal dominant optic atrophy linked to chromosome 3q28

21 January 2018

1 reference

Regulation of OPA1 processing and mitochondrial fusion by m-AAA protease isoenzymes and OMA1

21 January 2018

1 reference

ER-mitochondria contacts couple mtDNA synthesis with mitochondrial division in human cells

21 January 2018

1 reference

Human mitochondrial ribosomes can switch their structural RNA composition.

21 January 2018

1 reference

Mutations in MTFMT underlie a human disorder of formylation causing impaired mitochondrial translation

21 January 2018

1 reference

Mitochondrial aminoacyl-tRNA synthetases in human disease

21 January 2018

1 reference

Synchronized mitochondrial and cytosolic translation programs

21 January 2018

1 reference

Use of whole-exome sequencing to determine the genetic basis of multiple mitochondrial respiratory chain complex deficiencies

21 January 2018

1 reference

A mitochondrial ribosomal and RNA decay pathway blocks cell proliferation.

21 January 2018

1 reference

Inhibition of mitochondrial translation as a therapeutic strategy for human acute myeloid leukemia

21 January 2018

1 reference

Mitochondrial ribosomal RNA gene mutation in a patient with sporadic aminoglycoside ototoxicity

21 January 2018

1 reference

Mitochondrial ribosome assembly in health and disease

21 January 2018

1 reference

Whole-exome sequencing identifies a mutation in the mitochondrial ribosome protein MRPL44 to underlie mitochondrial infantile cardiomyopathy

21 January 2018

1 reference

Structure of the large ribosomal subunit from human mitochondria.

21 January 2018

1 reference

Mutations in C12orf65 in patients with encephalomyopathy and a mitochondrial translation defect

21 January 2018

1 reference

Quality control of mitochondrial protein synthesis is required for membrane integrity and cell fitness.

21 January 2018

1 reference

Mitochondrial DNA Replication Defects Disturb Cellular dNTP Pools and Remodel One-Carbon Metabolism

21 January 2018

1 reference

Ubiquilins Chaperone and Triage Mitochondrial Membrane Proteins for Degradation.

21 January 2018

1 reference

Mitochondrial protein synthesis in HeLa cells

21 January 2018

1 reference

New roles for mitochondrial proteases in health, ageing and disease

21 January 2018

1 reference

A mutation in the tRNA(Leu)(UUR) gene associated with the MELAS subgroup of mitochondrial encephalomyopathies

21 January 2018

1 reference

Mutation in mitochondrial tRNALeu(UUR) gene in a large pedigree with maternally transmitted type II diabetes mellitus and deafness

21 January 2018

1 reference

Myoclonic epilepsy and ragged-red fiber disease (MERRF) is associated with a mitochondrial DNA tRNA(Lys) mutation

21 January 2018

1 reference

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

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

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

21 January 2018

1 reference

Loss of the m-AAA protease subunit AFG₃L₂ causes mitochondrial transport defects and tau hyperphosphorylation

21 January 2018

1 reference

Spastic paraplegia and OXPHOS impairment caused by mutations in paraplegin, a nuclear-encoded mitochondrial metalloprotease

21 January 2018

1 reference

Mutations in the mitochondrial protease gene AFG3L2 cause dominant hereditary ataxia SCA28.

21 January 2018

1 reference

The YTA10-12 complex, an AAA protease with chaperone-like activity in the inner membrane of mitochondria

21 January 2018

1 reference

Inducible proteolytic inactivation of OPA1 mediated by the OMA1 protease in mammalian cells

21 January 2018

1 reference

Mitochondrial myopathy induces a starvation-like response

21 January 2018

1 reference

Tissue-specific loss of DARS2 activates stress responses independently of respiratory chain deficiency in the heart

21 January 2018

1 reference

Identification of the cis-acting endoplasmic reticulum stress response element responsible for transcriptional induction of mammalian glucose-regulated proteins. Involvement of basic leucine zipper transcription factors

21 January 2018

1 reference

A mitochondrial specific stress response in mammalian cells

21 January 2018

1 reference

ClpP mediates activation of a mitochondrial unfolded protein response in C. elegans

21 January 2018

1 reference

Mitochondrial import efficiency of ATFS-1 regulates mitochondrial UPR activation.

21 January 2018

1 reference

Evaluating and responding to mitochondrial dysfunction: the mitochondrial unfolded-protein response and beyond

21 January 2018

1 reference

The Cell-Non-Autonomous Nature of Electron Transport Chain-Mediated Longevity

21 January 2018

1 reference

Muscle mitohormesis promotes cellular survival via serine/glycine pathway flux.

21 January 2018

1 reference

One-Carbon Metabolism in Health and Disease

21 January 2018

1 reference

Quantitative flux analysis reveals folate-dependent NADPH production.

21 January 2018

1 reference

Serine, glycine and one-carbon units: cancer metabolism in full circle

21 January 2018

1 reference

Characterization of the usage of the serine metabolic network in human cancer

21 January 2018

1 reference

mTORC1 induces purine synthesis through control of the mitochondrial tetrahydrofolate cycle

21 January 2018

1 reference

Spatial colocalization and functional link of purinosomes with mitochondria.

21 January 2018

1 reference

Administration of deoxyribonucleosides or inhibition of their catabolism as a pharmacological approach for mitochondrial DNA depletion syndrome.

