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Lecture 22

PSYC 211 Lecture 22: PSYC211 November 29th
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Department
Psychology
Course Code
PSYC 211
Professor
Jonathan Britt

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November 29th
Degenerative disorders
There are many different kinds of neurodegenerative disease, such as prion diseases, Alzheimer's,
Parkinson's, Huntington's, and ALS.
Some of these conditions injure particular kinds of cells, others are more widespread.
Degeneration is typically the result of apoptosis, which is triggered by collections (aggregates) of
misfolded proteins that disrupt normal cellular function.
Transmissible spongiform encephalopathy
Contagious brain disease, such as mad cow and Creutzfeldt-Jacob disease, whose degenerative
process gives the brain a sponge-like appearance
Caused by accumulation of misfolded prion protein
Spongiform means that when they die their brain resembles a sponge
Their brain has holes in it due to neurons that are not there anymore
Prion
Protein that can exist in two forms that differ only in their three-dimensional shape
The prion protein is at risk of being misfolded
When a misfolded protein bumps into another well-folded one, it can cause this one to
also become misfolded
This creates clumps of misfolded proteins that all bind together
Accumulation of misfolded prion protein is responsible for transmissible spongiform
encephalopathies
However, they are not the most prominent of the degenerative disorders
Sporadic disease (another word for idiopathic diseases)
Disease that is not obviously caused by heredity or an infectious agent
Has an unknown cause
Prion protein diseases can be genetic or sporadicand both forms can be transmitted to others
by means of a simple misfolded prion protein
But prion proteins often fall under the sporadic category
Can be transmitted when an individual eats the neural tissue of another individual
This cannot be prevented by cooking the neural tissue
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So once there is an outbreak, it becomes very dangerous
Misfolded proteins often encourage more misfolding to occur. Thus, most neurodegenerative
diseases can easily spread from neuron to neuron and from brain to brain.
Bottom panel: anytime regular prion proteins was made in the cell, it would immediately be
degraded even if the protein was well folded
Dementia
Loss of cognitive abilities such as memory, perception, verbal ability, and judgment that is
caused by an organic brain disorder.
Common causes are multiple strokes and Alzheimer's disease
Alzheier’s is a for of deetia
Dementia is typically seen in old age
Blanket term for several different types of degenerative disorders
Alzheimer's disease
Degenerative brain disorder typically caused by misfolded b-amyloid protein
Clump together in the brain and causes neurons to undergo apoptosis
You do’t hae holes i the rai i this ase eause the lups do’t dissipate
Causes progressive memory loss, motor deficits, and eventual death
Alzheier’s disease
Occurs in approximately 10 percent of the population above the age of sixty-five and almost 50 percent
of people older than eighty-five years
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find more resources at oneclass.com
Produces severe degeneration within and around the hippocampus, neocortex (especially the
association cortex of the frontal and temporal lobes), and other places
Degeneration produces progressive loss of memory and other mental functions
Brai of Alzheier’s patiet ersus oral rai
Alzheier’s disease AD is haraterised  progressie eor loss ad other mental functions.
β-amyloid (Aβ)
Protein found in excessive amounts in brains of patients with Alzheimer's disease
When it clumps together because it is misfolded, it forms amyloid plaques
Amyloid plaque
Extracellular deposit containing a dense core of β-amyloid protein surrounded by degenerating
axons and dendrites and activated microglia and reactive astrocytes
Start to be surrounded by other neural tissues (axons, dendrites, etc.)
Tau protein
Protein that is a component of microtubules, part of the cell's transport mechanism. Tau
protein becomes hyper-phosphorylated in Alzheimer's disease, which disrupts transport.
I Alzheier’s, the Tau proteis hae tos of phosphate groups put o the
They break down over time and reforms neurofibrillary tangles
Neurofibrillary tangle
Dying neuron containing intracellular accumulations of twisted Tau protein filaments that
formerly served as cell's internal skeleton
This begins to clump up as well
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Description
th November 29 Degenerative disorders There are many different kinds of neurodegenerative disease, such as prion diseases, Alzheimer's, Parkinson's, Huntington's, and ALS. Some of these conditions injure particular kinds of cells, others are more widespread. Degeneration is typically the result of apoptosis, which is triggered by collections (aggregates) of misfolded proteins that disrupt normal cellular function. Transmissible spongiform encephalopathy Contagious brain disease, such as mad cow and Creutzfeldt-Jacob disease, whose degenerative process gives the brain a sponge-like appearance Caused by accumulation of misfolded prion protein Spongiform means that when they die their brain resembles a sponge Their brain has holes in it due to neurons that are not there anymore Prion Protein that can exist in two forms that differ only in their three-dimensional shape The prion protein is at risk of being misfolded When a misfolded protein bumps into another well-folded one, it can cause this one to also become misfolded This creates clumps of misfolded proteins that all bind together Accumulation of misfolded prion protein is responsible for transmissible spongiform encephalopathies However, they are not the most prominent of the degenerative disorders Sporadic disease (another word for idiopathic diseases) Disease that is not obviously caused by heredity or an infectious agent Has an unknown cause Prion protein diseases can be genetic or sporadicand both forms can be transmitted to others by means of a simple misfolded prion protein But prion proteins often fall under the sporadic category Can be transmitted when an individual eats the neural tissue of another individual This cannot be prevented by cooking the neural tissue So once there is an outbreak, it becomes very dangerous Misfolded proteins often encourage more misfolding to occur. Thus, most neurodegenerative diseases can easily spread from neuron to neuron and from brain to brain. Bottom panel: anytime regular prion proteins was made in the cell, it would immediately be degraded even if the protein was well folded Dementia Loss of cognitive abilities such as memory, perception, verbal ability, and judgment that is caused by an organic brain disorder. Common causes are multiple strokes and Alzheimer's disease Alzheimers is a form of dementia Dementia is typically seen in old age Blanket term for several different types of degenerative disorders Alzheimer's disease Degenerative brain disorder typically caused by misfolded b-amyloid protein Clump together in the brain and causes neurons to undergo apoptosis You dont have holes in the brain in this case because the clumps dont dissipate Causes progressive memory loss, motor deficits, and eventual death Alzheimers disease Occurs in approximately 10 percent of the population above the age of sixty-five and almost 50 percent of people older than eighty-five years Produces severe degeneration within and around the hippocampus, neocortex (especially the association cortex of the frontal and temporal lobes), and other places Degeneration produces progressive loss of memory and other mental functions Brain of Alzheimers patient versus normal brain Alzheimers disease (AD) is characterised by progressive memory loss and other mental functions. -amyloid (A) Protein found in excessive amounts in brains of patients with Alzheimer's disease When it clumps together because it is misfolded, it forms amyloid plaques Amyloid plaque Extracellular deposit containing a dense core of -amyloid protein surrounded by degenerating axons and dendrites and activated microglia and reactive astrocytes Start to be surrounded by other neural tissues (axons, dendrites, etc.) Tau protein Protein that is a component of microtubules, part of the cell's transport mechanism. Tau protein becomes hyper-phosphorylated in Alzheimer's disease, which disrupts transport. In Alzheimers, the Tau proteins have tons of phosphate groups put on them They break down over time and reforms neurofibrillary tangles Neurofibrillary tangle Dying neuron containing intracellular accumulations of twisted Tau protein filaments that formerly served as cell's internal skeleton This begins to clump up as well -amyloid precursor protein (APP) Protein that is the precursor for -amyloid protein. The gene for this protein is located on chromosome 21, which is the one duplicated (triplicated) in down syndrome. Secretase
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