Targeting proteins to the ciliary membrane.
Most vertebrate cell types display solitary nonmotile cilia on their surface that serve as cellular antennae to sense the extracellular environment. These organelles play key roles in the development of mammals by coordinating the actions of a single cell with events occurring around them. Severe defects in cilia lead to midgestational lethality in mice while more subtle defects lead to pathology in most organs of the body. These pathologies range from cystic diseases of the kidney, liver, and pancreas, to retinal degeneration, to bone and skeletal defects, hydrocephaly, and obesity. The sensory functions of cilia rely on proteins localized specifically to the ciliary membrane. Even though the ciliary membrane is a subdomain of the plasma membrane and is continuous with the plasma membrane, cells have the ability to specifically localize proteins to this domain. In this chapter, we will review what is currently known about the structure and function of the ciliary membrane. We will further discuss ongoing work to understand how the ciliary membrane is assembled and maintained, and discuss protein machinery that is thought to play a role in sorting or trafficking proteins to the ciliary membrane.
Pazour
Gregory J
GJ
Program in Molecular Medicine, University of Massachusetts Medical School, Biotech II, Worcester, Massachusetts, USA.
Bloodgood
Robert A
RA
D000818:Animals; D002462:Cell Membrane; D002923:Cilia / Q000502:physiology*; D021381:Protein Transport; D011506:Proteins / Q000378:metabolism* / Q000648:ultrastructure
D011506:Proteins