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Block 8 week 5 > Parks-Neurodegenerative Disease > Flashcards

Parks-Neurodegenerative Disease Flashcards

1
Q

What are the 2 major ways to deal with misfolded or worn out proteins?

A 
ubiquitination and proteasomes
or lysosomes  (autophagy)
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2
Q

What is the protein and location of protein involved with alzheimers?

A

Ab TAU

extracellular neuron

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3
Q

What is the protein and location of protein involved with Parkinson Diseease?

A

alpha-synuclein

Neurons

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4
Q

What is the protein and location of protein involved with amytrophic lateral sclerosis?

A

TDP-43
SOD-I (familial disease)
neurons

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5
Q

What is the protein and location of protein involved with Huntington diseease?

A

Huntingtin

neurons

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6
Q

How do you form inclusion bodies?

A

failed proteasomal degreadation results in soluble aggregates that are autphagycotized and results in inclusion bodies

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7
Q

Are there cellular consequences to protein aggregation and inclusions?

A
  1. often they elicit a stress reaction from the cell.
  2. often directly TOXIC to neuron
  3. Some aggregates are capable of behaving like prions. Aggregates derived from one neuron taken up by another neuron giving rise to more aggregates.
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8
Q

What does amyloidogenic proteins (misfoled proteins) turn into?

A

fibrils by forming beta sheet structures

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9
Q

What are the two major histological components of alzheimers?

A

neurofibrillary tangles

amyloid plaques

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10
Q

Where does the progressive disease of alzheimers begin?

A

in the hippocampus

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11
Q

What is the average time for alzheimers to progress to severe AD and death? How long can the progression last?

A

8 yrs

20 yrs

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12
Q

How does amyloid cause neuronal damage in alzheimers?

A

Amyloid precursor protein is cut by the secretase cleavage. This creates an AB monomers which will combine with other AB monomers and form oligomers and then aggregate into plaques and tangles and you end up with neuronal damage

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13
Q

What makes up an amyloid (senile) plaque?

A

composed of dystrophic neuritic processes, B-amyloid peptide, microglial cells, and astrocytes and their processes

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14
Q

What are neurofibrillary tangles made up of?

A

paired helical filaments (PHFs) of hyperphosphorylated tau protein

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15
Q

(blank) are intracellular aggregates of actin and actin-associated proteins first observed in neurons.

A

Hirano bodies

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16
Q

Explain why Tau is awesome until its not. How does it form neurofibrillary tangles?

A

Tau is a microtubile stabilizing protein. When Amyloid beta builds up in alzheimers it activated a kinase thus phosphorylating other proteins. It hyperphosphorylates Tau which makes it fall off of microtubles and thus disassembles the microtuble. In addition the tau protein then pairs up and turns into a helical filament and then forms neurofibrillary tangles.

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17
Q

What is the amyloid cascade hypothesis?

A
  • > increased production/reduced degredation of AB
  • > plaque formation
  • > hyperphosphorylation of tau
  • > NFT formation
  • > synaptic dysfunction and neuron loss
  • > memory loss/cognitive deficits
  • > psychobehavioral impairment
  • > death
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18
Q

What does alzheimer do to your synapses?

A

causes them to dysfunction

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19
Q

How do you check fr the present of beta-amyloid in the brain?

A

with amyvid (fluroescent biomarker) and a PET scan

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20
Q

What is a problem with the Amyloid PET (PIB) scan?

A

there is a lot of overlap between amyloid found in alzheimers patients and older normal people
i.e normal elderly patients have some amyloid plaques and NFT too!!

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21
Q

What are three biomarkers used to check for Alzheimers?

A
  • low beta-amyloid 1-42
  • high total tau protein
  • elevated phosphorylated tau 181p
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22
Q

What are the signs and symptoms of Parkinsons?

A
  • tremor
  • masked like facies
  • stooped posture
  • short shuffling gate
  • hips and knees slightly flexed
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23
Q

The (Blank) modulate the output signals from motor neurons and help coordinate and regulate motor behavior

A

basal ganglia

24
Q

What kind of inclusion bodies will you see in parkinsons?

A

alpha-synuclein

25
Q

The largest concentration of dopaminergic neuron cell bodies in the rbain is in the (Blank). Where do these neurons project?
What do they do?

A

substantia nigra
corpus striatum
coordinate movement (if degenerated cause severe tremors and muscle rigidity)

26
Q

Other dopaminergic neurons in the CNS are involved in motivation and reward and hyperactivity of these neurons is associated with (blank) behavior

A

addictive

i.e. cocain buildups dopamine in limbic system by inhibiting Na dependent dopamine reuptake

27
Q

What is the main region affected by parkinsons?

What are other regions?

A

substantia nigra

  • striatum (caudate, putamen, substantia nigra)
  • locus ceruleus
  • raphe nuclei
  • brainstem
  • globus pallidus
  • thalamus
  • motor cortex
28
Q

Although Parkinson’s usually shows motor-coordination type signs and symptoms, alpha synuclein can spread and do what?
What does this lead to?

