Nuclear factor ? B expression in patients with sporadic amyotrophic lateral sclerosis and hereditary amyotrophic lateral sclerosis with optineurin mutations
Abstract
Nuclear factor κ B (NF-κB) is involved in the pathogenesis of a number of neurodegenerative disorders with neuroinflammation. In order to clarify the role of NF-κB in ALS, immunohistochemical studies with an antibody that recognizes the p65 subunit of NF-κB were performed on the spinal anterior horn of 4 patients with sporadic ALS (sALS), 1 patient with optineurin-mutated ALS (OPTN-ALS), and 3 normal controls (NC). In patients with sALS or OPTN-ALS, the expression pattern of NF-κB was altered when compared to that of NC; NF-κB immunoreactivity tended to be absent from neuronal nucleus and was increased in microglia. The down-regulation of NF-κB in neuronal nucleus might contribute to a loss of neuroprotection, or neurons with nuclear NF-κB might be lost immediately after its activation. The microglial induction of NF-κB might contribute to neuroinflammation. In conclusion, NF-κB signaling pathway could have a key role in the pathomechanism of ALS.
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ALS-associated OPTN mutations enhance microglial NFKB1 expression.
“ALS-associated OPTN mutations enhance microglial NFKB1 expression [260]”
From Autophagy and ALS: mechanistic insights and therapeutic implications (2022) · cites this paper as [260]
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- Authors
- Sako, W., Ito, H., Yoshida, M., Koizumi, H., Kamada, M., Fujita, K., Hashizume, Y., Izumi, Y., & Kaji, R.
- Affiliations
- Tokushima University (JP)
- Venue
- Clinical Neuropathology
- Publisher
- Dustri-Verlgag Dr. Karl Feistle
- Published
- 2012-11-01
- Volume
- 31
- Issue
- 11
- Pages
- 418-423
- ISSN
- 0722-5091 (ISSN-L)
- OpenAlex
- W2058094550
- Cited by
- 30
- References
- 0
- Citations by year
- 2026: 1 · 2025: 2 · 2023: 3 · 2022: 2 · 2021: 2 · 2020: 6 · 2019: 2 · 2018: 4 · 2016: 1 · 2015: 1 · 2014: 3 · 2013: 2 · 2012: 1
- FWCI
- 1.47
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Clinical Neuropathology (publishedVersion)
- PubMed (publishedVersion)
- Funders
- Japan Society for the Promotion of Science (23659458)
- Topics
- Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
- Life Sciences › Neuroscience › Neurology › Neuroinflammation and Neurodegeneration Mechanisms
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Cancer Research › NF-κB Signaling Pathways
- Keywords
- nuclear factor-kappaB, amyotrophic lateral sclerosis, sporadic ALS, optineurin, microglia, neuroinflammation, neuroprotection, NF-κB p65, neurodegeneration
- MeSH terms
- Aged, Aged, 80 and over, Amyotrophic Lateral Sclerosis, Female, Humans, Immunohistochemistry, Male, Middle Aged, Spinal Cord, Blotting, Western, NF-kappa B, Cell Cycle Proteins, Membrane Transport Proteins, Transcription Factor TFIIIA
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