Proteinopathies

Question 1

List four neurodegenerative diseases the protein aggregates are found in

Answer 1

Alzheimer’s, Parkinson’s Huntington’s disease, ALS

Question 2

List the clinical phenotype and molecular pathology for parkinson

Answer 2

Clinical phenotype: MSA, PSP, PD, and LBD
Molecular pathology: alpha-synuclein

Question 3

List the clinical phenotype and molecular pathology for alzheimer’s disease

Answer 3

clinical phenotype: LO, AD, EO, PCA
Molecular path: alpha beta 42, p-Tau

Question 4

What are biomarkers for DOPA?

Answer 4

Flurodopa PET, Troda SPECT

Question 5

List the clinical phenotype and molecular pathology for FTD-ALS spectrum

Answer 5

Molecular pathology: Tau, TDP-43, FUS, UPS
Clinical phenotype: PPA (iogopenic and semantic and nonfluent), bvFTD, fvAD, ALS

Question 6

What are biomarkers for amyloid?

Answer 6

ABeta 42 in CSF
AMyloid PET (PIB, AV45, etc)

Question 7

What are biomarkers for Tau?

Answer 7

p-tau in CSF, tau PET (Flortaucipir, etc)

Question 8

What are biomarkers for neurodegeneration?

Answer 8

MRI scan
T-Tau in CSF
FDG-PET

Question 9

PH-Tau injection leads to __ __ 6 months post-injection

Answer 9

cognitive decline

Question 10

Tau binds tightly to __

Answer 10

microtubules

Question 11

PH-Tau disrupts __ and induces __ __

Answer 11

microtubules
membrane blebbing

Question 12

T/F: PH-Tau toxic behavior can be transferred from cell to cell

Answer 12

True! like a prion-effect

Question 13

PH-Tau __ neighboring cells and disrupts __

Answer 13

invades, microtubules

Question 14

Transfer of PH-Tau between cells is __ __

Answer 14

cell specific

Question 15

Recombinant Tau and PH-Tau enter cells that express __ __

Answer 15

muscarinic receptors

Question 16

Recombinant tau and PH-tau enter __ neurons within __ __

Answer 16

primary
24 hours

Question 17

Treatment of PH-Tau with __ blocks the __

Answer 17

atropine, uptake

Question 18

In Alzheimer Disease P-Tau as PH-Tau is uptaken in the __ and destroys __

Answer 18

neurons; microtubules

Question 19

T/F: atropine treatment blocks AD P-tau uptake

Answer 19

true

Question 20

tcDCs reduces __ __ and upregulates HSP70 __

Answer 20

Tau accumulation
expression

Question 21

Tau pathology is __ and is pivotal in the pathogenesis of __ and other tauopathies

Answer 21

primary
AD

Question 22

Abnormal __ of tau is an early step in the process of __ degeneration

Answer 22

hyperphosphorylation
neurofibrillary

Question 23

The combination of __ at Thr212, 231, and SER199 and 262 in the same tau molecule can trigger __ in __ and __

Answer 23

phosphorylation
toxicity
vitro, in vivo

Question 24

List four new inducible mouse model recapitulates

Answer 24

oligomeric tau
truncated tau
early cognitive impairment
neuronal loss and astrocytes activation

Question 25

Tau can be __ from one __ to the next. Blocking __ receptors with __ resulted in __ of tau uptake

Answer 25

transferred, neuron
muscarinic, atropine
prevention