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BMS3 > Neurodegeneration > Flashcards

Neurodegeneration Flashcards

1
Q

Neurodegenerative disease include:

A 
Alzheimer's
Parkinson's
Huntington's
ALS/MND
Spinal muscular atrophy
Creutzfeldt-Jakob
Multiple sclerosis
Other dementias
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2
Q

Protein aggregates in diseases are:

A

beta amyloid plaques
TAU tangles
alpha synuclein
TDP43

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3
Q

The most common cause of dementia is..

A

Alzheimer’s (62%)

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4
Q

Other dementias are..

A

vascular
mixed - AD/vascular
dementia with Lewy bodies
fronto-temporal lobe

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5
Q

Alzheimer’s mostly develops after the age of..

A

65

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6
Q

AD is not inherited but people with … have a higher risk. Why?

A

Down’s syndrome. They have 3 copies of APP (AD gene)

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7
Q

Genes predisposing AD are..

A

APP (amyloid precursor protein), APOE4 (carriers show more beta amyloid), PSEN1 and PSEN2 which encode components of the amyloid pathway.

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8
Q

The early symptoms of AD are:

A

Memory lapse due to damage in the hippocampus.
Problem-solving skills.
Language skills.
Visuospatial skills.

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9
Q

Other AD symptoms which can be subtle are:

A 
inattentiveness
cognitive dulling
emotional withdrawal (apathy)
agitation
anxiety
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10
Q

Symptoms in later stages of AD become more … and include..

A

Severe:
delusions/hallucinations
dyspraxia

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11
Q

Pathologies in AD:

A 
Cerebral atrophy
Neurofibrillary tangles
Plaques
Cerebral amyloid angiopathy
Gliosis
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12
Q

Pathologies of AD are staged by

A

Braak staging

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13
Q

B amyloid plaques can reside… They are detectable by..

A

In a neuron or diffuse extracellularly. IHC and silver stains

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14
Q

Neuritic plaques are surrounded by..

A

dystrophic cell body projections which label with TAU protein

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15
Q

Amyloid is thought to cause..(3)

A

induce neuronal death by apoptosis.
cause oxidative damage.
dysregulate Ca levels - injurying organelles.

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16
Q

Amyloid’s effect on neuronal circuits could develop before..

A

cells are lost (ex vivo rat shows synaptic loss as a response to amyloid levels)

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17
Q

TAU tangles are found..

A

found with plaques (normal ageing) and are intracellular

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18
Q

TAU tangles are seen in which diseases:

A

AD, FTDP, coriticobasal degeneration and Pick’s disease.

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19
Q

amyloid angiopathy is… and can cause..

A

amyloid deposition in blood vessels which cause cerebral haemorrhage

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20
Q

PSEN mutations cause…important for..

A

lysosomal dysfunction by changing the pH of lysosomes. Involved in autophagy.

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21
Q

In AD, mutations in … affect motor proteins from attaching cargo

A

GSK

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22
Q

Braak staging shows..

A

spatiotemporal pattern of degeneration

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23
Q

in AD, degeneration of the cerebrum usually starts in the..

A

temporal and parietal lobes. Other variants include visual - posterior occipito-parietal/temporal areas

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24
Q

25% of AD patients develop..

A

Parkinsonism

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25
Q

AD can be mistaken for: (6)

A 
Major depression
Cerebrovascular disease
Inflammatory/metabolic disorders
Changes to medial temporal due to infection or vascular insults
Stroke
Tumour
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26
Q

Current therapies targeting amyloid in AD..

A

have failed to stop cognitive deficits despite reduction in amyloid.

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27
Q

Ways of looking into pathogenesis are:

A

GWAS, immune system pathway mapping, cholestrol metabolism , synaptic dysfunction and TREM2 variants (microglia and inflammation).

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28
Q

3 major therapies used in AD currently are…

A

AChE inhibitors
NMDAr antagonists
Amyloid targets

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29
Q

ACh inhibs cause … in AD

A

increase ACh levels to stabilise cognitive function, mainly lost from the nucleus basalis of Meynert (Aricept)

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30
Q

NMDAr antagonists are .. and cause..

A

anti-glutamate drugs which slow progression by reducing Ca, mitochondrial dysfunction and oxidants (Memantine)

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31
Q

2 monoclonal antibodies used in AD to target beta amyloid:

A

Aducanumab

Solanezumab

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32
Q

Aducanumab is a human antibody which has..

A

a high affinity for aggregated forms. Shown success by reducing neuronal death (through microglia).

