(Q38477484)

3 September 2017

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title

New roles for mitochondrial proteases in health, ageing and disease (English)

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3 September 2017

3

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3 September 2017

Pedro M Quirós

1

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author name string

Thomas Langer

series ordinal

2

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PubMed ID

25970558

3 September 2017

language of work or name

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13 May 2015

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Nature Reviews Molecular Cell Biology

3 September 2017

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3 September 2017

16

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3 September 2017

6

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3 September 2017

345-359

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https://scigraph.springernature.com/pub.10.1038/nrm3984

3 September 2017

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21 January 2018

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Protein degradation within mitochondria: versatile activities of AAA proteases and other peptidases

21 January 2018

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Proteolytic control of mitochondrial function and morphogenesis

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Mitochondrial quality control: a matter of life and death for neurons

21 January 2018

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Mitochondrial matrix proteases as novel therapeutic targets in malignancy.

21 January 2018

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Mitochondrial proteases and cancer

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The hallmarks of aging

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The Degradome database: mammalian proteases and diseases of proteolysis

21 January 2018

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MEROPS: the database of proteolytic enzymes, their substrates and inhibitors

21 January 2018

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A mitochondrial protein compendium elucidates complex I disease biology

21 January 2018

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Molecular and functional analyses of the human and mouse genes encoding AFG3L1, a mitochondrial metalloprotease homologous to the human spastic paraplegia protein

21 January 2018

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Chaperone-protease networks in mitochondrial protein homeostasis

21 January 2018

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Mitochondrial protein import: from proteomics to functional mechanisms

21 January 2018

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Mitochondrial processing peptidases

21 January 2018

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Processing peptidases in mitochondria and chloroplasts

21 January 2018

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Glycine-rich loop of mitochondrial processing peptidase alpha-subunit is responsible for substrate recognition by a mechanism analogous to mitochondrial receptor Tom20.

21 January 2018

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A mitochondrial protease with two catalytic subunits of nonoverlapping specificities.

21 January 2018

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Mitochondrial inner membrane protease promotes assembly of presequence translocase by removing a carboxy-terminal targeting sequence.

21 January 2018

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Mitochondrial protein turnover: role of the precursor intermediate peptidase Oct1 in protein stabilization

21 January 2018

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Global analysis of the mitochondrial N-proteome identifies a processing peptidase critical for protein stability

21 January 2018

1 reference

An unusual peptide deformylase features in the human mitochondrial N-terminal methionine excision pathway

21 January 2018

1 reference

Mitochondrial AAA proteases--towards a molecular understanding of membrane-bound proteolytic machines

21 January 2018

1 reference

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

21 January 2018

1 reference

YME1L controls the accumulation of respiratory chain subunits and is required for apoptotic resistance, cristae morphogenesis, and cell proliferation

21 January 2018

1 reference

Nonsense mutations in the COX1 subunit impair the stability of respiratory chain complexes rather than their assembly

21 January 2018

1 reference

Early complex I assembly defects result in rapid turnover of the ND1 subunit

21 January 2018

1 reference

The ATP-dependent Lon protease of Mus musculus is a DNA-binding protein that is functionally conserved between yeast and mammals

21 January 2018

2 references

https://pubmed.ncbi.nlm.nih.gov/25970558

21 January 2018

inferred from PubMed ID database lookup

12 December 2020

based on heuristic

Multitasking in the mitochondrion by the ATP-dependent Lon protease

1 reference

Oxygen-sensitive mitochondrial accumulation of cystathionine β-synthase mediated by Lon protease

21 January 2018

1 reference

Mitochondrial matrix proteostasis is linked to hereditary paraganglioma: LON-mediated turnover of the human flavinylation factor SDH5 is regulated by its interaction with SDHA.

21 January 2018

1 reference

Diphenylarsinic acid promotes degradation of glutaminase C by mitochondrial Lon protease

21 January 2018

1 reference

HIF-1 regulates cytochrome oxidase subunits to optimize efficiency of respiration in hypoxic cells

21 January 2018

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Lon protease preferentially degrades oxidized mitochondrial aconitase by an ATP-stimulated mechanism

21 January 2018

1 reference

Turnover of mitochondrial steroidogenic acute regulatory (StAR) protein by Lon protease: the unexpected effect of proteasome inhibitors.

