Exam 4 (Prions) Flashcards
PrPc stands for:
Cellular prion protein
What gene codes for PrPc:
PRNP gene
_____ is found in the membrane of many eukaryotic cells, including humans:
PrPc
What causes PrPc to become PrPsc:
a conformational change
PrPsc is soluble or insoluble:
insoluble
____ initiates an autocatalytic reaction leading to the accumulation of ____ protein in the CNS—- leading to neurodegeneration:
PrPsc
amyloid
Is the function of PrPc fully understood?
No, but hypothesized to help protect neurons and aid in synapse formation
List the 3 forms of CJD:
1) Sporadic
2) Hereditary
3) Acquired
This type of CJD develops with no known history:
Sporadic
This type of CJD has a family hx of PRNP gene mutation:
Hereditary
This type of CJD is transmitted via exposure to brain/nervous tissue:
Acquired
T/F
Acquired CJD has been transmitted by dural implants, contaminated pituitary growth hormone, and cornea transplants:
True
___ in 1 million people get CJD annually, and the US has ___ cases per year:
1 in 1 million
300
CJD typically manifests around age ___, with death occurring within ___ months:
60’s
6 months
CJD is resistant to these two things:
Formalin fixation
Paraffin embedding
The median age of death of CJD patients in the US is ____, while the median age of death of vCJD patients in the UK is ____:
US: 68 years
UK: 28 years
How is vCJD diagnosis confirmed:
Through examination of brain tissue obtained by biopsy or at autopsy
The incubation period for vCJD is unknown, but presumed to be:
years/decades
Which presents with prominent psychiatric/sensory symptoms, and delayed onset neurologic abnormalities:
vCJD
T/F
vCJD has a diffusely abnormal non-diagnostic EEG:
True
The 1993 outbreak of vCJD in the UK may have resulted from:
the feeding of scrapie-containing sheep meat-and-bone meal to cattle
The vCJD outbreak in the UK may have resulted from the feeding of scrapie-containing sheep meat-and-bone meal to cattle, and was thought to be amplified by:
by feeding rendered bovine meat-and-bone meal to young calves
The number of cases of vCJD in the UK has decreased since 2000, likely due to:
Changes in cattle feeding practices
This disease was linked to cannibalism among the indigenous people of Papua New Guinea:
Kuru
Kuru was linked cannabalism among the indigenous people of:
Papua New Guinea
The incubation period of Kuru typically lasted ___years, but has been reported to be as long as ___ years:
10-13 years
50 years
The practice of cannibalism stopped in this decade:
1960’s
This is a prion disease interfering with sleep w/ severe mental deterioration:
Fatal Familial Insomnia
T/F
FFI is almost always inherited via autosomal dominant pattern, with a genetic defect in PRNP gene coding for PrPc:
True
How does FFI differ from other prion diseases:
FFI primarily affects the thalamus, which controls sleep
FFI typically begins at this age and death occurs within ____ months of symptom onset:
30’s
death within 7-73 months
What type of scan can be used to detect abnormalities in FFI:
PET scan
A PET scan may be used to detect abnormalities in this autosomal dominant inherited prion disease:
FFI
_____ is a prion disease that causes loss of coordination (clumsiness), followed by slowly delayed mental dysfunction:
Gerstmann-Straussler-Scheinker (GSS)
You would see deterioration of the cerebellum, responsible for muscle tone, coordination, and balance with this prion disease:
GSS
GSS affects people in their ____, and is typically fatal within ___ years:
40’s
5 years
GSS is inherited in this pattern, thought some cases can be sporadic:
autosomal dominant
T/F
All GSS cases are inherited:
False.
Some cases can be sporadic.
When and Where was CWD first identified:
1960’s in Colorado
___ is only found in deer and elk:
CWD
There is growing concern over the transmission of ___ from deer to livestock: cattle and sheep:
CWD
Is there documented transmission to humans like Madcow with vCJD:
NO!
This prion disease is seen worldwide and infects sheep and goats:
Scrapies
Scrapies infects ___ and ____:
sheep and goats
