BETHESDA, Md., July 6, 2010 It's safe to say that cilia, the hairlike appendages jutting out from the smooth surfaces of most mammalian cells, have long been misunderstood underestimated, even.
Not to be confused with their whiplike cousins flagella, which propel sperm, one type of cilia has been known to serve as microscopic conveyor belts. (Picture cilia reaching up like concertgoers supporting a crowd-surfer.) But for decades another type of cilia, known as "primary" cilia, was believed to serve little to no purpose. Despite the fact that almost every cell found in vertebrates has at least one primary cilium, the organ was regarded as merely an evolutionary relic the cellular equivalent to the human appendix.
Of late, however, it has become increasingly clear that primary cilia serve as powerful communication hubs. (After all, they do sort of look like antennae.) Disruptions in the activity of cilia are now understood to lead to a whole class of diseases dubbed ciliopathies, and researchers are hustling to figure out what makes them tick.
One group of scientists in Japan last month marked a milestone in the pursuit to reveal cilia's secrets. In study results that were fast-tracked for publication and deemed a "Paper of the Week" by the Journal of Biological Chemistry, they report that they have identified a long-elusive enzyme necessary for the proper regulation of cilia.
The Hamamatsu University School of Medicine team is optimistic that the discovery may aid in the development of therapies for those with visual and hearing maladies caused by cilia dysfunction.
"Our finding might give insights into the sensory defects associated with problems in cilia function. For example, patients with some syndromes have genetic defects in cilia functions that result in retinal degeneration," explains Mitsutoshi Setou, who oversaw the team's work. "Also, age-dependent visual loss or hearing loss is known to be r
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American Society for Biochemistry and Molecular Biology