ALS Flashcards
1
Q
Degenerative disease of CNS
A
Neuronal loss is selective
No initiating factor
Slow deterioration of body function
Proteins that normally form filaments collapse and form perinuclear bundles
2
Q
Most common neuromuscular disease
A
Amyotrophic lateral sclerosis —> Lou Gehrig
Terminal - unknown cause
3
Q
ALS characterized by
A
Degernation and scarring of neurons in
- spinal cord (ant horn cells)
- brainstem (corticobulbar and corticospinal)
- brain (betz cells in motor cortex)
4
Q
Average age of onset - ALS
A
Mid to late 50s
Men slightly more that women
5
Q
SODs
A
Super oxide dismutases - group of enzymes that eliminated O2 free radicals
Implicated in neurodegneration - possible cause of ALS
6
Q
Three forms of SODS
A
CuZnSOD
MnSOD
ECSOD
7
Q
SOD1
A
Gene on chromosome 1 encodes CuZnSOD
20% of people w/ ALS have mutation of this gene. May lead to increase in free radicals
