WTH? Maple Syrup Urine Disease?

WTH? Maple Syrup Urine Disease?

Before I continue on our caffeinated adventure, I wanted to post on something I found fascinating.  Not that all of what I post isn’t fascinating, but, I.. yeah.

Okay, so, Maple Syrup Urine Disease (MSUD)!

Yup. You read that right. What the hell is that?  You’re making crap up.

Nope, I’m totally not.  The name comes from one of its unique side effects.

Anyway, here it is.

On a previous post, I went into detail on BCAAs. If you haven’t checked it out, do it. In this post, I will go into detail on something I find interesting about them.

What do BCAAs and maple syrup urine have in common? MSUD is caused by an inability to break down BCAAs.

A not so fun fact on BCAAs: There are people in the world who are unable to process BCAAs properly because of mutations in certain genes.  Because of these mutations, the enzymes necessary to break down the BCAAs are not able to function properly, which causes these aminos, as well as their by-products, to build up within the body because the body is unable to clear them.

This excessive accumulation of BCAAs is incredibly toxic to the brain and various other organs, which can cause a host of unpleasant side effects.  One of the main side effects, which is unique to this disease, is maple syrup-scented urine, which gives it one of its names: maple syrup urine disease.  It also gives sweat and earwax a nice mapley syrupy scent as well. How nice. Worst case, this disease could lead to brain cell damage and death.

The side effects are a bit too long to list, so here’s a link to some more information if this has piqued your interest.

There is no cure, and it requires a lifelong avoidance of BCAA containing foods.  Those who are afflicted have to adhere to an incredibly strict diet.

It appears that liver transplants can be an incredibly effective treatment for those with MSUD, even allowing them to follow a normal diet!

However, it doesn’t mean they are cured of it.  MSUD can still be passed on to their children as well,  but it seems that both parents of the child have to have the mutated gene to pass it on.

Isn’t that interesting!?

Now, before you go thinking you have MSUD, it’s incredibly unlikely that you do.  It’s an inherited genetic defect, so if you had it there’s a pretty strong chance you would know by now.  Especially considering BCAAs reside within the vast majority of protein-containing foods.

Here’s a quick fun fact.

There are supplements, such as Fenugreek, that could alter the taste of certain… things…

It’s Semen.  It can possibly give semen a somewhat maple syrup taste.  Though, I wouldn’t… like… put it on pancakes… or .. ah…uh…


Don’t Judge me.


Here’s the article where I stumbled upon that info.  Don’t ask me why it just caught my eye.

I wanted to do something different today, so I hope you enjoyed it, and if you have any questions, feel free to send me an email or leave a comment below!  Don’t forget to subscribe. 🙂

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