21 January 2018

1 reference

Lon peptidase 1 (LONP1)-dependent breakdown of mitochondrial 5-aminolevulinic acid synthase protein by heme in human liver cells

21 January 2018

1 reference

Mitochondrial Lon protease regulates mitochondrial DNA copy number and transcription by selective degradation of mitochondrial transcription factor A (TFAM)

21 January 2018

1 reference

ATP-Dependent Lon Protease Controls Tumor Bioenergetics by Reprogramming Mitochondrial Activity

21 January 2018

1 reference

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

21 January 2018

1 reference

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

21 January 2018

1 reference

Role of the novel metallopeptidase Mop112 and saccharolysin for the complete degradation of proteins residing in different subcompartments of mitochondria

21 January 2018

1 reference

Amyloid-β peptide induces mitochondrial dysfunction by inhibition of preprotein maturation.

21 January 2018

1 reference

Degradation of the amyloid beta-protein by the novel mitochondrial peptidasome, PreP

21 January 2018

1 reference

Characterization of a mitochondrial metallopeptidase reveals neurolysin as a homologue of thimet oligopeptidase

21 January 2018

1 reference

HTRA proteases: regulated proteolysis in protein quality control

21 January 2018

1 reference

HtrA2 deficiency causes mitochondrial uncoupling through the F₁F₀-ATP synthase and consequent ATP depletion

21 January 2018

1 reference

Prohibitins interact genetically with Atp23, a novel processing peptidase and chaperone for the F1Fo-ATP synthase.

21 January 2018

1 reference

Regulation of mitochondrial phospholipids by Ups1/PRELI-like proteins depends on proteolysis and Mdm35.

21 January 2018

1 reference

AFG3L2 supports mitochondrial protein synthesis and Purkinje cell survival

21 January 2018

1 reference

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

21 January 2018

1 reference

Purkinje neuron Ca2+ influx reduction rescues ataxia in SCA28 model

21 January 2018

1 reference

Stress-regulated translational attenuation adapts mitochondrial protein import through Tim17A degradation.

21 January 2018

1 reference

TRIAP1/PRELI complexes prevent apoptosis by mediating intramitochondrial transport of phosphatidic acid

21 January 2018

1 reference

Phosphorylation of human TFAM in mitochondria impairs DNA binding and promotes degradation by the AAA+ Lon protease

21 January 2018

1 reference

DNA and RNA binding by the mitochondrial lon protease is regulated by nucleotide and protein substrate

21 January 2018

1 reference

HtrA2/Omi deficiency causes damage and mutation of mitochondrial DNA.

21 January 2018

1 reference

Qri7/OSGEPL, the mitochondrial version of the universal Kae1/YgjD protein, is essential for mitochondrial genome maintenance

21 January 2018

1 reference

LACTB is a filament-forming protein localized in mitochondria

21 January 2018

1 reference

Mitonuclear protein imbalance as a conserved longevity mechanism

21 January 2018

1 reference

Surveillance-Activated Defenses Block the ROS–Induced Mitochondrial Unfolded Protein Response

21 January 2018

1 reference

A mitochondrial specific stress response in mammalian cells

21 January 2018

1 reference

The mitochondrial unfolded protein response, a conserved stress response pathway with implications in health and disease

21 January 2018

1 reference

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

21 January 2018

1 reference

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

21 January 2018

1 reference

Multilayered genetic and omics dissection of mitochondrial activity in a mouse reference population

21 January 2018

1 reference

StAR enhances transcription of genes encoding the mitochondrial proteases involved in its own degradation

21 January 2018

1 reference

Mitochondrial dysfunction triggered by loss of HtrA2 results in the activation of a brain-specific transcriptional stress response

21 January 2018

1 reference

Mitochondrial form and function

21 January 2018

1 reference

Mitochondrial dynamics and inheritance during cell division, development and disease

21 January 2018

1 reference

The i-AAA protease YME1L and OMA1 cleave OPA1 to balance mitochondrial fusion and fission

21 January 2018

1 reference

Regulation of mitochondrial morphology through proteolytic cleavage of OPA1

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

Proteolytic cleavage of Opa1 stimulates mitochondrial inner membrane fusion and couples fusion to oxidative phosphorylation

21 January 2018

1 reference

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

21 January 2018

1 reference

Loss of mitochondrial protease OMA1 alters processing of the GTPase OPA1 and causes obesity and defective thermogenesis in mice

21 January 2018

1 reference

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

21 January 2018

1 reference

New roles for OMA1 metalloprotease: From mitochondrial proteostasis to metabolic homeostasis

21 January 2018

1 reference

Dynamic survey of mitochondria by ubiquitin.

