Exam 4 (Prions)

Question 1

PrPc stands for:

Answer 1

Cellular prion protein

Question 2

What gene codes for PrPc:

Answer 2

PRNP gene

Question 3

_____ is found in the membrane of many eukaryotic cells, including humans:

Answer 3

PrPc

Question 4

What causes PrPc to become PrPsc:

Answer 4

a conformational change

Question 5

PrPsc is soluble or insoluble:

Answer 5

insoluble

Question 6

____ initiates an autocatalytic reaction leading to the accumulation of ____ protein in the CNS—- leading to neurodegeneration:

Answer 6

PrPsc

amyloid

Question 7

Is the function of PrPc fully understood?

Answer 7

No, but hypothesized to help protect neurons and aid in synapse formation

Question 8

List the 3 forms of CJD:

Answer 8

1) Sporadic
2) Hereditary
3) Acquired

Question 9

This type of CJD develops with no known history:

Answer 9

Sporadic

Question 10

This type of CJD has a family hx of PRNP gene mutation:

Answer 10

Hereditary

Question 11

This type of CJD is transmitted via exposure to brain/nervous tissue:

Answer 11

Acquired

Question 12

T/F

Acquired CJD has been transmitted by dural implants, contaminated pituitary growth hormone, and cornea transplants:

Answer 12

True

Question 13

___ in 1 million people get CJD annually, and the US has ___ cases per year:

Answer 13

1 in 1 million

300

Question 14

CJD typically manifests around age ___, with death occurring within ___ months:

Answer 14

60’s

6 months

Question 15

CJD is resistant to these two things:

Answer 15

Formalin fixation

Paraffin embedding

Question 16

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 ____:

Answer 16

US: 68 years
UK: 28 years

Question 17

How is vCJD diagnosis confirmed:

Answer 17

Through examination of brain tissue obtained by biopsy or at autopsy

Question 18

The incubation period for vCJD is unknown, but presumed to be:

Answer 18

years/decades

Question 19

Which presents with prominent psychiatric/sensory symptoms, and delayed onset neurologic abnormalities:

Answer 19

vCJD

Question 20

T/F

vCJD has a diffusely abnormal non-diagnostic EEG:

Answer 20

True

Question 21

The 1993 outbreak of vCJD in the UK may have resulted from:

Answer 21

the feeding of scrapie-containing sheep meat-and-bone meal to cattle

Question 22

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:

Answer 22

by feeding rendered bovine meat-and-bone meal to young calves

Question 23

The number of cases of vCJD in the UK has decreased since 2000, likely due to:

Answer 23

Changes in cattle feeding practices

Question 24

This disease was linked to cannibalism among the indigenous people of Papua New Guinea:

Answer 24

Kuru

Question 25

Kuru was linked cannabalism among the indigenous people of:

Answer 25

Papua New Guinea

Question 26

The incubation period of Kuru typically lasted ___years, but has been reported to be as long as ___ years:

Answer 26

10-13 years

50 years

Question 27

The practice of cannibalism stopped in this decade:

Answer 27

1960’s

Question 28

This is a prion disease interfering with sleep w/ severe mental deterioration:

Answer 28

Fatal Familial Insomnia

Question 29

T/F

FFI is almost always inherited via autosomal dominant pattern, with a genetic defect in PRNP gene coding for PrPc:

Answer 29

True

Question 30

How does FFI differ from other prion diseases:

Answer 30

FFI primarily affects the thalamus, which controls sleep

Question 31

FFI typically begins at this age and death occurs within ____ months of symptom onset:

Answer 31

30’s

death within 7-73 months

Question 32

What type of scan can be used to detect abnormalities in FFI:

Answer 32

PET scan

Question 33

A PET scan may be used to detect abnormalities in this autosomal dominant inherited prion disease:

Answer 33

FFI

Question 34

_____ is a prion disease that causes loss of coordination (clumsiness), followed by slowly delayed mental dysfunction:

Answer 34

Gerstmann-Straussler-Scheinker (GSS)

Question 35

You would see deterioration of the cerebellum, responsible for muscle tone, coordination, and balance with this prion disease:

Answer 35

GSS

Question 36

GSS affects people in their ____, and is typically fatal within ___ years:

Answer 36

40’s

5 years

Question 37

GSS is inherited in this pattern, thought some cases can be sporadic:

Answer 37

autosomal dominant

Question 38

T/F

All GSS cases are inherited:

Answer 38

False.

Some cases can be sporadic.

Question 39

When and Where was CWD first identified:

Answer 39

1960’s in Colorado

Question 40

___ is only found in deer and elk:

Answer 40

CWD

Question 41

There is growing concern over the transmission of ___ from deer to livestock: cattle and sheep:

Answer 41

CWD

Question 42

Is there documented transmission to humans like Madcow with vCJD:

Answer 42

NO!

Question 43

This prion disease is seen worldwide and infects sheep and goats:

Answer 43

Scrapies

Question 44

Scrapies infects ___ and ____:

Answer 44

sheep and goats