What codon 129 does

Dr. Brian Appleby posted an interesting blog this morning about genetic susceptibility to kuru.  I thought it was interesting to learn more about what codon 129 is responsible for since wee see this on our NPDPSC autopsy paperwork.  As someone who lost a mother at age 56 to fCJD, I thought it was interesting to learn that codon 129 affects age of onset of the disease.  I’ll have to find my mom’s paperwork, because I can’t remember what she was on codon 129.

First “24,” Now “The Simpsons” Tackle Prion Disease

Another TV show is tackling the difficult and complicated matter of prion diseases.  This time it’s another Fox show, “The Simpsons,” however, the show does nothing to help my cause of educating people about CJD.  Instead, it does everything to reinforce stereotypes about “mad cow disease.”  Lisa Simpson is the only one who speaks the truth, calling it an “abomination” to feed cows to cows.

I found the clip on Ecorazzi, which does blog about the show with class.  Thank you, Ecorazzi for understanding that prion diseases are no laughing matter.

1. Vegetarians can, and have, contracted CJD.

2. CJD does not make every one of its victims “mad” or aggressive.  Usually when BSE affects a cow, it does alter the personality of the animal. A normally calm cow can become aggressive, but a normally aggressive cow can become calm.

3.  People do not get “mad cow disease.”  BSE is “mad cow disease” and affects cows.  If a person eats tainted meat and contracts a prion disease from it, it is called “vCJD.”

To be honest, I’m intrigued as to what’s going on at Fox.

That’s two shows now working CJD into their plots, even if not calling the disease by its proper name.  I also find it ironic that this ‘Simpsons’ episode shows cannibalism–when cannibalism in Papua New Guinea a couple hundred years ago is believed to have been the beginning of prion disease with Kuru.  There is another interesting moment where a brain comes out of a skull and gets covered with glasses and dentures, in effect personifying the brain.  (Relax, it’s a cartoon).  This also jumped out at me since CJD is a brain disease.  Seeing this reinforces what I’ve believed since “24” saw Jack Bauer get CJD, that someone at Fox lost someone to CJD.

Who are you?

I see your grief working its way out through your art.  We need to talk–you can do so much for this cause that we share.