Associated Papers
Deposition of CENP-A Cse4 is enhanced by mutations in the AAA+ ATPase domain of ATAD2 Yta7.
Kaur N et al.
Genetics, 2026
Proteasome inhibition by VR23 enhances autophagic clearance of FUSP525L-mediated persistent stress granule in SH-SY5Y cells.
Kim SH et al.
Molecular Brain, 2026
Aberrant nuclear pore complex degradation contributes to neurodegeneration in VCP disease.
Dubey SK et al.
Neuron, 2025
Cardiomyopathy in valosin-containing protein multisystem proteinopathy: Evaluation, diagnosis, and management.
Chan JM et al.
American Heart Journal Plus : Cardiology Research and Practice, 2025
Antisense oligonucleotides targeting valosin-containing protein ameliorate muscle pathology and molecular defects in cell and mouse models of multisystem proteinopathy.
Pal P et al.
Clinical and Translational Medicine, 2025
Challenges of modelling TDP-43 pathology in mice.
Armas JMB et al.
Mammalian Genome : Official Journal of the International Mammalian Genome Society, 2025
Targeting mitophagy in neurodegenerative diseases.
Antico O et al.
Nature Reviews. Drug Discovery, 2025
2024 VCP International Conference: Exploring multi-disciplinary approaches from basic science of valosin containing protein, an AAA+ ATPase protein, to the therapeutic advancement for VCP-associated multisystem proteinopathy.
Peck A et al.
Neurobiology of Disease, 2025
A systematic review and functional in-silico analysis of genes and variants associated with amyotrophic lateral sclerosis.
Arreola-Aldape CA et al.
Frontiers in Neuroscience, 2025
Loss of function of VCP/TER94 causes neurodegeneration.
Tsumaki K et al.
Disease Models & Mechanisms, 2024
Tau accumulation is cleared by the induced expression of VCP via autophagy.
Giong HK et al.
Acta Neuropathologica, 2024
VCP/p97 UFMylation stabilizes BECN1 and facilitates the initiation of autophagy.
Wang Z et al.
Autophagy, 2024
Fatty links between multisystem proteinopathy and small VCP-interacting protein.
Ramzan F et al.
Cell Death Discovery, 2024
VCP activator reverses nuclear proteostasis defects and enhances TDP-43 aggregate clearance in multisystem proteinopathy models.
Phan JM et al.
The Journal of Clinical Investigation, 2024
Valosin-Containing Protein (VCP): A Review of Its Diverse Molecular Functions and Clinical Phenotypes.
Pontifex CS et al.
International Journal of Molecular Sciences, 2024
p37 regulates VCP/p97 shuttling and functions in the nucleus and cytosol.
Wrobel L et al.
Science Advances, 2024
Elevated 4R tau contributes to endolysosomal dysfunction and neurodegeneration in VCP-related frontotemporal dementia.
Hung C et al.
Brain : a Journal of Neurology, 2024
Astrocyte-Neuron Interactions Contributing to Amyotrophic Lateral Sclerosis Progression.
Jensen BK
Advances in Neurobiology, 2024
Amplifying the Heat Shock Response Ameliorates ALS and FTD Pathology in Mouse and Human Models.
Ahmed M et al.
Molecular Neurobiology, 2023
An autosomal-dominant childhood-onset disorder associated with pathogenic variants in VCP.
Mah-Som AY et al.
American Journal of Human Genetics, 2023
Therapeutic developments for valosin-containing protein mediated multisystem proteinopathy.
Boock V et al.
Current Opinion in Neurology, 2023
Clinical Classification of Variants in the Valosin-Containing Protein Gene Associated With Multisystem Proteinopathy.
Schiava M et al.
Neurology. Genetics, 2023
Sex influences clinical phenotype in valosin-containing protein mutations: A case family report and systematic literature review.
Leccese D et al.
Clinical Neurology and Neurosurgery, 2023
Valosin containing protein (VCP): initiator, modifier, and potential drug target for neurodegenerative diseases.
Chu S et al.
