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Adv Exp Med Biol. 2016;854:627-33. doi: 10.1007/978-3-319-17121-0_83.

The Role of Intraflagellar Transport in the Photoreceptor Sensory Cilium.

Author information

1
Ocular Genomics Institute, and Berman-Gund Laboratory for the Study of Retinal Degenerations, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, 243 Charles St., 5th Floor, 563C, 02114, Boston, MA, USA. dtaub1230@gmail.com.
2
Ocular Genomics Institute, and Berman-Gund Laboratory for the Study of Retinal Degenerations, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, 243 Charles St., 5th Floor, 563C, 02114, Boston, MA, USA. qin_liu@meei.harvard.edu.

Abstract

The photoreceptor is a complex specialized cell in which a major component responsible for visual transduction is the photoreceptor sensory cilium (PSC). Building and maintenance of the PSC requires the transport of large proteins along microtubules that extend from the inner segments to the outer segments. A key process, termed intraflagellar transport (IFT), has been recognized as an essential phenomenon for photoreceptor development and maintenance, and exciting new studies have highlighted its importance in retinal and cilia related diseases. This review focuses on the important roles of IFT players, including motor proteins, IFT proteins, and photoreceptor-specific cargos in photoreceptor sensory cilium. In addition, specific IFT components that are involved in inherited human diseases are discussed.

KEYWORDS:

Cilia; Inherited retinal degeneration; Intraflagellar transport (IFT); Photoreceptor; Protein transport

PMID:
26427468
DOI:
10.1007/978-3-319-17121-0_83
[Indexed for MEDLINE]

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