Mohawk. Sonic hedgehog. Lunatic fringe. Mothers against decapentaplegia. Believe it or not, these are all names of genes. 

 

Drosophila melanogaster is a fruit fly that has been extensively used as a model organism for genetic analysis because of its incredible level of similarity to humans. Believe it or not, but over 75% of known human genes have a homologue in Drosophila. Fruit flies are easy to care for, and they also reproduce very quickly and in large quantities. This makes them ideal model organisms for genetic experimentation.

 

What’s in a name?

 

Researchers who find new genes are given the privilege to name their discovery.  Some are named based on the resultant phenotype if the gene is mutated, such as Tinman; based on the character from The Wizard of Oz. A fly that is a mutant for Tinman will not develop a heart. Van Gogh creates a swirly pattern on the wings, reminiscent of the masterpiece Starry Night. A mutation at Braniac would cause excessive amounts of brain cells and result in an enlarged head.

 

In 1980, a mutation was discovered that gave the drosophila many points on the drosophila larvae, reminiscent of a hedgehog. Three homologues were later discovered: desert, Indian (both species of hedgehog), and Sonic, based off of Sega’s video game character. An inhibitor of Sonic hedgehog was named Robotnikinin; an homage to Dr. Robotnik, Sonic’s nemesis in the game.

 

The scientists who give genes unorthodox names do so to make the gene stand out, to make it easy to remember, or even just to break up the monotony of working in the lab every day. It all seemed frivolously harmless, until the same genes were found in humans. 

 

Physicians have had to explain serious congenital deformities to distraught parents using the correct names of the genes, which has not been well received. The attached picture shows diprosopus, an overexpression of sonic hedgehog. The black arrow in the middle is pointing to a third orbital cavity, and the two white arrows point to two separate nasal cavities. Many people were understandably upset that such extreme craniofacial patterning defects were associated with a joke name.

 

For clinical sensitivity, steps were taken to prohibit scientists from using silly names for human genes. The offensive names are known by their abbreviations, such as Sonic hedgehog (SHH), lunatic fringe (LFNG), and mohawk (MKX).