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33
Q

Solanezumab is a humanised antibody which has a higher affinity for … than…

A

Soluble monomeric forms rather than aggregated forms. In phase 3 trials but doesnt show many benefits.

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34
Q

Verubecestat in AD is a small molecule which targets..

A

BACE1 and BACE2, amyloid production pathway. Phase2/3 trials but show low efficiency

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35
Q

A core CSF biomarker for AD is…and shows

A

Abeta42. shows total tau, cortical amyloid deposition

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36
Q

temporal lobe lesions affect..

A 
hearing
language
object recognition
memory
limbic system
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37
Q

Motor neurons travel from .. to..

A

Run from the brain, through the brainstem and spinal cord, and innervate effector muscles

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38
Q

Cell bodies of motor neurons are within..

A

the brain or spinal cord

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39
Q

Complex networks of motor neurons develops in… and…

A

In the embryo and do not get replaced

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40
Q

Motor neurons is caused by..in what tract

A

motor neurons die, muscles stop receiving signals from the brain and waste away.
Corticospinal tract.

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41
Q

MND involves degeneration of … and … which cause either…

A

UMN - motor cortex
LMN - anterior horns of spinal cord
Bulbar neurons - motor nuclei
UMN primarily causes lateral sclerosis. LMN cause muscular atrophy.

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42
Q

MND is associated with other diseases:

A

FTD
cognitive problems
Parkinsonism
oculomotor pathologies

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43
Q

MND affects … in the UK. and has a life expectancy of…

A

5000 people in the UK. Life expectancy of 2-4 years, can be up to 10 but rare.

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44
Q

Usual onset of MND is..

A

60-80 years, but all ages can be affected

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45
Q

Death from MND happens due to..

A

respiratory failure
bulbar dysfunction
bronchopneumonia

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46
Q

…% of MND is genetic. Genes are.. and are involved in 3 aspects..

A 
10%. C9ORF - intron expansion.
SOD1
TARDBP
FUS
protein homeostasis, RNA binding proteins and cytoskeletal proteins
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47
Q

Early symptoms of MND:

A

general weakness of limbs (75%).
Bulbar muscles - medulla (20%).
Respiratory onset (5%)

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48
Q

Symptoms of upper MND:

A

weakness and spasticity - constantly contracted and stiff.

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49
Q

Symptoms of lower MND:

A

weakness due to wasting of muscle and fasiculations - floppy limb

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50
Q

Symptoms of bulbar onset (MND):

A

slurred speech, dysphagia, jaw spasms, tongue fasiculations, teeth grinding

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51
Q

In MND, protein inclusions of…are seen in..

A

TDP43 seen in nuclei and neurofilaments

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52
Q

In mutant SOD1 (MND), mitochondria is seen to accumulate..and are also..

A

in cell bodies instead of along the axon. Seen with vacuoles and swelling in dendrites

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53
Q

Mutations affecting axonal transport in MND are…and leads to..

A

in dynactin complex components. This leads to build up of toxic waste

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54
Q

Intronic expansion mutations of C9ORF in MND show..

A

intra-nuclear RNA foci and cytoplasmic inclusions of p62 - ubiquitin binding protein. Inactivation causes abnormal microglia

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55
Q

MND patients with mutations of SOD1 and Fus are distinct because..

A

they show inclusions of abnormal SOD1 and Fus proteins since they fail to fold properly

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56
Q

Treatment for MND works by..eg..

A

reducing excitation and glutamate - Riluzole

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57
Q

Improving survival in later stages of MND is done by..

A

non-invasive ventilation - tube through trachea

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58
Q

Treatments in clinical trials for MND are..

A

antisense oligonucleotides and monoclonal antibodies through lumbar puncture

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59
Q

Current studies for MND..

A

iPSCs used in drug screens to study toxic glial cells by converting fibroblasts to neural progenitors.

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60
Q

What is spinal muscular atrophy?

A

Recessive motor neuron disease typically in infants.

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61
Q

What is mutated in spinal muscular atrophy?

A

SMN1 gene; SMN protein is not produced

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62
Q

In Spinal muscular atrophy, SMN2 is being overexpressed by … to cause…

A

antisense oligonucleotide called Nusinersen. This affects splicing of SMN2 so more SMN protein is made. It is administered by lumbar puncture.

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63
Q

Other ways of increasing SMN expression in SMA…

A

Antibodies

Viral vectors

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64
Q

Loss of … is seen in the spinal cord in MND

A

white matter

65
Q

Gene silencing of SOD1 is being studied through..

