Abstract
The precise trafficking and spatial organization of signaling molecules within cells is critical for many fundamental cellular processes. Two interconnected microtubule-based organelles, the centrosome and primary cilium, have been making headlines recently due to their role as central “hubs” for coordinating such signaling events. The centrosome is the major microtubule-nucleating center in animal cells, which polarizes microtubule arrays and thereby directs microtubule-based trafficking toward itself and its associated structure, the primary cilium. [1]. The primary cilium is a tiny hair-like sensory organelle that is templated by one of two centrioles, core elements of the centrosome, and protrudes above the apical surface of almost every cell in the human body (Figure 1). Together, the centrosome and cilium mediate the initiation and transmission of extracellular signals to the interior of the cell, thus controlling many aspects of cell physiology [2], [3]. Defects in the structure and/or function of these organelles result in human disease conditions termed “ciliopathies,” a heterogeneous group of disorders with phenotypes including cystic kidneys; digit, bone, and brain anomalies; infertility; and even cancer [4], [5].
Highlights
Human diseases associated with centrosome-cilium aberrations generally result from defects in the proper assembly of the centrosome or cilium, or the trafficking of proteins to these structures
Centriolar satellites are generally defined by PCM1, a large scaffolding protein reported to function in dynein-dependent, microtubule-based trafficking of centrosomal and ciliary proteins [7,8]
The proteome of centriolar satellites remains unknown, a number of proteins have been shown to associate with PCM1, many of which are mutated in patients with ciliopathies
Summary
Human diseases associated with centrosome-cilium aberrations generally result from defects in the proper assembly of the centrosome or cilium, or the trafficking of proteins to these structures. Centriolar satellites are generally defined by PCM1, a large scaffolding protein reported to function in dynein-dependent, microtubule-based trafficking of centrosomal and ciliary proteins [7,8]. The functional significance of centriolar satellites in mammalian development and ciliogenesis in vivo remains unclear.
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