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RISS 인기검색어
- 검색키워드
- 저자 : Moya Kenneth L (검색결과 2 건)
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Engrailed protects mouse midbrain dopaminergic neurons against mitochondrial complex I insults
Alvarez-Fischer, Daniel,Fuchs, Julia,Castagner, Fran챌ois,Stettler, Olivier,Massiani-Beaudoin, Olivia,Moya, Kenneth L,Bouillot, Colette,Oertel, Wolfgang H,Lomb챔s, Anne,Faigle, Wolfgang,Joshi, Rajiv L,H Nature Publishing Group, a division of Macmillan P 2011 NATURE NEUROSCIENCE Vol.14 No.10
Mice heterozygous for the homeobox gene Engrailed-1 (En1) display progressive loss of mesencephalic dopaminergic (mDA) neurons. We report that exogenous Engrailed-1 and Engrailed-2 (collectively Engrailed) protect mDA neurons from 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP), a mitochondrial complex I toxin used to model Parkinson's disease in animals. Engrailed enhances the translation of nuclearly encoded mRNAs for two key complex I subunits, Ndufs1 and Ndufs3, and increases complex I activity. Accordingly, in vivo protection against MPTP by Engrailed is antagonized by Ndufs1 small interfering RNA. An association between Engrailed and complex I is further confirmed by the reduced expression of Ndufs1 and Ndufs3 in the substantia nigra pars compacta of En1 heterozygous mice. Engrailed also confers in vivo protection against 6-hydroxydopamine and 慣-synuclein-A30P. Finally, the unilateral infusion of Engrailed into the midbrain increases striatal dopamine content, resulting in contralateral amphetamine-induced turning. Therefore, Engrailed is both a survival factor for adult mDA neurons and a regulator of their physiological activity.
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Graded Otx2 activities demonstrate dose-sensitive eye and retina phenotypes
Bernard, Clé,mence,Kim, Hyoung-Tai,Torero Ibad, Raoul,Lee, Eun Jung,Simonutti, Manuel,Picaud, Serge,Acampora, Dario,Simeone, Antonio,Di Nardo, Ariel A.,Prochiantz, Alain,Moya, Kenneth L.,Kim, Ji Oxford University Press 2014 Human Molecular Genetics Vol.23 No.7
<P>In the human, mutations of <I>OTX2</I> (<I>Orthodenticle homeobox 2</I> transcription factor) translate into eye malformations of variable expressivity (even between the two eyes of the same individual) and incomplete penetrance, suggesting the existence of subtle thresholds in OTX2 activity. We have addressed this issue by analyzing retinal structure and function in six mutant mice with graded Otx2 activity: <I>Otx2<SUP>+/+</SUP></I>, <I>Otx2<SUP>+/AA</SUP></I>, <I>Otx2<SUP>+/GFP</SUP></I>, <I>Otx2<SUP>AA/AA</SUP></I>, <I>Otx2<SUP>AA/GFP</SUP></I> and <I>Otx2<SUP>GFP/GFP</SUP></I>. Null mice (<I>Otx2<SUP>GFP/GFP</SUP></I>) fail to develop the head and are embryonic lethal, and compound heterozygous <I>Otx2<SUP>AA/GFP</SUP></I> mice show a truncated head and die at birth. All other genotypes develop until adulthood. We analyzed eye structure and visual physiology in the genotypes that develop until adulthood and report that phenotype severity parallels Otx2 activity. <I>Otx2<SUP>+/AA</SUP></I> are only mildly affected whereas <I>Otx2<SUP>+/GFP</SUP></I> are more affected than <I>Otx2<SUP>+/AA</SUP></I> but less than <I>Otx2<SUP>AA/AA</SUP></I> mice. <I>Otx2<SUP>AA/AA</SUP></I> mice later manifest the most severe defects, with variable expressivity. Electrophysiological and histological analyses of the mouse retina revealed progressive death of bipolar cells and cone photoreceptors that is both Otx2 activity- and age-dependent with the same ranking of phenotypic severity. This study demonstrates the importance of gene dosage in the development of age-dependent pathologies and underscores the fact that small gene dosage differences can cause significant pathological states.</P>
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