A

using viral vectors, adenovirus. This can cross the BBB

66
Q

Parkinson’s is characterised by neuronal loss in… which causes..

A

the substantia nigra. Reduces levels of dopamine

67
Q

symptoms of Parkinson’s develop after..

A

80% of neurons are lost

68
Q

general symptoms of Parkinson’s are..

A

stiffness, slow movements and tremors.

69
Q

Other symptoms of PD are:

A 
brady/akinesia.
small-stepped gait.
stooped posture.
reduced arm swing.
repeated movements.
tremor at rest
rigidity - dull ache
70
Q

Parkinson’s tremor has features of..

A

present at rest and unilateral

71
Q

Later staged symptoms of PD are:

A 
on/off dyskinesia.
choreiform movements (jerky).
depression.
dementia.
anxiety.
hallucinations.
constipation.
urinary incontinence
72
Q

conditions with similar symptoms to PD are:

A

Normal pressure hydrocephalus - corrected by draining of CSF.
Essential tremor in the elderly - not at rest and no increased tone

73
Q

Pathology of substantia nigra in Parkinson’s shows..

A

cell loss and Lewy bodies of a synuclein.

TDP43 deposits in nuclei.

74
Q

Risk factors of PD are genetic and enrivonmetal. Toxins from pesticides such as MPTP have shown to … in dopaminergic neurons

A

inhibit Cl- channels - Cl- cant move in, can’t repolarise

75
Q

Imaging has shown the mitochondrial network in PD is..

A

hyperfused, meaning lower activity of mitochondria

76
Q

PINK and Parkin are regulators of..

A

mitophagy - degradation of mitochondria.

77
Q

Loss of PINK1 generally causes..

A

mitochondrial dysfunction and increased sensitivity to oxidative stress.

78
Q

PINK is normally degraded constantly. When mitochondria are non-functional…

A

PINK phosphorylates Parkin and ubiquitin on mitochondrial membranes. This prevents mitophagy.

79
Q

LRRK2 mutants in PD inhibit..

A

autophagy

80
Q

Main treatments for PD focus on..

A

compensating the loss of dopamine

81
Q

The 3 types of dopamine treatment are:

A

LDOPA
Dopamine agonists
COMT/MAO-B inhibitors

82
Q

Anti-cholinergics are also used in PD to..

A

reduce excitation in tremors. Not good, have side effects on cognition

83
Q

L-DOPA is…and how is it taken?

A

the precursor of dopamine. It comes in 3 different preparations:
kick start morning
release throughout day
slow release at night

84
Q

Dopamine agonists such as Ropinirole, Praipexole and Rotigotine are used as..

A

substitues for dopamine, targeting postsynaptic dopamine receptors. They are a first choice drug when treating younger patients (under 60).

85
Q

MAO-B and COMT inhibitors both inhibit..

A

enzymes which breakdown dopamine - catechol-o-methyl-transferase and monoamino-oxidase (also ALDH).

86
Q

Gene therapies in PD aim to…and use lentivirus to deliver..

A

replace dopamine. Lentivirus delivers tyrosine hydroxylase - enzyme which converts L-tyrosine to L-DOPA. Tested in small and large animals, and injected into putamen of Parkinson’s patients.

87
Q

Oxidative stress pathway has been targeted in PD by..which targets..

A

Small molecules target the Nrf2/ARE pathway, activating protection against oxidative stress in neuronal cultures.

88
Q

Long term medication in PD causes..

A

wearing off effect - on/off phases of dyskinesia

89
Q

Huntington’s causes death …. after onset

A

15-20 years

90
Q

the HD gene htt is..

A

located on chromosome 4 and is a trinucleotide polyglutamate repeat disorder (CAG)

91
Q

Huntington’s is a … inherited disease of..

A

monogenic, autosomal dominant - passed on from either parent. Severe atrophy of the striatum and cortex.

92
Q

Normal amount of CAG repeat is … too many causes what in HD?

A

35 repeats. Over 40 repeats cause over-production of huntingtin protein.

93
Q

Age of onset of HD is.. correlates to..

A

30-50 years. number of CAG repeats

94
Q

Regional patterning of huntingtin is mainly seen in..

A

the striatum but also in cortex

95
Q

Physical symptoms of HD are:

A

Chorea - progressive.
Dystonia - fixed posture.
Myoclonus - jerky
Tics

96
Q

Other symptoms of HD are:

A

Cognitive impairments:
slowness of thoughts.
apathy.
subcortical dementia

97
Q

Chorea in HD occurs due to..

