Neurodegeneration Flashcards
1
Q
What are the 8 hallmarks of neurodegenerative disease?
A
- Pathological protein aggregation
- Synaptic and neuronal network dysfunction
- Aberrant proteostasis
- Cytoskeletal abnormalities
- Altered energy homeostasis
- DNA and RNA defects
- Inflammation
- Neuronal cell death
2
Q
ALS involves the loss of which motor neurons - upper or lower?
A
Both
3
Q
What percentage of ALS cases are genetic and what percentage are sporadic?
A
10% genetic
90% sporadic
4
Q
Which gender is more likely to get sporadic ALS?
A
Males
5
Q
What is the average age of onset of ALS?
A
Middle-to-late 50s
6
Q
What is the life expectancy of ALS from symptom onset?
A
2-5 years
7
Q
What is the neuropathological hallmark of ALS?
A
Aggregation and accumulation of misfolded proteins leading to intracellular inclusions in motor neurons
8
Q
Which misfolded protein is involved in ALS?
A
SOD1
9
Q
In ALS, astrocytes and microglia undergo which processes?
A
Astrocytic gliosis
Microglial infiltration
10
Q
A
