prion diseases


In 2010, Sonia Vallabh watched her mom, Kamni Vallabh, die in a really horrible way.

First, her mom’s memory started to go, then she lost the ability to reason. Sonia says it was like watching someone get unplugged from the world. By the end, it was as if she was stuck between being awake and asleep. She was confused and uncomfortable all the time.

“Even when awake, was she fully or was she really? And when asleep, was she really asleep?” says Sonia.

The smart, warm, artistic Kamni – just 51 years old — was disappearing into profound dementia.

“I think until you’ve seen it, it’s hard to actually imagine what it is for a person to be alive and their body is moving around, but their brain is not there anymore,” says Eric Minikel, Sonia’s husband.

In less than a year, Sonia’s mom died.

An autopsy showed Kamni had died from something rare — a prion disease. Specifically, one called fatal familial insomnia because in some patients it steals the ability to fall asleep.

Basically, certain molecules had started clumping together in Kamni’s brain, killing her brain cells. It was all because of one tiny error in her DNA — an “A” where there was supposed to be a “G,” a single typo in a manuscript of 6 billion letters.

Sonia sent a sample of her own blood to a lab, where a test confirmed she inherited the same mutation. The finding threw the family into grief all over again.

Today, Sonia and her husband live and work in Cambridge, Mass., where they are both doctoral students in the lab of Stuart Schreiber, a Harvard professor of chemistry and chemical biology. Over the past several years, the couple has completely redirected their careers and their lives toward this single goal: to prevent prion disease from ever making Sonia sick.

A Couple’s Quest To Stop A Rare Disease Before It Takes One Of Them

Photos: Kayana Szymczak for NPR

The brain above illustrates the degenerative changes and atrophy caused by Creutzfeld-jakob Disease, a form of transmissible spongiform encephalopathy. When ‘mad cow disease’ is transmitted to humans it is classified as a varient of CJD. Typically CJD is transmitted through exposure to infected tissue usually through medical procedures, however there can be a other causes - such as hereditary CJD. A person is infected with a type of protein known as a ‘prion’, these occur normally within humans but the infectious form is folded in an abnormal way, which then alters normal, healthy prions in the cells of the host.  It is thought that the neuronal loss and damage is caused by a build up of these proteins in the brain.

CJD is marked by a quick onset of neurological symptoms and a rapidly progressing dementia and decline of neurological functioning. Initially, individuals experience problems with muscular coordination; personality changes, including impaired memory, judgment, and thinking; and impaired vision. People with the disease also may experience insomnia, depression, or unusual sensations. As the illness progresses, mental impairment becomes severe. Individuals often develop involuntary muscle jerks called myoclonus, and they may go blind. They eventually lose the ability to move and speak and enter a coma. CJD is fatal, and there are no treatments for the condition. 

Fatal Familial Insomnia

FFI is an inherited prion disease that affects the thalamus. A person suffering from this disease loses the ability to sleep. This causes dementia and hallucinations. The person typically dies within 18 months. There is no known cure for the disease.

The disease is very rare and has only been recorded in 100 people.

anonymous asked:

I'm writing a character that doesn't sleep until she literally can't stay awake and passes out from exhaustion, because she's afraid of sleeping. Are there any conditions that cause this and if someone is aware of the problem, how can they help her?

Oh, boy, Anon.  You have finally given me an excuse to talk about one of my favorite diseases.

So there’s this thing called “Fatal Familial Insomnia.” (FFI)  In short, you stop being able to sleep.  You go crazy.  Then you die.  

There are four stages to this disease:

  • 4 months - First, the character gets chronic insomnia.  They start getting paranoid, develop phobias, and begin having panic attacks.  They start experiencing severe physical symptoms (excessive sweating, constipation, etc.)
  • 5 months - The character begins having hallucinations and their panic attacks get much worse.
  • 3 months - The character rapidly loses weight and is completely unable to sleep.
  • Over the course of the next 6 months, the character develops dementia, becoming completely unresponsive and mute.  
  • Then they die.  Do not pass go, do not collect $200.  