21 January 2018

1 reference

Fibroblast growth factor 21 induction by activating transcription factor 4 is regulated through three amino acid response elements in its promoter region

21 January 2018

1 reference

FGF-21 as a biomarker for muscle-manifesting mitochondrial respiratory chain deficiencies: a diagnostic study

21 January 2018

1 reference

FGF21 is a biomarker for mitochondrial translation and mtDNA maintenance disorders

21 January 2018

1 reference

FGF-21 as a novel metabolic regulator

21 January 2018

1 reference

Hepatic fibroblast growth factor 21 is regulated by PPARalpha and is a key mediator of hepatic lipid metabolism in ketotic states

21 January 2018

1 reference

The fasting polypeptide FGF21 can enter brain from blood

21 January 2018

1 reference

Growth differentiation factor 15 as a useful biomarker for mitochondrial disorders.

21 January 2018

1 reference

TGF-b superfamily cytokine MIC-1/GDF15 is a physiological appetite and body weight regulator

21 January 2018

1 reference

Imbalanced OPA1 processing and mitochondrial fragmentation cause heart failure in mice

21 January 2018

1 reference

Molecular insights into the klotho-dependent, endocrine mode of action of fibroblast growth factor 19 subfamily members

21 January 2018

1 reference

Creatine supplementation and exercise performance: recent findings

21 January 2018

1 reference

A new pathway for mitochondrial quality control: mitochondrial-derived vesicles

21 January 2018

1 reference

Deciphering the Molecular Signals of PINK1/Parkin Mitophagy.

21 January 2018

1 reference

How mitochondria produce reactive oxygen species

21 January 2018

1 reference

Unraveling the biological roles of reactive oxygen species

21 January 2018

1 reference

ROS-dependent signal transduction

21 January 2018

1 reference

MtDNA mutagenesis impairs elimination of mitochondria during erythroid maturation leading to enhanced erythrocyte destruction.

21 January 2018

1 reference

Regulation of oxidative stress by ATM is required for self-renewal of haematopoietic stem cells

21 January 2018

1 reference

Metabolic requirements for the maintenance of self-renewing stem cells

21 January 2018

1 reference

Hypoxia as a therapy for mitochondrial disease.

21 January 2018

1 reference

Leigh syndrome: neuropathology and pathogenesis.

21 January 2018

1 reference

In vivo correction of COX deficiency by activation of the AMPK/PGC-1α axis

21 January 2018

1 reference

Ketogenic diet slows down mitochondrial myopathy progression in mice

21 January 2018

1 reference

mTOR inhibition alleviates mitochondrial disease in a mouse model of Leigh syndrome

21 January 2018

1 reference

Modified Atkins diet induces subacute selective ragged-red-fiber lysis in mitochondrial myopathy patients.

21 January 2018

1 reference

Parkinsonism, premature menopause, and mitochondrial DNA polymerase gamma mutations: clinical and molecular genetic study.

21 January 2018

1 reference

Familial parkinsonism and ophthalmoplegia from a mutation in the mitochondrial DNA helicase twinkle

21 January 2018

1 reference

High levels of mitochondrial DNA deletions in substantia nigra neurons in aging and Parkinson disease

21 January 2018

1 reference

Muscle mitochondrial stress adaptation operates independently of endogenous FGF21 action

21 January 2018

1 reference

mtRF1a is a human mitochondrial translation release factor decoding the major termination codons UAA and UAG

21 January 2018

1 reference

Codon-reading specificities of mitochondrial release factors and translation termination at non-standard stop codons

21 January 2018

1 reference

Ribosome rescue and translation termination at non-standard stop codons by ICT1 in mammalian mitochondria.

21 January 2018

1 reference

An autosomal dominant disorder with multiple deletions of mitochondrial DNA starting at the D-loop region

21 January 2018

1 reference

Effective treatment of mitochondrial myopathy by nicotinamide riboside, a vitamin B3

21 January 2018

1 reference

High mitochondrial DNA copy number has detrimental effects in mice

31 March 2020

1 reference

Tissue distribution and effects on mitochondrial protein synthesis of tetracyclines after prolonged continuous intravenous administration to rats

31 March 2020

1 reference

Antibiotic effects on mitochondrial translation and in patients with mitochondrial translational defects

31 March 2020

1 reference

The A3243G tRNALeu(UUR) mutation induces mitochondrial dysfunction and variable disease expression without dominant negative acting translational defects in complex IV subunits at UUR codons

31 March 2020

1 reference

Mitochondrial dysfunction remodels one-carbon metabolism in human cells

31 March 2020

1 reference

Metabolic enzyme expression highlights a key role for MTHFD2 and the mitochondrial folate pathway in cancer

31 March 2020

1 reference

Somatic Progenitor Cell Vulnerability to Mitochondrial DNA Mutagenesis Underlies Progeroid Phenotypes in Polg Mutator Mice

31 March 2020

1 reference

Effect of bezafibrate treatment on late-onset mitochondrial myopathy in mice

31 March 2020

1 reference

NAG-1/GDF15 transgenic mouse has less white adipose tissue and a reduced inflammatory response

31 March 2020

1 reference

12 December 2020

Distribution and threshold expression of the tRNA(Lys) mutation in skeletal muscle of patients with myoclonic epilepsy and ragged-red fibers (MERRF)

1 reference

12 December 2020

Pearson syndrome and mitochondrial encephalomyopathy in a patient with a deletion of mtDNA.

1 reference

12 December 2020

Benign infantile mitochondrial myopathy due to reversible cytochrome c oxidase deficiency

1 reference

12 December 2020

Identifiers

DOI

10.1038/NRM.2017.66

1 reference

7 February 2018

1 reference

7 February 2018