A

loss of inhibitory GABA neurons in basal ganglia; dopaminergic neurons more active causing movement

98
Q

Pathologies seen in HD:

A

Atrophy of cerebrum, sulci and caudate nucleus.
Astrogliosis - vast proliferation.
Reduced astrocyte response to amyloid
Protein aggregates

99
Q

Protein aggregates seen in Huntington’s are:

A

TAU
Beta amyloid
Alpha synuclein
Huntingtin

100
Q

Expansions of trinucleotide repeats cause… Common expansions are..

A

Gain of function, autosomal dominant effects. Found in promoters, UTRs, introns and exons. Common repeats are polyglutamine and polyalanine.

101
Q

Post mortem tissue of HD has shown abnormal..

A

mitochondrial morphology; less cristae, with prominent defects in CII, changes in mtDNA and Ca handling.

102
Q

Mice with mutant htt have shown..

A

localisation of htt on outer mitochondrial membranes with CII defects and reduced O2 consumption. This correlates to CAG repeat length.

103
Q

JNK is mutated in HD. It is an abnormally active…involved in..

A

Kinase involved in regulation of phosphorylation of kinesin and dynein activity.

104
Q

It is thought that Htt has roles in:

A 
Transcriptional regulation.
Protein homeostasis.
Oxidative stress.
Axonal transport.
Synaptic transmission
Suppression of apoptosis
105
Q

HD is treated by..

A

Antipsychotics: presynaptic release of dopamine, postsynaptic receptor activation.
Interference of vesicle storage. Tetrabenazine

106
Q

Tetrabenazine works by reducing..

A

uptake of dopamine by presynaptic vesicles and depleting dopamine storage.

107
Q

Tetrabenazine is not great as..

A

not a lot of improvement.

Many adverse effects - sedation, depression, anxiety, insomnia

108
Q

Sulpiride, Olanzapine, Haloperidol work in HD as…

A

dopamine receptor antagonists to reduce abnormal movement

109
Q

Dystonia (abnormal tone and posture) can be alleviated by..

A

Botulinum toxins and Clonazepam

110
Q

Trials in HD are targeting mutant htt protein with..

A

small molecules and antisense oligonucleotides

111
Q

Microglia are immune cells of the CNS. They make up…of the CNS and have … origin

A

1-15%. Originate from myeloid lineage (not neuronal).

112
Q

Microglia respond in… and indicate..

A

a sensitive but non-specific way. They are pathological markers for disease

113
Q

Features of TDP43 are..

A

amorphous, disordered, and have prion-like domains

114
Q

Prions are..and have …formation

A

RNA binding proteins which self replicate and transmit disease. They form an amyloid fibril-like formation.

115
Q

Lewy body dementia has symptoms of..

A

Fluctuating cognitive decline.
Visual hallucinations.
Parkinsonism.
Neuroleptic sensitivity.

116
Q

In Lewy body dementia, Lewy bodies are found… and are made of..

A

neurons of neocortex, limbic system and brainstem.

They are made of alpha synuclein

117
Q

Vascular dementia is caused by..

A

infarcts in small or large vessel. Dementia is dependent on the location of infarct.

118
Q

Risk factors of vascular dementia are..

A

atherosclerosis; smoking, alcohol, hypertension, cardiac disease, diabetes and genetics.

119
Q

Gliosis is..and can lead to..

A

proliferation of several types of glial cells (astrocytes, microglia and oligodendrocytes). Can lead to scarring

120
Q

Astrocytes provide..

A

trophic support to neurons and show response to damage

121
Q

Astrocytes expressing SOD1 mutations in MND are..

A

selectively toxic to motor neurons by releasing factors.

122
Q

Glial cells expressing mutant htt in HD have reduced..

A

ability to protect htt mutated neurons

123
Q

Microglia respond to damage by..which causes..

A

retracting and thickening their processes. They form a rounded amoeboid-like shape, and express different molecules to induce astrocytes.

124
Q

Microglia have 3 states:

A

M0 - resting
M1 - active, toxic
M2 - active, wound healing

125
Q

Neurodegenerative diseases are characteristed by clumps of … proteins. This also means there is reduced..

A

Ubiquinated. There is reduced function of ubiquitin proteasome systems

126
Q

Protein inclusions start in specific areas due to selective vulnerability, and spread..how..

A

through the brain in a stepwise fashion, correlating with disease progression

127
Q

Selective vulnerability in AD is in..

