Please use this identifier to cite or link to this item: https://doi.org/10.1038/srep33830
Title: Mitochondrial pathogenic mechanism and degradation in optineurin E50K mutation-mediated retinal ganglion cell degeneration
Authors: Shim, M.S
Takihara, Y 
Kim, K.-Y
Iwata, T
Yue, B.Y.J.T
Inatani, M
Weinreb, R.N
Perkins, G.A
Ju, W.-K
Issue Date: 2016
Citation: Shim, M.S, Takihara, Y, Kim, K.-Y, Iwata, T, Yue, B.Y.J.T, Inatani, M, Weinreb, R.N, Perkins, G.A, Ju, W.-K (2016). Mitochondrial pathogenic mechanism and degradation in optineurin E50K mutation-mediated retinal ganglion cell degeneration. Scientific Reports 6 : 33830. ScholarBank@NUS Repository. https://doi.org/10.1038/srep33830
Rights: Attribution 4.0 International
Abstract: Mutations in optineurin (OPTN) are linked to the pathology of primary open angle glaucoma (POAG) and amyotrophic lateral sclerosis. Emerging evidence indicates that OPTN mutation is involved in accumulation of damaged mitochondria and defective mitophagy. Nevertheless, the role played by an OPTN E50K mutation in the pathogenic mitochondrial mechanism that underlies retinal ganglion cell (RGC) degeneration in POAG remains unknown. We show here that E50K expression induces mitochondrial fission-mediated mitochondrial degradation and mitophagy in the axons of the glial lamina of aged E50K â 'tg mice in vivo. While E50K activates the Bax pathway and oxidative stress, and triggers dynamics alteration-mediated mitochondrial degradation and mitophagy in RGC somas in vitro, it does not affect transport dynamics and fission of mitochondria in RGC axons in vitro. These results strongly suggest that E50K is associated with mitochondrial dysfunction in RGC degeneration in synergy with environmental factors such as aging and/or oxidative stress. © 2016 The Author(s).
Source Title: Scientific Reports
URI: https://scholarbank.nus.edu.sg/handle/10635/178862
ISSN: 20452322
DOI: 10.1038/srep33830
Rights: Attribution 4.0 International
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