Emerging functions of the VCP/p97 AAA-ATPase in the ubiquitin system

Inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia is caused by mutant valosin-containing protein

Watts, G. D. J., Wymer, J., Kovach, M. J., Mehta, S. G., Mumm, S., Darvish, D., Pestronk, A., Whyte, M. P., & Kimonis, V. E. (2004) · Nature Genetics · doi:10.1038/ng1332 · Cited by 1,391

Proteasome and p97 mediate mitophagy and degradation of mitofusins induced by Parkin

Tanaka, A., Cleland, M. M., Xu, S., Narendra, D. P., Suen, D.-F., Karbowski, M., & Youle, R. J. (2010) · Journal of Cell Biology · doi:10.1083/jcb.201007013 · Cited by 1,369

Exome Sequencing Reveals VCP Mutations as a Cause of Familial ALS

Johnson, J. O., Mandrioli, J., Benatar, M., Abramzon, Y., Van Deerlin, V. M., Trojanowski, J. Q., Gibbs, J. R., Brunetti, M., Gronka, S., Wuu, J., et al. (2010) · Neuron · doi:10.1016/j.neuron.2010.11.036 · Cited by 1,235

Mutations in the endosomal ESCRTIII-complex subunit CHMP2B in frontotemporal dementia

Skibinski, G., Parkinson, N. J., Brown, J. M., Chakrabarti, L., Lloyd, S. L., Hummerich, H., Nielsen, J. E., Hodges, J. R., Spillantini, M. G., Thusgaard, T., et al. (2005) · Nature Genetics · doi:10.1038/ng1609 · Cited by 822

Valosin-containing protein (VCP) is required for autophagy and is disrupted in VCP disease

Ju, J.-S., Fuentealba, R. A., Miller, S. E., Jackson, E., Piwnica-Worms, D., Baloh, R. H., & Weihl, C. C. (2009) · Journal of Cell Biology · doi:10.1083/jcb.200908115 · Cited by 494

VCP/p97 is essential for maturation of ubiquitin-containing autophagosomes and this function is impaired by mutations that cause IBMPFD

Tresse, E., Salomons, F. A., Vesa, J., Bott, L. C., Kimonis, V., Yao, T.-P., Dantuma, N. P., & Taylor, J. P. (2010) · Autophagy · doi:10.4161/auto.6.2.11014 · Cited by 437

A Stress-Responsive System for Mitochondrial Protein Degradation

Heo, J.-M., Livnat-Levanon, N., Taylor, E. B., Jones, K. T., Dephoure, N., Ring, J., Xie, J., Brodsky, J. L., Madeo, F., Gygi, S. P., et al. (2010) · Molecular Cell · doi:10.1016/j.molcel.2010.10.021 · Cited by 304

Cdc48/p97 promotes reformation of the nucleus by extracting the kinase Aurora B from chromatin

Ramadan, K., Bruderer, R., Spiga, F. M., Popp, O., Baur, T., Gotta, M., & Meyer, H. H. (2007) · Nature · doi:10.1038/nature06388 · Cited by 261

The AAA-ATPase p97 is essential for outer mitochondrial membrane protein turnover

Xu, S., Peng, G., Wang, Y., Fang, S., & Karbowski, M. (2011) · Molecular Biology of the Cell · doi:10.1091/mbc.e10-09-0748 · Cited by 243

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

Ritz, D., Vuk, M., Kirchner, P., Bug, M., Schütz, S., Hayer, A., Bremer, S., Lusk, C., Baloh, R. H., Lee, H., et al. (2011) · Nature Cell Biology · doi:10.1038/ncb2301 · Cited by 224

The changing scene of amyotrophic lateral sclerosis

Robberecht, W., & Philips, T. (2013) · Nature Reviews Neuroscience · doi:10.1038/nrn3430 · Cited by 957

