Structure and function of the AAA+ ATPase p97/Cdc48p.
Di Xia,Wai Kwan Tang,Yihong Ye +2 more
TLDR
This review summarizes the current understanding of the structure and function of this essential cellular chaperoning system.About:
This article is published in Gene.The article was published on 2016-05-25 and is currently open access. It has received 137 citations till now. The article focuses on the topics: AAA proteins & Proteasome.read more
Citations
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Journal ArticleDOI
Molecular Mechanism of Substrate Processing by the Cdc48 ATPase Complex
TL;DR: In this paper, the Cdc48 ATPase and its cofactors extract polyubiquitinated proteins from membranes or macromolecular complexes, but how they perform these functions is unclear.
ComponentDOI
Substrate processing by the Cdc48 ATPase complex is initiated by ubiquitin unfolding
Edward C. Twomey,Zhejian Ji,Thomas E. Wales,Nicholas O. Bodnar,Scott B. Ficarro,Scott B. Ficarro,Jarrod A. Marto,Jarrod A. Marto,John R. Engen,Tom A. Rapoport +9 more
TL;DR: Cryo-EM structures of the Cdc48 ATPase in complex with Ufd1/Npl4 and poly-ubiquitinated substrate show that the CDC48 complex initiates substrate processing by unfolding a ubiquitin molecule.
Journal ArticleDOI
Folding and Misfolding of Human Membrane Proteins in Health and Disease: From Single Molecules to Cellular Proteostasis
Justin T. Marinko,Hui Huang,Wesley D. Penn,John A. Capra,Jonathan P. Schlebach,Charles R. Sanders +5 more
TL;DR: This review comprehensively outline current perspectives on the folding and misfolding of integral MPs as well as the mechanisms of cellular MP quality control and highlights new opportunities for innovations that bridge the molecular understanding of the energetics of MP folding with the nuanced complexity of biological systems.
Journal ArticleDOI
Multiprotein complexes governing Wnt signal transduction.
Melissa V. Gammons,Mariann Bienz +1 more
TL;DR: Recent advances that have highlighted mechanistic principles governing the assembly and function of three multiprotein complexes that have key roles in transducing Wnt signals from the plasma membrane to the cell nucleus are focused on.
Journal ArticleDOI
A Mighty "Protein Extractor" of the Cell: Structure and Function of the p97/CDC48 ATPase.
TL;DR: The current understanding of the structure and function of this important cellular machinery is summarized and the relevant clinical implications are discussed.
References
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Journal ArticleDOI
Inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia is caused by mutant valosin-containing protein.
Giles D. J. Watts,Jill Wymer,Margaret J. Kovach,Sarju G. Mehta,Steven Mumm,Daniel Darvish,Alan Pestronk,Michael P. Whyte,Virginia Kimonis +8 more
TL;DR: Identification of VCP as causing IBMPFD has important implications for other inclusion-body diseases, including myopathies, dementias and Paget disease of bone (PDB), as it may define a new common pathological ubiquitin-based pathway.
Journal ArticleDOI
Proteasome and p97 mediate mitophagy and degradation of mitofusins induced by Parkin
Atsushi Tanaka,Megan M. Cleland,Shan-Shan Xu,Derek P. Narendra,Der-Fen Suen,Mariusz Karbowski,Richard J. Youle +6 more
TL;DR: The Parkin ubiquitin ligase marks the mitofusins Mfn1 and Mfn2 for proteasome-dependent degradation, promoting disposal of damaged mitochondria by preventing their fusion with healthy organelles.
Journal ArticleDOI
Exome Sequencing Reveals VCP Mutations as a Cause of Familial ALS
Janel O. Johnson,Jessica Mandrioli,Michael Benatar,Yevgeniya Abramzon,Vivianna M. Van Deerlin,John Q. Trojanowski,J. Raphael Gibbs,J. Raphael Gibbs,Maura Brunetti,Susan Gronka,Joanne Wuu,Jinhui Ding,Leo McCluskey,Maria Martinez-Lage,Dana Falcone,Dena G. Hernandez,Dena G. Hernandez,Sampath Arepalli,Sean Chong,Jennifer C. Schymick,Jeffrey D. Rothstein,Francesco Landi,Yong Dong Wang,Andrea Calvo,Gabriele Mora,Mario Sabatelli,Maria Rosaria Monsurrò,Stefania Battistini,Fabrizio Salvi,Rossella Spataro,Patrizia Sola,Giuseppe Borghero,Giuliana Galassi,Sonja W. Scholz,Sonja W. Scholz,J. Paul Taylor,Gabriella Restagno,Adriano Chiò,Bryan J. Traynor,Bryan J. Traynor +39 more
TL;DR: Exome sequencing data broaden the phenotype of IBMPFD to include motor neuron degeneration, suggest that VCP mutations may account for ∼1%-2% of familial ALS, and provide evidence directly implicating defects in the ubiquitination/protein degradation pathway in motor neurons degeneration.
partners transport proteins from the ER into the cytosol
TL;DR: This work proposes that the Cdc48/p97–Ufd1–Npl4 complex extracts proteins from the ER membrane for cytosolic degradation, and demonstrates that it requires the interacting partners Ufd1 and Npl4.
Journal ArticleDOI
The AAA ATPase Cdc48/p97 and its partners transport proteins from the ER into the cytosol
TL;DR: In this paper, it was shown that the Cdc48/p97-Ufd1/Npl4 complex can extract proteins from the endoplasmic reticulum for cytosolic degradation.
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