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Exp Neurobiol 2015; 24(1): 1-7
Published online March 31, 2015
https://doi.org/10.5607/en.2015.24.1.1
© The Korean Society for Brain and Neural Sciences
Shynrye Lee and Hyung-Jun Kim*
Korea Brain Research Institute, Research Division, Daegu 700-010, Korea
Correspondence to: *To whom correspondence should be addressed.
TEL: 82-70-4496-3296, FAX: 82-53-428-1851
e-mail: kijang1@kbri.re.kr
ALS is a fatal adult-onset motor neuron disease. Motor neurons in the cortex, brain stem and spinal cord gradually degenerate in ALS patients, and most ALS patients die within 3~5 years of disease onset due to respiratory failure. The major pathological hallmark of ALS is abnormal accumulation of protein inclusions containing TDP-43, FUS or SOD1 protein. Moreover, the focality of clinical onset and regional spreading of neurodegeneration are typical features of ALS. These clinical data indicate that neurodegeneration in ALS is an orderly propagating process, which seems to share the signature of a seeded self-propagation with pathogenic prion proteins.
Keywords: ALS (Amyotrophic lateral sclerosis), SOD1, TDP-43, FUS, Plion-like phenomena, Misfolded protein aggregates