Alcohol-abuse drug disulfiram targets cancer via p97 segregase adaptor NPL4

Skrott, Z., Mistrik, M., Andersen, K. K., Friis, S., Majera, D., Gursky, J., Ozdian, T., Bartkova, J., Turi, Z., Moudry, P., et al. (2017) · Nature · doi:10.1038/nature25016 · Cited by 807

Autophagy-dependent cell death

Denton, D., & Kumar, S. (2019) · Cell Death & Differentiation · doi:10.1038/s41418-018-0252-y · Cited by 777

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

Buchan, J. R., Kolaitis, R.-M., Taylor, J. P., & Parker, R. (2013) · Cell · doi:10.1016/j.cell.2013.05.037 · Cited by 737

Mechanisms and functions of ribosome-associated protein quality control

Joazeiro, C. A. P. (2019) · Nature Reviews Molecular Cell Biology · doi:10.1038/s41580-019-0118-2 · Cited by 456

A proximity labeling strategy provides insights into the composition and dynamics of lipid droplet proteomes

Bersuker, K., Peterson, C. W. H., To, M., Sahl, S. J., Savikhin, V., Grossman, E. A., Nomura, D. K., & Olzmann, J. A. (2018) · Developmental Cell · doi:10.1016/j.devcel.2017.11.020 · Cited by 346

Autophagy-dependent cell death—where.; how and why a cell eats itself to death

Bialik, S., Dasari, S. K., & Kimchi, A. (2018) · Journal of Cell Science · doi:10.1242/jcs.215152 · Cited by 295

BOK Is a Non-canonical BCL-2 Family Effector of Apoptosis Regulated by ER-Associated Degradation

Llambi, F., Wang, Y.-M., Victor, B., Yang, M., Schneider, D. M., Gingras, S., Parsons, M. J., Zheng, J. H., Brown, S. A., Pelletier, S., et al. (2016) · Cell · doi:10.1016/j.cell.2016.02.026 · Cited by 261

Detection and Clearance of Damaged Lysosomes by the Endo-Lysosomal Damage Response and Lysophagy

Papadopoulos, C., & Meyer, H. (2017) · Current Biology · doi:10.1016/j.cub.2017.11.012 · Cited by 230

VCP recruitment to mitochondria causes mitophagy impairment and neurodegeneration in models of Huntington’s disease

Guo, X., Sun, X., Hu, D., Wang, Y.-J., Fujioka, H., Vyas, R., Chakrapani, S., Joshi, A. U., Luo, Y., Mochly-Rosen, D., et al. (2016) · Nature Communications · doi:10.1038/ncomms12646 · Cited by 200

Autophagy and ALS: mechanistic insights and therapeutic implications

Chua, J. P., De Calbiac, H., Kabashi, E., & Barmada, S. J. (2022) · Autophagy · doi:10.1080/15548627.2021.1926656 · Cited by 189

Progressive Motor Neuron Pathology and the Role of Astrocytes in a Human Stem Cell Model of VCP-Related ALS

Hall, C. E., Yao, Z., Choi, M., Tyzack, G. E., Serio, A., Luisier, R., Harley, J., Preza, E., Arber, C., Crisp, S. J., et al. (2017) · Cell Reports · doi:10.1016/j.celrep.2017.05.024 · Cited by 179

ULK1 and ULK2 Regulate Stress Granule Disassembly Through Phosphorylation and Activation of VCP/p97

Wang, B., Maxwell, B. A., Joo, J. H., Gwon, Y., Messing, J., Mishra, A., Shaw, T. I., Ward, A. L., Quan, H., Sakurada, S. M., et al. (2019) · Molecular Cell · doi:10.1016/j.molcel.2019.03.027 · Cited by 185

Pathogenic VCP Mutations Induce Mitochondrial Uncoupling and Reduced ATP Levels

Bartolome, F., Wu, H.-C., Burchell, V. S., Preza, E., Wray, S., Mahoney, C. J., Fox, N. C., Calvo, A., Canosa, A., Moglia, C., et al. (2013) · Neuron · doi:10.1016/j.neuron.2013.02.028 · Cited by 156

