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PSYC 211 > Lecture 20: Neurodegenerative Disorders > Flashcards

Lecture 20: Neurodegenerative Disorders Flashcards

1
Q

what causes Down syndrome?

A

the possession of an extra 21st chromosome

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2
Q

what type of disorder is Down syndrome?

A

congenital (one is born with it)

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3
Q

symptoms of Down syndrome

A

moderate to severe intellectual disability and physical abnormalities

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4
Q

neurodegeneration in Down syndrome

A

After age 30, the brain of a person with Down syndrome begins to degenerate like Alzheimer’s disease

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5
Q

what type of disorder is MS?

A

sporadic

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6
Q

what is MS?

A

An autoimmune demyelinating disease that occurs in people’s late 20s or 30s

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7
Q

what causes MS symptoms?

A

At scattered locations in the CNS, myelin sheaths are attacked by the person’s immune system, leaving hard patches of debris called sclerotic plaques. Normal transmission of neural messages through demyelinated axons is interrupted

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8
Q

cycles of MS symptoms

A

Symptoms go through cycles of flare-ups and then decrease after varying periods. In most cases, this pattern (remitting-relapsing MS) is followed by progressive MS later in the course of the disease

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9
Q

progressive MS

A

characterized by a slow, continuous increase in symptoms

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10
Q

who is most likely to get MS?

A

females who live farther from the equator.

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11
Q

why are people who live father from the equator more likely to get MS

A

Hygiene hypothesis: It is likely that some diseases contracted during childhood spent in regions where viruses are prevalent cause a person’s immune system to attack their myelin. It is more dangerous to get these viruses as an adult when the immune system gets confused and can cause MS

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12
Q

two treatments approved for MS

A

Interferon β & Glatiramer acetate

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13
Q

Interferon β:

A

a protein that modulates immune system activity

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14
Q

Glatiramer acetate

A

peptides that mimic myelin (decoy approach)

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15
Q

what typically causes neurodegeneration

A

apoptosis, which is triggered by collections of misfolded proteins that disrupt normal cell function

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16
Q

prion

A

Misfolded proteins with the ability to transmit their misfolded shape onto normal variants of the same protein

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17
Q

Transmissible spongiform encephalopathy

A

contagious brain disease (including mad cow disease and Creutzfeldt-Jakob disease) whose degenerative process gives the brain a sponge-like appearance.

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18
Q

what causes transmissible spongiform encephalopathy

A

Accumulation of misfolded prion protein

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19
Q

what differentiates prion protein disease from other neurodegenerative diseases?

A

it’s contagious & it’s the only infectious agent that’s just a protein

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20
Q

what causes Huntington’s disease?

A

Caused by one dominant mutation in the Huntingtin gene. Over time, aggregates of Huntingtin protein form in the basal ganglia, causing neurodegeneration

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21
Q

symptoms of Huntington’s disease

A

severe lack of coordination, uncontrollable jerky limb movements, and eventually dementia and death

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22
Q

lifespan of people with Huntington’s

A

Symptoms usually begin between 30-50 and death follows 15-20 years later

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23
Q

what part of the brain degenerates in someone with huntington’s

A

the basal ganglia

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24
Q

basal ganglia function

A

help control movements

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25
Q

wild Huntingtin vs. mutation Huntingtin

A

The wild-type Huntingtin in unaffected people has under 35 glutamines, but the mutation Huntingtin has over 39. This causes long mutant proteins that aggregate and form clumps. Eventually, they get so big that they undergo apoptosis

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26
Q

why do mutant Huntingtin genes keep persisting?

A

it’s hard to duplicate DNA that has a string of the same letters over and over again.

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27
Q

where is Huntingtin protein heavily expressed?

A

in the input nuclei of the basal ganglia (the striatum, caudate nucleus, and putamen)

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28
Q

antisense gene therapy

A

Inject antisense DNA or RNA into the spinal cord (intrathecally) to prevent the affected RNA from being translated into a protein

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29
Q

treatment for Huntington’s

A

no treatment but some optimism about antisense gene therapy

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30
Q

what causes Parkinson’s disease

A

combination of genetic and enviornmental factors

31
Q

neurobiological mechanism of Parkinson’s

A

Associated with the degeneration of dopamine neurons in the substantia nigra in the midbrain

32
Q

when do Parkinson’s symptoms appear

A

after age 60

33
Q

symptoms of Parkinson’s

A

muscular rigidity, slowness of movement, difficulty walking, and eventually dementia

34
Q

what causes the death of dopamine neurons?