21 January 2018

1 reference

A small natural molecule promotes mitochondrial fusion through inhibition of the deubiquitinase USP30

21 January 2018

1 reference

Regulation of mitochondrial morphology by USP30, a deubiquitinating enzyme present in the mitochondrial outer membrane

21 January 2018

1 reference

21 January 2018

Mechanisms of mitophagy

1 reference

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

21 January 2018

1 reference

Mitochondrial processing peptidase regulates PINK1 processing, import and Parkin recruitment

21 January 2018

1 reference

Mitochondrial membrane potential regulates PINK1 import and proteolytic destabilization by PARL

21 January 2018

1 reference

PINK1-Parkin pathway activity is regulated by degradation of PINK1 in the mitochondrial matrix

21 January 2018

1 reference

Inactivation of Omi/HtrA2 protease leads to the deregulation of mitochondrial Mulan E3 ubiquitin ligase and increased mitophagy

21 January 2018

1 reference

The serine protease HtrA2/Omi cleaves Parkin and irreversibly inactivates its E3 ubiquitin ligase activity

21 January 2018

1 reference

Parkin, PINK1, and DJ-1 form a ubiquitin E3 ligase complex promoting unfolded protein degradation

21 January 2018

1 reference

Rhomboid protease PARL mediates the mitochondrial membrane potential loss-induced cleavage of PGAM5

21 January 2018

1 reference

A regulatory signaling loop comprising the PGAM5 phosphatase and CK2 controls receptor-mediated mitophagy.

21 January 2018

1 reference

The mitochondrial phosphatase PGAM5 functions at the convergence point of multiple necrotic death pathways

21 January 2018

1 reference

Impaired OMA1-dependent cleavage of OPA1 and reduced DRP1 fission activity combine to prevent mitophagy in cells that are dependent on oxidative phosphorylation

21 January 2018

1 reference

Proteolytic processing of Atg32 by the mitochondrial i-AAA protease Yme1 regulates mitophagy

21 January 2018

1 reference

Self-consumption: the interplay of autophagy and apoptosis

21 January 2018

1 reference

Activation of mitochondrial protease OMA1 by Bax and Bak promotes cytochrome c release during apoptosis

21 January 2018

1 reference

Stress-induced OMA1 activation and autocatalytic turnover regulate OPA1-dependent mitochondrial dynamics

21 January 2018

1 reference

OMA1 mediates OPA1 proteolysis and mitochondrial fragmentation in experimental models of ischemic kidney injury

21 January 2018

1 reference

Hax1-mediated processing of HtrA2 by Parl allows survival of lymphocytes and neurons

21 January 2018

1 reference

A serine protease, HtrA2, is released from the mitochondria and interacts with XIAP, inducing cell death

21 January 2018

1 reference

The Wilms' tumor suppressor protein WT1 is processed by the serine protease HtrA2/Omi

21 January 2018

1 reference

The proteases HtrA2/Omi and UCH-L1 regulate TNF-induced necroptosis

21 January 2018

1 reference

Alternative germ cell death pathway in Drosophila involves HtrA2/Omi, lysosomes, and a caspase-9 counterpart

21 January 2018

1 reference

Mitochondrial rhomboid PARL regulates cytochrome c release during apoptosis via OPA1-dependent cristae remodeling

21 January 2018

1 reference

OPA1 controls apoptotic cristae remodeling independently from mitochondrial fusion

21 January 2018

1 reference

The role of mitochondria in aging

21 January 2018

1 reference

Mitochondrial protein quality control during biogenesis and aging

21 January 2018

1 reference

Oxidative stress and mitochondrial protein quality control in aging

21 January 2018

1 reference

Importance of the lon protease in mitochondrial maintenance and the significance of declining lon in aging.

21 January 2018

1 reference

Upregulation of the mitochondrial Lon Protease allows adaptation to acute oxidative stress but dysregulation is associated with chronic stress, disease, and aging.

