4.5 Article

The VCP/p97 system at a glance: connecting cellular function to disease pathogenesis

Journal

JOURNAL OF CELL SCIENCE
Volume 127, Issue 18, Pages 3877-3883

Publisher

COMPANY BIOLOGISTS LTD
DOI: 10.1242/jcs.093831

Keywords

ALS; Cdc48; IBMPFD; VCP; p97; Ubiquitin

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Funding

  1. Deutsche Forschungsgemeinschaft
  2. Muscular Dystrophy Association
  3. National Institutes of Health

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The ATPase valosin-containing protein (VCP)/p97 has emerged as a central and important element of the ubiquitin system. Together with a network of cofactors, it regulates an ever-expanding range of processes that stretch into almost every aspect of cellular physiology. Its main role in proteostasis and key functions in signaling pathways are of relevance to degenerative diseases and genomic stability. In this Cell Science at a Glance and the accompanying poster, we give a brief overview of this complex system. In addition, we discuss the pathogenic basis for VCP/p97-associated diseases and then highlight in more detail new exciting links to the translational stress response and RNA biology that further underscore the significance of the VCP/p97 system.

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