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Block 8 Week 5 Colt > Neurodegenerative Diseases - Parks > Flashcards

Neurodegenerative Diseases - Parks Flashcards

1
Q

AD, PD, HD, and ALS all show a progressive loss of (blank) in specific areas, along with loss of glial cells

A

neurons

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2
Q

What two proteins are in the inclusions in AD?

A

AB protein

Tau protein

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3
Q

Where are the inclusions located in AD?

A

amyloid plaques are extracellular

tau protein is intraneuronal

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4
Q

What is the protein in the inclusions in PD?

A

Alpha-synuclein

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5
Q

Where are the inclusions located in PD?

A

inside neurons

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6
Q

What are the two proteins involved in the inclusions in ALS?

A

TDP-43

SOD1

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7
Q

where are the inclusions located in ALS?

A

inside the neurons

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8
Q

What is the protein in the inclusion in HD?

A

huntingtin

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9
Q

where are the inclusions located in HD?

A

inside the neurons

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10
Q

what are the two fates of old or misfolded proteins?

A

Ubiquitinated and sent to proteosome

autophagy via lysosomes if they form aggregates

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11
Q

what class of proteins form aggregates?

A

globular proteins

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12
Q

Describe the formation of an inclusion body?

A
  1. wear/tear exposes hydrophobic surfaces
  2. small soluble oligomers form; resistant to degradation
  3. soluble aggregates of the oligomers form
  4. large aggregates become inclusion bodies
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13
Q

At what point in inclusion body formation is the oligomer subject to autophagy mediated degradation?

A

formation of soluble aggregates

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14
Q

T/F: individual oligomers can be the toxic substance even before inclusion formation

A

true

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15
Q

Lewy bodies are seen in which disease?

A

PD

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16
Q

What types of neurons are involved in PD?

A

dopaminergic

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17
Q

Where is the greatest neuronal loss in PD?

A

substantia nigra

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18
Q

protein aggregation and inclusion elicits a (blank) repsonse from the cell

A

stress response

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19
Q

inclusions are (directly/indirectly) toxic to neurons

A

directly

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20
Q

Some aggregates are capable of behaving like (blanks); aggregates from one neurons can be taken up by another neuron leading to more aggregates

A

prions

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21
Q

in the neurodegen dzs, misfolded proteins from what conformation?

A

b-sheets and amyloid fibrillar aggregates

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22
Q

(deep/cortical) neurons are filled with tangles and extracellular plaques

A

cortical

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23
Q

neurofibrillary tangles are outside or inside the cell?

A

inside

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24
Q

Where does AD begin in the brain?

