prion prez

what is a normal cellular prion protein

proteins found on the surface of many cells, especially neurons in the brain.

what is the normal function of a prion protein

Though scientists are still trying to figure out the full extent of its functions, Prion proteins play a role in various neurological functions such as cell-to-cell signaling

how do the infectious form of prion proteins convert cellular prion proteins

The infectious variant of this protein does not contain genetic material, but changes the 3-D structure of the normal cellular prion proteins by altering how a portion of the amino acid chain folds when the proteins come into contact.

what is an affect of the infectious prion proteins

the infectious prion protein is richer in beta pleated sheets in the secondary structure, they tend to clump together and stack like pieces of paper. This tendency results in disrupted neuronal functions, triggering cellular stress and leading to neurodegenerative diseases

what are transmissible spongiform encephalopathies and which diseases are in this family

CJD, Kuru, FFI, Mad Cow Disease

CJD

rare, rapidly progressive, and fatal human prion disease that causes severe cognitive decline

Kuru

fatal prion disease historically transmitted through ritualistic consumption of infected human brain tissue

FFM Fatal Familial Insomnia

rare, inherited prion disease caused by a mutation in the PRNP gene that leads to progressively worsening insomnia

Mad Cow Disease (bse)

bovine spongiform encephalopathy, prion disease affecting cattle that causes progressive neurological deterioration and can be transmitted to humans through contaminated beef products

1 in how many people develop a TSE and is it curable?

500,000, incurable

early 1700s

scrappie discovered in sheep

early 1900s

creutzfeldt and jakob coin the term CJD to describe scrappie and its symptoms

mid 1900s

Kuru is described and spreads through cannibalistic practices in New Guinea, Kuru and CJD can spread to monkeys

late 1900s

1. Prion protein is cloned and found to be normally occurring

2. FDA bans mammalian proteins from cattle feed to prevent spread of mad cow disease

early 2000s

Antisense oligonucleotides extend prognosis in mice with infectious prions by reducing production of normal prion proteins

2022

Cryo-EM helps scientists map out the structure of prions at a near atomic resolution

2026

ION 717 trial reopens, similar to antisense oligonucleotides, reduces production of prion proteins that can be converted into infectious prion proteins. Treatment is delivered intrathecally