Journal article
Chaperones and retinal disorders
Advances in protein chemistry and structural biology, Vol.114, pp.85-117
2019
DOI: 10.1016/bs.apcsb.2018.09.001
PMID: 30635087
Abstract
Defects in protein folding and trafficking are a common cause of photoreceptor degeneration, causing blindness. Photoreceptor cells present an unusual challenge to the protein folding and transport machinery due to the high rate of protein synthesis, trafficking and the renewal of the outer segment, a primary cilium that has been modified into a specialized light-sensing compartment. Phototransduction components, such as rhodopsin and cGMP-phosphodiesterase, and multimeric ciliary transport complexes, such as the BBSome, are hotspots for mutations that disrupt proteostasis and lead to the death of photoreceptors. In this chapter, we review recent studies that advance our understanding of the chaperone and transport machinery of phototransduction proteins.
Details
- Title: Subtitle
- Chaperones and retinal disorders
- Creators
- Maxim Sokolov - Department of Ophthalmology, West Virginia University, Morgantown, WV, United StatesRavi P Yadav - Department of Molecular Physiology and Biophysics, The University of Iowa Carver College of Medicine, Iowa City, IA, United StatesCeline Brooks - Department of Ophthalmology, West Virginia University, Morgantown, WV, United StatesNikolai O Artemyev - Department of Molecular Physiology and Biophysics, The University of Iowa Carver College of Medicine, Iowa City, IA, United States; Department of Ophthalmology and Visual Sciences, The University of Iowa Carver College of Medicine, Iowa City, IA, United States. Electronic address: nikolai-artemyev@uiowa.edu
- Resource Type
- Journal article
- Publication Details
- Advances in protein chemistry and structural biology, Vol.114, pp.85-117
- Publisher
- Netherlands
- DOI
- 10.1016/bs.apcsb.2018.09.001
- PMID
- 30635087
- ISSN
- 1876-1623
- eISSN
- 1876-1631
- Language
- English
- Date published
- 2019
- Academic Unit
- Molecular Physiology and Biophysics; Iowa Neuroscience Institute; Ophthalmology and Visual Sciences
- Record Identifier
- 9984070827002771
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