Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What two proteins are in the inclusions in AD?

A

AB protein

Tau protein

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Where are the inclusions located in AD?

A

amyloid plaques are extracellular

tau protein is intraneuronal

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What is the protein in the inclusions in PD?

A

Alpha-synuclein

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Where are the inclusions located in PD?

A

inside neurons

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

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

A

TDP-43

SOD1

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

where are the inclusions located in ALS?

A

inside the neurons

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What is the protein in the inclusion in HD?

A

huntingtin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

where are the inclusions located in HD?

A

inside the neurons

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

what class of proteins form aggregates?

A

globular proteins

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

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

A

formation of soluble aggregates

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

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

A

true

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Lewy bodies are seen in which disease?

A

PD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What types of neurons are involved in PD?

A

dopaminergic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Where is the greatest neuronal loss in PD?

A

substantia nigra

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

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

A

stress response

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

inclusions are (directly/indirectly) toxic to neurons

A

directly

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

in the neurodegen dzs, misfolded proteins from what conformation?

A

b-sheets and amyloid fibrillar aggregates

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

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

A

cortical

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

neurofibrillary tangles are outside or inside the cell?

A

inside

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Where does AD begin in the brain?

A

hippocampus – temporal lobe

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

When are AZtherapies effective in treating AD?

A

only during MCI stage

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

an amyloid plaque as a (blank) core

A

beta-amyloid core

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

formation of amyloid plaques leads to the phsycial disruption of…

A

dendrites

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

formation of amyloid plaques elicits what “healing” response from the brain?

A

gliosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

What protein makes up the neurofibrillary tangles?

A

tau

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

Whence is the origin of AB protein?

A

amyloid precursor protein APP; a transmembrane synaptic protein

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

APP is cleaved by (blanks) a, b, and g, forming AB monomers

A

secretases

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

which secretase is most important in the formation of amyloid plaques?

A

secretase b

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

what is the result of the formation of AB oligomers?

A

formation of aggregates, amyloid fibrils, and neuronal damage

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

what is the function of normal tau?

A

a microtubular protein that is part of the cytoskeleton

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

Amyloid beta activates a (blank) which (blanks) tau protein

A

kinase; phosphorylates tau

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

What happens when tau becomes hyperphosphorylated?

A

falls off the microtubules

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

Tau that has fallen off the microtubules forms (blanks) which form neurofibrillary tangles

A

paired helical fragments

38
Q

Tau is interfering with microtubules and intracellular traffic while the plaques are interfering with….

A

dendritic connections

39
Q

do tangles form before or after plaques?

A

after

40
Q

t/F: plaque formation is a part of normal aging

A

true

41
Q

Radioactive imaging tracers that bind to beta amyloid can be visualized with….

A

PET

42
Q

T/F: AD pathology overlaps with normal people

A

true

43
Q

T/F: tangles are present in the normal aging brain

A

true

44
Q

What are the three CSF biomarkers that help in the Dx of AD?

A

LOW beta-amyloid 1-42
HIGH total tau
elevated phosphorylated tau

45
Q

Why is CSF beta amyloid low?

A

the amyloid is being deposited in the plaques and not in circulation

46
Q

The CSF signature of AD has a low (specificity/sensitivity)

A

specificity

47
Q

T/F: AD has motor components

A

false

48
Q

PD involves what set of nuclei in the brain?

A

basal ganglia

49
Q

the largest concentration of dopaminergic neurons in the brain are found in the…

A

substantia nigra

50
Q

Where do the axons of the substantia nigra project?

A

corpus striatum

51
Q

Degeneration of projections from the substantia nigra to the corpus striatum leads to what Sx?

A

severe tremors

muscle rigidity

52
Q

Hyperactivity in substantia nigra neurons is associated with….

A

addictive behaviors

53
Q

Cocaine causes extracellular buildup of DA in the…

A

limbic system

54
Q

how does cocaine cause the buildup of DA in the limbic system?

