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Article Publish Status: FREE
Abstract Title:

Bee venom effects on ubiquitin proteasome system in hSOD1G85R-expressing NSC34 motor neuron cells.

Abstract Source:

BMC Complement Altern Med. 2013 ;13:179. Epub 2013 Jul 18. PMID: 23866691

Abstract Author(s):

Seon Hwy Kim, So Young Jung, Kang-Woo Lee, Sun Hwa Lee, MuDan Cai, Sun-Mi Choi, Eun Jin Yang

Article Affiliation:

Seon Hwy Kim

Abstract:

BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that results from a progressive loss of motor neurons. Familial ALS (fALS) is caused by missense mutations in Cu, Zn-superoxide dismutase 1 (SOD1) that frequently result in the accumulation of mutant protein aggregates that are associated with impairments in the ubiquitin-proteasome system (UPS). UPS impairment has been implicated in many neurological disorders. Bee venom (BV) extracted from honey bees has been used as a traditional medicine for treating inflammatory diseases and has been shown to attenuate the neuroinflammatory events that occur in a symptomatic ALS animal model.

METHODS: NSC34 cells were transiently transfected with a WT or G85R hSOD1-GFP construct for 24 hrs and then stimulated with 2.5 μg/ml BV for 24 hrs. To determine whether a SOD1 mutation affects UPS function in NSC34 cells, we examined proteasome activity and performed western blotting and immunofluorescence using specific antibodies, such as anti-misfolded SOD1, anti-ubiquitin, anti-GRP78, anti-LC3, and anti-ISG15 antibodies.

RESULTS: We found that GFP-hSOD1G85R overexpression induced SOD1 inclusions and reduced proteasome activity compared with the overexpression of GFP alone in NSC34 motor neuronal cells. In addition, we also observed that BV treatment restored proteasome activity and reduced the accumulation of ubiquitinated and misfolded SOD1 in GFP-hSOD1G85R-overexpressing NSC34 motor neuronal cells. However, BV treatment did not activate the autophagic pathway in these cells.

CONCLUSION: Our findings suggest that BV may rescue the impairment of the UPS in ALS models.

Study Type : In Vitro Study

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