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PDBsum entry 1soa

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protein links
Protein binding PDB id
1soa
Jmol
Contents
Protein chain
187 a.a. *
Waters ×268
* Residue conservation analysis
PDB id:
1soa
Name: Protein binding
Title: Human dj-1 with sulfinic acid
Structure: RNA-binding protein regulatory subunit. Oncogene chain: a. Engineered: yes
Source: Homo sapiens. Human. Organism_taxid: 9606. Expressed in: escherichia coli bl21(de3). Expression_system_taxid: 469008.
Biol. unit: Dimer (from PDB file)
Resolution:
1.20Å     R-factor:   0.113     R-free:   0.141
Authors: R.Canet-Aviles,M.A.Wilson,D.W.Miller,R.Ahmad,C.Mclendon, S.Bandyopadhyay,M.J.Baptista,D.Ringe,G.A.Petsko,M.R.Cookson
Key ref:
R.M.Canet-Avilés et al. (2004). The Parkinson's disease protein DJ-1 is neuroprotective due to cysteine-sulfinic acid-driven mitochondrial localization. Proc Natl Acad Sci U S A, 101, 9103-9108. PubMed id: 15181200 DOI: 10.1073/pnas.0402959101
Date:
13-Mar-04     Release date:   22-Jun-04    
PROCHECK
Go to PROCHECK summary
 Headers
 References

Protein chain
Pfam   ArchSchema ?
Q99497  (PARK7_HUMAN) -  Protein DJ-1
Seq:
Struc:
189 a.a.
187 a.a.*
Key:    PfamA domain  Secondary structure  CATH domain
* PDB and UniProt seqs differ at 1 residue position (black cross)

 Gene Ontology (GO) functional annotation 
  GO annot!
  Cellular component     membrane   11 terms 
  Biological process     response to stress   65 terms 
  Biochemical function     protein binding     31 terms  

 

 
DOI no: 10.1073/pnas.0402959101 Proc Natl Acad Sci U S A 101:9103-9108 (2004)
PubMed id: 15181200  
 
 
The Parkinson's disease protein DJ-1 is neuroprotective due to cysteine-sulfinic acid-driven mitochondrial localization.
R.M.Canet-Avilés, M.A.Wilson, D.W.Miller, R.Ahmad, C.McLendon, S.Bandyopadhyay, M.J.Baptista, D.Ringe, G.A.Petsko, M.R.Cookson.
 
  ABSTRACT  
 
Loss-of-function DJ-1 mutations can cause early-onset Parkinson's disease. The function of DJ-1 is unknown, but an acidic isoform accumulates after oxidative stress, leading to the suggestion that DJ-1 is protective under these conditions. We addressed whether this represents a posttranslational modification at cysteine residues by systematically mutating cysteine residues in human DJ-1. WT or C53A DJ-1 was readily oxidized in cultured cells, generating a pI 5.8 isoform, but an artificial C106A mutant was not. We observed a cysteine-sulfinic acid at C106 in crystalline DJ-1 but no modification of C53 or C46. Oxidation of DJ-1 was promoted by the crystallization procedure. In addition, oxidation-induced mitochondrial relocalization of DJ-1 and protection against cell death were abrogated in C106A but not C53A or C46A. We suggest that DJ-1 protects against neuronal death, and that this is signaled by acidification of the key cysteine residue, C106.
 
  Selected figure(s)  
 
Figure 1.
Fig. 1. Generation of stable dimeric cysteine mutants of human DJ-1. (a) The crystal structure of human DJ-1 with cysteine residues highlighted. Two cysteine residues, C46 and C53, whose sidechains are shown in red, are present in a -turn close to the dimer interface. A third cysteine, C106, is within the center of the dimer. (b) Transient transfections of V5-tagged cysteine mutants into M17 cells. Because some variants, notably C46A, were unstable, samples in lanes 7-11 were treated with 5 µM MG132 for 24 h. Western blots were probed with anti-DJ-1 (Upper) to visualize both transfected DJ-1 (arrows) and endogenous DJ-1 (arrowhead). The blot was reprobed with -actin as a loading control (Lower, open arrowhead). Markers on the right of the blot are in kilodaltons. (c) To assess whether these variants form dimers, transfected cells were treated with the crosslinker disuccinimidyl suberate before extraction and Western blotting for V5 (Upper) or DJ-1 (Lower). Monomeric V5-tagged protein is indicated with an arrow, and endogenous DJ-1 in the cells indicated by an arrowhead. The V5-tagged proteins formed dimers with either endogenous or transfected DJ-1, indicated by lines on the left of the blot. Blots are representative of at least duplicate experiments. To confirm these results, we coimmunoprecipitated V5-tagged DJ-1 with endogenous protein (d). (Upper) Lysates of transfected cells probed with V5 or DJ-1 to demonstrate input levels of proteins. (Lower) Lysates immunoprecipitated withV5 antibody and blotted with DJ-1 antibody to show interaction of endogenous DJ-1 with the tagged proteins.
Figure 4.
Fig. 4. DJ-1 localizes to the outer mitochondrial membrane after oxidative stress. (a) M17 cells were transfected with V5-tagged DJ-1 variants as indicated and stained for DJ-1 (V5, green) and mitotracker (red). After exposure to PQ2+ (Lower), DJ-1 colocalized with mitochondria much more extensively than under control conditions (Upper), as evidenced by the yellow color where staining overlaps. The C106A and C106D variants did not relocalize to mitochondria, whereas C53A and C46A did. This finding is quantified in b by counting the proportion of V5-positive cells that showed strong colocalization with mitotracker. Representative data are shown from at least three experiments for each construct, performed in duplicate. (c) DJ-1 is not imported into mitochondria. Cells transfected with WT DJ-1 were exposed to PQ2+ and mitochondrial fractions prepared. Duplicate samples were either untreated (-, lanes 1 and 2) or subjected to limited proteolysis with trypsin (+, lanes 3 and 4) and blotted for V5 (Top), the outer mitochondrial membrane protein VDAC1 (Middle), or the inner mitochondrial membrane protein Tim23 (Bottom).
 
