Abstract:
:Recent successes in identifying the genes and associated proteins underlying several familial neurodegenerative conditions have not always resulted in accounts as to why the associated patterns of neuronal damage are so specific and limited. Here, with reference to Huntington's disease, we present a general scheme to show how the mutant protein could interact with associated proteins to form an aggregation product. This could lead to neuronal death by direct actions on caspases, or by raising the levels of intracellular calcium ions and reactive oxygen species above a threshold that cannot be resisted by the protection normally conferred by endogenous factors such as calcium binding proteins, free radical scavengers and trophic factors. The local distributions of vulnerability and protective factors could ultimately dictate the pattern of damage induced by the mutant gene.
journal_name
IUBMB Lifejournal_title
IUBMB lifeauthors
Mitchell IJ,Griffiths MRdoi
10.1080/1521654031000153012keywords:
subject
Has Abstractpub_date
2003-06-01 00:00:00pages
293-8issue
6eissn
1521-6543issn
1521-6551journal_volume
55pub_type
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