Final Exam Flashcards
Prions are made of _______.
Proteins
Viroids only infect _______.
Plants
Prions cause _____.
TSEs. Transmissible spongiform encephalopathies.
(T/F) Prions invoke immune responses like viruses.
False
We discussed 5 kinds of human prion diseases. List them.
Kuru, CJD, vCJD, GSS, and FFI
We discussed 4 animal prion diseases. List them.
Scrapie, BSE, FSE, and CWD
Where did Kuru originate?
In the South Fore people.
South Fore people practiced _________, a likely cause for the emergence of Kuru.
Endocannabalism
Kuru was originally known as _______ sickness.
Laughing
In the 1950s, _______ traveled to New Guinea to study Kuru.
Gadjusek
Gadjusek was the first to say that Kuru is _________.
Transmissible
What are the 3 stages of Kuru infection?
Ambulant, Sedentary, and Terminal
What is the length of the incubation period for Kuru?
Very long incubation period
_______ initiated research on Kuru, CJD, and Scrapie in 1972, later winning a Nobel prize for isolating a single infectious particle he named “prion”
Pruisner
Prion stands for?
Proteinaceous infectious particle (PrP)
What are the 2 forms of a prion?
PrPc (normal cellular protein) and PrPres (mutated)
In 1994, _______ used a yeast protein called ____ as a model organism for prion work, helping Pruisner’s work to be accepted.
Wickner, Ure2P
(T/F) Prions are not very resistant to routine inactivation methods.
False
PrP is encoded by the ______ gene.
PRNP
How does PrPres destroy neurons in the brain?
By accumulating into clumps
The misfolded protein structure of a prion has a high content of _____ _____.
Beta sheets
How are more proteins misfolded after initial infection with a prion?
The misfolded protein molecule catalyzes the misfiling of other proteins
PrPc may bind to ______ and be shuttled into lysosomes where it is degraded.
Copper
Prions infect which 4 regions of the brain?
Cerebrum, cerebellum, thalamus, and spinal cord
What are the 3 ways TSEs can arise?
Infectious, sporadic, and inherited.
Which TSEs are inherited?
CJD, GSS, and FFI
Which TSEs are infectious?
vCJD, Kuru, and CJD acquired via iatrogenic infection
Which TSEs are sporadic?
Sporadic (normal) CJD
How do eaten prions travel from the stomach to the brain?
Via splanchnic or vagus nerves present in the abdominal region
(T/F) The infectious dose of a prion is unknown.
True
(T/F) Prions are known not to exist in the blood.
False.
Which form of CJD (vCJD vs. sporadic CJD) has a longer incubation period?
Sporadic CJD
What is the average age at death for vCJD patients?
28
What is the average age at death for sporadic CJD patients?
68
How does one make a definite diagnosis of vCJD?
Prion-positive immunostaining of biopsy material from tonsils, spleen, or lymph nodes
How can one make a definitive diagnosis of vCJD post-mortem?
Western blot analysis of brain tissue
Genetic studies are conducted to determine if a patient suspected of having vCJD is (homozygous/heterozygous) for _________ at codon ____.
Homozygous
Methionine
129
Upon autopsy, slices of brain contain _______ similar to a sponge after TSE infection.
Vacuoles
The PRNP gene exists at codons _____ and ____.
129 and 219
(T/F) Mutations in the PRNP gene result in spontaneous formation of PrPres.
True
Transmissibility of TSEs among same species is (easy/impossible). Explain why.
Easy. High degree of homology in prion protein
What is the likely origin of BSE?
Likely came from scrapie infected sheep, disease spread to cattle by eating contaminated bone meal derived from sheep offal
What is the likely origin of vCJD in humans?
Ingestion of beef contaminated with BSE agent.