(Q34357337)

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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

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1 August 2017

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

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1 August 2017

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Martin Hrabě de Angelis

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Martin Hrabé de Angelis

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Finja Büchel

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Michael Klinkenberg

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Michael Klinkenberg

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Andrea Hamann

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Ulrich Brandt

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Andreas S Reichert

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Andreas S Reichert

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Michel Mittelbronn

6

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Michel Mittelbronn

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1 August 2017

5

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Ilka Wittig

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Pawel Grzmil

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1 August 2017

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Stefan Dröse

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1 August 2017

Marina Jendrach

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Marina Jendrach

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1 August 2017

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Suzana Gispert

1

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1 August 2017

Dajana Parganlija

2

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1 August 2017

Sebastian Koob

8

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1 August 2017

Michael Walter

10

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1 August 2017

Thure Adler

12

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1 August 2017

Dirk H Busch

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1 August 2017

Andreas Zell

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Heinz D Osiewacz

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Georg Auburger

20

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publication date

12 July 2013

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1 August 2017

Human Molecular Genetics

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22

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1 August 2017

4871-4887

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Europe PubMed Central

PubMed publication ID

1 August 2017

AAA+ proteases: ATP-fueled machines of protein destruction

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Mitochondrial protein homeostasis: the cooperative roles of chaperones and proteases

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

ClpXP, an ATP-powered unfolding and protein-degradation machine

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Molecular determinants of complex formation between Clp/Hsp100 ATPases and the ClpP peptidase.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Role of the processing pore of the ClpX AAA+ ATPase in the recognition and engagement of specific protein substrates

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Degradation of Escherichia coli RecN aggregates by ClpXP protease and its implications for DNA damage tolerance.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Proteolysis of sigmaS (RpoS) and the general stress response in Escherichia coli

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Modulating substrate choice: the SspB adaptor delivers a regulator of the extracytoplasmic-stress response to the AAA+ protease ClpXP for degradation

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Rapid degradation of bacteriophage lambda O protein by ClpP/ClpX protease influences the lysis-versus-lysogenization decision of the phage under certain growth conditions of the host cells

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Derepression of bacteriophage mu transposition functions by truncated forms of the immunity repressor

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

A mitochondrial specific stress response in mammalian cells

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Stress induction of the Bacillus subtilis clpP gene encoding a homologue of the proteolytic component of the Clp protease and the involvement of ClpP and ClpX in stress tolerance

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Human CLPP reverts the longevity phenotype of a fungal ClpP deletion strain.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Mitochondrial stress signaling: a pathway unfolds.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Functional proteolytic complexes of the human mitochondrial ATP-dependent protease, hClpXP

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Decreased expression of the mitochondrial matrix proteases Lon and ClpP in cells from a patient with hereditary spastic paraplegia (SPG13).

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

The Hsp60-(p.V98I) mutation associated with hereditary spastic paraplegia SPG13 compromises chaperonin function both in vitro and in vivo.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Frataxin deficiency causes upregulation of mitochondrial Lon and ClpP proteases and severe loss of mitochondrial Fe-S proteins

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Emerging roles of mitochondrial proteases in neurodegeneration

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Mitochondrial processing peptidase regulates PINK1 processing, import and Parkin recruitment

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Loss of function mutations in the gene encoding Omi/HtrA2 in Parkinson's disease

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Mitochondrial membrane potential regulates PINK1 import and proteolytic destabilization by PARL

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Missense mutations in the AFG3L2 proteolytic domain account for ∼1.5% of European autosomal dominant cerebellar ataxias

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Whole-exome sequencing identifies homozygous AFG3L2 mutations in a spastic ataxia-neuropathy syndrome linked to mitochondrial m-AAA proteases

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

The m-AAA protease defective in hereditary spastic paraplegia controls ribosome assembly in mitochondria

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Mitochondrial quality control: an integrated network of pathways

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Mitochondrial protein quality control during biogenesis and aging

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Quality control of mitochondria: protection against neurodegeneration and ageing

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Animal models for respiratory chain disease

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Characterization of mouse Clpp protease cDNA, gene, and protein

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Molecular cloning and characterization of a mouse homolog of bacterial ClpX, a novel mammalian class II member of the Hsp100/Clp chaperone family

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

A mitochondrial protein compendium elucidates complex I disease biology

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Human and mouse mitochondrial orthologs of bacterial ClpX

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

The Perrault syndrome: clinical report and review

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

The perrault syndrome: Autosomal recessive ovarian dysgenesis with facultative, non-sex-limited sensorineural deafness

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Deafness, sensory neuropathy, and ovarian dysgenesis: a new syndrome or a broader spectrum of Perrault syndrome?

