The DOI matches its Crossref record; 11 of the 11 references it cites were found in a registry or an index.
Activation of the IκB Kinase Complex and Nuclear Factor-κB Contributes to Mutant Huntingtin Neurotoxicity
Open accessAbstract
Transcriptional dysregulation by mutant huntingtin (Htt) protein has been implicated in the pathogenesis of Huntington's disease (HD). We find that cultured cells expressing mutant Htt and striatal cells from HD transgenic mice have elevated nuclear factor-κB (NF-κB) activity. Furthermore, NF-κB is concentrated in the nucleus of neurons in the brains of HD transgenic mice. In inducible PC12 cells and in HD transgenic mice, mutant Htt activates the IκB kinase complex (IKK), a key regulator of NF-κB. Activation of IKK is likely mediated by direct interaction with mutant Htt, because the expanded polyglutamine stretch and adjacent proline-rich motifs in mutant Htt interact with IKKγ, a regulatory subunit of IKK. Activation of IKK may also influence the toxicity of mutant Htt, because expression of IKKγ promotes aggregation and nuclear localization of mutant Htt exon-1. Moreover, in acute striatal slice cultures, inhibition of IKK activity with an N-terminally truncated form of IKKγ blocks mutant Htt-induced toxicity in medium-sized spiny neurons (MSNs). In addition, blocking degradation of NF-κB inhibitors with a dominant-negative ubiquitin ligase β-transducin repeat-containing protein also reduces the toxicity of mutant Htt in MSNs. Therefore, aberrant NF-κB activation may contribute to the neurodegeneration induced by mutant Htt.
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Cites
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“PC12 cells, which express wild-type (WT) HDx1-enhanced green fluorescent protein (EGFP) with 25 glutamine repeats (25Qs) or mutant HDx1-EGFP with 103Qs in response to ecdysone, were kindly provided by Dr. E. Schweitzer (University of California Los Angeles).”
BackgroundMethodResultInfluentialNF-κB at the crossroads of life and death
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Cited by
Microglia in neurodegenerative diseases: mechanism and potential therapeutic targets
Gao, C., Jiang, J., Tan, Y., & Chen, S. (2023) · Signal Transduction and Targeted Therapy · doi:10.1038/s41392-023-01588-0 · Cited by 1,616
Innate immune activation in neurodegenerative disease
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Molecular Mechanisms and Potential Therapeutical Targets in Huntington's Disease
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Does neuroinflammation fan the flame in neurodegenerative diseases?
Frank-Cannon, T. C., Alto, L. T., McAlpine, F. E., & Tansey, M. G. (2009) · Molecular Neurodegeneration · doi:10.1186/1750-1326-4-47 · Cited by 751
Elusive roles for reactive astrocytes in neurodegenerative diseases
Ben Haim, L., Carrillo-de Sauvage, M.-A., Ceyzériat, K., & Escartin, C. (2015) · Frontiers in Cellular Neuroscience · doi:10.3389/fncel.2015.00278 · Cited by 453
Metadata
- Authors
- Khoshnan, A., Ko, J., Watkin, E. E., Paige, L. A., Reinhart, P. H., & Patterson, P. H.
- Venue
- The Journal of Neuroscience
- Publisher
- Society for Neuroscience
- Published
- 2004-09-15
- Volume
- 24
- Issue
- 37
- Pages
- 7999-8008
- ISSN
- 0270-6474 (ISSN-L)
- 1529-2401
- OpenAlex
- W2001086078
- Cited by
- 222
- References
- 11
- FWCI
- 1.97
- Type
- journal article
- Language
- en
- Open access
- hybrid
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- Khoshnan, A., Ko, J., Watkin, E. E., Paige, L. A., Reinhart, P. H., & Patterson, P. H. (2004). Activation of the IκB Kinase Complex and Nuclear Factor-κB Contributes to Mutant Huntingtin Neurotoxicity. The Journal of Neuroscience. https://doi.org/10.1523/jneurosci.2675-04.2004
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