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26 Cards in this Set
- Front
- Back
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AD: sporadic or genetic
course |
sporadic
10 years |
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NFT: unique to AD?
role of tau what does i |
nope. seen in other diseases too, but CHAR of AD
microtubule stability: there is abnormal modification of this leading to insoluble tau |
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neuropil threads
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bands of abnormal tau, within dendrites
more in AD |
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what is the AB hypothesis?
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ab is toxic and is the causative agent in AD
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neuritic plaques:
associated with what? |
NEURITES that are dystrophic == stain with silver
some of these have TAU |
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pick bodies contain?
pick cells? |
bodies: tau
cells: neurofilaments |
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picks or NFT more common
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NFT
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role of TDP43
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DNA binding protein- transcription factor
also seen in ALS |
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what is PrP
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normal component of neurons
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gold Std for CJD
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Western blot
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nvCJD
Age of onset clinical features |
Age: <40
Clin: Psych and ATAXIA |
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huntingtin: mechanism of action
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gain of function
N terminal fragment generated by proteases could be key. inactivated export signal leads it to build up in the neurons. |
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how does the atrophy spread in huntington's
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DORSAL to ventral!
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grading system?
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VON SATTEL
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causes of dementia in PD?
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Alzheimers!
LB disease |
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In huntingtin, which NT are affected
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GABA
Penkephalin less substance P loss of medium sized neurons |
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t/f striatal neurons atrophy in PD
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nope. they try to become more sensitive to Dopamine
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ALS: just UMN?
just LMN? |
UMN: primary lateral sclerosis
- hits CS tract LMN: primary muscular atrophy |
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Familial ALS caused by
is it mostly familial? |
Cu/Zn superoxide dismutase
nope: only 5% familial |
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sporadic and familial ALS caused by
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TDP-43
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sooner death for ALS?
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if you have the BULBAR form: start with CN involvement
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most common hereditary ataxia?
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FA!
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presentation of FA
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ataxic gait, cannot walk
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cause of death in FA
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hypertrophic cardiomyopathy
also high incidence of DM |
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which protein is lost in FA?
normal role? |
frataxin gene
need for iron homeostasis |
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mechanism of damage
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loss of function
poor mito iron metabolism leads to increased ROS = damage |
