Comprehensive structural characterization of the human AAA+ disaggregase CLPB in the apo- and substrate-bound states reveals a unique mode of action driven by oligomerization

Cryo-EM structures of human p97 double hexamer capture potentiated ATPase-competent state

(2022) Haishan Gao et al.   Cell Discovery

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

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

Selective TnsC recruitment enhances the fidelity of RNA-guided transposition

(2022) Florian T. Hoffmann et al.   NATURE

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

(2022) Ryan R. Cupo et al.   Cell Reports

Structural dynamics of AAA + ATPase Drg1 and mechanism of benzo-diazaborine inhibition

(2022) Chengying Ma et al.   Nature Communications

Gene Expression Changes and Associated Pathways Involved in the Progression of Prostate Cancer Advanced Stages

(2021) Elena A. Pudova et al.   Frontiers in Genetics

Affinity Capture of p97 with Small-Molecule Ligand Bait Reveals a 3.6 Å Double-Hexamer Cryoelectron Microscopy Structure

(2021) Md Rejaul Hoq et al.   ACS Nano

Dual proteome-scale networks reveal cell-specific remodeling of the human interactome

(2021) Edward L. Huttlin et al.   CELL

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

Common mutations of AAA ATPase p97 and inhibitor binding disrupt inter-domain coupling and subsequent allosteric activation

(2021) Brian Caffrey et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Highly accurate protein structure prediction with AlphaFold

(2021) John Jumper et al.   NATURE

Mechanistic insight into substrate processing and allosteric inhibition of human p97

(2021) Man Pan et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Cryo-electron microscopy structures of VCP/p97 reveal a new mechanism of oligomerization regulation

(2021) Guimei Yu et al.   iScience

Structures of AAA protein translocase Bcs1 suggest translocation mechanism of a folded protein

(2020) Wai Kwan Tang et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Structure of the Bcs1 AAA-ATPase suggests an airlock-like translocation mechanism for folded proteins

(2020) Lukas Kater et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

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

(2020) Jasenko Zivanov et al.   IUCrJ

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

ClpL is a functionally active tetradecameric AAA+ chaperone, distinct from hexameric/dodecameric ones

(2020) Gyuhee Kim et al.   FASEB JOURNAL

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

The Cryo-EM structure of AAV2 Rep68 in complex with ssDNA reveals a malleable AAA+ machine that can switch between oligomeric states

(2020) Vishaka Santosh et al.   NUCLEIC ACIDS RESEARCH

Cryo-EM structure of the essential ribosome assembly AAA-ATPase Rix7

(2019) Yu-Hua Lo et al.   Nature Communications

Targeting mitochondrial structure sensitizes acute myeloid leukemia to Venetoclax treatment

(2019) Xufeng Chen et al.   Cancer Discovery

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

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

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

Stairway to translocation: AAA+ motor structures reveal the mechanisms of ATP‐dependent substrate translocation

(2019) Stephanie N. Gates et al.   PROTEIN SCIENCE

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

(2019) Aaron Botham et al.   PROTEOMICS

Mechanical Protein Unfolding and Degradation

(2018) Adrian O. Olivares et al.   Annual Review of Physiology

Protein misfolding, aggregation, and conformational strains in neurodegenerative diseases

(2018) Claudio Soto et al.   NATURE NEUROSCIENCE

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

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

(2017) Stephanie N. Gates et al.   SCIENCE

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

(2016) Ayca Kiykim et al.   CLINICAL IMMUNOLOGY

Gctf: Real-time CTF determination and correction

(2016) Kai Zhang   JOURNAL OF STRUCTURAL 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

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

Targeting protein aggregation for the treatment of degenerative diseases

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

The renaissance of mitochondrial pH

(2012) Jaime Santo-Domingo et al.   JOURNAL OF GENERAL PHYSIOLOGY

An introduction to data reduction: space-group determination, scaling and intensity statistics

(2011) Philip R. Evans   ACTA CRYSTALLOGRAPHICA SECTION D-BIOLOGICAL CRYSTALLOGRAPHY

XDS

(2010) Wolfgang Kabsch   ACTA CRYSTALLOGRAPHICA SECTION D-BIOLOGICAL CRYSTALLOGRAPHY

PHENIX: a comprehensive Python-based system for macromolecular structure solution

(2010) Paul D. Adams et al.   ACTA CRYSTALLOGRAPHICA SECTION D-BIOLOGICAL CRYSTALLOGRAPHY

Structure and function of the molecular chaperone Hsp104 from yeast

(2009) Valerie Grimminger-Marquardt et al.   BIOPOLYMERS

Adapting the machine: adaptor proteins for Hsp100/Clp and AAA+ proteases

(2009) Janine Kirstein et al.   NATURE REVIEWS MICROBIOLOGY