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Crystal Structure of Filamentous Aggregates of Human DJ-1 Formed in an Inorganic Phosphate-dependent Manner*

By Sun-Shin Cha, Ha Il Jung, Hyesung Jeon, Young Jun An, In-Kwon Kim, Sanguk Yun, Hyun Jin Ahn, Kwang Chul Chung, Sang Hee Lee, Pann-Ghill Suh and Sa-Ouk Kang


Mutations in the DJ-1 gene have been implicated in the autosomal recessive early onset parkinsonism. DJ-1 is a soluble dimeric protein with critical roles in response to oxidative stress and in neuronal maintenance. However, several lines of evidence suggest the existence of a nonfunctional aggregated form of DJ-1 in the brain of patients with some neurodegenerative diseases. Here, we show that inorganic phosphate, an important anion that exhibits elevated levels in patients with Parkinson disease, transforms DJ-1 into filamentous aggregates. According to the 2.4-Å crystal structure, DJ-1 dimers are linearly stacked through Pi-mediated interactions to form protofilaments, which are then bundled into a filamentous assembly

Topics: Protein Structure and Folding
Publisher: American Society for Biochemistry and Molecular Biology
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Provided by: PubMed Central
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