By Yishane Lee

At a presentation Hearing Restoration Project scientist Neil Segil, Ph.D., made to a small audience at the HHF offices earlier this year, I was surprised (and delighted) to learn that a gene had the name of Lunatic Fringe—which is not to be confused with the Manic Fringe or the Radical Fringe. Dr. Segil mentioned the gene because it can affect the Notch signaling pathway, a form of cell communication that creates mosaic patterns of different cell types, like the kind we see in the inner ear for hearing. I wondered, how did the gene get this name?

The privilege for people who discover things is that they get to name it—be it a star, mountain, or gene. And the scientists who worked on genetically mapping the fruit fly, which has been studied for over a century, have a sense of humor.

Besides the fringe genes, there are these fruit fly genes that have been found or created: the Tinman refers to a gene that makes it hard to develop a heart; the Groucho Marx refers to a gene that produces excess facial bristles; the Cheap Date gene means the fruit fly is extra-sensitive to alcohol; the Ken and Barbie fruit fly lacks genitalia; and fruit flies with the I’m Not Dead Yet (INDY) gene live longer than usual. (See a list of funny fruit fly gene names.)

The problem, though, is that we discover we actually share quite a lot of ancestry—and therefore, genes—with the fruit fly, including those that cause hereditary diseases, telling a patient that he has a genetic condition related to a mutation in the Lunatic Fringe gene is not going to, well, fly. (Two faulty copies of the Lunatic Fringe gene, which encodes the development of the limbs and other parts of the body, leads to spinal defect known as spondylocostal dysostosis.)

So while these catchy names and the conditions they refer to are easy to remember, a few years ago the Human Genome Organization (HUGO) gene naming committee was petitioned to change them. And, faster than you can say Sonic Hedgehog (another gene name that was changed), the Lunatic Fringe—at least officially—became the LNFG.

Read more about genes related to hearing loss, including the HRP’s use of next-generation DNA sequencing.

Tell us what your favorite gene name is here in the comments!