A

entorhinal cortical and hippocampal CA1 neurons. These are particularly vulnerable to energy deprivation.

128
Q

Selective vulnerability in HD is in..

A

spiny striatal neurons which express Cav1.3 channels; mediate Ca influx

129
Q

Selective vulnerability in MND is in…

A

Spinal motor neurons are prone to hyperexcitation as they have low expression of GABA receptors. They express Cav1.3 channels which mediate Ca influx

130
Q

Pathology is thought to spread throughout the brain by..

A

affected neurons produce toxins, picked up by surrounding neurons.
OR areas get affected in turn

131
Q

Amyloid fibril formations have the ability to recruit other proteins to become amyloidogenic in order to build what structure?

A

twisted, non-branching filaments of beta sheets. Structures are fibrillar or multimeric.

132
Q

Spread of aggregates requires neurons to..

A

release and uptake from other neurons. May occur by secretory/endocytic pathways or across synapses.

133
Q

TAU, alpha synuclein and TDP43 can be transported to other neurons how?

A

Through the axon antero/retrogradely. Seen through in vitro studies.

134
Q

Injection of TAU or synuclein into the brain causes aggregates where?

A

in anatomical structures distant from site of injection, implying that neuronal connections are involved in spread of aggregates.

135
Q

Recent studies have shown TAU, beta amyloid and synuclein have different conformational variants which show different…

A

seeding properties and different levels of neurotoxicity. This contributes to heterogeneity of neurodegen diseases.

136
Q

Evidence to suggest that protein aggregates are toxic are..

A

Proteins are mutated in familial diseases

137
Q

Evidence suggesting protein aggregates are not toxic is..

A

model organism studies have shown toxicity in the absence of aggregates, suggesting they may be protective.

138
Q

Longer protein aggregates have a slower turnover which potentially..

A

overwhelms protein homeostasis and degradation pathways

139
Q

Aggregates interact with other molecules, and can … other proteins

A

sequester. This affect transcription.

140
Q

Aggregates interfere with what processes?

A

Axonal transport

Nucleocytoplasmic

141
Q

In MS, remyelination of oligodendrocytes has been looked at through

A

stem cell screening

142
Q

Mitochondrial dysfunction affects..

A

Glucose and oxygen supply.

Ca handling

143
Q

Lysosomal degradation pathway is activated in cells in response to stresses:

A

when a cell is starved.
When damaged constituents are present (eg. aggregates).
To defend themselves from pathogens

144
Q

Autophagy is regulated by..

A

basal turnover and stress responses

145
Q

mTOR activity in autophagy is regulated by signals of:

A 
nutrients.
Growth factors.
stress pathways
energy pathways
Inositol pathways
cAMP, Ca and JNK pathways
146
Q

In neurodegen diseases, autophagy is observed as dysfunctional as..

A

protein aggregates are tagged with ubiquitin but are not apoptosed

147
Q

p62 is a receptor for … which is seen to be defective in neurodegen diseases.

A

autophagosomes

148
Q

Knockouts in mice have shown that loss of essential autophagy proteins cause..

A

accumulation of aggregates and neuronal loss. Neurons are sensitive to loss of autophagy

149
Q

Autophagy has been stimulated by … called rapalogs. These are inhibitors of…

A

small molecules which are inhibitors of mTOR

150
Q

Axonal transport works by components:

A

microtubules
Kinesin
dynein

151
Q

molecular motors work as..

A

ATPases, converting chemical energy to mechanical energy

152
Q

Dynein has other chains which bind to..

A

cargo and dynactin - factor for cargo binding

153
Q

Fast axonal transport is for..

A

synaptic vesicles and mitochondria (50-400mm/day)

154
Q

Slow axonal transport pauses between movements and moves..

A

proteins and larger molecules

155
Q

Abnormal and excessive activation of kinases in motor proteins are hallmarks of neurodegen diseases. EG:

A 
p38
MAPK
JNK
GSK
(In AD, MNS and HD)
156
Q

Kinase mutations phosphorylate kinesin and dynein. Mutations cause mitochondria to…kinesin to be..and prevent cargo…

A

cluster around the nucleus.

Kinesin is released from microtubules (preventing transport). Prevents cargo from attaching to motor proteins.

157
Q

When …… are mutated, it causes microtubules to become unstable, reducing transport

A

TAU, Spastin and Hsps

158
Q

Gene therapy involves the use of… to carry genes or molecules

A

vectors: plasmids, nanoparticles, viral - lentiviral or adenoviral

BMS3 (6 decks)

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