title: Donating Otx2 to support neighboring neuron survival.
abstract: Mutations of orthodentricle homeobox 2 (OTX2) in human and mice often cause retinal dystrophy and nyctalopia, suggesting a role of OTX2 in mature retina, in addition to its functions in the development of the eye and retina. In support of this, the number of bipolar cells in Otx2＋/- post-natal mouse retina was found to be significantly lower than normal. Degeneration of the cells becomes greater as the mice age, leading to the loss of vision. Especially, the type-2 OFF-cone bipolar cells, which do not express Otx2 mRNA but carry Otx2 protein, are most sensitive to Otx2 haplodeficiency. Interestingly, this bipolar cell subpopulation imports Otx2 protein from photoreceptors to protect itself from glutamate excitotoxicity in the dark. Moreover, in the bipolar cells, the exogenous Otx2 relocates to the mitochondria to support mitochondrial ATP synthesis. This novel mitochondrial activity of exogenous Otx2 highlights the therapeutic potential of Otx2 protein transduction in retinal dystrophy. [BMB Reports 2016; 49(2): 69-70].
authors.affiliation: Department of Biological Sciences, Korea Advanced Institute of Science and Technology (KAIST), Daejeon 34141, Korea.
authors.forename: Hyoung-Tai
authors.initials: HT
authors.lastname: Kim
authors.affiliation: Center for Interdisciplinary Research in Biology (CIRB), College de France, Paris, France.
authors.forename: Alain
authors.initials: A
authors.lastname: Prochiantz
authors.affiliation: Department of Biological Sciences, Korea Advanced Institute of Science and Technology (KAIST), Daejeon 34141, Korea.
authors.forename: Jin Woo
authors.initials: JW
authors.lastname: Kim
mesh_terms: D000818:Animals; D002470:Cell Survival; D018345:Mice, Knockout; D009154:Mutation / Q000235:genetics; D009474:Neurons / Q000166:cytology* / Q000378:metabolism*; D000066829:Neuroprotection; D051857:Otx Transcription Factors / Q000235:genetics / Q000378:metabolism*; D051245:Retinal Bipolar Cells / Q000378:metabolism
chemical_list: D051857:Otx Transcription Factors; C497660:Otx2 protein, mouse