Molecular Neurodegeneration, 2023
Yeast Models of Amyotrophic Lateral Sclerosis Type 8 Mimic Phenotypes Seen in Mammalian Cells Expressing Mutant VAPBP56S.
Stump AL et al.
Biomolecules, 2023
Novel VCP activator reverses multisystem proteinopathy nuclear proteostasis defects and enhances TDP-43 aggregate clearance.
Phan JM et al.
Biorxiv : the Preprint Server for Biology, 2023
Novel Variants in the VCP Gene Causing Multisystem Proteinopathy 1.
Columbres RCA et al.
Genes, 2023
Prevalence of Frontotemporal Dementia in Females of 5 Hispanic Families With R159H VCP Multisystem Proteinopathy.
Shmara A et al.
Neurology. Genetics, 2023
Autophagy and neurodegeneration: Unraveling the role of C9ORF72 in the regulation of autophagy and its relationship to ALS-FTD pathology.
Diab R et al.
Frontiers in Cellular Neuroscience, 2023
VCP-related myopathy: a case series and a review of literature.
Iannibelli E et al.
Acta Myologica : Myopathies and Cardiomyopathies : Official Journal of the Mediterranean Society of Myology, 2023
Skeletal muscle cell protein dysregulation highlights the pathogenesis mechanism of myopathy-associated p97/VCP R155H mutations.
Luzzi A et al.
Frontiers in Neurology, 2023
Selenoprotein S: A versatile disordered protein.
Ghelichkhani F et al.
Archives of Biochemistry and Biophysics, 2022
The functional importance of VCP to maintaining cellular protein homeostasis.
Ahlstedt BA et al.
Biochemical Society Transactions, 2022
Proteostasis impairment and ALS.
Lambert-Smith IA et al.
Progress in Biophysics and Molecular Biology, 2022
A clinicopathologic study of malignancy in VCP-associated multisystem proteinopathy.
Shmara A et al.
Orphanet Journal of Rare Diseases, 2022
Autophagy Dysfunction in ALS: from Transport to Protein Degradation.
Cozzi M et al.
Journal of Molecular Neuroscience : Mn, 2022
CRISPR/Cas9-Mediated Constitutive Loss of VCP (Valosin-Containing Protein) Impairs Proteostasis and Leads to Defective Striated Muscle Structure and Function In Vivo.
Voisard P et al.
International Journal of Molecular Sciences, 2022
AAA-ATPase valosin-containing protein binds the transcription factor SREBP1 and promotes its proteolytic activation by rhomboid protease RHBDL4.
Shibuya K et al.
The Journal of Biological Chemistry, 2022
Multisystem Proteinopathy Due to VCP Mutations: A Review of Clinical Heterogeneity and Genetic Diagnosis.
Pfeffer G et al.
Genes, 2022
Astrocytes display cell autonomous and diverse early reactive states in familial amyotrophic lateral sclerosis.
Taha DM et al.
Brain : a Journal of Neurology, 2022
VCP suppresses proteopathic seeding in neurons.
Zhu J et al.
Molecular Neurodegeneration, 2022
Clinical, Biochemical, Radiological, and Genetic Analyses of a Patient with VCP Gene Variant-Induced Paget's Disease of Bone.
Zhang Y et al.
Calcified Tissue International, 2022
Valosin Containing Protein (VCP): A Multistep Regulator of Autophagy.
Ferrari V et al.
International Journal of Molecular Sciences, 2022
Phenotype of VCP Mutations in Chinese Amyotrophic Lateral Sclerosis Patients.
Feng SY et al.
Frontiers in Neurology, 2022
The Role of VCP Mutations in the Spectrum of Amyotrophic Lateral Sclerosis-Frontotemporal Dementia.
Scarian E et al.
Frontiers in Neurology, 2022
Loss-of-function mutation in VCP mimics the characteristic pathology as in FTLD-TARDBP.
Wani A et al.
Autophagy, 2021
Nearly 30 Years of Animal Models to Study Amyotrophic Lateral Sclerosis: A Historical Overview and Future Perspectives.
Bonifacino T et al.
International Journal of Molecular Sciences, 2021