Immunohistochemical study on the distribution of alpha-synuclein in the central nervous system of transgenic mice expressing a human Cu/Zn superoxide dismutase mutation.
We used the SOD1(G93A) transgenic mice as an in vivo model of amyotrophic lateral sclerosis (ALS) and performed immunohistochemical studies to investigate whether alpha-synuclein is involved in the pathogenesis of ALS. In the spinal cord of transgenic mice, immunohistochemistry showed intense staining of alpha-synuclein mainly in the anterior horn. In the hippocampus of transgenic mice, differential increases in the staining density of alpha-synuclein were observed. In the cerebellar cortex of transgenic mice, the prominent immunostaining of alpha-synuclein was found in the molecular and granular layers. The present study provides the first in vivo evidence that alpha-synuclein immunoreactivity was increased in the central nervous system of SOD(G93A) transgenic mice, suggesting that alpha-synuclein might play an important role in the pathogenesis of ALS. However, the functional implications of these increases require elucidation.
Chung
Yoon Hee
YH
Department of Anatomy, Seoul National University College of Medicine, 28 Yongon-Dong, Chongno-Gu, Seoul 110-799, South Korea.
Joo
Kyeung Min
KM
Kim
Myeung Ju
MJ
Cha
Choong Ik
CI
D000409:Alanine / Q000235:genetics; D000818:Animals; D002490:Central Nervous System / Q000033:anatomy & histology / Q000378:metabolism*; D004195:Disease Models, Animal; D005998:Glycine / Q000235:genetics; D006801:Humans; D007150:Immunohistochemistry / Q000379:methods*; D051379:Mice; D008822:Mice, Transgenic; D009154:Mutation; D009419:Nerve Tissue Proteins / Q000378:metabolism*; D051381:Rats; D013482:Superoxide Dismutase / Q000096:biosynthesis / Q000235:genetics*; D051843:Synucleins; D014018:Tissue Distribution; D051844:alpha-Synuclein
D009419:Nerve Tissue Proteins; C497604:SNCA protein, human; C497605:Snca protein, mouse; C497606:Snca protein, rat; D051843:Synucleins; D051844:alpha-Synuclein; D013482:Superoxide Dismutase; D000409:Alanine; D005998:Glycine