Crystal Structure of Filamentous Aggregates of Human DJ-1 Formed in an Inorganic Phosphate-dependent Manner
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SCOPUS
- Title
- Crystal Structure of Filamentous Aggregates of Human DJ-1 Formed in an Inorganic Phosphate-dependent Manner
- Authors
- Cha, SS; Il Jung, H; Jeon, H; An, YJ; Kim, IK; Yun, S; Ahn, HJ; Chung, KC; Lee, SH; Suh, PG; Kang, SO
- Date Issued
- 2008-12-05
- Publisher
- AMER SOC BIOCHEMISTRY MOLECULAR BIOLO
- 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.
- Keywords
- FAMILIAL PARKINSONS-DISEASE; MULTIPLE SYSTEM ATROPHY; OXIDATIVE STRESS; DROSOPHILA DJ-1; ALPHA-SYNUCLEIN; PROTEIN DJ-1; CELL-DEATH; FERTILITY; L166P; INACTIVATION
- URI
- https://oasis.postech.ac.kr/handle/2014.oak/22376
- DOI
- 10.1074/JBC.M8042432
- ISSN
- 0021-9258
- Article Type
- Article
- Citation
- JOURNAL OF BIOLOGICAL CHEMISTRY, vol. 283, no. 49, page. 34069 - 34075, 2008-12-05
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