期刊
JOURNAL OF BIOLOGICAL CHEMISTRY
卷 283, 期 49, 页码 34069-34075出版社
AMER SOC BIOCHEMISTRY MOLECULAR BIOLOGY INC
DOI: 10.1074/jbc.M804243200
关键词
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资金
- Functional Proteomics Center Program
- Korea Ministry of Science and Technology
- Marine & Extreme Genome Research Center Program
- Ministry of Land, Transport, and Maritime Affairs, Republic of Korea
- BK21 Project, Republic of Korea
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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