These tools will no longer be maintained as of December 31, 2024. Archived website can be found here. PubMed4Hh GitHub repository can be found here. Contact NLM Customer Service if you have questions.


Pubmed for Handhelds

PUBMED FOR HANDHELDS

Search MEDLINE/PubMed

  • Title: Endoplasmic reticulum stress is accompanied by activation of NF-κB in amyotrophic lateral sclerosis.
    Author: Prell T, Lautenschläger J, Weidemann L, Ruhmer J, Witte OW, Grosskreutz J.
    Journal: J Neuroimmunol; 2014 May 15; 270(1-2):29-36. PubMed ID: 24666819.
    Abstract:
    BACKGROUND: Recent studies have indicated that endoplasmic reticulum (ER) stress is involved in the pathogenesis of amyotrophic lateral sclerosis (ALS). ER stress occurs when the ER-mitochondria calcium cycle is disturbed and misfolded proteins accumulate in the ER. To cope with ER stress, cells activate the unfolded protein response (UPR). Accumulating evidence from non-neuronal cell models suggests that there is extensive cross-talk between the UPR and the NF-κB pathway. METHODS: Here we investigated the expression of NF-κB and the main UPR markers X-box binding protein 1 (XBP1), basic leucine-zipper transcription factor 6 (ATF6) and phosphorylated eukaryotic initiation factor-2α (p-eIF2) in mutated SOD1(G93A) cell models of ALS, as well as their modulation by lipopolysaccharide and ER-stressing (tunicamycin) stimuli. RESULTS: Expression of NF-κB was enhanced in the presence of SOD1(G93A). Lipopolysaccharide did not induce the UPR in NSC34 cells and motor neurons in a mixed motor neuron-glia coculture system. The induction of the UPR by tunicamycin was accompanied by activation of NF-κB in NSC34 cells and motor neurons. CONCLUSION: Our data linked two important pathogenic mechanisms of ALS, ER stress and NF-κB signalling, in motor neurons.
    [Abstract] [Full Text] [Related] [New Search]