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Bye Bye, Sonic Hedgehog

sonichedgehog.jpgI planned to make a good start for Gene Genie but my first gene related post is about a farewell. Sonic Hedgehog, one of the most famous genes, will lost his name. According to the Discover article:

An international committee of human geneticists recently voted to change the names of a few human genes because the current, sometimes flamboyant names—like Sonic hedgehog—are either offensive or embarrassing… The vote was conducted by the HUGO Gene Nomenclature Committee, which establishes naming protocols for human genes.

The gene map locus is 7q 36 . Sonic Hedgehog regulates vertebrate organogenesis, the growth of digits on limbs and organisation of the nervous system (brain, spinal cord, thalamus, zona limitans interthalamica). Additionally, fruit flies with a mutation in this gene don’t have external genitalia.

The so called hedgehog gene (hh) was first identified in 1978 as a morphogen. Sonic got his weird name after a phenomenon well described in the Wikipedia article: the hh loss of function mutant phenotype causes the embryos to be covered with denticles (small pointy projections), much like a hedgehog.

What kind of disorders is this gene responsible for?

  • Holoprosencephaly type 3: cerebral disorder characterized by the failure of the prosencephalon (forebrain) development in the embryo.
  • Ocular Colobomas: eye related malformations that result from a defect in the fusion of the fetal optic fissure
  • Preaxial Polydactyly II: anatomical variant consisting of more than the usual number of digits on the hands and/or feet.
  • Cleft Lip and/or Palate

shhmap.jpg
Click to enlarge!

1732_fs.jpg
Zebrafish embryo with a stain that shows where the hedgehog protein is acting during development.

References :

Update! One additional sentence from the Wikipedia article: many “[call] it inappropriate that patients with ‘a serious illness or disability are told that they or their child have a mutation in a gene such as Sonic hedgehog.’” (Thank you, Darmok, for the suggestion!)

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17 Comments Post a comment
  1. It’s about time. I always thought Sonic hedgehog was a ridiculously frivolous name for such an important gene. Some whimsicality is fine, but I also feel that there should be some respectability as well; as the Wikipedia article states, many “[call] it inappropriate that patients with ‘a serious illness or disability are told that they or their child have a mutation in a gene such as Sonic hedgehog.’”

    February 6, 2007
  2. I must update my post with that sentence…

    February 6, 2007
  3. Phil #

    This is ridiculous.

    “Ma’am, I’m sorry, but your son has a mutation of the gene 1335424353243t545324324ddg.”

    Is that somehow more comforting than

    “I’m sorry, your son has a mutation of the gene ‘Sonic hedgehog.’”

    If doctors are worried the patients aren’t going to take it well, why talk about which gene the mutation is on? Why not just refer to it as “Shh.” Or say “an important morphogen.” Or say “a defect in the Gli pathway.”

    Buncha stuffed-shirted killjoys…

    February 9, 2007
  4. You’re righ, but I think that usually they only talk about the malfunction, not the gene itself.

    February 10, 2007
  5. It should be possible to find a name which is easy to remember and also describes the gene’s function. I do believe that medicine should carry a certain degree of formality.

    I’m sorry for those who look for joy in whimsical gene names, but perhaps they will be able to find alternate sources of joy.

    February 11, 2007
  6. Attis #

    That just called into my mind an old story. Professor Corbató, the lead developer of the legendary MULTICS operating system said one of the reasons why MULTICS has been able to stay alive for more than 30 years was the policy to avoid funny names. On the contrary, the GNU and Linux community enjoys using these names. GNU still has a library called libiberty.so that would be linked into a program by the -Liberty switch. Technical fields always have had the tendency for puns and insider jokes, and getting deprived of them will also hurt people. Including me. I liked that name.

    February 13, 2007
  7. Sue Pattison #

    For the layman, the language of science, is obscure, unrecognisable, and beyond grasp. I think gene names like Sonic hedgehog, remind us and our patients, science is for everyone, it is human, and those who make discoveries are human as well. A little language everyone can understand, I think is helpful, we shoudl strive for more descriptive words in all our medicine, otherwsie we set ourselves up for elitism by language, when we should be breaking down barriers.

    May 2, 2007
  8. Danny Coyle #

    I agree with Sue on this point. And besides, Sonic Hedgehog isn’t just a mutation. There is a lot of research going into it to make it possibly even speed up the production of stem cells in the brain! And ironicly, that fits our blue, spiky friend quite well!
    I say let Sonic have his gene! And hey, maybe we’ll let Mario have one too…

    September 19, 2007
  9. I’m afraid I didn’t listen much to my boring Genetics and Biology professors way back in college. That’s why I was really amazed upon seeing the photo of a developing embryo with the stained hedgehog. But one struck my mind – why was it called as such? Hedge because it acts as support. What about the hog? Surely scientists have this strange way of naming things.

    November 8, 2007
  10. Jeff, a Sonic Hedgehog patient advocate #

    I belong to an organization of people with Gorlin Syndrome, or Nevoid Basal Cell Carcinoma Syndrome, which stems from the sonic hedgehog mutation. We actually can identify much better with a friendly sounding name to a most severe chronic condition which includes oral keratocysts, medulloblastoma (pediatric brain tumor) and loads of invasive basal cell carcinomas. No one takes it lightly due the name of sonic hedgehog, but it makes it more human, more easily articulated for the layperson. Our Executive Director is a patient herself, with severe morbidity, however she has a collection of homemade hedgehogs, knit, carved etc., that she uses to teach the affected children about our condition. We fully support the hedgehog name for this mutation. We have a LIfe Support Network, and are proactive and supportive. Please don’t ding the name, empower it!

    January 12, 2008
  11. Anonymous #

    I think that whoever finds it should get to name it whatever they want as long as its not blatantly offensive.

    March 9, 2009
  12. Anonymous #

    i think people are retards

    November 17, 2010
  13. Hi there! This is kind of off topic but I need some advice from an established blog. Is it very hard to set up your own blog? I’m not very techincal but I can figure things out pretty quick. I’m thinking about setting up my own but I’m not sure where to begin. Do you have any ideas or suggestions? Many thanks.

    September 21, 2011
  14. Adebayo #

    No fair….completely unfair. if you find it you name it, plus the name is slightly descriptive and complicated names are more scary, more ominous and very hard to relate with. i wish that that council respected the original name considering that it wasn’t offensive and completely unrelated to the gene or gene mutation.

    November 24, 2012

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