LONP1 Is Required for Maturation of a Subset of Mitochondrial Proteins, and Its Loss Elicits an Integrated Stress Response

Multi-omics analysis identifies ATF4 as a key regulator of the mitochondrial stress response in mammals

(2017) Pedro M. Quirós et al.   JOURNAL OF CELL BIOLOGY

The Transcription Factor ATF5 Mediates a Mammalian Mitochondrial UPR

(2016) Christopher J. Fiorese et al.   CURRENT BIOLOGY

Mitochondrial unfolded protein response controls matrix pre-RNA processing and translation

(2016) Christian Münch et al.   NATURE

The N-terminal domain plays a crucial role in the structure of a full-length human mitochondrial Lon protease

(2016) Sami Kereïche et al.   Scientific Reports

The ubiquitin kinase PINK1 recruits autophagy receptors to induce mitophagy

(2015) Michael Lazarou et al.   NATURE

FASTKD2 is an RNA-binding protein required for mitochondrial RNA processing and translation

(2015) Johannes Popow et al.   RNA

Mitochondrial RNA Granules Are Centers for Posttranscriptional RNA Processing and Ribosome Biogenesis

(2015) Hana Antonicka et al.   Cell Reports

Effect of Lon protease knockdown on mitochondrial function in HeLa cells

(2013) Aurélien Bayot et al.   BIOCHIMIE

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

(2013) Brian F. Teske et al.   MOLECULAR BIOLOGY OF THE CELL

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

Mitochondrial processing peptidase regulates PINK1 processing, import and Parkin recruitment

(2012) Andrew W Greene et al.   EMBO REPORTS

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

(2012) Bin Lu et al.   MOLECULAR CELL

Mitochondrial Import Efficiency of ATFS-1 Regulates Mitochondrial UPR Activation

(2012) A. M. Nargund et al.   SCIENCE

Multitasking in the mitochondrion by the ATP-dependent Lon protease

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

Transcriptional and Cellular Responses to Defective Mitochondrial Proteolysis in Fission Yeast

(2011) Suranjana Guha et al.   JOURNAL OF MOLECULAR BIOLOGY

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

Mitochondrial membrane potential regulates PINK1 import and proteolytic destabilization by PARL

(2010) Seok Min Jin et al.   JOURNAL OF CELL BIOLOGY

Crystal Structures of Bacillus subtilis Lon Protease

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

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

PINK1 Is Selectively Stabilized on Impaired Mitochondria to Activate Parkin

(2010) Derek P. Narendra et al.   PLOS BIOLOGY

MTERF2 is a nucleoid component in mammalian mitochondria

(2009) Mina Pellegrini et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Elucidation of separate, but collaborative functions of the rRNA methyltransferase-related human mitochondrial transcription factors B1 and B2 in mitochondrial biogenesis reveals new insight into maternally inherited deafness

(2009) Justin Cotney et al.   HUMAN MOLECULAR GENETICS