Trafficking of Membrane-Associated Proteins to Cone Photoreceptor Outer Segments Requires the Chromophore 11-cis-Retinal

Lecithin retinol acyl transferase (LRAT) and retinal pigment epithelium protein 65 (RPE65) are key enzymes of the retinoid cycle. In Lrat(-/-) and Rpe65(-/-) mice, models of human Leber congenital amaurosis, the retinoid cycle is disrupted and 11-cis-retinal, the chromophore of visual pigments, is n...

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Veröffentlicht in:	The Journal of neuroscience 2008-04, Vol.28 (15), p.4008-4014
Hauptverfasser:	Zhang, Houbin, Fan, Jie, Li, Sha, Karan, Sukanya, Rohrer, Baerbel, Palczewski, Krzysztof, Frederick, Jeanne M, Crouch, Rosalie K, Baehr, Wolfgang
Format:	Artikel
Sprache:	eng
Schlagworte:	Acyltransferases - deficiency Acyltransferases - metabolism Animals Blindness - congenital Blindness - genetics Carrier Proteins - genetics Carrier Proteins - metabolism cis-trans-Isomerases Disease Models, Animal Eye Proteins - genetics Eye Proteins - metabolism Guanylate Cyclase - metabolism Membrane Proteins - metabolism Mice Mice, Knockout Nerve Degeneration - etiology Nerve Degeneration - physiopathology Protein Isoforms - metabolism Protein Transport - drug effects Receptors, Cell Surface - metabolism Retinal Cone Photoreceptor Cells - metabolism Retinal Cone Photoreceptor Cells - physiopathology Retinal Pigments - metabolism Retinaldehyde - deficiency Retinaldehyde - metabolism Retinaldehyde - pharmacology Rod Opsins - metabolism Time Factors Vision, Ocular
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