21 January 2018

1 reference

Increasing organismal healthspan by enhancing mitochondrial protein quality control.

21 January 2018

1 reference

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

21 January 2018

1 reference

Loss of HtrA2/Omi activity in non-neuronal tissues of adult mice causes premature aging

21 January 2018

1 reference

The mitochondrial protease AFG3L2 is essential for axonal development

21 January 2018

1 reference

Evolutionary conserved longevity genes and human cognitive abilities in elderly cohorts

21 January 2018

1 reference

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

21 January 2018

1 reference

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

21 January 2018

1 reference

Oxidative stress is involved in age-dependent spermatogenic damage of Immp2l mutant mice

21 January 2018

1 reference

Mitochondrial aminopeptidase deletion increases chronological lifespan and oxidative stress resistance while decreasing respiratory metabolism in S. cerevisiae

21 January 2018

1 reference

Monogenic mitochondrial disorders

21 January 2018

1 reference

Mutations in the DJ-1 gene associated with autosomal recessive early-onset parkinsonism

21 January 2018

1 reference

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

21 January 2018

1 reference

Mitochondrial serine protease HTRA2 p.G399S in a kindred with essential tremor and Parkinson disease

21 January 2018

1 reference

Loss of Omi mitochondrial protease activity causes the neuromuscular disorder of mnd2 mutant mice

21 January 2018

1 reference

Neuroprotective role of the Reaper-related serine protease HtrA2/Omi revealed by targeted deletion in mice

21 January 2018

1 reference

Functional alteration of PARL contributes to mitochondrial dysregulation in Parkinson's disease

21 January 2018

1 reference

The role of PARL and HtrA2 in striatal neuronal injury after transient global cerebral ischemia

21 January 2018

1 reference

Intragenic deletions affecting two alternative transcripts of the IMMP2L gene in patients with Tourette syndrome.

21 January 2018

1 reference

A novel approach of homozygous haplotype sharing identifies candidate genes in autism spectrum disorder

21 January 2018

1 reference

Interstitial 7q31.1 copy number variations disrupting IMMP2L gene are associated with a wide spectrum of neurodevelopmental disorders.

21 January 2018

1 reference

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

21 January 2018

1 reference

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

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 SPG7 gene cause chronic progressive external ophthalmoplegia through disordered mitochondrial DNA maintenance

21 January 2018

1 reference

Spastic paraplegia type 7 is associated with multiple mitochondrial DNA deletions

21 January 2018

1 reference

Axonal degeneration in paraplegin-deficient mice is associated with abnormal mitochondria and impairment of axonal transport

21 January 2018

1 reference

Genetic interaction between the m-AAA protease isoenzymes reveals novel roles in cerebellar degeneration

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

Haploinsufficiency of AFG3L2, the gene responsible for spinocerebellar ataxia type 28, causes mitochondria-mediated Purkinje cell dark degeneration.

21 January 2018

1 reference

SPG7 variant escapes phosphorylation-regulated processing by AFG3L2, elevates mitochondrial ROS, and is associated with multiple clinical phenotypes

21 January 2018

1 reference

Regulation of skeletal muscle oxidative capacity and insulin signaling by the mitochondrial rhomboid protease PARL.

21 January 2018

1 reference

Neurolysin knockout mice generation and initial phenotype characterization

21 January 2018

1 reference

Variations in DNA elucidate molecular networks that cause disease

21 January 2018

1 reference

CODAS syndrome is associated with mutations of LONP1, encoding mitochondrial AAA+ Lon protease.

21 January 2018

1 reference

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

21 January 2018

1 reference

Mitochondrial complex III deficiency caused by a homozygous UQCRC2 mutation presenting with neonatal-onset recurrent metabolic decompensation

21 January 2018

1 reference

Individuals with mutations in XPNPEP3, which encodes a mitochondrial protein, develop a nephronophthisis-like nephropathy

21 January 2018

1 reference

Hallmarks of Cancer: The Next Generation

21 January 2018

1 reference

21 January 2018

Mitochondria and cancer

1 reference

Down-regulating overexpressed human Lon in cervical cancer suppresses cell proliferation and bioenergetics

21 January 2018

1 reference

Overexpression of Lon contributes to survival and aggressive phenotype of cancer cells through mitochondrial complex I-mediated generation of reactive oxygen species.

