Photoreceptor intersegmental transport and retinal degeneration: a conserved pathway common to motile and sensory cilia

JC Besharse, SA Baker, K Luby-Phelps… - Retinal Degenerations …, 2003 - Springer
JC Besharse, SA Baker, K Luby-Phelps, GJ Pazour
Retinal Degenerations: Mechanisms and Experimental Therapy, 2003Springer
During early development of rod and cone photoreceptors, the outer segment (OS)
assembles on the apical cell surface by an elaborate process of membrane and protein
transport followed by membrane reorganization to form the highly organized stack of
membrane disks found in the OS of mature cells. The process of membrane and protein
transport continues in mature photoreceptors as the molecular components of the OS
turnover at a prodigious rate for the life of the cell (Young, 1967). Intersegmental transport …
Abstract
During early development of rod and cone photoreceptors, the outer segment (OS) assembles on the apical cell surface by an elaborate process of membrane and protein transport followed by membrane reorganization to form the highly organized stack of membrane disks found in the OS of mature cells. The process of membrane and protein transport continues in mature photoreceptors as the molecular components of the OS turnover at a prodigious rate for the life of the cell (Young, 1967). Intersegmental transport between the inner segment (IS) and the OS is a major logistical problem as all OS macromolecules are synthesized in the IS and must be transported to the OS. Elucidating the molecular pathways involved in intersegmental transport will ultimately require a refined understanding of the photoreceptor connecting cilium as this structure is the only stable connecting link between the IS and the OS and is the likely transport corridor (reviewed in Besharse and Horst, 1990). In addition, understanding the details of these pathways is likely to provide an improved understanding of photoreceptor degeneration diseases that involve trafficking between the two segments.
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