Exam 3 Flashcards
The primary structure of a protein can be defined as
a. the final structure of a protein folded in 3 dimensions
b. the complex the protein forms with other subunits
c. the sequence of nucleotides in the DNA
d. the sequence of amino acids in the polypeptide
d. the sequence of amino acids in the polypeptide
Amyloid Beta Plaques result from
a. prion induced misfolding of APP
b. protease cleavage of APP then beta fragment aggregation
c. inappropriate ubiquitin targeted degradation of APP
d. gamma secretase targeting of a-synuclein
b. protease cleavage of APP then beta fragment aggregation
Post mortem diagnosis of Parkinson’s requires identification of
a. amyloid plaques
b. necrosis
c. lewey bodies
d. huntingtin mutation
c. Lewey bodies
Parkinsons disease deficits are largely limited to
a. reactive movements
b. volitional movements
c. involuntary reactions
d. reflexes
b. volitional movements
Huntingtin’s disease results from
a. mutation of ubiquitin gene
b. mutation of the gene for huntingtin
c. mutation of the gene for the CAG protein complex
d. none of the above
b. mutation of the gene for Huntingtin
Alzheimer’s disease is characterized by
a. lewey bodies
b. Tau neurofibrillary tangles
c. spongiform holes in the brain
d. prion induced protein misfolding
b. tau neurofibrillary tangles
Successful pharmacological clearance of amyloid plaques in Alzheimer’s sufferers resulted in
a. clearance or resolution of symptoms
b. acceleration of symptoms
c. remission of symptoms
d. not effect on symptoms
d. not effect symptoms
What makes Kuru unique as a human prion disease
a. it doesn’t involve the prion protein
b. it was primarily transmitted through cannabilism
c. it is genetically transmitted
d. it only affects cows
b. it was primarily transmitted through cannabilism
The infectious agent in prion diseases is
a. a virus
b. a normal protein
c. a misfolded protein
d. cow brains
c. a misfolded protein
What is the role of genetics in prion diseases
a. it is a genetic disorder where a mutation is responsible for all cases
b. there is no role, it is a transmissible diseases that requires a pathogen
c. there is a complex interplay between genetics and environment
d. none of these
c. There is a complex interplay between genetics and environment
The difference between PrP^c and PrP^sc is
a. the amino acid sequence
b. the number of CAG repeats
c. the conformation of the protein
d. fatal familial insomnia results from one and Creutzfeldt-Jakob disease results from the other
c. the conformation of the protein
What is a protein secondary structure
a. beta loop
b. alpha helix
c. pore loop motif
d. none of the above
b. alpha helix
Protein folding occurs as a progressive process. Which are protein that aid in this process as the folding protein is produced
a. telomerases
b. chaperone proteins
c. bell hoop proteins
d. valet proteins
e. Tau
b. chaperone proteins
Ubiquitin is a common protein that may play a role in a couple of the neurodegenerative disorders. The basic role of ubiquitin is
a. to activate gene transcription
b. to target proteins for degradation
c. to target proteins to the nucleus
d. to block gene transcription
b. to target proteins for degradation
Excitation in a neuron caused by the release of inhibition is called
a. excitation
b. disinhibition
c. inhibition
d. disexcitation
b. disinhibition
A) Cortex > B) Caudate > C) Globus Pallidus (IS) > D) Thalamus
Using the connection above, the activation of cell A does what to the activation of cell C?
a. decreases it
b. increases it
c. does nothing
d. reclassifies it as inhibitory
a. Decreases it
Using diagram above, activation of cell A (cortex) does what to the activation of cell D (thalamus)?
a. decreases it
b. increases it
c. does nothing
d. reclassifies it as inhibitory
B. increases it
Same as above, which cell is inhibited as a result of the activation of cell A (cortex)
a. A (cortex)
b.B (caudate/putamen)
c. C (globus pallidus)
d. D (thalamus)
c. C globus pallidus
Same as above, which cell is disinhibited as a result of activation of cell A (cortex)?
a. A (cortex)
b. B (caudate/putamen)
c. C (globus pallidus)
d. D (thalamus)
d. D (thalamus)
Using the connection diagram which cells are tonic active in that system
a. A and B
b. B and C
c. B and D
d. C and D
C (globus pallidus) and D (thalamus)
Using the diagram what pathway is shown
a. direct
b. indirect
c. hyperdirect
d. none of these
a. direct
Using the diagram, activation of cell A will result in
a. increased muscle tone
b. decreased muscle tone
c. blockade of movement
d. tremors
a. increased muscle tone
Describe the infectious agent in prion diseases and how it causes infection in acquired or transmitted cases?
The infectious agent in prion diseases is a misfolded protein that lacks nucleic acid, and it is transmitted when the PrP^c protein misfolds in a way that causes others to misfold and aggregate
How is the affected gene different in an individual who has huntington’s disease?
the affected gene (huntingtin) is different because it has an abnormally large amount of repeating DNA sequences called CAG repeats
Very breifly define or describe a protein’s primary, secondary, tertiary, and quaternary structure
A protein’s primary structure is a sequence of amino acids, a secondary structure is a beta sheet or alpha helix, a tertiary structure is a three-dimensional structure of the polypeptide chain, and the quaternary structure is a structure formed by the interactions between multiple polypeptide chains
Briefly describe the relationship between the level of activation of the GPi and muscle tone
The level of activation in the GPi affects the amount of movement from the muscle tone. In the direct pathway the striatum inhibits the GPi cell’s normally tonic active state which inhibits the thalamus, so now with it being inhibited the thalamus is able to send glutamate to the motor cortex and movement happens. In the indirect pathway the subthalamic nucleus blocks the inhibition of the GPe cells so the GPi cells are disinhibited and will continue to fire GABA onto the thalamus preventing movement
Which site is affected by Parkinson’s disease
substania nigra compacta
How does the affected area lead to the symptoms of Parkinson’s disease? (Include NT involved and how they’re involved)
There is a loss of dopamine functioning in the substania nigra which leads to a loss of excitation and disinhibition of volitional movement initiation (weakens direct pathway)
Which site is affected by Huntington’s disease
striatum
How does the affected area lead to the symptoms of Huntinton’s disease? (Include NT involved and how they’re involved)
Symptoms arise from a combo of neuronal degeneration in striatum and neurotransmitter dysregulation (GABA, DA, Glu, and ACh)
Loss of GABAergic MSNs in striatum reduces inhibitory output to the GPe, this disinhibition of the GPe causes excessive inhibition of the subthalamic nucleus impairing normal movement control, DA signaling may become imbalanced contributing to chorea
What is the role of Dopamine in the direct pathway
Dopamine in the direct pathway increases the excitability of the striatum which makes glutamate receptors more sensitive. It also amplifies pathway’s excitatory input and reduces inhibitory output from GPi to thalamus
What is the role of dopamine in the indirect pathway? Be precise
dopamine reduces the activity of D-2 receptor expressing neurons in striatum which disinhibits the GPe, diminishing GPi activity and facilitates voluntary movement by decreasing thalamic inhibition