There’s no cure.  Taking sleeping pills doesn’t work.  In fact, being sedated makes the disease worse, and shortens the time until death.  The only thing that’s even slightly helped someone with this disease is spending nights in complete sensory deprivation chambers.  Even then, they still died within a few years.

FFI is an incredibly rare disease, and it’s only found in about 40 families in the world - hence the “familial” part of the name.  Only about 100 people in the world today have tested positive for this disease.

So what causes such a horrifying thing?


What the fuck is a prion, you ask?

Well, it’s something that can spread an infectious disease, just like bacteria or viruses.  But prions are different in that they’re made from something that’s usually normal inside the body - a protein - that has just been made wrong.  And it begins to spread its wrongness to other proteins.  These proteins basically begin to eat holes in your character’s brain.


Some prion diseases are fairly well known, such as Mad Cow disease, Creutzfeldt-Jakob disease, kuru, and scrapie. But FFI will always be my favorite.

This is probably not what you wanted, but I will take any excuse I can to talk about this disease. >_> Prions are terrifying and awesome.  But mostly terrifying.

Even still, looking at the symptoms experienced by people with FFI, who have severe, long-term insomnia, might help you writing your character.  If that still isn’t what you’re looking for, I should have the Demystifying the DSM for Sleep Disorders (including insomnia) early-ish next year :).

Get some sleep, y’all.  WHILE YOU CAN.

For more info:

angelicuscadere  asked:

For a sentient specie of omnivorous raptors (think feathered dino) with social complexity, technology and population density similar to humans ~300 years ago, what would be the most prominent health issues for the common folk?

I’m using birds and alligators as references for most things anatomy, so what would be avian/crocodilian equivalent to fleas, flu, cholera, measles, or other highly contagious and common ailments? (They have both feathers and scales)  They have had little to no contact with any large mammals over the course of their evolution - upon contact with mammals (including humans), would that make them less or more susceptible to be affected by human illness, or a random mix? I know this is very broad so I’m not expecting a detailed answer - I was just hoping you could give me some pointers as to what kind of diseases to investigate and inspire myself from.   Thank you! I really love your script blog! :)


Yay raptors! I hope you like info-dumps.

Originally posted by gifovea

If I assume a similar medical scene to the 1700-1800′s, I’d first broadly group the common diseases into parasitic, bacterial, viral and fungal. Most of these species don’t congregate in terribly large numbers, except in farms and fortunately for your writing, both birds and crocodiles are bred on farms in large numbers to give you disease examples that are probably common at high densities with sub-optimal hygiene. I will link to other sites for the most interesting ones.

Parasites are the group that were extremely common before effective medication, and also the most externally obvious. They’re also potential vectors for the other groups, to spread disease from one raptor to another (think about how mosquitoes do this today).

External parasites are your equivalent to fleas. Avians can get fleas, but mites and lice are far more common. Almost all wild birds are harboring some kind of feather lice. Reptiles commonly get ticks. Scaly leg mite might give you inspiration for a suitably interesting looking disease.

Internal parasites get a bit more variable, depending on the internal anatomy of your raptor species. Almost everything can get intestinal worms (because almost everything has intestines). Where exactly in the intestines they live will depend on anatomy, and young won’t get any placental transmission from their mother if they lay eggs. Worms like Heterakis can transmit other diseases to certain species too.

Birds get respiratory parasites, which are quite unique. Air sac mites may be relevant if your raptors have them, and gapeworm is one of my personal favorites. (Yes, I have favorite parasites. I’m not weird.)

Moving onto bacterial diseases, Cholera was a big killer of humans, and poultry have Fowl Cholera of their own. Botulism toxin kills a lot of birds that congregate around waterways, but interestingly birds and reptiles seem very resistant to tetanus.

Gut pathogens like salmonella are common in reptiles and birds, and are not species specific. These things can get into just about anything, but they are often host adapted. This means the usual species they infect doesn’t get as severe pathology as a new species. This may be relevant for your mammals who encounter this species, as it’s commonly spread by poor hygiene practices.

Psittacosis is a bacterial disease that you should definitely look into. It can affect both humans and parrots, and can be lethal in both. It was historically something of a mystery disease for a while, and worth reading about.

Most species (honestly, probably all species but we haven’t bothered to look) have a poxvirus of their own. Some of these poxviruses will cross species (eg goats and sheep) and will vary in how virulent they are (smallpox vs chickenpox). They hang around in the environment for a really long time and are difficult to exterminate. Your species probably has one, but despite the name not all poxviruses present with pox on the skin.

If your species is feathered, then Psittacine Beak and Feather Disease is simply fascinating and visually dramatic. It’s a chronic disease and may fill a similar social role as leprosy

Influenza viruses commonly affect many species of birds and will also potentially cross over to humans or other mammals. Human and mammal influenza can also cross over into birds. When you get an influenza type into a ‘new’ species, death rates are typically higher.

Most concerning, however, is when you have two different influenza strains infect the same individual, recombine by infecting the same cell, and then by chance produce a totally new strain of influenza which may then infect any species that could have been infected by either parent virus. Immunity to on strain of influenza offers little protection against another.  This is why bird flu outbreaks are such a concern.

I noticed you said no contact with large mammals over their evolution. If they’re farming, what’s eating their stored food? Rats are common and disease vectors to boot, if they have no rats, what do they have instead? Something will be taking advantage of food stores, and will be relevant to the diseases in the population.

And I don’t know if you considered it, but crocodillians tend to be cannibalistic. If they are, then you could potentially have a tapeworm species that spends it’s entire life cycle within this species. It matures and drops cysts in the intestine of one individual, those cysts are eaten by a second individual (faecal contamination of food most likely), then forming cysts in muscle or meat tissue, and when the 2nd individual is eaten by a 3rd individual, those cysts mature into the adult tapeworm to live inside their intestine, and the cycle begins again. There may also be a prion disease, though they are rare.

Pathophysiology of the Blight and Red Lyrium

All right!  This will be my last science meta post about Thedas for a while, unless something else strikes me.  As always, thanks to everyone who’s shown an interest in these sorts of posts.  I hope they’re entertaining and interesting, I know I’ve had a ton of fun thinking about them.

Part 1: Lyrium Addiction and Withdrawal

Part 2: Biology and Life History of Lyrium, and Dwarven Physiology

The Blight is two different entities: one is the coordinated attack on the surface world by darkspawn led by a tainted Old God, the other is the disease that results in infection and the creation of wastelands.  We’ll be talking about the latter.

The Blight pathogen has to be something pretty elastic; it is able to infect animals, plants, and fungus.  Our options for infectious agents include bacteria, viruses, fungi, parasites, and prions.  

Parasites are typically fairly host-specific, sticking to well-established life cycles, so they’re out; there’s no parasite in nature that would infect both plants and animals, and when they do infect animals of different classes, typically it’s just a handful (i.e. mosquitoes and dogs both are affected by heartworm, not mosquitoes, houseflies, beetles, ants, dogs, bears, birds, lizards, etc.).  

Viruses are more diversified than parasites; consider rabies virus, which is capable of infecting all mammals.  However, plants and fungi, as well as birds and reptiles, would be safe from rabies.  There are certainly viruses that jump entire classes of creatures - West Nile infects birds and mammals – but I really can’t name one that jumps kingdoms, affecting Animalia AND Plantae, for example; making it more doubtful that the Blight is a virus, in my mind.  

Keep reading

Orphan Black: Helsinki -- Episode 112
Tatiana Is Everyone: An Orphan Black Podcast
Orphan Black: Helsinki -- Episode 112

Orphan Black: Helsinki – Episode 112

Stephanie and Kris discuss the Helsinki series of Orphan Black comics, including the context we have for them from the show and how the comics give us new context for what we’ve seen on the show.

