Loss of CLPP alleviates mitochondrial cardiomyopathy without affecting the mammalian UPRmt. (Q37061723)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:27154400%20AND%20SRC:MED&resulttype=core&format=json

4 April 2020

title

Loss of CLPP alleviates mitochondrial cardiomyopathy without affecting the mammalian UPRmt (English)

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4 April 2020

1 reference

1 reference

6

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4 April 2020

Aleksandra Trifunovic

9

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4 April 2020

author name string

Dominic Seiferling

1

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4 April 2020

Karolina Szczepanowska

2

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4 April 2020

Christina Becker

3

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4 April 2020

Katharina Senft

4

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4 April 2020

Steffen Hermans

5

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4 April 2020

Tim König

7

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Alexandra Kukat

8

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publication date

6 May 2016

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4 April 2020

number of pages

12

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based on heuristic

inferred from page(s)

published in

EMBO Reports

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4 April 2020

volume

17

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issue

7

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4 April 2020

page(s)

953-964

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describes a project that uses

4 April 2020

ImageJ

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11 June 2022

https://www.ebi.ac.uk/europepmc/webservices/rest/PMC4931557/fullTextXML

based on heuristic

inferred from PubMed Central ID database lookup

cites work

The loss of LRPPRC function induces the mitochondrial unfolded protein response

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Mitochondria Retrograde Signaling and the UPR mt: Where Are We in Mammals?

29 September 2017

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Mutations in Twinkle primase-helicase cause Perrault syndrome with neurologic features

29 September 2017

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DNAJC19, a mitochondrial cochaperone associated with cardiomyopathy, forms a complex with prohibitins to regulate cardiolipin remodeling

29 September 2017

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

29 September 2017

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Genome engineering using the CRISPR-Cas9 system

29 September 2017

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The accumulation of misfolded proteins in the mitochondrial matrix is sensed by PINK1 to induce PARK2/Parkin-mediated mitophagy of polarized mitochondria

29 September 2017

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Loss of mitochondrial peptidase Clpp leads to infertility, hearing loss plus growth retardation via accumulation of CLPX, mtDNA and inflammatory factors

29 September 2017

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CHOP induces activating transcription factor 5 (ATF5) to trigger apoptosis in response to perturbations in protein homeostasis

29 September 2017

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Mitonuclear protein imbalance as a conserved longevity mechanism

29 September 2017

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Mutations in LARS2, encoding mitochondrial leucyl-tRNA synthetase, lead to premature ovarian failure and hearing loss in Perrault syndrome

29 September 2017

1 reference

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Perrault syndrome is caused by recessive mutations in CLPP, encoding a mitochondrial ATP-dependent chambered protease

29 September 2017

1 reference

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LRPPRC is necessary for polyadenylation and coordination of translation of mitochondrial mRNAs

29 September 2017

1 reference

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Mutations in mitochondrial histidyl tRNA synthetase HARS2 cause ovarian dysgenesis and sensorineural hearing loss of Perrault syndrome

29 September 2017

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The matrix peptide exporter HAF-1 signals a mitochondrial UPR by activating the transcription factor ZC376.7 in C. elegans

29 September 2017

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Inhibition of mitochondrial function induces an integrated stress response in oligodendroglia

29 September 2017

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Discovery of genes activated by the mitochondrial unfolded protein response (mtUPR) and cognate promoter elements

29 September 2017

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Ubiquitin-like protein 5 positively regulates chaperone gene expression in the mitochondrial unfolded protein response

29 September 2017

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Somatic mtDNA mutations cause aging phenotypes without affecting reactive oxygen species production

29 September 2017

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29 September 2017

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29 September 2017

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LON is the master protease that protects against protein aggregation in human mitochondria through direct degradation of misfolded proteins

29 September 2017

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Variable and tissue-specific subunit composition of mitochondrial m-AAA protease complexes linked to hereditary spastic paraplegia

27 June 2018

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

27 June 2018

1 reference

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Accumulation of pathogenic DeltamtDNA induced deafness but not diabetic phenotypes in mito-mice

4 September 2018

1 reference

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Compartment-specific perturbation of protein handling activates genes encoding mitochondrial chaperones

4 September 2018

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4 September 2018

Identifiers

DOI

10.15252/EMBR.201642077

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:27154400%20AND%20SRC:MED&resulttype=core&format=json

4 April 2020

1 reference

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4 April 2020

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