So, you know in Game of Thrones, there’s
this character—smells really bad, they call him “Reek”?
Turns out, that could actually be caused by medical conditions that we know of,
medical conditions that cause you to emit odors that go way beyond the typical stinky armpit.
In some cases, you might reek of boiled cabbage,
or sweaty feet, or even rotting fish.
These conditions are rare,
but their symptoms can be pungent,
and sometimes also downright dangerous.
Unusual body odors are often a sign of a bigger
problem—specifically, a defect in the way
your body is breaking down, or metabolizing, your food.
For example, there’s the condition known
as trimethylaminuria—
also known as “fish odor syndrome”.
Patients with this condition are said to smell
like decomposing fish, because their bodies
don’t break down a compound called trimethylamine,
which emits the je ne sais quoi of fishiness.
Now, everyone’s body produces trimethylamine—specifically, in the gut, where bacteria excrete it
while helping us digest foods like eggs, liver, and fish.
Normally, having all that trimethylamine
in your body is not a problem,
because it’s converted into an odorless molecule,
thanks to a special enzyme in the liver,
known as a flavin-containing monooxygenase.
But people with fish odor syndrome can’t
metabolize the smelly compound, because they
have mutations in the gene that produces that enzyme.
Without enough of that working enzyme, the
trimethylamine builds up, and has nowhere
to go but out with your bodily fluids—in
your sweat, urine, even on your breath.
But people with the condition do have some options.
They can change their diets so there are fewer
of the precursor chemicals
that get broken down into trimethylamine.
It’s one of the only times your doctor
will actually tell you not to eat your broccoli,
or your brussels sprouts!
Infusions of antibiotics can also help wipe out some of the bacteria that are making the trimethylamine.
These rarely solve the problem entirely, but
the good news is that apart from the smell,
there isn’t any major health problem associated
with fish odor syndrome.
Which is not the case for a disorder that gives people the distinctive whiff of sweaty feet.
This condition, known as isovaleric acidemia,
can cause brain damage, and even death,
particularly in young children.
Here, patients have a genetic mutation that
leads to a deficiency in an enzyme called
isovaleric co-enzyme A dehydrogenase.
This enzyme is important because it helps
break down the amino acid leucine.
Without this enzyme, leucine can only be broken
down part-way.
And the compound that’s left over from this
process, an acid called isovaleric acid,
starts to build up.
Isovaleric acid smells kind of like cheese,
and it’s the same chemical
that makes your sweaty feet smell.
The bacteria hiding out between
your toes produce this acid
when they’re chomping away on leucine.
But while isovaleric acid isn’t exactly
pleasant to smell outside your body,
it can be downright damaging to the inside.
It’s not exactly clear why, but a build-up
of isovaleric acid tends to have the most
dramatic effects on the central nervous system.
In large amounts, it’s toxic to neurons, which can result in developmental delays in many patients.
And because this enzyme deficiency makes it
difficult to digest breast milk or formula,
dangerous symptoms can start
appearing very soon after birth.
In severe cases, infants just a few days old
will refuse to eat and begin to have seizures.
There is, so far, no cure for isovaleric acidemia,
but some treatments—
like avoiding foods rich in leucine, and taking supplements of other, non-threatening amino acids—
can help keep patients safe.
Finally, peculiar symptoms and even stranger
smells can result from another, similar disorder
known as hypermethioninemia.
In this case, the problem is having too much
of a different amino acid: methionine.
Methionine is the rare amino acid that contains
sulfur, an element known for its pungent odor.
And when methionine isn’t metabolized properly
in your body, it can result in large amounts
of dimethylsulfide, which produces a smell
similar to boiled cabbage.
Sometimes the condition comes about just because
you’ve eaten too much methionine,
which is in protein-rich foods, like meat and cheese.
But if the cause is genetic, it can be due
to mutations in one of several genes
that are responsible for making the enzymes that help break down methionine.
Without those enzymes, patients sometimes have that cabbagey smell
in their sweat, breath, or urine.
And strangely, not everyone with the disease
has symptoms—in fact, most people don’t.
But in some, it can be serious.
In severe cases, the inability to process
methionine can lead to neurological problems
and muscle weakness, among other problems
in the nervous system.
Again, treatment usually involves avoiding
foods that contain methionine, as well as
taking supplements to make sure that the body
is getting what it needs.
So, run-of-the-mill BO is nothing compared
to the very real medical conditions that can
create unpleasant smells.
There are a lot of things that can go wrong
when your body metabolizes food,
and weird odors are just one way
to help spot and diagnose them.
This episode of SciShow is brought to you
by 23andMe, a personal genetic analysis company
created to help people understand their DNA.
The name ‘23andMe’ comes from the fact that human DNA is organized into 23 pairs of chromosomes.
Through genetic analysis, 23andMe users can
see which regions around the world their ancestors
came from, and learn how their DNA influences
facial features, hair,
sense of taste and smell, sleep quality and more.
You can also connect with people who share
similar DNA; and also learn how your DNA may
influence your health and wellness.
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