A

aggregation of the protein alpha-synuclein, which eventually causes dopamine neurons to undergo apoptosis

35
Q

Alpha-synuclein

A

protein heavily expressed in midbrain dopamine neurons. Its function isn’t entirely clear. Abnormal accumulations are associated with dopamine neuron degeneration in Parkinson’s

36
Q

Lewy body

A

an aggregate of misfolded alpha-synuclein protein. Found in the cytoplasm of midbrain dopamine neurons in people with Parkinson’s disease

37
Q

what type of mutations are alpha-synuclein

A

dominant

38
Q

Ubiquitin

A

Protein that is put on old misfolded proteins, which targets them for degradation. Ubiquitinated proteins get brought to proteasomes, which break them into their constituent amino acids for recycling

39
Q

Parkin

A

Protein that plays a critical role in ubiquitination. Mutated Parkin is a cause of familial Parkinson’s disease. If Parkin is defective, misfolded proteins accumulate, aggregate, and eventually kill the cell

40
Q

Proteasome

A

Organelle responsible for destroying ubiquitinated proteins within a cell

41
Q

what type of mutation is Parkin

A

recessive

42
Q

toxic gain of function

A

a genetic disorder caused by a dominant gene mutation that produces a protein with toxic effects

43
Q

loss of function

A

a genetic disorder caused by a recessive gene mutation that fails to produce a protein that is necessary to avoid proteins

44
Q

treatment of Parkinson’s

A

We can take L-DOPA, a precursor to dopamine, which, when it enters the brain, is readily converted to dopamine

45
Q

alternative treatment for Parkinson’s

A

Damaging the globus pallidus or disrupting subthalamic nucleus activity seems to relieve symptoms of Parkinson’s disease by removing one of the brakes on motor behaviour. This can be done via brain lesions and deep brain stimulation

46
Q

deep brain stimulation

A

Damaging the globus pallidus or disrupting subthalamic nucleus activity seems to relieve symptoms of Parkinson’s disease by removing one of the brakes on motor behaviour

47
Q

dementia

A

Progressive impairments to memory, thinking, and behaviour that affects the ability to perform everyday activities as a result of a neurological disorder

48
Q

Alzheimer’s

A

a neurodegenerative disorder that causes progressive memory loss, motor deficits, and eventual death

49
Q

prevalence of Alzheimer’s

A

This occurs in ~10% of the population above the age of 65 and ~50% of the population above the age of 85

50
Q

what protein condition is associated with Alzheimer’s

A

aggregates of misfolded beta-amyloid protein and severe degeneration within and around the hippocampus and neocortex

51
Q

Beta-amyloid precursor protein (APP)

A

a protein that is the precursor for the beta-amyloid protein. (The gene for this protein is located on chromosome 21)

52
Q

secretase

A

class of enzymes that cut the beta-amyloid precursor protein into smaller fragments including beta-amyloid

53
Q

presenillin

A

a protein that forms part of the secretases that cut APP

54
Q

what happens when there are mutations in presenillin

A

cause it to preferentially generate the abnormal long-form beta-amyloid, which causes early onset Alzheimer’s disease

55
Q

Apolipoprotein E (ApoE)

A

a glycoprotein that transports cholesterol in the blood and plays a role in cellular repair

56
Q

result of E4 allele of the apoE gene

A

increases the risk of late-onset Alzheimer’s

57
Q

Beta-amyloid

A

Protein found in excessive amounts in the brains of patients with Alzheimer’s

58
Q

Amyloid plaque

A

extracellular aggregation of beta-amyloid protein surrounded by glial cells and degenerating neurons

59
Q

Tau protein

A

microtubule protein that becomes hyper-phosphorylated in Alzheimer’s disease, disrupting intracellular transport

60
Q

Neurofibrillary tangle

A

intracellular accumulation of twisted Tau protein in dying neurons

61
Q

risk factors for Alzheimer’s

A

traumatic brain injury, obesity, hypertension, high cholesterol levels, and diabetes

62
Q

who is LESS likely to get Alzheimer’s

A

well-educated people

63
Q

immunotherapy approach for Alzheimer’s

A

get antibodies to recognize and destroy Beta-amyloid proteins either by sensitizing the patient’s immune system to these proteins or by directly injecting antibodies that are made elsewhere

64
Q

Amyotrophic Lateral Sclerosis (ALS)

A

A degenerative disorder that attacks the spinal cord and cranial nerve motor neurons

65
Q

symptoms of ALS

A

Symptoms include spasticity (increased tension of muscles, causing stiff and awkward movements), exaggerated stretch reflexes, progressive weakness, muscle atrophy, and finally paralysis

66
Q

what type of disease is ALS

A

sporadic

67
Q

10-20% of ALS cases are caused by ____

A

a mutation in the gene that produces the enzyme superoxide dismutase 1 (SOD1) found on chromosome 21. This mutation causes a toxic gain of function that leads to protein misfolding and aggregation, impaired axonal transport, and mitochondrial dysfunction

68
Q

ALS & FTD

A

ALS and frontotemporal dementia (FTD) are now considered to be part of a common disease spectrum (FTD-ALS) because of genetic, clinical, and pathological similarities

69
Q

drug treatment for ALS

A

reduces glutamate-induced excitotoxicity, which extends life by about 2-3 months

70
Q

average lifespan following an ALS diagnosis

A

2-4 years

71
Q

why are late-onset disorders so prevalent

A

The environmental risk factors associated with late-onset disorders were not present in ancestral environments due to the rapid increase in human lifespan

72
Q

most genes in the human genome have ___

A

gone to fixation because they promoted survival and reproduction under ancestral conditions

73
Q

what genes are schizophrenia associated with?

A

hundreds of relatively common gene variants, which each create a small statistical increase in the risk of developing schizophrenia

74
Q

a common theory for the prevalence of schizophrenia

A

certain combinations of these genes might be advantageous for reproductive success. If this were true, the siblings of schizophrenics who don’t have the disease should have higher reproductive success, but they don’t

PSYC 211 (23 decks)

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