Context for Helsinki from the Series

  • A large number of self-aware clones (and civilians) had been killed in a short period of time
  • Ferdinand coordinated the operation that killed the clones
  • MK (Veera Suominen) survived and sought vengeance against Ferdinand, particularly for killing her friend Niki
  • Articles about the death of the “twins” are shown in 4x04 “From Instinct to Rational Control”

Context for Helsinki from Orphan Black #5

  • Rachel encountered Veera at the lab that exploded, causing her parents’ “deaths”
  • Rachel blamed Veera for that occurrence, slapping her burned, healing face as she recovered at Dyad
  • Veera is revealed to have survived the Helsinki protocol at the end of the issue.

MK’s (Veera’s) Background

  • Raised by Matti Suominen, whom she calls her “legal guardian” – is he actually her uncle? Why was she not placed with parents? Or did she have parents before whatever brought her to the Dyad labs in Orphan Black #5?
  • She was homeschooled.
  • The series hinted that MK is neuroatypical, and she tells Niki that she has Asperger’s.

Clone Support Group

  • Katja, Ania, Sofia, the twins (Fay & Femke), Justyna
  • Reminiscent of clone club (which is obviously the idea)
  • Both Sofia and Justyna had different body types than the majority of the clones, which is a luxury of the comic medium.

Dr. Dmitri Volkov

  • So many labs! Stephanie had assumed from what was shown in Orphan Black #5 that Veera and Niki had met either before the lab explosion that “killed” Rachel’s parents or right after that.
  • However! They met in a different context entirely.
  • Jade suggests that Dmitri was purposefully making his clone test subjects sick.
  • Do Jade’s statements and the scene between Marion Bowles and Susan Duncan suggest that they knew about the prion disease and were trying to fix that flaw to create more clones?
  • Dmitri was associated with the Cold River project (“the place of screams”), which Sarah found out about in 2x06 by following Maggie Chen’s trail.
  • The deformed fetuses in Dr. Volkov’s lab evoke the Brightborn babies.
  • Effie in the body bag is reminiscent of Jennifer Fitzsimmons.

Rachel (and Ferdinand)

  • Kris is so deeply uncomfortable with the way Rachel is depicted in these issues. She’s supposed to be 16 or 17!!!!
  • Stephanie appreciated how Rachel’s switch from brunette to blonde was incorporated.

Stray Thoughts

  • SO many people following other people! The Clone Project has a lot of money.
  • More shifty monitors who turn out to be not so bad? Maybe?

Cameos by Clone Club

  • Niki saw a young Sarah with Mrs. S and Felix in a park
  • Helena kills Ania
How Orphan Black trashed my favourite character.

Though I’m made up of 90% Delphine Cormier salt, there’s still 10% of my saltine self that remembers Cosima Niehaus. I spent season 3 watching in horror as my favourite character - to that point, Cosima Niehaus - became increasingly more unrecognizable to me, inching away from the vibrant, expressive, inquisitive clone who drew me into the show, for the sake of creating artificial drama. I could understand Sarah’s behaviour, I could understand Rachel’s behaviour, I could understand Delphine’s behaviour, I could even understand Alison’s behaviour (as much as I didn’t care for her storyline), but I could not, for the life of me, understand Cosima’s. “Getting over someone by getting under someone else” as an entire season arc did not cut it for me - not for this character. It’s really easy for people to dismiss any criticism of season 3 plots as “you’re just crazy shippers who can’t see outside cophine”, but I very much beg to differ. My investment in the ship has made me even more concerned about the individual plots and characterizations of Cosima and Delphine, and how their development fits with what has been built up in past seasons.

Onward, to the tolling of the bell. 

  • Cosima is a communicator who has had no problem lashing out at Delphine in the past. Why did their break-up consist of 0 communication? That’s not Cosima.

