Unique structural features govern the activity of a human mitochondrial AAA+ disaggregase, Skd3

Chaperones directly and efficiently disperse stress-triggered biomolecular condensates

(2022) Haneul Yoo et al.   MOLECULAR CELL

Human mitochondrial AAA+ ATPase SKD3/CLPB assembles into nucleotide-stabilized dodecamers

(2022) Zachary Spaulding et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

AAA+ proteins: one motor, multiple ways to work

(2022) JiaBei Lin et al.   BIOCHEMICAL SOCIETY TRANSACTIONS

(Dis)Solving the problem of aberrant protein states

(2021) Charlotte M. Fare et al.   Disease Models & Mechanisms

Heterozygous Variants of CLPB are a Cause of Severe Congenital Neutropenia

(2021) Julia T Warren et al.   BLOOD

Neutropenia and intellectual disability are hallmarks of biallelic and de novo CLPB deficiency

(2021) Saskia B. Wortmann et al.   GENETICS IN MEDICINE

Highly accurate protein structure prediction with AlphaFold

(2021) John Jumper et al.   NATURE

DAXX represents a new type of protein-folding enabler

(2021) Liangqian Huang et al.   NATURE

AlphaFold Protein Structure Database: massively expanding the structural coverage of protein-sequence space with high-accuracy models

(2021) Mihaly Varadi et al.   NUCLEIC ACIDS RESEARCH

Structures of the ATP-fueled ClpXP proteolytic machine bound to protein substrate

(2020) Xue Fei et al.   eLife

CLPB (caseinolytic peptidase B homolog), the first mitochondrial protein refoldase associated with human disease

(2020) Dagmara Mróz et al.   BIOCHIMICA ET BIOPHYSICA ACTA-GENERAL SUBJECTS

The mutational constraint spectrum quantified from variation in 141,456 humans

(2020) Konrad J. Karczewski et al.   NATURE

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

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

Functional analysis of proposed substrate-binding residues of Hsp104

(2020) Matthew K. Howard et al.   PLoS One

Skd3 (human ClpB) is a potent mitochondrial protein disaggregase that is inactivated by 3-methylglutaconic aciduria-linked mutations

(2020) Ryan R Cupo et al.   eLife

A High-Density Human Mitochondrial Proximity Interaction Network

(2020) Hana Antonicka et al.   Cell Metabolism

Human CLPB forms ATP-dependent complexes in the mitochondrial intermembrane space

(2020) Indhujah Thevarajan et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

UCSF ChimeraX : Structure visualization for researchers, educators, and developers

(2020) Eric F. Pettersen et al.   PROTEIN SCIENCE

UniProt: the universal protein knowledgebase in 2021

(2020) et al.   NUCLEIC ACIDS RESEARCH

TRIM11 Prevents and Reverses Protein Aggregation and Rescues a Mouse Model of Parkinson’s Disease

(2020) Guixin Zhu et al.   Cell Reports

Spiraling in Control: Structures and Mechanisms of the Hsp104 Disaggregase

(2019) James Shorter et al.   Cold Spring Harbor Perspectives in Biology

Hsp104 and Potentiated Variants Can Operate as Distinct Nonprocessive Translocases

(2019) Clarissa L. Durie et al.   BIOPHYSICAL JOURNAL

Targeting mitochondrial structure sensitizes acute myeloid leukemia to Venetoclax treatment

(2019) Xufeng Chen et al.   Cancer Discovery

The EMBL-EBI search and sequence analysis tools APIs in 2019

(2019) Fábio Madeira et al.   NUCLEIC ACIDS RESEARCH

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

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

Macromolecular structure determination using X-rays, neutrons and electrons: recent developments in Phenix

(2019) Dorothee Liebschner et al.   Acta Crystallographica Section D-Structural Biology

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

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

Probing the rice Rubisco–Rubisco activase interaction via subunit heterooligomerization

(2019) Devendra Shivhare et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Global Interactome Mapping of Mitochondrial Intermembrane Space Proteases Identifies a Novel Function for HTRA2

(2019) Aaron Botham et al.   PROTEOMICS

SWISS-MODEL: homology modelling of protein structures and complexes

(2018) Andrew Waterhouse et al.   NUCLEIC ACIDS RESEARCH

cisTEM, user-friendly software for single-particle image processing

(2018) Timothy Grant et al.   eLife

ISOLDE: a physically realistic environment for model building into low-resolution electron-density maps

(2018) Tristan Ian Croll   Acta Crystallographica Section D-Structural Biology

PARL deficiency in mouse causes Complex III defects, coenzyme Q depletion, and Leigh-like syndrome

(2018) Marco Spinazzi et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Structure of Calcarisporiella thermophila Hsp104 Disaggregase that Antagonizes Diverse Proteotoxic Misfolding Events

(2018) Karolina Michalska et al.   STRUCTURE

A scoring system predicting the clinical course of CLPB defect based on the foetal and neonatal presentation of 31 patients

