Submitted by Blakut t3_11mpj0v in askscience
-Metacelsus- t1_jbke35s wrote
Reply to comment by iayork in Are prions/prion diseases transmittable from an infected human mother to a fetus? by Blakut
> prion-strain specific
What is a "prion strain"? Are there different misfolded forms of PRNP?
iayork t1_jbkfq5k wrote
Yes, certainly. That's why there are multiple different prion diseases in humans, such as Creutzfeldt-Jakob Disease, Gerstmann-Straussler-Scheinker Syndrome,Fatal Familial Insomnia, and Kuru.
>Although PrPC is encoded by the host genome, prions themselves encipher many phenotypic TSE variants, known as prion strains. Prion strains are TSE isolates that, after inoculation into distinct hosts, cause disease with consistent characteristics, such as incubation period, distinct patterns of PrPSc distribution and spongiosis and relative severity of the spongiform changes in the brain. The existence of such strains poses a fascinating challenge to prion research.
Radirondacks t1_jbkje31 wrote
Isn't FFI an example of what OP was wondering about? Or is it different because it's the gene responsible for causing it that's passed down, not the proteins themselves?
Lil_Pharma00 t1_jbl467w wrote
I’m only in undergrad, but I do all of my projects and papers on prions and I am under the impression the second thing you said is true, it’s just the genetic basis.
aTacoParty t1_jboeyi1 wrote
FFI is caused by a mutation in the PrP gene that predisposes it to misfolding causing disease. If this DNA mutation is not passed down to the fetus, then it won't get the disease even if the mother has FFI. The misfolded protein itself will not be passed from mother to fetus.
Radirondacks t1_jbol4sk wrote
I see, thank you!
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