  • She puts her new hookup above researching her biology - something that was so important to her in the last two seasons - to the point where Scott starts being affected by her behaviour. Scott. Scott has no obligation to be there trying to help her, but as her friend, he’s there working on the science alongside her and for her, partaking in every poor decision she makes.
  • Cosima brought her new girlfriend to Dyad. Cosima willingly, willfully, brought her new girlfriend to Dyad - the actual belly of the beast. For what reason? After a couple of weeks of dating, you bring your new girlfriend to the hub of illegal cloning, run by people who have tried to capture and control you and your sisters multiple times, and where your ex-girlfriend now sits as director? The only possible motivation here would be spite, spite that blocks the fact that she’s pulling this new girlfriend into danger and any other logical thought Cosima would have normally had.

  • “Excuse me, are you stalking me now?” Obviously Delphine had her own hidden motivations for showing up - some more nice out-of-character shit writing - but really? After the history of that relationship, really?
  • Cosima tells Delphine “if you’re not going to be with me, then let me go” - meaning, very clearly, “I choose you, but if you don’t want me, then let me go to my second choice”. Would second choice be happy about this?

  • Cosima and Delphine share this ridiculous moment of passion - dams breaking open, feelings pouring out - and then instead of being like “fuck, what is going on here, we need to talk”, seeing how much just mentioning her near-death experience affected Delphine, she is back at second choice’s house the next day. Is this not unfair to second choice?
  • Cosima goes out of her way all season to hide Castor info and everything else from Delphine, when Delphine is quickly able to spot the prion disease, has ample knowledge of the clones, is invested, has resources at her disposal as director, sees what Rachel is capable of, and has already involved Sarah and Alison to shut down Helsinki. A little bit of communication at the beginning of the season - in the normal Cosima fashion - would have prevented so much of the mess. None of this made any sense for this character.

  • Despite all the evidence that shows Delphine’s willingness to help and protect the clones (I really have no patience for the “Delphine is a stalker that doesn’t respect Cosima’s autonomy” bullshit, but if you must), she and the other clones choose to ally themselves with Rachel, who has repeatedly tried to *kill them*, stole Kira, unabashedly smashed the bone marrow for Cosima’s treatment, tried to force surgery on Sarah, and initiated Helsinki v.2. They couldn’t come up with any other deal aside from “we’ll spring you from Dyad”? Scott and Cosima came up with the pencil plan last season, why go this other route now - especially after the Helsinki plot? I thought the first episode pretty strongly solidified the alliance between Delphine, Sarah and the other clones. They decided not to trust Delphine anyway, ignoring the time Delphine spent as Cosima’s doctor, basically providing her with palliative care for who knows how long, out of spite. For no logical reason.

It’s really shitty to lose your favourite character to bizarre writing choices. The above were the moments that really struck me as being unpalatable. Perhaps it’s just a change in what I personally can relate to now based on my real-life experience, but when I look at a character like Sarah, I can understand why she makes the choices she does. With Delphine, I can relate to the choices she’s made. So in that regard, it’s a personal thing - personal in the sense of what I can relate to and sympathize with. But that certainly doesn’t imply that the writing was solid and my tastes or life views are the reasons season 3 rang false for me - I think it just implies that I’m no longer able to give the writing a pass now that it’s gone and destroyed the main reason I could ignore/let slide the writing quality in the first place, which was Cosima Niehaus.

Godspeed, Cosima Niehaus.   (… new band name)

ELI5: Getting sick from a 'bacteria' vs. 'virus'

Bacteria produce harmful chemicals as part of their life processes. They use up your body’s resources (like eating your sugar or even eating your cells) and spit out toxic waste. Sometimes that waste is specifically designed to protect the bacteria by killing your immune system cells that try to attack it. But it also just basically poops all up in your body, which causes some damage. The symptoms of bacterial infections are related to what waste products the bacteria produces and where the bacteria is living. Your body fights bacterial infections by basically eating them, along with some other toxic chemicals that destroy them.