Modulation of the proteostasis network promotes tumor resistance to oncogenic KRAS inhibitors

Lv, X., Lu, X., Cao, J., Luo, Q., Ding, Y., Peng, F., Pataer, A., Lu, D., Han, D., Malmberg, E., et al. (2023) · Science · doi:10.1126/science.abn4180 · Cited by 123

Type 2 deiodinase polymorphism causes ER stress and hypothyroidism in the brain

Jo, S., Fonseca, T. L., Bocco, B. M. L. C., Fernandes, G. W., McAninch, E. A., Bolin, A. P., Da Conceição, R. R., Werneck-de-Castro, J. P., Ignacio, D. L., Egri, P., et al. (2018) · Journal of Clinical Investigation · doi:10.1172/jci123176 · Cited by 107

p97/VCP promotes degradation of CRBN substrate glutamine synthetase and neosubstrates

Nguyen, T. V., Li, J., Lu, C.-C. (., Mamrosh, J. L., Lu, G., Cathers, B. E., & Deshaies, R. J. (2017) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1700949114 · Cited by 85

Beyond antioxidant genes in the ancient Nrf2 regulatory network

Lacher, S. E., Lee, J. S., Wang, X., Campbell, M. R., Bell, D. A., & Slattery, M. (2015) · Free Radical Biology and Medicine · doi:10.1016/j.freeradbiomed.2015.06.044 · Cited by 77

Intracellular Cholesterol Synthesis and Transport

Shi, Q., Chen, J., Zou, X., & Tang, X. (2022) · Frontiers in Cell and Developmental Biology · doi:10.3389/fcell.2022.819281 · Cited by 73

The MTR4 helicase recruits nuclear adaptors of the human RNA exosome using distinct arch-interacting motifs

Lingaraju, M., Johnsen, D., Schlundt, A., Langer, L. M., Basquin, J., Sattler, M., Heick Jensen, T., Falk, S., & Conti, E. (2019) · Nature Communications · doi:10.1038/s41467-019-11339-x · Cited by 55

Lipid droplets as signaling platforms linking metabolic and cellular functions

Arrese, E. L., Saudale, F. Z., & Soulages, J. L. (2014) · Lipid Insights · doi:10.4137/lpi.s11128 · Cited by 54

Perturbation of ubiquitin homeostasis promotes macrophage oxidative defenses

Charbonneau, M.-E., Passalacqua, K. D., Hagen, S. E., Showalter, H. D., Wobus, C. E., & O’Riordan, M. X. D. (2019) · Scientific Reports · doi:10.1038/s41598-019-46526-9 · Cited by 24

A genome-scale drug discovery pipeline uncovers therapeutic targets and a unique p97 allosteric binding site in Schistosoma mansoni

(2025) · doi:10.1073/pnas.2505710122 · Cited by 8

Differential reliance of CTD-nuclear envelope phosphatase 1 on its regulatory subunit in ER lipid synthesis and storage

(2024) · doi:10.1091/mbc.e23-09-0382 · Cited by 8

Specific proteolysis mediated by a p97-directed proteolysis-targeting chimera (p97-PROTAC)

Salinas-Rebolledo, C., Blesa, J., Valenzuela-Nieto, G., Schwefel, D., López-González del Rey, N., Méndez-Ruette, M., Burkhalter, J., Carrazana, E., Díaz-Tejeda, F., Arias Catalán, I., et al. (2025) · eLife · doi:10.7554/elife.101496 · Cited by 3

Assessing the helical stability of polyXYs at the boundaries of intrinsically disordered regions with MD simulations

Gonçalves-Kulik, M., Baptista, L. A., Schmid, F., & Andrade-Navarro, M. A. (2025) · Computational and Structural Biotechnology Reports · doi:10.1016/j.csbr.2025.100054