Franz, Andre, Ackermann, Leena ORCID: 0000-0001-6405-900X and Hoppe, Thorsten ORCID: 0000-0002-4734-9352 (2014). Create and preserve: Proteostasis in development and aging is governed by Cdc48/p97/VCP. Biochim. Biophys. Acta-Mol. Cell Res., 1843 (1). S. 205 - 216. AMSTERDAM: ELSEVIER SCIENCE BV. ISSN 1879-2596

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Abstract

The AAA-ATPase Cdc48 (also called p97 or VCP) acts as a key regulator in proteolytic pathways, coordinating recruitment and targeting of substrate proteins to the 26S proteasome or lysosomal degradation. However, in contrast to the well-known function in ubiquitin-dependent cellular processes, the physiological relevance of Cdc48 in organismic development and maintenance of protein homeostasis is less understood. Therefore, studies on multicellular model organisms help to decipher how Cdc48-dependent proteolysis is regulated in time and space to meet developmental requirements. Given the importance of developmental regulation and tissue maintenance, defects in Cdc48 activity have been linked to several human pathologies including protein aggregation diseases. Thus, addressing the underlying disease mechanisms not only contributes to our understanding on the organism-wide function of Cdc48 but also facilitates the design of specific medical therapies. In this review, we will portray the role of Cdc48 in the context of multicellular organisms, pointing out its importance for developmental processes, tissue surveillance, and disease prevention. This article is part of a Special Issue entitled: Ubiquitin-Proteasome System. Guest Editors: Thomas Sommer and Dieter H. Wolf. (C) 2013 Elsevier B.V. All rights reserved.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Franz, AndreUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Ackermann, LeenaUNSPECIFIEDorcid.org/0000-0001-6405-900XUNSPECIFIED
Hoppe, ThorstenUNSPECIFIEDorcid.org/0000-0002-4734-9352UNSPECIFIED
URN: urn:nbn:de:hbz:38-451001
DOI: 10.1016/j.bbamcr.2013.03.031
Journal or Publication Title: Biochim. Biophys. Acta-Mol. Cell Res.
Volume: 1843
Number: 1
Page Range: S. 205 - 216
Date: 2014
Publisher: ELSEVIER SCIENCE BV
Place of Publication: AMSTERDAM
ISSN: 1879-2596
Language: English
Faculty: Faculty of Mathematics and Natural Sciences
Divisions: Faculty of Mathematics and Natural Sciences > Department of Biology > Institute for Genetics
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
VALOSIN-CONTAINING-PROTEIN; INCLUSION-BODY MYOPATHY; SELECTIVE CHAPERONE CDC-48/P97; MITOCHONDRIAL QUALITY-CONTROL; ENDOPLASMIC-RETICULUM STRESS; P97 AAA-ATPASE; NF-KAPPA-B; CAENORHABDITIS-ELEGANS; FRONTOTEMPORAL DEMENTIA; AURORA-BMultiple languages
Biochemistry & Molecular Biology; Cell BiologyMultiple languages
Refereed: Yes
URI: http://kups.ub.uni-koeln.de/id/eprint/45100

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