Crystal Structure of Filamentous Aggregates of Human DJ-1 Formed in an Inorganic Phosphate-dependent Manner
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Title
- Crystal Structure of Filamentous Aggregates of Human DJ-1 Formed in an Inorganic Phosphate-dependent Manner
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Author(s)
- Cha, Sun-Shin; Il Jung, Ha; Jeon, Hyesung; An, Young Jun; Kim, In-Kwon; Yun, Sanguk; Ahn, Hyun Jin; Chung, Kwang Chul; Lee, Sang Hee; Suh, Pann-Ghill; Kang, Sa-Ouk
- KIOST Author(s)
- An, Young Jun(안영준)
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Alternative Author(s)
- 차선신; 정하일; 안영준
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Publication Year
- 2008-12
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Abstract
- 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-angstrom crystal structure, DJ-1 dimers are linearly stacked through P-i-mediated interactions to form protofilaments, which are then bundled into a filamentous assembly.
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ISSN
- 0021-9258
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URI
- https://sciwatch.kiost.ac.kr/handle/2020.kiost/4411
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DOI
- 10.1074/jbc.M804243200
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Bibliographic Citation
- Journal of Biological Chemistry, v.283, no.49, pp.34069 - 34075, 2008
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Publisher
- American Society for Biochemistry and Molecular Biology Inc.
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Type
- Article
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Language
- English
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Document Type
- Article
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