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Fish Smell Syndrome (Trimethylaminuria)

Min Bannister

Possessed dog
Joined
Sep 5, 2003
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5,994
I have never heard of this before. This unfortunate lady lacks an enzyme which breaks down trimethylamine in her gut causing her to smell of rotten fish!
https://www.bbc.co.uk/news/uk-england-bristol-51006604
A woman who has a syndrome that causes her to smell of rotten fish says it is like "living with a death sentence".
Paula Thomas, 45, from Bristol, has trimethylaminuria (TMAU), which prevents the body from breaking down materials found in certain foods.
Mrs Thomas said she missed school plays to avoid being in crowded places, had been made to get off buses, and felt racially abused because of her smell.
Here is a more in-depth discussion of the condition.
https://rarediseases.org/rare-diseases/trimethylaminuria/
Trimethylaminuria is a rare disorder in which the body's metabolic processes fail to alter the chemical trimethylamine. Trimethylamine is notable for its unpleasant smell. It is the chemical that gives rotten fish a bad smell. When the normal metabolic process fails, trimethylamine accumulates in the body, and its odor is detected in the person's sweat, urine and breath. The consequences of emitting a foul odor can be socially and psychologically damaging among adolescents and adults.
The genetic or primary form of this disorder is transmitted as an autosomal recessive trait. The metabolic deficiency occurs as a result of a failure in the cell to make a specific protein, in this case the enzyme flavin-containing monooxygenase 3 (FMO3). Enzymes are nature's catalysts and act to speed up biochemical processes. Without this enzyme, foods containing carnitine, choline and/or trimethylamine N-oxide are processed to trimethylamine and no further, causing a strong fishy odor.
 
I have never heard of this before.

I had heard of it because I once saw a highly-exploitative, though entertaining, tv documentary, subtly entitled something like Britain's Smelliest People!

Remedies, iirc, ranged from careful regulation to the diet to the surgical removal of the sweat-glands!

There were several consultations with medics included, the camera closing in for reaction-shots as the docs struggled to control their gag reflexes, confronted by the mephitic reek of their clients . . . :eek:
 
I noticed a very strong smell of fish in town today, nowhere near any food establishments*. Twice as I passed the same spot. Now I wonder if it had anything to do with the little old ladies standing outside.


*In fact, the only place in town with raw fish is the fishmongers stall in the market but he never smells.
 
I have never heard of this before. This unfortunate lady lacks an enzyme which breaks down trimethylamine in her gut causing her to smell of rotten fish!
Nah, she's either a Lovecraftian Deep One or a sex worker; possibly both. This whole enzyme thing is just an elaborate cover story to hide her Lovecraft.
 
She sleeps with the fishes

Or possibly the fetishists? I bet there are Trimethylaminuriaphiles* out there! :oops:

*Edit 10.01.2020, 12.13 am, They have yet to form an online community. I was afraid of what I might find, Googling the above word and related ones, but drew a blank!
 
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In primary school we had a visiting art teacher who always smelled of bad fish so perhaps that's what she had. I remember she always wore lots of fur as well.
The boys thought it might have been her period but it was stronger than that,
 
Yup, there is indeed (as we know) a medical condition which makes sufferers reek. Now and then it comes up as a Reader's Amazing Story in the misery mags.

Nothing to do with hygiene, mostly diet. Patients are hugely grateful for a diagnosis and help with their diet after years of humiliation.

I really feel for them, it must be awful to know you've showered and put on clean clothes but still stink and everyone despises you.
 
Reminds me of the old joke about the man who complains to his doctor that he feels great but he smells bad. Looking in his medical books, the doctor replies, "It says here, you're a vagina."
 
On a somewhat related note ...

There's a recently-discovered human genetic mutation that prevents people from smelling the stinky fish aroma of trimethylamine - the same substance that underlies the syndrome described here.

This means there are folks who wouldn't be put off by someone with trimethylaminuria.
People With This Mutation Can’t Smell Stinky Fish

Researchers in Iceland have identified a new mutant superpower — but the genetic trait probably won’t be granting anyone admission to the X-Men.

A small contingent of the world’s population carries a mutation that makes them immune to the odious funk that wafts off fish, according to a study of some 11,000 people published Thursday in the journal Current Biology. The trait is rare, but potent: When faced with a synthetic odor that would put many people off their lunch, some test subjects smelled only the pleasant aroma of caramel, potato or rose.

The vast majority of people aren’t so lucky. Nearly 98 percent of Icelanders, the research said, are probably as put off by the scent as you’d expect. The mutation is thought to be even rarer in populations in other countries. ...

Study participants were asked to take a whiff of six Sniffin’ Sticks — pens imbued with synthetic odors resembling the recognizable scents of cinnamon, peppermint, banana, licorice, lemon and fish. They were asked to identify the smell, then rate its intensity and pleasantness. ...

The reek of fish, however, was mostly recognizable and received by far the lowest pleasantness ratings among the six sticks. But a small group of people consistently tolerated or even welcomed the piscine perfume: those born with a genetic mutation that incapacitated a gene called TAAR5.

TAAR5 helps make a protein that recognizes a chemical called trimethylamine, or TMA, that is found in rotten and fermented fish and certain animal bodily fluids, including human sweat and urine. ...

FULL STORY:
https://www.nytimes.com/2020/10/08/science/smell-fish-genes.html

PUBLISHED REPORT:
https://www.cell.com/current-biology/fulltext/S0960-9822(20)31343-9
 
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This Smithsonian Magazine article about the genetic mutation gives some additional context.
If Rotten Fish Smell Like Roses to You, a Genetic Mutation Might Be to Blame

A new study in Iceland found a connection between a person’s ability to sniff stinky fish and a gene called TAAR5

There are two kinds of people in the world: Those who can smell stinky fish, and those lucky enough not to. A new study published in Current Biology on Thursday shows how a rare genetic mutation makes some people immune to the nauseating aroma of rotten fish, Nature News reports.

About 11,000 people participated in the study by providing DNA samples and putting their noses to the test. When presented with a each of six odors, each participant took a whiff and tried to identify it. For many people, the rotten fish smell was easy to identify and incredibly unpleasant, but a small group labeled the scent as something neutral.

A look in their DNA revealed that the group shared a genetic mutation in common. They all had at least one broken version of a gene called TAAR5. ...

The gene makes the tool that cells in your nose use to identify a rank chemical called trimethylamine, or TMA, which is also found in faeces, blood and bad breath, Donna Lu writes for New Scientist. TMA is a red flag for iffy food, and people’s disgusted reaction to its sickening smell helps them avoid danger.

“TAAR5 is a very conserved gene, so it’s very similar across species, probably because it has been important to protect us against harmful microorganisms,” says deCODE neuroscientist Rosa Gisladottir to New Scientist. ...

FULL STORY: https://www.smithsonianmag.com/smar...mell-roses-mutation-might-be-blame-180976029/
 
Maybe it's with other kinds of smells as well? My sense of smell is very strong but there used to be an old lady who lived around here who was called The goat lady because she apparently smelled so bad and kept goats as well.
Now I found her quite a pleasant person to talk to and could never smell her at all, but businesses and the bus driver used to spray after she was there.
I was shopping once with a neighbour and stopped to chat to her and my neighbour was retching and trying to drag me away.
 
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