Structures of the human LONP1 protease reveal regulatory steps involved in protease activation

LONP1 and mtHSP70 cooperate to promote mitochondrial protein folding

(2021) Chun-Shik Shin et al.   Nature Communications

A processive rotary mechanism couples substrate unfolding and proteolysis in the ClpXP degradation machinery

(2020) Zev A Ripstein et al.   eLife

Estimation of high-order aberrations and anisotropic magnification from cryo-EM data sets in RELION-3.1

(2020) Jasenko Zivanov et al.   IUCrJ

Conformational plasticity of the ClpAP AAA+ protease couples protein unfolding and proteolysis

(2020) Kyle E. Lopez et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

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

(2020) Mia Shin et al.   Science Advances

Quality control of the mitochondrial proteome

(2020) Jiyao Song et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Non-uniform refinement: adaptive regularization improves single-particle cryo-EM reconstruction

(2020) Ali Punjani et al.   NATURE METHODS

Unique Structural Features of the Mitochondrial AAA+ Protease AFG3L2 Reveal the Molecular Basis for Activity in Health and Disease

(2019) Cristina Puchades et al.   MOLECULAR CELL

Structure of the Cdc48 segregase in the act of unfolding an authentic substrate

(2019) Ian Cooney et al.   SCIENCE

Substrate processing by the Cdc48 ATPase complex is initiated by ubiquitin unfolding

(2019) Edward C. Twomey et al.   SCIENCE

Structural basis for substrate gripping and translocation by the ClpB AAA+ disaggregase

(2019) Alexandrea N. Rizo et al.   Nature Communications

The molecular principles governing the activity and functional diversity of AAA+ proteins

(2019) Cristina Puchades et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

SWISS-MODEL: homology modelling of protein structures and complexes

(2018) Andrew Waterhouse et al.   NUCLEIC ACIDS RESEARCH

Real-space refinement in PHENIX for cryo-EM and crystallography

(2018) Pavel V. Afonine et al.   Acta Crystallographica Section D-Structural Biology

Substrate-engaged 26S proteasome structures reveal mechanisms for ATP-hydrolysis–driven translocation

(2018) Andres H. de la Peña et al.   SCIENCE

New tools for automated high-resolution cryo-EM structure determination in RELION-3

(2018) Jasenko Zivanov et al.   eLife

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

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

MotionCor2: anisotropic correction of beam-induced motion for improved cryo-electron microscopy

(2017) Shawn Q Zheng et al.   NATURE METHODS

Achieving better-than-3-Å resolution by single-particle cryo-EM at 200 keV

(2017) Mark A Herzik et al.   NATURE METHODS

UCSF ChimeraX: Meeting modern challenges in visualization and analysis

(2017) Thomas D. Goddard et al.   PROTEIN SCIENCE

Ratchet-like polypeptide translocation mechanism of the AAA+ disaggregase Hsp104

(2017) Stephanie N. Gates et al.   SCIENCE

Structure of the mitochondrial inner membrane AAA+ protease YME1 gives insight into substrate processing

(2017) Cristina Puchades et al.   SCIENCE

Structure of a AAA+ unfoldase in the process of unfolding substrate

(2017) Zev A Ripstein et al.   eLife

Mitochondrial Lon protease in human disease and aging: Including an etiologic classification of Lon-related diseases and disorders

(2016) Daniela A. Bota et al.   FREE RADICAL BIOLOGY AND MEDICINE

Structural Basis for the Magnesium-Dependent Activation and Hexamerization of the Lon AAA+ Protease

(2016) Shih-Chieh Su et al.   STRUCTURE

Structural Insights into the Allosteric Operation of the Lon AAA+ Protease

(2016) Chien-Chu Lin et al.   STRUCTURE

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

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

(2015) Marcello Pinti et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

CTFFIND4: Fast and accurate defocus estimation from electron micrographs

(2015) Alexis Rohou et al.   JOURNAL OF STRUCTURAL BIOLOGY

New roles for mitochondrial proteases in health, ageing and disease

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

Mapping Residual Structure in Intrinsically Disordered Proteins at Residue Resolution Using Millisecond Hydrogen/Deuterium Exchange and Residue Averaging

(2014) Theodore R. Keppel et al.   JOURNAL OF THE AMERICAN SOCIETY FOR MASS SPECTROMETRY

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

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

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

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

HDX Workbench: Software for the Analysis of H/D Exchange MS Data

(2012) Bruce D. Pascal et al.   JOURNAL OF THE AMERICAN SOCIETY FOR MASS SPECTROMETRY

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

(2012) Bin Lu et al.   MOLECULAR CELL

AAA+ Proteases: ATP-Fueled Machines of Protein Destruction

(2011) Robert T. Sauer et al.   Annual Review of Biochemistry

Multitasking in the mitochondrion by the ATP-dependent Lon protease

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

Protein unfolding and degradation by the AAA+ Lon protease

(2011) Eyal Gur et al.   PROTEIN SCIENCE

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

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

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

Determination of amide hydrogen exchange by mass spectrometry: A new tool for protein structure elucidation

(2010) Zhongqi Zhang et al.   PROTEIN SCIENCE

Structure of the catalytic domain of the human mitochondrial Lon protease: Proposed relation of oligomer formation and activity

(2010) Javier García-Nafría et al.   PROTEIN SCIENCE

Appion: An integrated, database-driven pipeline to facilitate EM image processing

(2009) Gabriel C. Lander et al.   JOURNAL OF STRUCTURAL BIOLOGY

DoG Picker and TiltPicker: Software tools to facilitate particle selection in single particle electron microscopy

(2009) N.R. Voss et al.   JOURNAL OF STRUCTURAL BIOLOGY

The crystal structure of apo-FtsH reveals domain movements necessary for substrate unfolding and translocation

(2009) C. Bieniossek et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Recognition of misfolded proteins by Lon, a AAA+ protease

(2008) E. Gur et al.   GENES & DEVELOPMENT