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TABLE 1.2. Misfolded Proteins and Their Associated Neurodegenerative Diseases
Protein(s)
Disease(s)
Lesion(s)
Aβ and tau protein
AD, Down's syndrome
Senile plaques, NFTs
α-Synuclein
Parkinson's disease, dementia
with Lewy bodies
Lewy bodies and Lewy
neurites
Tau protein
FTLD
Tau inclusions
PrP
Transmissible prion disease
Amyloid plaques and prion
rods
Huntington
HD
Intranuclear inclusions
ABri/ADan and
tau protein
British and Danish familial
dementias
Amyloid plaques and NFTs
SOD-1
Motor neuron disease
SOD-1 inclusions
TDP-43
FTLD, motor neuron disease
Intracellular inclusions
Mutations in the genes encoding these proteins invariably give rise to familial forms of neurode-
generative disease.
Aβ, amyloid β-peptide; ADan, Danish dementia peptide; SOD-1, superoxide dismutase 1; TDP-43,
TAR DNA-binding protein 43; NFT, neurofibrillary tangle.
Adapted from Allsop et al. [26] with the permission of the Biochemical Society.
Figure 1.1. Scheme of oxidative stress biochemistry and neurotoxicity. HNE, 4-hydroxy-
2-nonenal; ONE, 4-oxo-2-nonenal (adapted from Sayre et al. [23] with the permission
of the American Chemical Society).
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