lecture 20 Flashcards
There are many different kinds of neurodegenerative disease, such as what
prion diseases, Huntington’s, Parkinson’s, Alzheimer’s, and ALS
Degeneration is typically the result of what
apoptosis, which is triggered by collections (aggregates) of misfolded proteins that disrupt normal cellular function
what is 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
what is a Prion
Protein that can exist in two forms that differ only in their three-dimensional shape
Accumulation of misfolded prion protein is responsible for what
transmissible spongiform encephalopathies
what kind of disease is a Sporadic disease
Prion protein disease
A Sporadic disease is one that is caused by what
not obviously caused by heredity or an infectious agent. Prion protein diseases can be genetic or sporadic—and both forms can be transmitted to others by means of a simple misfolded prion protein
Misfolded proteins often encourage what
more misfolding to occur
do most neurodegenerative diseases spread
Misfolded proteins often encourage more misfolding to occur. Thus, most neurodegenerative diseases can easily spread from neuron to neuron and from brain to brain
what is Huntington’s disease
an inherited gene disorder associated with neurodegeneration in areas of the basal ganglia.
Huntington’s disease happens to who
Symptoms usually begin between 30 and 50 years of age and death follows 15-20 years later.
Huntington’s is characterized by what
an increasingly severe lack of coordination, uncontrollable jerky limb movements, and eventually dementia followed by death
Movements in Huntington’s disease look like what
fragments of purposeful movements but occur involuntarily.
Huntington’s disease is caused by what
one dominant mutation in the Huntingtin gene and it affects 1 in 10,000 people
Huntingtin protein is heavily expressed where
in the input nuclei of the basal ganglia (the striatum - caudate nucleus and putamen).
Mutated Huntingtin protein tends to do what
aggregate (clump) and overtime this causes degeneration of neurons in these regions
is there a cure for Huntingtin’s
There is presently no cure (or treatment really) but antisense gene therapy is showing promise in clinical trials
what is ANTISENSE THERAPY
Antisense DNA can be administered (repeatedly) intracerebroventricularly. The hope for the relatively near future is that viral-mediated gene delivery and/or gene editing technologies will become a practical and effective approach to altering gene expression in brain tissue
Parkinson’s disease is what kind of disorder
another degenerative “movement” disorder
Parkinson’s disease is associated with degeneration of what
dopamine neurons in the midbrain, specifically in the substantia nigra
who does Parkinson’s disease impact
It affects 1% of the population. Symptoms usually appear after the age of 60.
what is Parkinson’s disease characterized by
It is characterized by shaking, muscular rigidity, slowness of movement, difficulty walking, and eventually dementia. Without treatment, people have increasing difficulty initiating purposeful movement
The causes of Parkinson’s disease are what
ly unknown (partly genetic, partly environmental),
The causes of Parkinson’s disease are largely unknown (partly genetic, partly environmental), however the death of midbrain dopamine neurons seems to relate to what
aggregation of the protein alpha-synuclein.
The protein alpha-synuclein is what
heavily expressed in dopamine neurons and it can aggregate (clump together). Overtime these clumps cause dopamine neurons to undergo apoptosis
Reduced dopamine signaling in the basal ganglia disrupts what
movement
is there a cure for Parkinson’s disease
There is presently no cure, but there are many ways to somewhat successfully treat the motor problems
what other symptoms occur in Parkinson’s disease
Cognitive, emotional, and sleep disturbances eventually develop as well and there are currently no good treatments for those symptoms
what are some parts to PARKINSON’S DISEASE
Alpha- synuclein Lewy body Ubiquitin Parkin Proteasome
what is Alpha- synuclein
Protein heavily express in midbrain dopamine neurons. Its function is not entirely clear. Abnormal accumulations are associated with dopamine neuron degeneration in Parkinson’s disease
what is Lewy body
Aggregate of misfolded alpha-synuclein protein; found in the cytoplasm of midbrain dopamine neurons in people with Parkinson’s disease