Viruses hijack the DNA in your cells to make more of the virus. They invade the cell and tell it to stop doing whatever it’s doing that your body needs it to do, and instead all it does is manufacture more of that virus. Eventually, the cell dies - usually by literally exploding - when it fills up with copies of the virus. Those viruses go on to infect other cells. Viral symptoms are caused by your body’s own attempt to kill them, and by the deaths of the cells they’re infecting. Your body fights viruses also by eating them, but it’s harder because they’re a lot smaller and have special protein shells that disguise them as “totally not a virus don’t eat me you guys”.

For extra fun, there are also prion diseases! Prions are proteins that folded the wrong way. When properly-folded proteins come into contact with prions, they re-fold into the same wrong shape as the prion. Your body can’t do anything about it because although it’s folded wrong, it’s still a protein that’s supposed to be there. Proteins are the way your body communicates and accomplishes certain things, so folding them wrong can really muck-up what is supposed to happen. In the case of Mad Cow Disease, as more and more proteins turn into prions, your brain turns to mush and gets holes in it until you go crazy and die.

If you think of your body as a factory that builds cars: bacteria are like a drunk hobo sneaking into your factory and dumping empty wine bottles into the machinery so it breaks. Viruses are like a roomba wandered in and reprogrammed your factory to start making more factory-invading roombas instead of cars. Prions are like a weird European car showed up and crashed into one of your factory’s cars after it left the factory, and now they both keep crashing into other cars (which then go on to crash into more cars) and also they all keep crashing into your factory.

Also fungal infections. Fungi can’t produce their own food, so they steal yours. Often that means invading parts of your body to get to it, and dumping toxic waste like bacteria. In the factory, a fungus would be someone building a shed attached to your factory and stealing your power so your factory doesn’t have enough to run and dumping garbage into your factory.

Also also, parasites. Parasites do the same thing as bacteria, but they’re [often] multicellular, so they’re much larger. Instead of a bunch of them, it’s usually a few big ones (although sometimes also a lot of them). In the factory, a parasite would be like the mafia moving into your factory, breaking stuff, and punching you right in the kidneys (or more likely, in the intestines) while they steal your money.

Explain Like I`m Five: good questions, best answers.

Eating brains helped Papua New Guinea tribe resist disease

Research involving a former brain-eating tribe from Papua New Guinea is helping scientists better understand mad cow disease and other so-called prion conditions and may also offer insights into Parkinson’s and dementia.

People of the Fore tribe, studied by scientists from Britain and Papua New Guinea, have developed genetic resistance to a mad cow-like disease called kuru, which was spread mostly by the now abandoned ritual of eating relatives’ brains at funerals.

Experts say the cannibalistic practice led to a major epidemic of kuru prion disease among the Fore people, which at its height in the late 1950s caused the death of up to 2% of the population each year.

In findings published in the scientific journal Nature, the researchers said they had identified the specific prion resistance gene – and found that it also protects against all other forms of Creutzfeldt-Jakob disease (CJD).

“This is a striking example of Darwinian evolution in humans, the epidemic of prion disease selecting a single genetic change that provided complete protection against an invariably fatal dementia,” said John Collinge of the Institute of Neurology’s prion unit at University College London, which co-led the work.

Papua New Guinea Photograph: Lloyd Jones/AAP Image

The Curse of the Boyfriend Sweater

“Consumption of troll liver is linked to a number of rare, incurable, and fatal diseases which infect and afflict the think pan and smooth muscle tissue, leading to slow, agonizing death.”

The tape measure wraps around a gray bicep, then slips away to hug Karkat’s chest.

“Cool story.”

“Prolapsed waste chutes and internal bleeding are not uncommon, although you’re more likely to die from heart failure or an aneurysm after a short bout of complete memory loss and insanity.”