(2017) Ewa Pronicka et al.   JOURNAL OF INHERITED METABOLIC DISEASE

PARL mediates Smac proteolytic maturation in mitochondria to promote apoptosis

(2017) Shotaro Saita et al.   NATURE CELL BIOLOGY

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

(2017) Shawn Q Zheng et al.   NATURE METHODS

cryoSPARC: algorithms for rapid unsupervised cryo-EM structure determination

(2017) Ali Punjani et al.   NATURE METHODS

Single-Dose Gene-Replacement Therapy for Spinal Muscular Atrophy

(2017) Jerry R. Mendell et al.   NEW ENGLAND JOURNAL OF MEDICINE

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

Severe congenital neutropenias

(2017) Julia Skokowa et al.   Nature Reviews Disease Primers

Novel CLPB mutation in a patient with 3-methylglutaconic aciduria causing severe neurological involvement and congenital neutropenia

(2016) Ayca Kiykim et al.   CLINICAL IMMUNOLOGY

Adenosine diphosphate restricts the protein remodeling activity of the Hsp104 chaperone to Hsp70 assisted disaggregation

(2016) Agnieszka Kłosowska et al.   eLife

Automated structure refinement of macromolecular assemblies from cryo-EM maps using Rosetta

(2016) Ray Yu-Ruei Wang et al.   eLife

CLPB Variants Associated with Autosomal-Recessive Mitochondrial Disorder with Cataract, Neutropenia, Epilepsy, and Methylglutaconic Aciduria

(2015) Carol Saunders et al.   AMERICAN JOURNAL OF HUMAN GENETICS

CLPB Mutations Cause 3-Methylglutaconic Aciduria, Progressive Brain Atrophy, Intellectual Disability, Congenital Neutropenia, Cataracts, Movement Disorder

(2015) Saskia B. Wortmann et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Bi-allelic CLPB mutations cause cataract, renal cysts, nephrocalcinosis and 3-methylglutaconic aciduria, a novel disorder of mitochondrial protein disaggregation

(2015) Marta Kanabus et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Disruption ofCLPBis associated with congenital microcephaly, severe encephalopathy and 3-methylglutaconic aciduria

(2015) José-Mario Capo-Chichi et al.   JOURNAL OF MEDICAL GENETICS

The Hsp104 N-Terminal Domain Enables Disaggregase Plasticity and Potentiation

(2015) Elizabeth A. Sweeny et al.   MOLECULAR CELL

Structural and Energetic Characterization of the Ankyrin Repeat Protein Family

(2015) R. Gonzalo Parra et al.   PLoS Computational Biology

Metabolic and Chaperone Gene Loss Marks the Origin of Animals: Evidence for Hsp104 and Hsp78 Chaperones Sharing Mitochondrial Enzymes as Clients

(2015) Albert J. Erives et al.   PLoS One

Potentiated Hsp104 Variants Antagonize Diverse Proteotoxic Misfolding Events

(2014) Meredith E. Jackrel et al.   CELL

Proteomic Mapping of the Human Mitochondrial Intermembrane Space in Live Cells via Ratiometric APEX Tagging

(2014) Victoria Hung et al.   MOLECULAR CELL

Mechanism of an ATP-independent Protein Disaggregase

(2013) Peera Jaru-Ampornpan et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

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

(2013) H.-W. Rhee et al.   SCIENCE

Hsp104 Suppresses Polyglutamine-Induced Degeneration Post Onset in a Drosophila MJD/SCA3 Model

(2013) Mimi Cushman-Nick et al.   PLoS Genetics

Operational Plasticity Enables Hsp104 to Disaggregate Diverse Amyloid and Nonamyloid Clients

(2012) Morgan E. DeSantis et al.   CELL

Features and development ofCoot

(2010) P. Emsley et al.   ACTA CRYSTALLOGRAPHICA SECTION D-BIOLOGICAL CRYSTALLOGRAPHY

ATP-independent reversal of a membrane protein aggregate by a chloroplast SRP subunit

(2010) Peera Jaru-Ampornpan et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Visualization of ATP levels inside single living cells with fluorescence resonance energy transfer-based genetically encoded indicators

(2009) H. Imamura et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The C-terminal Extension ofSaccharomyces cerevisiaeHsp104 Plays a Role in Oligomer Assembly†

(2008) Ryder G. Mackay et al.   BIOCHEMISTRY

Hsp104 antagonizes α-synuclein aggregation and reduces dopaminergic degeneration in a rat model of Parkinson disease

(2008) Christophe Lo Bianco et al.   JOURNAL OF CLINICAL INVESTIGATION

Coupling and Dynamics of Subunits in the Hexameric AAA+ Chaperone ClpB

(2008) Nicolas D. Werbeck et al.   JOURNAL OF MOLECULAR BIOLOGY