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Parkinson phenotype in aged PINK1-deficient mice is accompanied by progressive mitochondrial dysfunction in absence of neurodegeneration

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Mitochondria in innate immunity

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

The role of mitochondria in the mammalian antiviral defense system

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Circulating mitochondrial DAMPs cause inflammatory responses to injury

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Bioenergetic origins of complexity and disease

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Opa3, a novel regulator of mitochondrial function, controls thermogenesis and abdominal fat mass in a mouse model for Costeff syndrome

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Proteomic and metabolomic analyses of mitochondrial complex I-deficient mouse model generated by spontaneous B2 short interspersed nuclear element (SINE) insertion into NADH dehydrogenase (ubiquinone) Fe-S protein 4 (Ndufs4) gene

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Mitochondrial superoxide anion (O(2)(-)) inducible "mev-1" animal models for aging research

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

The GRACILE mutation introduced into Bcs1l causes postnatal complex III deficiency: a viable mouse model for mitochondrial hepatopathy

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Mitochondrial dysfunction and reduced prostaglandin synthesis in skeletal muscle of Group VIB Ca2+-independent phospholipase A2gamma-deficient mice

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Progressive loss of mitochondrial DNA in thymidine kinase 2-deficient mice

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Disruption of Supv3L1 damages the skin and causes sarcopenia, loss of fat, and death.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Role of human mitochondrial Nfs1 in cytosolic iron-sulfur protein biogenesis and iron regulation

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Mouse models for mitochondrial disease

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Strain-dependent high-level expression of a transgene for manganese superoxide dismutase is associated with growth retardation and decreased fertility

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Lack of mitochondrial trifunctional protein in mice causes neonatal hypoglycemia and sudden death

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Mitochondrial oxidative stress in mice lacking the glutathione peroxidase-1 gene

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Maternal germ-line transmission of mutant mtDNAs from embryonic stem cell-derived chimeric mice.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Targeted disruption of mouse conventional kinesin heavy chain, kif5B, results in abnormal perinuclear clustering of mitochondria

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Immotile sperm and infertility in mice lacking mitochondrial voltage-dependent anion channel type 3.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Neurologic dysfunction and male infertility in Drosophila porin mutants: a new model for mitochondrial dysfunction and disease

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Testis-specific cytochrome c-null mice produce functional sperm but undergo early testicular atrophy

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Depletion of selenoprotein GPx4 in spermatocytes causes male infertility in mice

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Mitochondrial glutathione peroxidase 4 disruption causes male infertility

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Short form glutathione peroxidase 4 is the essential isoform required for survival and somatic mitochondrial functions

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Developmentally regulated mitochondrial fusion mediated by a conserved, novel, predicted GTPase.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Mitochondria-related male infertility

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

A mutation in the inner mitochondrial membrane peptidase 2-like gene (Immp2l) affects mitochondrial function and impairs fertility in mice

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Mitochondrial peptidase IMMP2L mutation causes early onset of age-associated disorders and impairs adult stem cell self-renewal

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Mutations in the DBP-deficiency protein HSD17B4 cause ovarian dysgenesis, hearing loss, and ataxia of Perrault Syndrome

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

The mitochondrial connection in auditory neuropathy

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

A mutation in PNPT1, encoding mitochondrial-RNA-import protein PNPase, causes hereditary hearing loss

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Regulation of mitochondrial DNA copy number during spermatogenesis

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Is the mitochondrial cloud the selection machinery for preferentially transmitting wild-type mtDNA between generations? Rewinding Müller's ratchet efficiently

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Mitochondrial DNA depletion syndromes--many genes, common mechanisms

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Control of bacteriophage mu lysogenic repression.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Immune and non-immune functions of the immunoproteasome

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Primary follicular dystrophy with scarring dermatitis in C57BL/6 mouse substrains resembles central centrifugal cicatricial alopecia in humans

