Prion Diseases and Protein Misfolding

Dr. Moogly explains prion diseases, focusing on Kruetzfeldt Jakob disease, also known as transmissible spongiform encephalopathy. The video covers the pathophysiology, highlighting the conversion of normal prion protein (PRPC) to the disease-causing form (PRPSC), which aggregates and leads to neurological damage. The transmission of prion diseases through mutation or consumption of infected meat is discussed, emphasizing the role of PRPSC as an infectious agent. The video concludes with a summary of the key points.

What is another name for Kruetzfeldt Jakob Disease?

• Scrapie

• Transmissible Spongiform Encephalopathy

• Bovine Spongiform Encephalopathy

• Mad Cow Disease

In which species is the disease known as Scrapie?

• Humans

• Sheep

• Pigs

• Cows

What is the common term for diseases like Kruetzfeldt Jakob Disease, Bovine Spongiform Encephalopathy, and Scrapie?

• Bacterial Infections

• Genetic Disorders

• Viral Diseases

• Prion Diseases

What is the normal form of prion protein referred to as?

• PRP SC

• PRP C

• PRP B

• PRP A

What structural feature is rich in the normal prion protein?

• Gamma Turn

• Alpha Helix

• Beta Sheet

• Delta Loop

What happens to PRP C when it mutates?

• It becomes PRP SC

• It becomes PRP B

• It disintegrates

• It remains unchanged

What is the result of PRP SC aggregation?

• Improved brain function

• Enhanced memory

• Neurological damage

• Increased immunity

What was initially thought to be the infectious agent in prion diseases?

• Virus

• Bacteria

• Nucleic Acid

• All of the above

How can prion diseases be transmitted?

• Through air

• By eating infected meat

• Through water

• By direct contact

What structural change occurs in PRP C to become PRP SC?

• Alpha helix to beta sheet

• Delta loop to gamma turn

• Beta sheet to alpha helix

• Gamma turn to delta loop