A

‘spread’ like a prion and contribute to neuron loss in many areas of the cerebral cortex.

dementia

29
Q

ALS is a motor neuron disease of what?

A

both upper and lower motor neurons

30
Q

In ALS you get (blank) and (blank)

A

demyleination and sclerosis

31
Q

What does “amyotrophic” refer to?

A

muscle atrophy, weakness and fasciulation (shows the loss of lower motor neurons in ALS)

32
Q

What does “lateral sclerosis” refer to?

A

hardness to palpation of the lateral columns of the spinal cord in autopsy specimens where gliosis follows degeneration of corticospinal tracts-> resultin gin overactive tendon reflexes, hoffmann signs, clonus, babinski signs (shows the loss of upper motor neurons)

33
Q

In patients with typical ALS, the symptoms are primarily those of (blank)

A

weakness

34
Q

IN ALS, what is intact? What is gone?

A

Sensory

Motor neurons

35
Q

What tract does ALS affect? What will you find here?

A

Corticospinal tract
and anterior horn
Gliosis

36
Q

If you are looking at the anterior roots of a spinal cord of an ALS patient what will they look like?

A

be atrophic

37
Q

If you look at the cross section of a spinal cord in an ALS patient, what will the corticospinal tracts look like? Why?

A
  • demylination and expansion of the corticospinal tract

- cell body of motor neurons are dead leading to atrophy and demylination and the expansion i due to gliosis.

38
Q

In ALS, loss of lower motor neurons results in (blank).

A

denervation muscle atrophy

this is amyotrophy

39
Q

T or F

amyloid beta can have all sorts of configurations making it difficult to study.

A

T

40
Q

What can amyloid beta do to NMDA receptors?

A

increase calcium influx resulting in signaling cascades the result in synaptic impairment

41
Q

What are the two ways you can induce dopamine neuron death and thus get parkinsons?

A
  • Drug induced (MPTP, Rotenone)

- overexpression of alpha synuclein

42
Q

What are the 3 ways alpha-synuclein can damage dopamine neurons?

A
  • Activation of ROS (resulting in apoptosis cytotoxicity)
  • alpha-synuclein can form oligomers which are neurotoxic
  • alpha-synuclein can form oligromers and fibrils which can form lewy body-like inclusions and damage neurons
43
Q

(blank) are abnormal aggregates of protein that develop inside nerve cells in Parkinson’s disease

A

Lewy bodies

44
Q

What disease has early disturbance in attention and visual perception, typically has delirium, visual hallucinations, delusions, rigor, autonomic dysfunction, cortical and subcortical lewy bodies, neuritic plaques, cholinergic and dopaminergic deficits?

A

Dementia with Lewy bodies

45
Q

What disease has neuritic plaques, neurofibrillary tangles, cholinergic deficits, delusions and early impairment of memory and attention?

A

Alzheimers disease

46
Q

What disease has autonomic dysfunction, no early cognitive impairment, rigor, bradykinesia, tremor, subcortical lewy bodies, dopaminergic deficit?

A

Parkinson’s disease

47
Q

How can you differentiate between parkinsons and dementia with lewy bodies based on the lewy bodies?

A

parkinson’s lewy bodies are subcortical while dementia with lewy bodies has them located cortically and subcortically

48
Q

What wil you find within the anterior horn of the spinal cord?

A

lower motor neurons

49
Q

What causes ALS?

A

a defect in SOD1 gene (superoxide dismutase gene)

50
Q

WHat does superoxide dismutase do?

What happens if you dont have it?

A

it is an antioxidant (dismantles the superoxides) and prevents damage of the oxides to cells.
-you get decreased function of glutamate transport receptors on astrocytes resulting in increased glutamate in neuron synapses and thus excitotoxicity

51
Q

How can you treat ALS?

What does it do?

A

Riluzole

-inhibits glutamate release and blocks post-synaptic actions of NMDA receptors reducing the excitotoxicity

52
Q

Syndenham’s chorea which almost are dance-like movements that some patients get following a (blank) infection

A

Streptococcal infection.

53
Q

What part of the brain does huntingtons affect the most?

Why do you get choreo and movement problems in huntingtons?

A
  • mainly the caudate and some of the putamen (ie the striatum)
  • because it is affecting the basal ganglia
54
Q

What will the brain of a huntingtons patient look like?

A

you will have enlarged ventricles with decreased caudate size

55
Q

What is the three nucleotide sequence that is repeated in huntingtons disease?
How many times?
What does this repeated sequence code for?

A

CAG
36-121
a ton of glutamine which forms polyglutamine tract

56
Q

What happens when you get the polyglutamine tracts due to your messed up chromosome 4 in HD?

A

they aggregate and create inclusions in the nucleus and increase the decay rate of certain types of neurons

Block 8 week 5 (9 decks)

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