Molecular chaperones and photoreceptor function
| Autor: | Nele Schwarz, Michael E. Cheetham, John S. Bett, Maria Kosmaoglou |
|---|---|
| Rok vydání: | 2008 |
| Předmět: |
AIPL1
Rhodopsin genetic structures Protein aggregation Article Retina Photoreceptor cell 03 medical and health sciences Heat shock protein medicine Animals Humans Hsp Neurodegeneration 030304 developmental biology 0303 health sciences biology Retinal Degeneration 030302 biochemistry & molecular biology Retinal dystrophy Photoreceptor protein Sensory Systems RP2 Cell biology Co-chaperone Ophthalmology medicine.anatomical_structure Biochemistry Chaperone (protein) Molecular chaperone biology.protein Protein folding sense organs Molecular Chaperones Photoreceptor Cells Vertebrate |
| Zdroj: | Progress in Retinal and Eye Research |
| ISSN: | 1350-9462 |
| Popis: | Molecular chaperones facilitate and regulate protein conformational change within cells. This encompasses many fundamental cellular processes: including the correct folding of nascent chains; protein transport and translocation; signal transduction and protein quality control. Chaperones are, therefore, important in several forms of human disease, including neurodegeneration. Within the retina, the highly specialized photoreceptor cell presents a fascinating paradigm to investigate the specialization of molecular chaperone function and reveals unique chaperone requirements essential to photoreceptor function. Mutations in several photoreceptor proteins lead to protein misfolding mediated neurodegeneration. The best characterized of these are mutations in the molecular light sensor, rhodopsin, which cause autosomal dominant retinitis pigmentosa. Rhodopsin biogenesis is likely to require chaperones, while rhodopsin misfolding involves molecular chaperones in quality control and the cellular response to protein aggregation. Furthermore, the specialization of components of the chaperone machinery to photoreceptor specific roles has been revealed by the identification of mutations in molecular chaperones that cause inherited retinal dysfunction and degeneration. These chaperones are involved in several important cellular pathways and further illuminate the essential and diverse roles of molecular chaperones. |
| Databáze: | OpenAIRE |
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