Nuclear Factor κ B Expression in Patients With Sporadic Amyotrophic Lateral Sclerosis and Hereditary Amyotrophic Lateral Sclerosis With Optineurin Mutations

Clin Neuropathol. Nov-Dec 2012;31(6):418-23. doi: 10.5414/NP300493.

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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Aged, 80 and over
  • Amyotrophic Lateral Sclerosis / genetics*
  • Amyotrophic Lateral Sclerosis / metabolism*
  • Amyotrophic Lateral Sclerosis / pathology
  • Blotting, Western
  • Female
  • Humans
  • Immunohistochemistry
  • Male
  • Middle Aged
  • NF-kappa B / biosynthesis*
  • Spinal Cord / metabolism
  • Spinal Cord / pathology
  • Transcription Factor TFIIIA / genetics*

Substances

  • NF-kappa B
  • OPTN protein, human
  • Transcription Factor TFIIIA