Donating Otx2 to support neighboring neuron survival
Identifieur interne : 001922 ( Ncbi/Curation ); précédent : 001921; suivant : 001923Donating Otx2 to support neighboring neuron survival
Auteurs : Hyoung-Tai Kim [Corée du Sud] ; Alain Prochiantz [France] ; Jin Woo Kim [Corée du Sud]Source :
- BMB Reports [ 1976-6696 ] ; 2016.
Abstract
Mutations of
Url:
DOI: 10.5483/BMBRep.2016.49.2.006
PubMed: 26779998
PubMed Central: 4915118
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PMC:4915118Le document en format XML
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<author><name sortKey="Kim, Hyoung Tai" sort="Kim, Hyoung Tai" uniqKey="Kim H" first="Hyoung-Tai" last="Kim">Hyoung-Tai Kim</name>
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<sourceDesc><biblStruct><analytic><title xml:lang="en" level="a" type="main">Donating Otx2 to support neighboring neuron survival</title>
<author><name sortKey="Kim, Hyoung Tai" sort="Kim, Hyoung Tai" uniqKey="Kim H" first="Hyoung-Tai" last="Kim">Hyoung-Tai Kim</name>
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<author><name sortKey="Kim, Jin Woo" sort="Kim, Jin Woo" uniqKey="Kim J" first="Jin Woo" last="Kim">Jin Woo Kim</name>
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<front><div type="abstract" xml:lang="en"><p>Mutations of <italic>orthodentricle homeobox 2</italic>
(<italic>OTX2</italic>
) 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 <italic>Otx2</italic>
<sup>+/−</sup>
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 <italic>Otx2</italic>
mRNA but carry Otx2 protein, are most sensitive to <italic>Otx2</italic>
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]</p>
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