21 January 2018

1 reference

Silencing of mitochondrial Lon protease deeply impairs mitochondrial proteome and function in colon cancer cells

21 January 2018

1 reference

Effect of Lon protease knockdown on mitochondrial function in HeLa cells

21 January 2018

1 reference

The mitochondrial ATP-dependent Lon protease: a novel target in lymphoma death mediated by the synthetic triterpenoid CDDO and its derivatives

21 January 2018

1 reference

Lon protease: A key enzyme controlling mitochondrial bioenergetics in cancer

21 January 2018

1 reference

p53-mediated activation of the mitochondrial protease HtrA2/Omi prevents cell invasion

21 January 2018

1 reference

p53 is required for cisplatin-induced processing of the mitochondrial fusion protein L-Opa1 that is mediated by the mitochondrial metallopeptidase Oma1 in gynecologic cancers

21 January 2018

1 reference

MAP1D, a novel methionine aminopeptidase family member is overexpressed in colon cancer

21 January 2018

1 reference

Genome-wide association study on differentiated thyroid cancer

21 January 2018

1 reference

Targeting capacity and conservation of PreP homologues localization in mitochondria of different species

21 January 2018

1 reference

The Parkinson's disease protein DJ-1 is neuroprotective due to cysteine-sulfinic acid-driven mitochondrial localization

21 January 2018

1 reference

Biogenesis of the cytochrome bc(1) complex and role of assembly factors.

21 January 2018

1 reference

The identification of novel ovarian proteases through the use of genomic and bioinformatic methodologies

21 January 2018

1 reference

Calpains, mitochondria, and apoptosis

21 January 2018

1 reference

Calpain 10: a mitochondrial calpain and its role in calcium-induced mitochondrial dysfunction

21 January 2018

1 reference

The enigma of caspase-2: the laymen's view

21 January 2018

1 reference

Caspases 3 and 7: key mediators of mitochondrial events of apoptosis

21 January 2018

1 reference

Caspase-8 and bid: caught in the act between death receptors and mitochondria

21 January 2018

1 reference

Pre-processed caspase-9 contained in mitochondria participates in apoptosis

21 January 2018

1 reference

USP8 regulates mitophagy by removing K6-linked ubiquitin conjugates from parkin

21 January 2018

1 reference

Deubiquitinase USP9X stabilizes MCL1 and promotes tumour cell survival

21 January 2018

1 reference

The deubiquitinase USP15 antagonizes Parkin-mediated mitochondrial ubiquitination and mitophagy

21 January 2018

1 reference

Disruption of SUMO-specific protease 2 induces mitochondria mediated neurodegeneration

21 January 2018

1 reference

The SUMO protease SENP5 is required to maintain mitochondrial morphology and function

21 January 2018

1 reference

A cryptic mitochondrial targeting motif in Atg4D links caspase cleavage with mitochondrial import and oxidative stress

21 January 2018

1 reference

Alternative translation initiation generates a novel isoform of insulin-degrading enzyme targeted to mitochondria

21 January 2018

1 reference

Nardilysin facilitates complex formation between mitochondrial malate dehydrogenase and citrate synthase

21 January 2018

1 reference

Granzyme A cleaves a mitochondrial complex I protein to initiate caspase-independent cell death

21 January 2018

1 reference

Deregulation of mitochondrial membrane potential by mitochondrial insertion of granzyme B and direct Hax-1 cleavage

21 January 2018

1 reference

The regulatory crosstalk between kinases and proteases in cancer

21 January 2018

1 reference

Isolation and identification of a novel mitochondrial metalloprotease (PreP) that degrades targeting presequences in plants

31 March 2020

1 reference

The mitochondrial deubiquitinase USP30 opposes parkin-mediated mitophagy

31 March 2020

1 reference

Deletion of the mitochondrial Pim1/Lon protease in yeast results in accelerated aging and impairment of the proteasome

31 March 2020

1 reference

Axonal degeneration in paraplegin-deficient mice is associated with abnormal mitochondria and impairment of axonal transport

31 March 2020

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/25970558

inferred from PubMed ID database lookup

12 December 2020

based on heuristic

Identifiers

DOI

10.1038/NRM3984

1 reference

PubMed ID

25970558

Dimensions Publication ID

3 September 2017

0 references

1 reference

PubMed ID

25970558