Downregulation of apoptosis-inducing factor in Harlequin mice induces progressive and severe optic atrophy which is durably prevented by AAV2-AIF1 gene therapy

Brain

Volumn 135, Issue 1, 2012, Pages 35-52

  Bouaita, Aicha ^a,b,c,d   Augustin, Sébastien ^a,b,c,d   Lechauve, Christophe ^a,b,c,d   Cwerman Thibault, Hélne ^a,b,c,d   Bénit, Paule ^e   Simonutti, Manuel ^a,b,c,d   Paques, Michel ^a,b,c,d   Rustin, Pierre ^e,f   Sahel, José Alain ^a,b,c,d   Corral Debrinski, Marisol ^a,b,c,d  

^a INSERM   (France)

^b SORBONNE UNIVERSITÉ   (France)

^c CNRS   (France)

^d CENTRE HOSPITALIER NATIONAL D OPHTALMOLOGIE DES QUINZE VINGTS   (France)

^e HÔPITAL ROBERT DEBRÉ   (France)

^f UNIVERSITÉ PARIS DESCARTES   (France)

Author keywords

AAV mediated gene therapy; apoptosis inducing factor; Harlequin mice; optic atrophy; respiratory chain complex I; retinal ganglion cells

Indexed keywords

ALLOGRAFT INFLAMMATORY FACTOR 1; APOPTOSIS INDUCING FACTOR; MESSENGER RNA; PARVOVIRUS VECTOR; REDUCED NICOTINAMIDE ADENINE DINUCLEOTIDE DEHYDROGENASE (UBIQUINONE);

3' UNTRANSLATED REGION; ADENO ASSOCIATED VIRUS; ADENO ASSOCIATED VIRUS TYPE 2; ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; BLINDNESS; CELL ACTIVATION; CELL PROTECTION; CONFOCAL SCANNING LASER OPHTHALMOSCOPY; CONTROLLED STUDY; DOWN REGULATION; GLIA CELL; MALE; MICROGLIA; MOUSE; NEUROPROTECTION; NONHUMAN; OPTIC NERVE ATROPHY; OPTICAL COHERENCE TOMOGRAPHY; PHENOTYPE; PRIORITY JOURNAL; REAL TIME POLYMERASE CHAIN REACTION; RESPIRATORY CHAIN; RETINA GANGLION CELL; SCANNING LASER OPHTHALMOSCOPY; STEADY STATE; VIRAL GENE DELIVERY SYSTEM; VISION; VISUAL IMPAIRMENT;

EID: 84856728004     PISSN: 00068950     EISSN: 14602156     Source Type: Journal    
DOI: 10.1093/brain/awr290     Document Type: Article

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