title: Nuclear factor κ 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.
authors: Department of Clinical Neuroscience, The University of Tokushima Graduate School, Tokushima, Japan. dwsako@yahoo.co.jp
authors: Wataru
authors: W
authors: Sako
authors: Hidefumi
authors: H
authors: Ito
authors: Mari
authors: M
authors: Yoshida
authors: Hidetaka
authors: H
authors: Koizumi
authors: Masaki
authors: M
authors: Kamada
authors: Koji
authors: K
authors: Fujita
authors: Yoshio
authors: Y
authors: Hashizume
authors: Yuishin
authors: Y
authors: Izumi
authors: Ryuji
authors: R
authors: Kaji
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D000690:Amyotrophic Lateral Sclerosis / Q000235:genetics* / Q000378:metabolism* / Q000473:pathology; D015153:Blotting, Western; D018797:Cell Cycle Proteins; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D026901:Membrane Transport Proteins; D008875:Middle Aged; D016328:NF-kappa B / Q000096:biosynthesis*; D013116:Spinal Cord / Q000378:metabolism / Q000473:pathology; D035582:Transcription Factor TFIIIA / Q000235:genetics*
chemical_list: D018797:Cell Cycle Proteins; D026901:Membrane Transport Proteins; D016328:NF-kappa B; C409460:OPTN protein, human; D035582:Transcription Factor TFIIIA