Mitochondrial Lon protease is a gatekeeper for proteins newly imported into the matrix

Structural basis for distinct operational modes and protease activation in AAA+ protease Lon

(2020) Mia Shin et al.   Science Advances

Defective mitochondrial protease LonP1 can cause classical mitochondrial disease

(2018) Bradley Peter et al.   HUMAN MOLECULAR GENETICS

Mitochondrial protein transport in health and disease

(2018) Yilin Kang et al.   SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY

Large-scale aggregation analysis of eukaryotic proteins reveals an involvement of intrinsically disordered regions in protein folding

(2018) Eri Uemura et al.   Scientific Reports

Mitochondrial Chaperones in the Brain: Safeguarding Brain Health and Metabolism?

(2018) José Pedro Castro et al.   Frontiers in Endocrinology

Structural basis of mitochondrial transcription

(2018) Hauke S. Hillen et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Bi-allelic mutations of LONP1 encoding the mitochondrial LonP1 protease cause pyruvate dehydrogenase deficiency and profound neurodegeneration with progressive cerebellar atrophy

(2018) Graeme A M Nimmo et al.   HUMAN MOLECULAR GENETICS

Accurate Single-Sequence Prediction of Protein Intrinsic Disorder by an Ensemble of Deep Recurrent and Convolutional Architectures

(2018) Jack Hanson et al.   Journal of Chemical Information and Modeling

A novel mutation in the proteolytic domain of LONP1 causes atypical CODAS syndrome

(2017) Takehiko Inui et al.   JOURNAL OF HUMAN GENETICS

Acylglycerol Kinase Mutated in Sengers Syndrome Is a Subunit of the TIM22 Protein Translocase in Mitochondria

(2017) Milena Vukotic et al.   MOLECULAR CELL

Maintenance and Expression of Mammalian Mitochondrial DNA

(2016) Claes M. Gustafsson et al.   Annual Review of Biochemistry

Emerging role of Lon protease as a master regulator of mitochondrial functions

(2016) Marcello Pinti et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

The TIM23 mitochondrial protein import complex: function and dysfunction

(2016) Keren Demishtein-Zohary et al.   CELL AND TISSUE RESEARCH

Mitochondrial Quality Control Proteases in Neuronal Welfare

(2016) Roman M. Levytskyy et al.   Journal of Neuroimmune Pharmacology

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

New roles for mitochondrial proteases in health, ageing and disease

(2015) Pedro M. Quirós et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Mitochondrial Lon regulates apoptosis through the association with Hsp60–mtHsp70 complex

(2015) T-Y Kao et al.   Cell Death & Disease

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

(2015) Ayenachew Bezawork-Geleta et al.   Scientific Reports

Mutations to a glycine loop in the catalytic site of human Lon changes its protease, peptidase and ATPase activities

(2014) Ľuboš Ambro et al.   FEBS Journal

The Pyruvate Dehydrogenase Complexes: Structure-based Function and Regulation

(2014) Mulchand S. Patel et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Oxidative stress and mitochondrial protein quality control in aging

(2013) Eirini Lionaki et al.   Journal of Proteomics

Mitochondrial matrix proteases as novel therapeutic targets in malignancy

(2013) C A Goard et al.   ONCOGENE

Targeting Mitochondrial STAT3 with the Novel Phospho-Valproic Acid (MDC-1112) Inhibits Pancreatic Cancer Growth in Mice

(2013) Gerardo G. Mackenzie et al.   PLoS One

Proteomic Mapping of Mitochondria in Living Cells via Spatially Restricted Enzymatic Tagging

(2013) H.-W. Rhee et al.   SCIENCE

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

Chaperone–protease networks in mitochondrial protein homeostasis

(2012) Wolfgang Voos   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

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

(2012) Bin Lu et al.   MOLECULAR CELL

Matrix proteases in mitochondrial DNA function

(2011) Yuichi Matsushima et al.   Biochimica et Biophysica Acta-Gene Regulatory Mechanisms

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

Inactivation of brain mitochondrial Lon protease by peroxynitrite precedes electron transport chain dysfunction

(2008) Lee Stanyer et al.   NEUROCHEMISTRY INTERNATIONAL