What is Phenylketonuria?
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- เผยแพร่เมื่อ 26 ก.ค. 2021
- Have you ever looked at the label on a can or bottle of diet drink. If you have then you may find wording that looks something like this
Contains a source of phenylalanine. So what is phenylalanine and why is it important that they tell us about it. Let’s find out more.
Phenylalanine is a component of aspartame which is an artificial sweetener about 200 times sweeter than sugar. It is used extensively worldwide in products as wide ranging as drinks, cakes and even crisps, and is even used as a table top sweetener.
There is a genetic disorder called phenylketonuria This is a bit of a mouthful and so if often abbreviated to PKU. This is associated with a faulty PAH gene. Since I inherit 2 copies of the PAH gene (1 from mum and 1 from dad), as long as I inherited one working version of the PAH gene from either parent. I’ll be able to make phenylalanine hydroxylase normally. If however both of the versions of the PAH gene that I inherited are faulty, All the instructions that I have for that enzyme are faulty. This results in the enzyme having the wrong shape and therefore being unable to convert phenylalanine into tyrosine.
Links to PKU support organisations
National PKU Alliance (USA) www.npkua.org/
National Society for PKU (UK) www.nspku.org/ - วิทยาศาสตร์และเทคโนโลยี
Not completely sure if I ever saw this label, but I am happy to know this, thanks for letting me know.
Thank you very much for watching the video as always.
EXPLANATION WAS DAMN WONDERFUL
Phenylketonurics: Contains phenylalanine
I always wondered what the significance of that was from quite a young age. Appreciate the video!
great video. highly helpfull
Thank you.
that's a very informative video thank you
I've been diagnosed with PKU but never really told to be kept on a strict diet, the only thing I was told is to make sure my pregnancy is planned and that I do follow the diet before and during it. Is that right? shouldn't I follow the diet all the time? I'm 26 and the more I learn about PKU the more I'm worried about the symptoms and possible damage
Fun fact, if you disregard all chemistry and just follow grammatical rules, you divide these two words phenylalanine and phenolphtalein into syllables incorrectly (phe-ny-la-la-nine and phe-nolph-ta-le-in), with a misplaced division compared to the correct forms which counts the words as compond words (phenyl-alanine and phenol-phtalein)
First
Thank you for being first, and thank you for watching the video.
Great Video! Helped me understand PKU :)