VCP suppresses proteopathic seeding in neurons

Brazilin Removes Toxic Alpha-Synuclein and Seeding Competent Assemblies from Parkinson Brain by Altering Conformational Equilibrium

(2021) George R. Nahass et al.   JOURNAL OF MOLECULAR BIOLOGY

Neuronal VCP loss of function recapitulates FTLD-TDP pathology

(2021) Abubakar Wani et al.   Cell Reports

TDP-43 α-helical structure tunes liquid–liquid phase separation and function

(2020) Alexander E. Conicella et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The Parkinson’s Disease Protein LRRK2 Interacts with the GARP Complex to Promote Retrograde Transport to the trans-Golgi Network

(2020) Alexandra Beilina et al.   Cell Reports

Autosomal dominant VCP hypomorph mutation impairs disaggregation of PHF-tau

(2020) Nabil F. Darwich et al.   SCIENCE

VCP maintains lysosomal homeostasis and TFEB activity in differentiated skeletal muscle

(2019) Khalid Arhzaouy et al.   Autophagy

Detection of TAR DNA-binding protein 43 (TDP-43) oligomers as initial intermediate species during aggregate formation

(2019) Rachel L. French et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Unbiased Screens for Modifiers of Alpha-Synuclein Toxicity

(2019) Matthias Höllerhage et al.   Current Neurology and Neuroscience Reports

Linking the Endoplasmic Reticulum to Parkinson’s Disease and Alpha-Synucleinopathy

(2019) Emanuela Colla   Frontiers in Neuroscience

Compromised function of the ESCRT pathway promotes endolysosomal escape of tau seeds and propagation of tau aggregation

(2019) John J. Chen et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Multisystem Proteinopathy Mutations in VCP/p97 Increase NPLOC4·UFD1L Binding and Substrate Processing

(2019) Emily E. Blythe et al.   STRUCTURE

Triggered recruitment of ESCRT machinery promotes endolysosomal repair

(2018) Michael L. Skowyra et al.   SCIENCE

The heterozygous R155C VCP mutation: Toxic in humans! Harmless in mice?

(2018) Christoph S. Clemen et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

A druggable genome screen identifies modifiers of α-synuclein levels via a tiered cross-species validation approach

(2018) Maxime W.C. Rousseaux et al.   JOURNAL OF NEUROSCIENCE

Parkinson's disease and multiple system atrophy have distinct α-synuclein seed characteristics

(2018) Tritia R. Yamasaki et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Patient-derived frontotemporal lobar degeneration brain extracts induce formation and spreading of TDP-43 pathology in vivo

(2018) Sílvia Porta et al.   Nature Communications

A sensitive assay reveals structural requirements for α-synuclein fibril growth

(2017) Dhruva D. Dhavale et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Impaired endo-lysosomal membrane integrity accelerates the seeding progression of α-synuclein aggregates

(2017) Peizhou Jiang et al.   Scientific Reports

Valosin-containing protein (VCP/p97) inhibitors relieve Mitofusin-dependent mitochondrial defects due to VCP disease mutants

(2017) Ting Zhang et al.   eLife

Dementia with Lewy Bodies: Molecular Pathology in the Frontal Cortex in Typical and Rapidly Progressive Forms

(2017) Paula Garcia-Esparcia et al.   Frontiers in Neurology

TRIMs and Galectins Globally Cooperate and TRIM16 and Galectin-3 Co-direct Autophagy in Endomembrane Damage Homeostasis

(2016) Santosh Chauhan et al.   DEVELOPMENTAL CELL

VCP/p97 cooperates with YOD1, UBXD1 and PLAA to drive clearance of ruptured lysosomes by autophagy

(2016) Chrisovalantis Papadopoulos et al.   EMBO JOURNAL

Discovery of a First-in-Class, Potent, Selective, and Orally Bioavailable Inhibitor of the p97 AAA ATPase (CB-5083)

(2015) Han-Jie Zhou et al.   JOURNAL OF MEDICINAL CHEMISTRY

Spreading of pathology in neurodegenerative diseases: a focus on human studies

(2015) Johannes Brettschneider et al.   NATURE REVIEWS NEUROSCIENCE

Multisystem proteinopathy: Table

(2015) J. Paul Taylor   NEUROLOGY

Evidence for α-synuclein prions causing multiple system atrophy in humans with parkinsonism

(2015) Stanley B. Prusiner et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Alterations in stress granule dynamics driven by TDP-43 and FUS: a link to pathological inclusions in ALS?

