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

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

AAA+ proteins

(2020) Thiago V. Seraphim et al.   CURRENT BIOLOGY

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

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

Therapeutic Dissolution of Aberrant Phases by Nuclear-Import Receptors

(2019) Lin Guo et al.   TRENDS IN CELL BIOLOGY

Spiraling in Control: Structures and Mechanisms of the Hsp104 Disaggregase

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

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

α-Synuclein pathology in Parkinson’s disease and related α-synucleinopathies

(2019) Michael X. Henderson et al.   NEUROSCIENCE LETTERS

Mitoribosomal small subunit biogenesis in trypanosomes involves an extensive assembly machinery

(2019) Martin Saurer et al.   SCIENCE

Mining Disaggregase Sequence Space to Safely Counter TDP-43, FUS, and α-Synuclein Proteotoxicity

(2019) Amber Tariq et al.   Cell Reports

Loss of Dynamic RNA Interaction and Aberrant Phase Separation Induced by Two Distinct Types of ALS/FTD-Linked FUS Mutations

(2019) Amirhossein Ghanbari Niaki et al.   MOLECULAR CELL

An Early mtUPR: Redistribution of the Nuclear Transcription Factor Rox1 to Mitochondria Protects against Intramitochondrial Proteotoxic Aggregates

(2019) Daniel Poveda-Huertes et al.   MOLECULAR CELL

Structural and mechanistic insights into Hsp104 function revealed by synchrotron X-ray footprinting

(2019) Elizabeth A. Sweeny et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

(2019) Aaron Botham et al.   PROTEOMICS

Nuclear Import Receptor Inhibits Phase Separation of FUS through Binding to Multiple Sites

(2018) Takuya Yoshizawa et al.   CELL

Nuclear-Import Receptors Reverse Aberrant Phase Transitions of RNA-Binding Proteins with Prion-like Domains

(2018) Lin Guo et al.   CELL

Two distinct sites of client protein interaction with the chaperone cpSRP43

(2018) Camille Z. McAvoy et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Analysis of heat-induced protein aggregation in human mitochondria

(2018) Anne Wilkening et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Amyloid assembly and disassembly

(2018) Edward Chuang et al.   JOURNAL OF CELL SCIENCE

IUPred2A: context-dependent prediction of protein disorder as a function of redox state and protein binding

(2018) Bálint Mészáros et al.   NUCLEIC ACIDS RESEARCH

PANTHER version 14: more genomes, a new PANTHER GO-slim and improvements in enrichment analysis tools

(2018) Huaiyu Mi et al.   NUCLEIC ACIDS RESEARCH

The Gene Ontology Resource: 20 years and still GOing strong

(2018)   NUCLEIC ACIDS RESEARCH

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

FUS inclusions disrupt RNA localization by sequestering kinesin-1 and inhibiting microtubule detyrosination

(2017) Kyota Yasuda et al.   JOURNAL OF CELL BIOLOGY

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

20 years of the SMART protein domain annotation resource

(2017) Ivica Letunic et al.   NUCLEIC ACIDS RESEARCH

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

(2017) Stephanie N. Gates et al.   SCIENCE

Structure and Function of the Mitochondrial Ribosome

(2016) Basil J. Greber et al.   Annual Review of Biochemistry

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

(2016) Ayca Kiykim et al.   CLINICAL IMMUNOLOGY

Mechanistic Insights into Hsp104 Potentiation

(2016) Mariana P. Torrente et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Synchronized mitochondrial and cytosolic translation programs

(2016) Mary T. Couvillion et al.   NATURE

Disparate Mutations Confer Therapeutic Gain of Hsp104 Function

(2015) Meredith E. Jackrel et al.   ACS Chemical Biology

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

Reversible, Specific, Active Aggregates of Endogenous Proteins Assemble upon Heat Stress

(2015) Edward W.J. Wallace et al.   CELL

Mutation in NDUFA13/GRIM19 leads to early onset hypotonia, dyskinesia and sensorial deficiencies, and mitochondrial complex I instability

(2015) Claire Angebault et al.   HUMAN MOLECULAR 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

Crucial HSP70 co-chaperone complex unlocks metazoan protein disaggregation

(2015) Nadinath B. Nillegoda et al.   NATURE

Targeting protein aggregation for the treatment of degenerative diseases

(2015) Yvonne S. Eisele et al.   NATURE REVIEWS DRUG DISCOVERY

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

Potentiated Hsp104 variants suppress toxicity of diverse neurodegenerative disease-linked proteins