“So a Prion disease.” The waist, now. My hands linger. “Humans get those from eating brains, but the liver is considered a delicacy by the more refined cannibals.”

“That’s why I’m warning you.”

“I’m not going to eat you, Karkat. That’s some Silence of the Lambs shit. I hated that movie.”

“I know.”

Keep reading

Prion diseases aren’t too bad. Easy to avoid: Cook your meat. Don’t eat people. Try your hardest not to be an eastern European Jewish person
—  Oh and don’t age, or have shitty luck.
Genetic mutation blocks prion disease
Unknown mechanism helped some people in Papua New Guinea escape historic, deadly outbreak.

Scientists who study a rare brain disease that once devastated entire communities in Papua New Guinea have described a genetic variant that appears to stop misfolded proteins known as prions from propagating in the brain1.

Kuru was first observed in the mid-twentieth century among the Fore people of Papua New Guinea. At its peak in the late 1950s, the disease killed up to 2% of the group’s population each year. Scientists later traced the illness to ritual cannibalism2, in which tribe members ate the brains and nervous systems of their dead. The outbreak probably began when a Fore person consumed body parts from someone who had sporadic Creutzfeldt-Jakob disease (CJD), a prion disease that spontaneously strikes about one person in a million each year.

Scientists have noted previously that some people seem less susceptible to prion diseases if they have an amino-acid substitution in a particular region of the prion protein — codon 1293. And in 2009, a team led by John Collinge — a prion researcher at University College London who is also the lead author of the most recent analysis — found another protective mutation among the Fore, in codon 1274.

Continue Reading.

anonymous asked:

Hello ^.^ I hope you're okay with answering questions about human flesh? One of my chars is a cannibal and I was wondering which parts exactly are safe to eat? Thank you!

Ahhh, I knew cannibalism asks would come around again! (Actually apparently the “again” part is a lie. Congrats on being the first!)

Human tissue is extremely dangerous to eat, because unless your cannibal character takes the time to screen the body for bloodborne diseases like hepatitis, HIV, TB, etc., they can wind up with some very devastating illnesses. (They may or may not already have one or more of these, and they may not care; it will depend on the cannibal.)

Beyond the potential for infection…. well, humans are mammals, and we’re made of meat. What parts do you eat on a cow? Muscles, livers, kidneys, heart, tongue, entrails (cleaned out and made into sausage casings), marrow, etc should all be edible. I would stay away from brains, because brains are how prion diseases like Creutzfeld-Jakob and Bovine Spongiform Encephalopathy (BSE, or Mad Cow Disease) get transmitted.


By the way, it’s actually not uncommon for junior doctors and paramedics in training to get hungry during autopsies; it’s believed to be a genetic predisposition, and it really freaks some people out if they don’t know it’s possible or even relatively common.

Hope this helps your story! xoxo, Aunt Scripty

A Woman of Experience

So, this is just a little, un-beta’d something I seemed possessed to write.  My work-in-progress that started before S03 even began keeps growing longer, so I figured I’d better put something out there before I forget how to use and AO3.  This goes out to my lovely internet spouses at OBFrankenfics, particularly thatscomplex, geekspiralling and tatarrific.  I hope it raises your… spirits, a little.

Note: Smutty smut-smut. NSFW.  NSFDriving.  NSFmy teenage friends who will probably laugh at me and read it anyway.  You have been warned.

Cosima sat, laptop open, staring out the window.  She should be working on the cure for the clones’ disease.  Even without Rachel agreeing to decode the cipher, the Castor brain had given them good information.  They were probably working with a prion disease.  However, not only was it presenting symptoms much different from other prion-based conditions, but prion diseases generally had no known cure.  She had already been unable to concentrate on her work since that day, that talk in the hallway with Delphine, but now, with her symptoms coming and going again and again and her distrust of the labs at DYAD, she was wondering if she’d be spinning her wheels even if she could focus, until somebody convinced Rachel to…

There was a loud series of knocks on the door.  Cosima started, and her elbow bumped her tea mug, sending it to shatter on the floor. There went her plan of pretending the apartment was empty.  A familiar voice, the very one she was afraid she’d hear, especially with Shay not there, came through the door.