A

hippocampus – temporal lobe

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25
When are AZtherapies effective in treating AD?
only during MCI stage
26
an amyloid plaque as a (blank) core
beta-amyloid core
27
formation of amyloid plaques leads to the phsycial disruption of...
dendrites
28
formation of amyloid plaques elicits what "healing" response from the brain?
gliosis
29
What protein makes up the neurofibrillary tangles?
tau
30
Whence is the origin of AB protein?
amyloid precursor protein APP; a transmembrane synaptic protein
31
APP is cleaved by (blanks) a, b, and g, forming AB monomers
secretases
32
which secretase is most important in the formation of amyloid plaques?
secretase b
33
what is the result of the formation of AB oligomers?
formation of aggregates, amyloid fibrils, and neuronal damage
34
what is the function of normal tau?
a microtubular protein that is part of the cytoskeleton
35
Amyloid beta activates a (blank) which (blanks) tau protein
kinase; phosphorylates tau
36
What happens when tau becomes hyperphosphorylated?
falls off the microtubules
37
Tau that has fallen off the microtubules forms (blanks) which form neurofibrillary tangles
paired helical fragments
38
Tau is interfering with microtubules and intracellular traffic while the plaques are interfering with....
dendritic connections
39
do tangles form before or after plaques?
after
40
t/F: plaque formation is a part of normal aging
true
41
Radioactive imaging tracers that bind to beta amyloid can be visualized with....
PET
42
T/F: AD pathology overlaps with normal people
true
43
T/F: tangles are present in the normal aging brain
true
44
What are the three CSF biomarkers that help in the Dx of AD?
LOW beta-amyloid 1-42 HIGH total tau elevated phosphorylated tau
45
Why is CSF beta amyloid low?
the amyloid is being deposited in the plaques and not in circulation
46
The CSF signature of AD has a low (specificity/sensitivity)
specificity
47
T/F: AD has motor components
false
48
PD involves what set of nuclei in the brain?
basal ganglia
49
the largest concentration of dopaminergic neurons in the brain are found in the...
substantia nigra
50
Where do the axons of the substantia nigra project?
corpus striatum
51
Degeneration of projections from the substantia nigra to the corpus striatum leads to what Sx?
severe tremors | muscle rigidity
52
Hyperactivity in substantia nigra neurons is associated with....
addictive behaviors
53
Cocaine causes extracellular buildup of DA in the...
limbic system
54
how does cocaine cause the buildup of DA in the limbic system?
inhibition of Na dependent DA reuptake
55
T/F: PD neurons are fucntional, its just that they lack coordination
true
56
what are the two components of the striatum?
caudate nucleus | putamen
57
What contributes the most to neuronal death in PD?
alpha synuclein oligomers
58
Alpha synuclein forms what type of inclusion?
lewy body
59
t/F: alpha synuclein can spread like a prion
true
60
Prion like spread of alpha synuclein in PD leads to....
dementia
61
dementia with lewy bodies is clinically between which two diseases?
PD and AD
62
What are two notable physical findings in ALS?
muscle weakness | fasciculations
63
What physical changes happen to the spinal column in ALS?
hardening of the lateral columns of the spinal cord
64
Gliosis follows the degeneration of the (blank) tracts in ALS
corticospinal
65
ALS involves both (blank and blank) neurons
upper and lower motor neurons!!!!
66
(blank) degeneration in ALS leads to demyelination and scarring
Wallerian degeneration of the axons
67
where do motor signals originate?
precentral gyrus -- motor cortex
68
Signals from the precentral gyrus flow via which fibers on the way to the spinal cord?
posterior limb of internal capsule
69
where do the motor fibers decussate?
in the pyramids
70
Upper motor neurons travel down the spinal cord in which tract?
lateral corticospinal tract
71
Upper motor neurons synapse with lower motor neurons in the....
anterior horn of the spinal cord
72
Lower motor neurons then synapse directly on....
muscle fibers
73
on gross examination, what do you see on the anterior side of the spinal cord?
degeneration and atrophy of the roots compared to the posterior side
74
Stains of the spinal cord will show what changes in the posterior areas (corticospinal tracts)?
lighter in color from demyelination
75
Why are the corticospinal tracts ENLARGED in stains of spinal cord in ALS?
GLIOSIS
76
T/f: gliosis occurs in the anterior horns in ALS
true
77
Loss of lower motor neurons lead to...
denervation muscle atrophy aka amyotrophy
78
SOD1 aggregates leads to the increased release of which neurotransmitter?
glutamate
79
increased glutamate release from SOD1 aggregates in ALS leads to....
excitotoxicity
80
What are the two MOAs of Riluzole for ALS?
1. inhibits glutamate release | 2. blocks post-synaptic actions of NMDA receptors
81
Syndeham's choreas is seen post-(blank) infx
strep
82
PD is (hyper/hypo)kinetic and HD is (hyper/hypo)kinetic
PD is hypokinetic | HD is hyperkinetic (chorea)
83
HD involves which which portion of the striatum
most notably the caudate | but also the putamen
84
autopsies of HD brains will show what two changes?
atrophic caudate | enlargement of the lateral ventricles
85
HD is a CAG trinucleotide repeat disorder that causes the formation of poly(blank) residues in the huntingtin protein
polyglutamate
86
Where in the cell is the huntingtin inclusion?
INTRANUCLEARA
87
What is the outcome in patients with less than 26 repeats?
normal; no risk to offspring
88
What is the outcome in patients with 27-35 HD repeats/
intermediate; will not be affected; elevated risk but less than 50% to offspring
89
What is the outcome in patients with 36-49 HD repeats?
reduced penetrance; may or may not be affected; 50% to offspring
90
What is the outcome in patients with more than 40 HD repeats?
full penetrance; will be affected; 50% to offspring
91
Increasing number of HD repeats affects the clinical presentation of HD how?
more repeats means presentation at younger age

Block 8 Week 5 Colt (12 decks)

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