PARK7 (DJ-1)

DJ-1, FLJ34360, FLJ27376, PARK7, FLJ92274
PARK2 (Parkin), Targeting Oxidative Stress, Oxidative Stress, PARK6 (PINK1)

PARK7 was originally linked to a Dutch family exhibiting autosomal recessive, young onset parkinsonism (van Duijn et al., 2001). Mutations in the gene for DJ-1 were ultimately associated with the PARK7 locus (Bonifati et al., 2003). Affected carriers of DJ-1 mutations exhibit generally early age of PD onset and slow disease progression (Dekker et al., 2003).

DJ-1 is an atypical peroxiredoxin-like peroxidase that regulates androgen-receptor transcription, acts as a redox-dependent chaperone, and acts as a sensor for oxidative stress. Its chaperone activity is thought to be sensitive to redox conditions: DJ-1 contains several cysteine residues that undergo acidic shift when exposed to oxidative conditions (Abou-Sleiman et al., 2006; Zhou et al., 2006; Cookson, 2005).

Reference: 
Abou-Sleiman PM, Muqit MM, Wood NW. Expanding insights of mitochondrial dysfunction in Parkinson's disease. Nat Rev Neurosci. 2006;7(3):207-19.
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Zhou W, Zhu M, Wilson MA, Petsko GA, Fink AL. The oxidation state of DJ-1 regulates its chaperone activity toward alpha-synuclein. J Mol Biol. 2006;356(4):1036-48.
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Cookson MR. The biochemistry of Parkinson's disease. Annu Rev Biochem. 2005;74:29-52.
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van Duijn CM, Dekker MC, Bonifati V, Galjaard RJ, Houwing-Duistermaat JJ, Snijders PJ, et al. Park7, a novel locus for autosomal recessive early-onset parkinsonism, on chromosome 1p36. Am J Hum Genet. 2001;69(3):629-34.
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Bonifati V, Rizzu P, van Baren MJ, Schaap O, Breedveld GJ, Krieger E, et al. Mutations in the DJ-1 gene associated with autosomal recessive early-onset parkinsonism. Science. 2003;299(5604):256-9.
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Dekker M, Bonifati V, van Swieten J, Leenders N, Galjaard RJ, Snijders P, et al. Clinical features and neuroimaging of PARK7-linked parkinsonism. Mov Disord. 2003;18(7):751-7.
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Contributions:
Selenium double hit in substantia nigra
Hot Topics@SfN 2009
Michael T Rogan
20 Oct 2009
Farzad Mortazavi et al. reported in poster 531.5 that restricting dietary selenium has a dramatic ‘double hit’ effect on the DJ-1 knockout model of PD.  A loss-of-function DJ-1 ... 
Responses: 1
Workgroups:
Genetic Models of Parkinson's Disease Group
10 Jun 2009
This group aims to promote interactions, collaborations and the exchange of ideas of those members interested in developing or characterizing genetic disease models, particularly mouse models. ...