A

inhibition of Na dependent DA reuptake

55
Q

T/F: PD neurons are fucntional, its just that they lack coordination

A

true

56
Q

what are the two components of the striatum?

A

caudate nucleus

putamen

57
Q

What contributes the most to neuronal death in PD?

A

alpha synuclein oligomers

58
Q

Alpha synuclein forms what type of inclusion?

A

lewy body

59
Q

t/F: alpha synuclein can spread like a prion

A

true

60
Q

Prion like spread of alpha synuclein in PD leads to….

A

dementia

61
Q

dementia with lewy bodies is clinically between which two diseases?

A

PD and AD

62
Q

What are two notable physical findings in ALS?

A

muscle weakness

fasciculations

63
Q

What physical changes happen to the spinal column in ALS?

A

hardening of the lateral columns of the spinal cord

64
Q

Gliosis follows the degeneration of the (blank) tracts in ALS

A

corticospinal

65
Q

ALS involves both (blank and blank) neurons

A

upper and lower motor neurons!!!!

66
Q

(blank) degeneration in ALS leads to demyelination and scarring

A

Wallerian degeneration of the axons

67
Q

where do motor signals originate?

A

precentral gyrus – motor cortex

68
Q

Signals from the precentral gyrus flow via which fibers on the way to the spinal cord?

A

posterior limb of internal capsule

69
Q

where do the motor fibers decussate?

A

in the pyramids

70
Q

Upper motor neurons travel down the spinal cord in which tract?

A

lateral corticospinal tract

71
Q

Upper motor neurons synapse with lower motor neurons in the….

A

anterior horn of the spinal cord

72
Q

Lower motor neurons then synapse directly on….

A

muscle fibers

73
Q

on gross examination, what do you see on the anterior side of the spinal cord?

A

degeneration and atrophy of the roots compared to the posterior side

74
Q

Stains of the spinal cord will show what changes in the posterior areas (corticospinal tracts)?

A

lighter in color from demyelination

75
Q

Why are the corticospinal tracts ENLARGED in stains of spinal cord in ALS?

A

GLIOSIS

76
Q

T/f: gliosis occurs in the anterior horns in ALS

A

true

77
Q

Loss of lower motor neurons lead to…

A

denervation muscle atrophy aka amyotrophy

78
Q

SOD1 aggregates leads to the increased release of which neurotransmitter?

A

glutamate

79
Q

increased glutamate release from SOD1 aggregates in ALS leads to….

A

excitotoxicity

80
Q

What are the two MOAs of Riluzole for ALS?

A
  1. inhibits glutamate release

2. blocks post-synaptic actions of NMDA receptors

81
Q

Syndeham’s choreas is seen post-(blank) infx

A

strep

82
Q

PD is (hyper/hypo)kinetic and HD is (hyper/hypo)kinetic

A

PD is hypokinetic

HD is hyperkinetic (chorea)

83
Q

HD involves which which portion of the striatum

A

most notably the caudate

but also the putamen

84
Q

autopsies of HD brains will show what two changes?

A

atrophic caudate

enlargement of the lateral ventricles

85
Q

HD is a CAG trinucleotide repeat disorder that causes the formation of poly(blank) residues in the huntingtin protein

A

polyglutamate

86
Q

Where in the cell is the huntingtin inclusion?

A

INTRANUCLEARA

87
Q

What is the outcome in patients with less than 26 repeats?

A

normal; no risk to offspring

88
Q

What is the outcome in patients with 27-35 HD repeats/

A

intermediate; will not be affected; elevated risk but less than 50% to offspring

89
Q

What is the outcome in patients with 36-49 HD repeats?

A

reduced penetrance; may or may not be affected; 50% to offspring

90
Q

What is the outcome in patients with more than 40 HD repeats?

A

full penetrance; will be affected; 50% to offspring

91
Q

Increasing number of HD repeats affects the clinical presentation of HD how?

A

more repeats means presentation at younger age

Block 8 Week 5 Colt (12 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions