11.28 Neurodegenerative Disorders/Lab Flashcards
what protein is spliced differently in Alzheimer’s to make the defective amyloid protein?
amyloid precursor protein (APP)
what is Parkinson’s caused by?
degeneration of dopaminergic neurons in the substantial nigra
Infectious prion protein in CJD differs from normal by a _____________ change (_______ ____ to a ______ _______).
conformational
alpha helix, beta sheet
what is this histologically? disease?
neurofibrillary tangle
Alzheimer’s
what is this depicting? what disease?
spongiform degeneration with amyloid plaques
the new strian of CJD
what are the two proteins (not structures they make) that are accumulated in Alzheimer’s?
tau amyloid-beta peptide
what are the three forms (ways) of Creutzfeldt-Jacob Disease?
Inherited Spontaneous Transmissible
what is the most major feature in the pathogenesis of neurodegenerative disease?
accumulation of misfolded or aggregation-prone proteins
what do Lewy bodies contain?
alpha-synuclein
what are the two structures that can be seen in histology to make the diagnosis of Alzheimer’s?
neurofibrillary tangles neurotic plaques
what does Creutzfeldt-Jacob Disease present as?
rapidly progressing dementia with myoclonus and death an approx 7 months
what aggregated protein is seen in Parkinson’s
alpha-synuclein
spongiform encephalopathy is seen in _____ diseases
prion
what can you see in a spinal cord cut in ALS histology?
degeneration of corticospinal tract loss of LMN in ventral horn
subacute fatal disease of cerebellar degeneration with ataxia and dementia following ritualistic cannibalism
kuru
what is this histologically? what is it an aggregate of? disease?
Lewy body
alpha-synuclein
Parkinson’s
when Lewy bodies are seen in the cerebral cortex this is called:
Dementia with Lewy bodies
what is this histologically? disease?
neurotic plaques (amyloid beta core and taue containing processes on outside)
Alzheimer’s
what is the most common form of spongiform encephalopathy in humans?
Creutzfeldt-Jacob Disease
progressive neurodegenerative disease characterized by loss of UMNs and LMNs.
ALS (amyotrophic lateral sclerosis)
what is wrong with the prion in Creutzfeldt-Jacob Disease? what can it do to the host?
it has an abnormal conformational change it can cause a conformational change in endogenous prion molecules of the host
what are the inclusions in cells histologically of ALS?
TDP-43 inclusions in remaining motor neurons
what do neurotic plaques consist of? in what neurodegenerative disease are they found?
abnormal neurites (processes with tau) that surround a core of amyloid
neurofibrillary tangles are seen in _________. What abnormal aggregate of protein is inside them? Where?
Alzheimer’s tau cytoplasm
