| Genetic reactivation of cone photoreceptors restores visual responses in retinitis pigmentosa. | |
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MedLine Citation:
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PMID: 20576849 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Retinitis pigmentosa refers to a diverse group of hereditary diseases that lead to incurable blindness, affecting two million people worldwide. As a common pathology, rod photoreceptors die early, whereas light-insensitive, morphologically altered cone photoreceptors persist longer. It is unknown if these cones are accessible for therapeutic intervention. Here, we show that expression of archaebacterial halorhodopsin in light-insensitive cones can substitute for the native phototransduction cascade and restore light sensitivity in mouse models of retinitis pigmentosa. Resensitized photoreceptors activate all retinal cone pathways, drive sophisticated retinal circuit functions (including directional selectivity), activate cortical circuits, and mediate visually guided behaviors. Using human ex vivo retinas, we show that halorhodopsin can reactivate light-insensitive human photoreceptors. Finally, we identified blind patients with persisting, light-insensitive cones for potential halorhodopsin-based therapy. |
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Authors:
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Volker Busskamp; Jens Duebel; David Balya; Mathias Fradot; Tim James Viney; Sandra Siegert; Anna C Groner; Erik Cabuy; Valérie Forster; Mathias Seeliger; Martin Biel; Peter Humphries; Michel Paques; Saddek Mohand-Said; Didier Trono; Karl Deisseroth; José A Sahel; Serge Picaud; Botond Roska |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, Non-P.H.S. Date: 2010-06-24 |
Journal Detail:
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Title: Science (New York, N.Y.) Volume: 329 ISSN: 1095-9203 ISO Abbreviation: Science Publication Date: 2010 Jul |
Date Detail:
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Created Date: 2010-07-23 Completed Date: 2010-08-09 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 0404511 Medline TA: Science Country: United States |
Other Details:
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Languages: eng Pagination: 413-7 Citation Subset: IM |
Affiliation:
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Neural Circuit Laboratories, Friedrich Miescher Institute for Biomedical Research, Basel, Switzerland. |
| Data Bank Information | |
Bank Name/Acc. No.:
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GEO/GSE22338 |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Animals Dependovirus / genetics Disease Models, Animal Evoked Potentials, Visual Gene Therapy* Genetic Vectors Halobacteriaceae / genetics Halorhodopsins / genetics*, metabolism* Humans Light Mice Mice, Knockout Promoter Regions, Genetic Retina / physiology Retinal Cone Photoreceptor Cells / physiology* Retinal Ganglion Cells / physiology Retinitis Pigmentosa / physiopathology, therapy* Tissue Culture Techniques Transfection Vision, Ocular Visual Pathways / physiology |
| Chemical | |
Reg. No./Substance:
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0/Halorhodopsins |
| Comments/Corrections | |
Comment In:
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Science. 2010 Jul 23;329(5990):403-4
[PMID:
20651144
]
Nat Rev Neurosci. 2010 Aug;11(8):534 [PMID: 20672425 ] |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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