(2015) Anaïs Aulas et al.   Frontiers in Cellular Neuroscience

Addition of exogenous α-synuclein preformed fibrils to primary neuronal cultures to seed recruitment of endogenous α-synuclein to Lewy body and Lewy neurite–like aggregates

(2014) Laura A Volpicelli-Daley et al.   Nature Protocols

ATP6V0C Knockdown in Neuroblastoma Cells Alters Autophagy-Lysosome Pathway Function and Metabolism of Proteins that Accumulate in Neurodegenerative Disease

(2014) Leandra R. Mangieri et al.   PLoS One

Eukaryotic Stress Granules Are Cleared by Autophagy and Cdc48/VCP Function

(2013) J. Ross Buchan et al.   CELL

Structure-Activity Relationship Study Reveals ML240 and ML241 as Potent and Selective Inhibitors of p97 ATPase

(2013) Tsui-Fen Chou et al.   ChemMedChem

Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS

(2013) Hong Joo Kim et al.   NATURE

Covalent and allosteric inhibitors of the ATPase VCP/p97 induce cancer cell death

(2013) Paola Magnaghi et al.   Nature Chemical Biology

Binding of the Radioligand SIL23 to α-Synuclein Fibrils in Parkinson Disease Brain Tissue Establishes Feasibility and Screening Approaches for Developing a Parkinson Disease Imaging Agent

(2013) Devika P. Bagchi et al.   PLoS One

Genotype-phenotype studies of VCP-associated inclusion body myopathy with Paget disease of bone and/or frontotemporal dementia

(2012) SG Mehta et al.   CLINICAL GENETICS

Phenotypic variability in three families withvalosin-containing proteinmutation

(2012) S. Spina et al.   EUROPEAN JOURNAL OF NEUROLOGY

Suppression of dynamin GTPase decreases α-synuclein uptake by neuronal and oligodendroglial cells: a potent therapeutic target for synucleinopathy

(2012) Masatoshi Konno et al.   Molecular Neurodegeneration

A progressive translational mouse model of human valosin-containing protein disease: TheVCPR155H/+mouse

(2012) Angèle Nalbandian et al.   MUSCLE & NERVE

Endolysosomal sorting of ubiquitylated caveolin-1 is regulated by VCP and UBXD1 and impaired by VCP disease mutations

(2011) Danilo Ritz et al.   NATURE CELL BIOLOGY

Exogenous α-Synuclein Fibrils Induce Lewy Body Pathology Leading to Synaptic Dysfunction and Neuron Death

(2011) Laura A. Volpicelli-Daley et al.   NEURON

Reversible inhibitor of p97, DBeQ, impairs both ubiquitin-dependent and autophagic protein clearance pathways

(2011) T.-F. Chou et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Valosin-containing protein disease: Inclusion body myopathy with Paget’s disease of the bone and fronto-temporal dementia

(2009) Conrad C. Weihl et al.   NEUROMUSCULAR DISORDERS

Inhibition of p97-dependent Protein Degradation by Eeyarestatin I

(2008) Qiuyan Wang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Impaired Protein Aggregate Handling and Clearance Underlie the Pathogenesis of p97/VCP-associated Disease

(2008) Jeong-Sun Ju et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

TDP-43 accumulation in inclusion body myopathy muscle suggests a common pathogenic mechanism with frontotemporal dementia

(2008) C C Weihl et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY