Mitochondrial Lon protease at the crossroads of oxidative stress, ageing and cancer

CODAS Syndrome Is Associated with Mutations of LONP1, Encoding Mitochondrial AAA+ Lon Protease

(2015) Kevin A. Strauss et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Mutations inLONP1, a mitochondrial matrix protease, cause CODAS syndrome

(2015) Esra Dikoglu et al.   AMERICAN JOURNAL OF MEDICAL GENETICS PART A

Mitochondrial (dys)function - a factor underlying the variability of efavirenz-induced hepatotoxicity?

(2015) M Polo et al.   BRITISH JOURNAL OF PHARMACOLOGY

Transcriptional activation of LON Gene by a new form of mitochondrial stress: A role for the nuclear respiratory factor 2 in StAR overload response (SOR)

(2015) Assaf Bahat et al.   MOLECULAR AND CELLULAR ENDOCRINOLOGY

Inhibition of Lon blocks cell proliferation, enhances chemosensitivity by promoting apoptosis and decreases cellular bioenergetics of bladder cancer: potential roles of Lon as a prognostic marker and therapeutic target in baldder cancer

(2015) Yongzhang Liu et al.   Oncotarget

Adaptation of leukemia cells to hypoxic condition through switching the energy metabolism or avoiding the oxidative stress

(2014) Mineaki Goto et al.   BMC CANCER

Oxidative Post-Translational Modifications Develop LONP1 Dysfunction in Pressure Overload Heart Failure

(2014) Atsushi Hoshino et al.   Circulation-Heart Failure

Aging with HIV infection: A journey to the center of inflammAIDS, immunosenescence and neuroHIV

(2014) Milena Nasi et al.   IMMUNOLOGY LETTERS

Mitochondrial biogenesis-associated factors underlie the magnitude of response to aerobic endurance training in rats

(2014) Orsolya Marton et al.   PFLUGERS ARCHIV-EUROPEAN JOURNAL OF PHYSIOLOGY

Sirtuin-3 (SIRT3), a therapeutic target with oncogenic and tumor-suppressive function in cancer

(2014) Y Chen et al.   Cell Death & Disease

Inhibition of human mitochondrial peptide deformylase causes apoptosis in c-myc-overexpressing hematopoietic cancers

(2014) A Sheth et al.   Cell Death & Disease

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

(2014) Pedro M. Quirós et al.   Cell Reports

Crystallization and preliminary X-ray diffraction analysis of the α subdomain of Lon protease fromBrevibacillus thermoruber

(2013) Yu-Da Chen et al.   Acta Crystallographica Section F-Structural Biology and Crystallization Communications

Effect of Lon protease knockdown on mitochondrial function in HeLa cells

(2013) Aurélien Bayot et al.   BIOCHIMIE

Proteotoxic Stress Induces a Cell-Cycle Arrest by Stimulating Lon to Degrade the Replication Initiator DnaA

(2013) Kristina Jonas et al.   CELL

StAR Enhances Transcription of Genes Encoding the Mitochondrial Proteases Involved in Its Own Degradation

(2013) Assaf Bahat et al.   MOLECULAR ENDOCRINOLOGY

Disrupting Autophagy Restores Peroxisome Function to an Arabidopsis lon2 Mutant and Reveals a Role for the LON2 Protease in Peroxisomal Matrix Protein Degradation

(2013) L. M. Farmer et al.   PLANT CELL

Down-Regulating Overexpressed Human Lon in Cervical Cancer Suppresses Cell Proliferation and Bioenergetics

(2013) Xiaobo Nie et al.   PLoS One

Distinct quaternary structures of the AAA+ Lon protease control substrate degradation

(2013) E. F. Vieux et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

(2013) H. Teng et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Label-free quantitative proteomics of the lysine acetylome in mitochondria identifies substrates of SIRT3 in metabolic pathways

(2013) M. J. Rardin et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

(2013) C-W Cheng et al.   Cell Death & Disease

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

(2013) Jenny K. Ngo et al.   Redox Biology

Age-associated declines in mitochondrial biogenesis and protein quality control factors are minimized by exercise training

(2012) Erika Koltai et al.   AMERICAN JOURNAL OF PHYSIOLOGY-REGULATORY INTEGRATIVE AND COMPARATIVE PHYSIOLOGY

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

(2012) S. H. Bernstein et al.   BLOOD

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

(2012) Nika Erjavec et al.   FREE RADICAL BIOLOGY AND MEDICINE

Peroxisomal Proteostasis Involves a Lon Family Protein That Functions as Protease and Chaperone

(2012) Magdalena Bartoszewska et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Diphenylarsinic Acid Promotes Degradation of Glutaminase C by Mitochondrial Lon Protease

(2012) Kayoko Kita et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Phosphorylation of Human TFAM in Mitochondria Impairs DNA Binding and Promotes Degradation by the AAA+ Lon Protease

(2012) Bin Lu et al.   MOLECULAR CELL

Biological Roles of the Podospora anserina Mitochondrial Lon Protease and the Importance of Its N-Domain

(2012) Céline Adam et al.   PLoS One

The Lon Protease Is Essential for Full Virulence in Pseudomonas aeruginosa

(2012) Elena B. M. Breidenstein et al.   PLoS One

Proteomic Investigations of Lysine Acetylation Identify Diverse Substrates of Mitochondrial Deacetylase Sirt3

(2012) Eri Maria Sol et al.   PLoS One

Aerobic Glycolysis: Meeting the Metabolic Requirements of Cell Proliferation

(2011) Sophia Y. Lunt et al.   Annual Review of Cell and Developmental Biology

Multitasking in the mitochondrion by the ATP-dependent Lon protease

(2011) Sundararajan Venkatesh et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Hallmarks of Cancer: The Next Generation

(2011) Douglas Hanahan et al.   CELL

Two Proteases, Trypsin Domain-containing 1 (Tysnd1) and Peroxisomal Lon Protease (PsLon), Cooperatively Regulate Fatty Acid β-Oxidation in Peroxisomal Matrix

(2011) Kanji Okumoto et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Lon Peptidase 1 (LONP1)-dependent Breakdown of Mitochondrial 5-Aminolevulinic Acid Synthase Protein by Heme in Human Liver Cells

(2011) Qing Tian et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Targeting cancer metabolism: a therapeutic window opens

(2011) Matthew G. Vander Heiden   NATURE REVIEWS DRUG DISCOVERY

Structure of the N-terminal fragment ofEscherichia coliLon protease

(2010) Mi Li et al.   ACTA CRYSTALLOGRAPHICA SECTION D-BIOLOGICAL CRYSTALLOGRAPHY

Protein modification and replicative senescence of WI-38 human embryonic fibroblasts

(2010) Emad K. Ahmed et al.   AGING CELL

Upregulation of nuclear-encoded mitochondrial LON protease in HAART-treated HIV-positive patients with lipodystrophy: implications for the pathogenesis of the disease

(2010) Marcello Pinti et al.   AIDS

Diverse functions of mitochondrial AAA+ proteins: protein activation, disaggregation, and degradationThis paper is one of a selection of papers published in this special issue entitled 8th International Conference on AAA Proteins and has undergone the Journal's usual peer review process.

(2010) Kaye N. Truscott et al.   Biochemistry and Cell Biology

Obtusilactone A and (−)-sesamin induce apoptosis in human lung cancer cells by inhibiting mitochondrial Lon protease and activating DNA damage checkpoints

(2010) Hui-Min Wang et al.   CANCER SCIENCE

Crystal structure of Lon protease: molecular architecture of gated entry to a sequestered degradation chamber

(2010) Sun-Shin Cha et al.   EMBO JOURNAL

SDHA is a tumor suppressor gene causing paraganglioma

(2010) Nelly Burnichon et al.   HUMAN MOLECULAR GENETICS

Identification of Novel Oxidized Protein Substrates and Physiological Partners of the Mitochondrial ATP-dependent Lon-like Protease Pim1

(2010) Aurélien Bayot et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Crystal Structures of Bacillus subtilis Lon Protease

(2010) Ramona E. Duman et al.   JOURNAL OF MOLECULAR BIOLOGY

Activation of the mitochondrial protein quality control system and actin cytoskeletal alterations in cells harbouring the MELAS mitochondrial DNA mutation

(2010) Sandra Felk et al.   JOURNAL OF THE NEUROLOGICAL SCIENCES

Functional characterization of the promoter of the human Lon protease gene

(2010) Marcello Pinti et al.   MITOCHONDRION

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

(2010) Y. Matsushima et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Oxidized Mitochondrial Protein Degradation and Repair in Aging and Oxidative Stress

(2009) Nicolas Ugarte et al.   ANTIOXIDANTS & REDOX SIGNALING

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

(2009) Blanche Guillon et al.   FEBS Journal

Mitochondrial Lon protease is a human stress protein

(2009) Jenny K. Ngo et al.   FREE RADICAL BIOLOGY AND MEDICINE

Increasing organismal healthspan by enhancing mitochondrial protein quality control

(2009) Karin Luce et al.   NATURE CELL BIOLOGY

Arabidopsis LON2 Is Necessary for Peroxisomal Function and Sustained Matrix Protein Import

(2009) M. J. Lingard et al.   PLANT PHYSIOLOGY

In-Depth Proteome Analysis of Arabidopsis Leaf Peroxisomes Combined with in Vivo Subcellular Targeting Verification Indicates Novel Metabolic and Regulatory Functions of Peroxisomes

(2009) S. Reumann et al.   PLANT PHYSIOLOGY

Evidence That Two ATP-Dependent (Lon) Proteases in Borrelia burgdorferi Serve Different Functions

(2009) James L. Coleman et al.   PLoS Pathogens

Mitochondrial protein quality control: Implications in ageing

(2008) Bertrand Friguet et al.   Biotechnology Journal

Mitochondria—A Nexus for Aging, Calorie Restriction, and Sirtuins?

(2008) Leonard Guarente   CELL

The Biology of Cancer: Metabolic Reprogramming Fuels Cell Growth and Proliferation

(2008) Ralph J. DeBerardinis et al.   Cell Metabolism

Endurance Exercise as a Countermeasure for Aging

(2008) I. R. Lanza et al.   DIABETES

Contribution of Peroxisome-specific Isoform of Lon Protease in Sorting PTS1 Proteins to Peroxisomes

(2008) S. Omi et al.   JOURNAL OF BIOCHEMISTRY

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

(2008) J. Hansen et al.   NEUROSCIENCE

Role of Lon1 protease in post-germinative growth and maintenance of mitochondrial function in Arabidopsis thaliana

(2008) Stamatis Rigas et al.   NEW PHYTOLOGIST

Towards the control of intracellular protein turnover: Mitochondrial Lon protease inhibitors versus proteasome inhibitors

(2007) Aurélien Bayot et al.   BIOCHIMIE