(2014) M. E. Jackrel et al.   Disease Models & Mechanisms

Megabase-scale deletion using CRISPR/Cas9 to generate a fully haploid human cell line

(2014) Patrick Essletzbichler et al.   GENOME RESEARCH

Accurate Proteome-wide Label-free Quantification by Delayed Normalization and Maximal Peptide Ratio Extraction, Termed MaxLFQ

(2014) Jürgen Cox et al.   MOLECULAR & CELLULAR PROTEOMICS

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

(2014) Victoria Hung et al.   MOLECULAR CELL

MICU1 and MICU2 Finely Tune the Mitochondrial Ca2+ Uniporter by Exerting Opposite Effects on MCU Activity

(2014) Maria Patron et al.   MOLECULAR CELL

Disruption of the PRKCD–FBXO25–HAX-1 axis attenuates the apoptotic response and drives lymphomagenesis

(2014) Ursula Baumann et al.   NATURE MEDICINE

The metazoan protein disaggregase and amyloid depolymerase system

(2014) Mariana P Torrente et al.   Prion

MICU1 Controls Both the Threshold and Cooperative Activation of the Mitochondrial Ca2+ Uniporter

(2013) György Csordás et al.   Cell Metabolism

Conserved Distal Loop Residues in the Hsp104 and ClpB Middle Domain Contact Nucleotide-binding Domain 2 and Enable Hsp70-dependent Protein Disaggregation

(2013) Morgan E. DeSantis et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mechanism of an ATP-independent Protein Disaggregase

(2013) Thang X. Nguyen et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Hsp110 Is aBona FideChaperone Using ATP to Unfold Stable Misfolded Polypeptides and Reciprocally Collaborate with Hsp70 to Solubilize Protein Aggregates

(2013) Rayees U. H. Mattoo et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mechanism of an ATP-independent Protein Disaggregase

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

MICU2, a Paralog of MICU1, Resides within the Mitochondrial Uniporter Complex to Regulate Calcium Handling

(2013) Molly Plovanich et al.   PLoS One

Heat shock protein (Hsp) 70 is an activator of the Hsp104 motor

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

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

Pathological  -Synuclein Transmission Initiates Parkinson-like Neurodegeneration in Nontransgenic Mice

(2012) K. C. Luk et al.   SCIENCE

Small Heat Shock Proteins Potentiate Amyloid Dissolution by Protein Disaggregases from Yeast and Humans

(2012) Martin L. Duennwald et al.   PLOS BIOLOGY

NDUFB7 and NDUFA8 are located at the intermembrane surface of complex I

(2011) Radek Szklarczyk et al.   FEBS LETTERS

Inactivation of the Mitochondrial Carrier SLC25A25 (ATP-Mg2+/PiTransporter) Reduces Physical Endurance and Metabolic Efficiency in Mice

(2011) Rea P. Anunciado-Koza et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mutations in TTC19 cause mitochondrial complex III deficiency and neurological impairment in humans and flies

(2011) Daniele Ghezzi et al.   NATURE GENETICS

The Mammalian Disaggregase Machinery: Hsp110 Synergizes with Hsp70 and Hsp40 to Catalyze Protein Disaggregation and Reactivation in a Cell-Free System

(2011) James Shorter   PLoS One

MICU1 encodes a mitochondrial EF hand protein required for Ca2+ uptake

(2010) Fabiana Perocchi et al.   NATURE

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

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

Enzymatic assembly of DNA molecules up to several hundred kilobases

(2009) Daniel G Gibson et al.   NATURE METHODS

Prediction of Protein Binding Regions in Disordered Proteins

(2009) Bálint Mészáros et al.   PLoS Computational Biology

Peptide and Protein Binding in the Axial Channel of Hsp104

(2008) Ronnie Lum et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

Hax1-mediated processing of HtrA2 by Parl allows survival of lymphocytes and neurons

(2008) Jyh-Rong Chao et al.   NATURE

Pore loops of the AAA+ ClpX machine grip substrates to drive translocation and unfolding

(2008) Andreas Martin et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Nucleocytoplasmic Trafficking of the Molecular Chaperone Hsp104 in Unstressed and Heat-Shocked Cells

(2007) Johnny M. Tkach et al.   TRAFFIC