Trouble With Tribbles.

Blog entry

“Tribbles” is the name of a gene found in Drosophila. It’s also found in humans. Defects in the Tribble gene in humans results in a particularly aggressive form of leukemia, acute myelogenous leukemia (AML).

Troubling, indeed. I’m willing to bet that the Trekkies who named that gene in Drosophila didn’t bet on their gene taking the stage front and center in patients with cancer. But that is exactly what has begun to happen. There are a finite number of proteins on this planet. That means every living thing is composed of some sort of combination of these finite proteins, and the most important ones, tend to be the most highly conserved across species (meaning, the gene that makes the protein had changed little, if at all, from fruitfly to human). The result? Clinicians find themselves saying things like “Well, the leukemia you have is very aggressive and is the result of a defective Tribbles gene.” Or something like this: “You have breast cancer. We need to see if your lunatic fringe gene is working properly.”

Do you see the issue here? These genes, while whimsical and funny when describing fruitfly physiology or zebrafish abnormalities, lose all of their comedic value when a clinician has to tell a sick patient, or worse, the parents of a sick patient, that the reason for their suffering is largely based on a ridiculously named gene. It trivializes the patient’s condition, and in some cases, his or her integrity. Imagine having to sit down and keep saying to yourself “Tribbles. My leukemia was brought on by tribbles…”

As a scientist, I would personally understand the fact that people can name a gene anything they want and would not feel insulted if someone told me my Sonic Hedgehog is mutated. But most people do feel insulted. It’s something they have to live with: The single most awful thing to happen to them is named after a video game, or a Star Trek episode, or a Monty Python movie.

So why not conveniently leave this information out when counseling a patient? Because patients want to know. They want to know what went wrong, or what can be fixed, or simply where the problem lies.  Rightfully so. We are an educated society and most of us have access to the internet and will use it to find answers. So, clinicians are obligated to share that information, regardless of how embarrassing or pejorative it may be.

So, kudos to the Gene Police (Human Genome Organization Gene Nomenclature Committee) for moving to change some of these names. Personally, I would like to see the human gene (regardless of nucleic acid sequence, or homology to other species’ gene) renamed to describe function, or at the very least, to a name that will not insult a patient. Leave the Drosophila gene name alone. After all, we can all use a chuckle when we get into the third hour of our Pubmed search. These names can ease the tension in a lab, or simply conjure up thoughts of favorite childhood story characters. But, as I would say to my lab mates, “Leave it in the lab, man. Outside of these walls is a different world.”