NDLI: Altered cofactor regulation with disease-associated p97/VCP mutations

Content Provider	PubMed Central
Author	Zhang, Xiaoyi Gui, Lin Zhang, Xiaoyan Bulfer, Stacie L. Sanghez, Valentina Wong, Daniel E. Lee, Youjin Lehmann, Lynn Lee, James Siho Shih, Pei-yin Lin, Henry J. Iacovino, Michelina Weihl, Conrad C. Arkin, Michelle R. Wang, Yanzhuang Chou, Tsui-fen
Abstract	Age-associated degenerative diseases have similar pathogenic mechanisms related to defects in protein homeostasis. p97/VCP (valosin-containing protein) is essential for coordinating protein degradation and is mutated in a multisystem degenerative disease that affects the central nervous system, muscle, and bone. p97/VCP is an enzyme in the AAA ATPases (ATPases associated with diverse cellular activities) family, which takes apart ATP and uses this energy to perform pivotal functions. We found that p97/VCP cofactors control its enzymatic activity. p97/VCP disease mutants behave abnormally due to lack of appropriate control by these cofactors. Correcting the function of the disease-associated proteins may be a desirable approach to developing safe treatment for fatal degenerative diseases. The next steps are to screen and characterize large panels of compounds to identify potential drugs that may correct the malfunction.
Related Links	http://dx.doi.org/10.1073/pnas.1418820112
Starting Page	1705
File Format	PDF
ISSN	00278424
e-ISSN	10916490
Journal	Proceedings of the National Academy of Sciences of the United States of America
Issue Number	14
Volume Number	112
Language	English
Publisher	National Academy of Sciences
Publisher Date	2015-04-07
Access Restriction	Open
Rights Holder	National Academy of Sciences
Subject Keyword	General Research in Higher Education
Content Type	Text
Resource Type	Article
Subject	Multidisciplinary

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1	Ministry of Education (GoI), Department of Higher Education	Sanctioning Authority	https://www.education.gov.in/ict-initiatives
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3	National Digital Library of India Office, Indian Institute of Technology Kharagpur	The administrative and infrastructural headquarters of the project	Dr. B. Sutradhar bsutra@ndl.gov.in
4	Project PI / Joint PI	Principal Investigator and Joint Principal Investigators of the project	Dr. B. Sutradhar bsutra@ndl.gov.in Prof. Saswat Chakrabarti will be added soon
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Altered cofactor regulation with disease-associated p97/VCP mutations.

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Altered cofactor regulation with disease-associated p97/VCP mutations

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Altered cofactor regulation with disease-associated p97/VCP mutations.

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