Pael-R is accumulated in Lewy bodies of Parkinson's disease.

Murakami, Tetsuro; Shoji, Mikio; Imai, Yuzuru; Inoue, Haruhisa; Kawarabayashi, Takeshi; Matsubara, Etsuro; Harigaya, Yasuo; Sasaki, Atsushi et al. · Ann Neurol · 2004

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Abstract

We examined the distribution of Pael-R, a newly identified substrate for Parkin, in Parkinson's disease (PD) and multiple system atrophy (MSA). Pael-R, Parkin, alpha-synuclein, and ubiquitin accumulated in Lewy bodies (LBs) and neurites. Pael-R was localized in the core of LBs. Parkin and alpha-synuclein accumulated in the halo, neuronal cell bodies, and processes. These findings potentially suggest the involvement of Pael-R in LB formation, and protection role of Parkin in Pael-R-mediated neurotoxicity in PD. The absence of Pael-R and Parkin in glial cytoplasmic inclusions (GCIs) in MSA implies a distinct pathway involved in the formation of LBs and GCIs.

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