There are lots of other strange sounding proteins in biology, e.g Dickkopf (DKK), which is german for 'thick head', because increased Dickkopf-1 makes large heads in a certain tadpole species. There are also toll-like receptors ('toll' being german for 'yay'), because their discoverer, Nobel laurate Christiane Nüsslein-Volhard, was very excited when she found them.
There's a signal transduction pathway where a mitogen activated protein (MAP) is phosphorylated by a MAP kinase, which itself is phosphorylated by a MAP kinase kinase, which is phosphorylated by a MAP kinase kinase kinase, which is phosphorylated by (you guessed it) a MAP kinase kinase kinase kinase.
One of my personal favorite protein names is Mothers Against Decapentaplegic. As with most of the funny gene names it was coined by drosophila researchers.
While the name might be cute to a researcher, it puts clinicians in a difficult position. Imagine facing parents and having to break to them the terrible news that their child has a problem with their Sonic the Hedgehog gene. What an awful injection of whimsy when none is welcome.
The gene has been linked to a condition known as holoprosencephaly, which can result in severe brain, skull and facial defects, causing a few clinicians and scientists to criticize the name on the grounds that it sounds too frivolous. It has been noted that mention of a mutation in a sonic hedgehog gene might not be well received in a discussion of a serious disorder with a patient or their family.[17][89][90] This controversy has largely died down, and the name is now generally seen as a humorous relic of the time before the rise of fast, cheap complete genome sequencing and standardized nomenclature.[91] The problem of the "inappropriateness" of the names of genes such as "Mothers against decapentaplegic", "Lunatic fringe", and "Sonic hedgehog" is largely avoided by using standardized abbreviations when speaking with patients and their families.[92]
"While the name might be cute to a researcher, it puts clinicians in a difficult position... What an awful injection of whimsy when none is welcome." [1]
I can't imagine being placed in this position where I may have to occasionally abbreviate the name of this protein SHH. Very difficult, just awful. Not welcome!
I don't know about you, but when my doctor throws a bunch of acronyms at me, I always ask what they mean. If nothing else, it slows down the torrent of information enough for me to process what I'm hearing. But I know enough greek and latin roots that it usually helps my memory file the information away for future retrieval.
Sometimes doctors get offended at having to explain what's going on to the patient. Those are bad doctors.
> Sometimes doctors get offended at having to explain what's going on to the patient.
I didn't mean it that way. It's all about using an appropriate language and level of detail for each person. It's fun to talk about how Sonic the Hedgehog was immortalized in the human genome here on HN but in a clinical setting it's ultimately irrelevant and counterproductive.
Some people are curious and ask lots of questions. Many will be confused and tune you out completely if you start explaining technical stuff. It's the doctor's job to help them understand so they can make informed decisions. This is accomplished by tailoring the message to each recipient.
Our daughter was diagnosed at age 4 with Gorlin's Syndrome, which is caused by a defect in this gene. She has spinal deformities similar to spina-bifida, and thousands of Basal Cell Carcinomas (BCC's), which are skin cancer, but with a lower-case c. The experience of being given this news was numbing, and in light of all the information we received, a cute name for the gene involved barely registered one way or the other.
My wife joined the board of the Gorlin's Syndrome Alliance, which is a non-profit organization that helps people with this disease find help, and also tries to push medical science forward in ways that can help. Their mascot is a hedgehog (for obvious reasons) wearing a hat - because people who have this condition have to avoid UV exposure all the time. If you're interested, please check them out: https://gorlinsyndrome.org/ (Currently suffering slow server).
A bit of whimsy isn't the worst thing, when the news is otherwise pretty hard.
It sounds like a great tool to use to soften the blow when telling the child.
"Hey Timmy, we got our results back, and we found out you have the Sonic the Hedgehog gene. It might be scary but if you can be as brave and tough as Sonic everyone will be very proud of you."
As I said, too many techbros without tact or life experience on this site. Geneticists moved away from silly names because physicians found that mothers who had just given birth to a severely disabled child did not take the silly names well. It's one thing to work in fly genetics and another to be talking to a parent whose child has a mutation in the human homolog.
This is actual experience from clinicians working in genetic counseling.
Sometimes I wonder if I am a normal human being, and then I realize that I'm not. I must be some kind of superhuman.
Less than a year ago I was told I had a C6 and C7 fracture, a manubrial fracture, a scapular fracture, a few metacarpal fractures, rib fractures, a splenic laceration.
Despite the Corvette C6 and C7 being some of my favourite cars, when I was eventually discharged I could not find it in me to take my AR-15 to SF Gen and unleash retribution for the horrendous crime the doctors had done to me. In addition, a man ubrial fracture? So I'm not a man anymore?! Ugh. Urge to kill rising!
This came up on a slide in a biology lecture I attended. I was the only computer science student present and did a double take when I saw it. I asked the lecturer if I had read correctly and she confirmed. Nobody else in the room found it amusing. If anything I think they found the novelty names tiresome.
"Two of these genes, desert hedgehog and Indian hedgehog, were named for species of hedgehogs, while sonic hedgehog was named after the video game character Sonic the Hedgehog.[15][16] The gene was named by Robert Riddle, a postdoctoral fellow at the Tabin Lab, after his wife Betsy Wilder came home with a magazine containing an advert for the game Sonic the Hedgehog.[17][18][19] In the zebrafish, two of the three vertebrate hh genes are duplicated: SHH a[20] and SHH b[21] (formerly described as tiggywinkle hedgehog, named for Mrs. Tiggy-Winkle, a character from Beatrix Potter's books for children) and ihha and ihhb[22] (formerly described as echidna hedgehog, named for the spiny anteater and not for the character Knuckles the Echidna in the Sonic franchise)."
There was controversy surrounding the name. If you're a doctor telling parents that their child is sick because of a defect in the sonic hedgehog protein, they're going to think you're trolling them. So these days they just tend to use abbreviations like SHH, and for new genes and proteins there is a standard nomenclature.
One of the lessons we've learned since the 90s is that cute names (for genes, proteins, servers, programming languages, etc.) can be inadvertently problematic or made retroactively problematic, and are better off avoided.
I have proposed the use of "marginalizing" as a replacement for "offensive" in the sense of "reinforces systemic biases against certain groups of people", after I saw character on a British procedural say "Don't call somebody 'hot' or 'a lush'; it's marginalising."
How is the list of biological processes ordered? Why is negative regulation of cell differentiation so far away from positive regulation of cell differentiation?
