feb '09 Nature:
Good and bad cell death by Donald W. Nicholson:
...
Whereas Alzheimer's disease affects neurons and synaptic junctions of the cerebral cortex, Huntington's disease is characterized by progressive and inexorable deterioration of neurons that project to the striatum region of the brain. Caspase-6-mediated breakdown of huntingtin, the protein that is mutated in Huntington's disease, is necessary for neuronal dysfunction and degeneration in this disorder. Whether the circuitry involved in APP cleavage, DR6 triggering and caspase activation have broad, overlapping mechanistic commonalities in the development of the nervous system, response to injury and disease-associated neurodegeneration is not known. But the links are intriguing and warrant further attention.
--proposed: loss of NGF (nerve growth factor) resulting in APP (amyloid-beta precursor protein) cleavage to generate N-APP (amino terminal portion of APP) that binds to DR6 (death receptor) and activate caspases
on a side note,
- domain swapping between amyloid (misfolded proteins) and prions have been linked to Alzheimer's as well
- not all amyloids are bad, some are functional, maybe nanomaterials?
http://repositorio-aberto.up.pt/bitstream/10216/7161/10/Text.pdf
Huntington’s disease is an autosomal-dominant, progressive neurodegenerative
disorder which affects 4-7 in every 100 000 individuals, worldwide.
Huntington’s disease designation comes from the name of an English doctor, who
vividly characterized this disorder, George Huntington. Several doctors in the 19th century
noticed the hereditary nature of the disease but it was George Huntington who introduced
the term “chorea”, from the Greek word for dance, to describe the involuntary “dance-like”
movements shown by his patients (Elliotson, 1832; Hayden, 1981; Harper P in Bates,
2002; Folstein, 1989).
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