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Autophagy proteins regulate innate immune responses by inhibiting the release of mitochondrial DNA mediated by the NALP3 inflammasome

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

SCID dogs: similar transplant potential but distinct intra-uterine growth defects and premature replicative senescence compared with SCID mice

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Interleukin 10 regulates inflammatory cytokine synthesis to protect against lipopolysaccharide-induced abortion and fetal growth restriction in mice.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Large-scale gene trapping in C57BL/6N mouse embryonic stem cells.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Analysis of Relative Gene Expression Data Using Real-Time Quantitative PCR and the 2−ΔΔCT Method

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Proteolytic processing of OPA1 links mitochondrial dysfunction to alterations in mitochondrial morphology

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Bioconductor: open software development for computational biology and bioinformatics

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Summaries of Affymetrix GeneChip probe level data

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

DNA extraction procedures meaningfully influence qPCR-based mtDNA copy number determination

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Impaired learning and motor behavior in heterozygous Pafah1b1 (Lis1) mutant mice

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

K+-independent actions of diazoxide question the role of inner membrane KATP channels in mitochondrial cytoprotective signaling.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Mitochondrial dysfunction: an early event in Alzheimer pathology accumulates with age in AD transgenic mice

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Blue native PAGE

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Quantification of muscle mitochondrial oxidative phosphorylation enzymes via histochemical staining of blue native polyacrylamide gels.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Functional assays in high-resolution clear native gels to quantify mitochondrial complexes in human biopsies and cell lines

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

SPICE: exploration and analysis of post-cytometric complex multivariate datasets

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Exercise early in life in rats born small does not normalize reductions in skeletal muscle PGC-1α in adulthood

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

New Insights into Staphylococcus aureus Stress Tolerance and Virulence Regulation from an Analysis of the Role of the ClpP Protease in the Strains Newman, COL, and SA564

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Proteomic Profiling of ClpXP Substrates after DNA Damage Reveals Extensive Instability within SOS Regulon

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Import into Mitochondria, Folding and Retrograde Movement of Fumarase in Yeast

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDT338

21 January 2018

Practical pathology of aging mice

1 reference

https://pubmed.ncbi.nlm.nih.gov/23851121

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Distinctive types of ATP-dependent Clp proteases in cyanobacteria

1 reference

https://pubmed.ncbi.nlm.nih.gov/23851121

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The mitochondrion--a Trojan horse that kicks off inflammation?

1 reference

https://pubmed.ncbi.nlm.nih.gov/23851121

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

A retrospective study of idiopathic ulcerative dermatitis in mice with a C57BL/6 background.

1 reference

https://pubmed.ncbi.nlm.nih.gov/23851121

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Perrault syndrome: Evidence for progressive nervous system involvement

1 reference

https://pubmed.ncbi.nlm.nih.gov/23851121

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Morphogenesis of the mitochondrial alterations in muscle diseases

1 reference

https://pubmed.ncbi.nlm.nih.gov/23851121

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Characterization of Chloroplast Clp proteins in Arabidopsis: Localization, tissue specificity and stress responses

1 reference

https://pubmed.ncbi.nlm.nih.gov/23851121

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

gamma-glutamyltranspeptidase-deficient knockout mice as a model to study the relationship between glutathione status, mitochondrial function, and cellular function

1 reference

https://pubmed.ncbi.nlm.nih.gov/23851121

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Maintenance of mitochondrial genome distribution by mitochondrial AAA+ protein ClpX

1 reference

https://pubmed.ncbi.nlm.nih.gov/23851121

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Mitochondrial DNA mutations in disease and aging.

1 reference

https://pubmed.ncbi.nlm.nih.gov/23851121

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Linear models and empirical bayes methods for assessing differential expression in microarray experiments

1 reference

https://pubmed.ncbi.nlm.nih.gov/23851121

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Infection with cytotoxic T-lymphocyte escape mutants results in increased mortality and growth retardation in mice infected with a neurotropic coronavirus.

1 reference

https://pubmed.ncbi.nlm.nih.gov/23851121

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Identifiers

10.1093/HMG/DDT338

1 reference

Europe PubMed Central

PubMed publication ID

1 August 2017

PubMed publication ID

1 reference

Europe PubMed Central

PubMed publication ID

1 August 2017

ResearchGate publication ID

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