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The Ubiquitin-Proteasome System in Huntington’s Disease : Are Proteasomes Impaired, Initiators of Disease, or Coming to the Rescue?
Joint Authors
Schipper-Krom, Sabine
Reits, Eric A. J.
Juenemann, Katrin
Source
Biochemistry Research International
Issue
Vol. 2012, Issue 2012 (31 Dec. 2012), pp.1-12, 12 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2012-09-24
Country of Publication
Egypt
No. of Pages
12
Main Subjects
Abstract EN
Huntington’s disease is a progressive neurodegenerative disease, caused by a polyglutamine expansion in the huntingtin protein.
A prominent hallmark of the disease is the presence of intracellular aggregates initiated by N-terminal huntingtin fragments containing the polyglutamine repeat, which recruit components of the ubiquitin-proteasome system.
While it is commonly thought that proteasomes are irreversibly sequestered into these aggregates leading to impairment of the ubiquitin-proteasome system, the data on proteasomal impairment in Huntington’s disease is contradictory.
In addition, it has been suggested that proteasomes are unable to actually cleave polyglutamine sequences in vitro, thereby releasing aggregation-prone polyglutamine peptides in cells.
Here, we discuss how the proteasome is involved in the various stages of polyglutamine aggregation in Huntington’s disease, and how alterations in activity may improve clearance of mutant huntingtin fragments.
American Psychological Association (APA)
Schipper-Krom, Sabine& Juenemann, Katrin& Reits, Eric A. J.. 2012. The Ubiquitin-Proteasome System in Huntington’s Disease : Are Proteasomes Impaired, Initiators of Disease, or Coming to the Rescue?. Biochemistry Research International،Vol. 2012, no. 2012, pp.1-12.
https://search.emarefa.net/detail/BIM-502058
Modern Language Association (MLA)
Schipper-Krom, Sabine…[et al.]. The Ubiquitin-Proteasome System in Huntington’s Disease : Are Proteasomes Impaired, Initiators of Disease, or Coming to the Rescue?. Biochemistry Research International No. 2012 (2012), pp.1-12.
https://search.emarefa.net/detail/BIM-502058
American Medical Association (AMA)
Schipper-Krom, Sabine& Juenemann, Katrin& Reits, Eric A. J.. The Ubiquitin-Proteasome System in Huntington’s Disease : Are Proteasomes Impaired, Initiators of Disease, or Coming to the Rescue?. Biochemistry Research International. 2012. Vol. 2012, no. 2012, pp.1-12.
https://search.emarefa.net/detail/BIM-502058
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-502058