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19 Cards in this Set
- Front
- Back
The accumulation of which protein causes amyloid deposit?
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alpha beta (aB)
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Parkinson's results from what?
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The death of dopamine neurons in the SN.
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What are the deposits found in neurons in PD called? What do they consist of?
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Lewy bodies. Synuclein.
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Prions are infectious protein particles that are responsible for these infectious diseases?
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Scrapie and mad cow disease.
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What are the clinical signs of AD?
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Memory loss, cognitive defects, and dementia
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Mutations in the prion gene give rise to what neurodegenerative disorders?
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Creutzfield Jakob disease, Gerstmann-Straussler-Scheinker disease, and Fatal Familial Insominia
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What are the most vulnerable neurons to AD?
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hippocampus, entorhinal cortex, neocortex, and basal forebrain cholinergic system
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Where is the amyloid deposit derived from? What cleaves the APP?
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APP- amyloid precursor protein. Secretase
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How is the dystrophin protein arranged? What is the problem in DMD?
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It binds cytoskeletal actin at one end and the dystroglycan/sarcoglycan at the other. Muscle destruction due to instability of this connection and membrane disruption.
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Why is Becker MD a milder disorder?
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In its mutation, retains the translational reading frame and can produce dystrophin.
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Why is Becker MD a milder disorder?
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In its mutation, retains the translational reading frame and can produce dystrophin.
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How many cases of DMD are new mutations aka somatic mosaics?
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1/3
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What causes Congenital Muscular dystrophy?
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Mutations in laminin-2 which is link to the basement membrane of muscle.
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What can mutations in the MPZ gene cause?
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The same clinical chx's seen in PMP22 mutations.
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What do these different variations of CMT affect: HMSN, HSN, and HMN?
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Various portions of the nerve. Just the sensory nerve. The motor nerve.
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In general, the CMT Type I syndrome are of what inheritance and show what defect?
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Dominant and demyelination.
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THE CMT Type II disorders are of what inheritance and the defect is seen where?
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Dominant; axonal.
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The CMT Type IV disorders are of what inheritance and have this fx?
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Recessive; demyelination.
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What do the affected neurons in AD contain?
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tau and ubiquitin with neurofibrillary tangles.
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