Pharmacological modulation of the retinal unfolded protein response in bardet-biedl syndrome reduces apoptosis and preserves light detection ability

Journal of Biological Chemistry

Volumn 287, Issue 44, 2012, Pages 37483-37494

  Mockel, Anais ^a   Obringer, Cathy ^a   Hakvoort, Theodorus B M ^b   Seeliger, Mathias ^c   Lamers, Wouter H ^b   Stoetzel, Corinne ^c   Dollfus, Hélène ^a,d   Marion, Vincent ^a  

^a UNIVERSITÉ DE STRASBOURG   (France)

^b UNIVERSITY OF AMSTERDAM   (Netherlands)

^c UNIVERSITY OF TÜBINGEN   (Germany)

^d UNIVERSITY HOSPITAL OF STRASBOURG   (France)

Author keywords

[No Author keywords available]

Indexed keywords

LIGHT DETECTION; PHARMACOLOGICAL REDUCTION; PHOTORECEPTOR CELL; PROTEIN ACCUMULATION; RETINAL DEGENERATIONS; UNFOLDED PROTEIN RESPONSE;

OPHTHALMOLOGY; PROTEINS; VISION;

CELL DEATH;

CASPASE 12; CASPASE 3; CASPASE 6; CASPASE 7; CASPASE INHIBITOR; GUANABENZ; MEMBRANE PROTEIN; PROTEIN BBS12; PROTEIN CHOP10; RETINA S ANTIGEN; RHODOPSIN; UNCLASSIFIED DRUG; VALPROIC ACID;

ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; APOPTOSIS; ARTICLE; BARDET BIEDL SYNDROME; CELL DEATH; CONTROLLED STUDY; ENDOPLASMIC RETICULUM; EX VIVO STUDY; IMMUNOFLUORESCENCE; IN VIVO STUDY; LIGHT ADAPTATION; MOUSE; NONHUMAN; PHENOTYPE; PHOTORECEPTOR; PRIORITY JOURNAL; PROTEIN DEPLETION; PROTEIN FOLDING; PROTEIN LOCALIZATION; PROTEIN PHOSPHORYLATION; RETINITIS PIGMENTOSA; SIGNAL TRANSDUCTION; TRANSMISSION ELECTRON MICROSCOPY;

ANIMALS; APOPTOSIS; BARDET-BIEDL SYNDROME; BIOLOGICAL TRANSPORT; CASPASE 12; CASPASE INHIBITORS; CHAPERONINS; CILIA; CYTOPROTECTION; ENDOPLASMIC RETICULUM STRESS; GUANABENZ; KINETICS; MICE; MICE, INBRED C57BL; MICE, KNOCKOUT; PHENOTYPE; PHOTORECEPTOR CELLS; RETINA; SIGNAL TRANSDUCTION; TISSUE CULTURE TECHNIQUES; UNFOLDED PROTEIN RESPONSE; VALPROIC ACID; VISION, OCULAR;

EID: 84868232817     PISSN: 00219258     EISSN: 1083351X     Source Type: Journal    
DOI: 10.1074/jbc.M112.386821     Document Type: Article

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