“Cos-i-ma,” came Delphine’s voice, muffled but still clearly concerned.  "What was that?  Are you alright?“

"Shit,” Cosima mumbled to herself.  She hesitated long enough that there was another pounding on the door.

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anonymous asked:

i get that u dont like reed richards and ur entitled to ur opinion but u dont have to be so fuckin snide about it

The very first time Reed Richards saved the world from an alien invasion, he hypnotized the invaders into thinking they were cows and then released them into the food supply, which resulted in an outbreak of superpowered prion disease.

There already is a strong undertow pulling HRC toward “reaching out” to the GOP, toward governing from “the middle,” and toward not accelerating the now-rapid descent of the Republican Party into the final madness of the prion disease it has welcomed so warmly into itself ever since the late 1970s. Ryan Lizza of The New Yorker even posited that, as a gesture of good faith, HRC should allow the Republicans to pick a Supreme Court justice, a stratagem that has been proven to work only on The West Wing, which was not a documentary series.

Are You Ready for the End of the Republican Party?

Sorter Ryan Lizza: “Hillary Clinton should save the Republican party from Donald Trump because BOTH SIDES BOTH SIDES BOTH SIDES BIPARTISANSHIP BOTH SIDEZZZzZZZzzzzzzz”

Ryan Lizza may want to top huffing paint. I hear it does long term damage and can turn your brain into David Broder.

Identification First Potentially Effective Therapy for Human Prion Disease

Human diseases caused by misfolded proteins known as prions are some of most rare yet terrifying on the planet—incurable with disturbing symptoms that include dementia, personality shifts, hallucinations and coordination problems. The most well-known of these is Creutzfeldt-Jakob disease, which can be described as the naturally occurring human equivalent of mad cow disease.

Now, scientists from the Florida campus of The Scripps Research Institute (TSRI) have for the first time identified a pair of drugs already approved for human use that show anti-prion activity and, for one of them, great promise in treating these universally fatal disorders.

The study, led by TSRI Professor Corinne Lasmézas and performed in collaboration with TSRI Professor Emeritus Charles Weissmann and Director of Lead Identification Peter Hodder, was published this week online ahead of print by the journal Proceedings of the National Academy of Sciences.

The new study used an innovative high-throughput screening technique to uncover compounds that decrease the amount of the normal form of the prion protein (PrP, which becomes distorted by the disease) at the cell surface. The scientists found two compounds that reduced PrP on cell surfaces by approximately 70 percent in the screening and follow up tests.

The two compounds are already marketed as the drugs tacrolimus and astemizole.

Tacrolimus is an immune suppressant widely used in organ transplantation. Tacrolimus could prove problematic as an anti-prion drug, however, because of issues including possible neurotoxicity.

However, astemizole is an antihistamine that has potential for use as an anti-prion drug. While withdrawn voluntarily from the U.S. over-the-counter market in 1999 because of rare cardiac arrhythmias when used in high doses, it has been available in generic form in more than 30 countries and has a well-established safety profile. Astemizole not only crosses the blood-brain barrier, but works effectively at a relatively low concentration.

Lasmézas noted that astemizole appears to stimulate autophagy, the process by which cells eliminate unwanted components. “Autophagy is involved in several protein misfolding neurodegenerative diseases such as Alzheimer’s, Parkinson’s and Huntington’s diseases,” she said. “So future studies on the mode of action of astemizole may uncover potentially new therapeutic targets for prion diseases and similar disorders.”

The study noted that eliminating cell surface PrP expression could also be a potentially new approach to treat Alzheimer’s disease, which is characterized by the build-up of amyloid β plaque in the brain. PrP is a cell surface receptor for Aβ peptides and helps mediate a number of critical deleterious processes in animal models of the disease.