  Figures were selected by an automated process.  

Literature references that cite this PDB file's key reference

  PubMed id Reference
19683835 A.Shtifman, N.Zhong, J.R.Lopez, J.Shen, and J.Xu (2011).
Altered Ca2+ homeostasis in the skeletal muscle of DJ-1 null mice.
  Neurobiol Aging, 32, 125-132.  
20497343 D.A.Hinkle, S.J.Mullett, B.E.Gabris, and R.L.Hamilton (2011).
DJ-1 expression in glioblastomas shows positive correlation with p53 expression and negative correlation with epidermal growth factor receptor amplification.
  Neuropathology, 31, 29-37.  
21034221 J.M.Shulman, P.L.De Jager, and M.B.Feany (2011).
Parkinson's disease: genetics and pathogenesis.
  Annu Rev Pathol, 6, 193-222.  
21160133 J.W.Gao, T.Yamane, H.Maita, S.Ishikawa, S.M.Iguchi-Ariga, X.P.Pu, and H.Ariga (2011).
DJ-1-Mediated Protective Effect of Protocatechuic Aldehyde Against Oxidative Stress in SH-SY5Y Cells.
  J Pharmacol Sci, 115, 36-44.  
20940149 K.J.Thomas, M.K.McCoy, J.Blackinton, A.Beilina, M.van der Brug, A.Sandebring, D.Miller, D.Maric, A.Cedazo-Minguez, and M.R.Cookson (2011).
DJ-1 acts in parallel to the PINK1/parkin pathway to control mitochondrial function and autophagy.
  Hum Mol Genet, 20, 40-50.  
  21461368 P.C.Keane, M.Kurzawa, P.G.Blain, and C.M.Morris (2011).
Mitochondrial dysfunction in Parkinson's disease.
  Parkinsons Dis, 2011, 716871.  
  21219333 S.J.Mullett, and D.A.Hinkle (2011).
DJ-1 deficiency in astrocytes selectively enhances mitochondrial Complex I inhibitor-induced neurotoxicity.
  J Neurochem, 117, 375-387.  
20089532 C.P.Ramsey, E.Tsika, H.Ischiropoulos, and B.I.Giasson (2010).
DJ-1 deficient mice demonstrate similar vulnerability to pathogenic Ala53Thr human alpha-syn toxicity.
  Hum Mol Genet, 19, 1425-1437.  
  20577640 D.M.Branco, D.M.Arduino, A.R.Esteves, D.F.Silva, S.M.Cardoso, and C.R.Oliveira (2010).
Cross-talk between mitochondria and proteasome in Parkinson's disease pathogenesis.
  Front Aging Neurosci, 2, 17.  
20225270 D.Rogers, and N.F.Schor (2010).
The child is father to the man: developmental roles for proteins of importance for neurodegenerative disease.
  Ann Neurol, 67, 151-158.  
19680261 E.Giaime, C.Sunyach, C.Druon, S.Scarzello, G.Robert, S.Grosso, P.Auberger, M.S.Goldberg, J.Shen, P.Heutink, J.Pouysségur, G.Pagès, F.Checler, and C.Alves da Costa (2010).
Loss of function of DJ-1 triggered by Parkinson's disease-associated mutation is due to proteolytic resistance to caspase-6.
  Cell Death Differ, 17, 158-169.  
20376509 E.M.Cornejo Castro, J.Waak, S.S.Weber, F.C.Fiesel, P.Oberhettinger, M.Schütz, I.B.Autenrieth, W.Springer, and P.J.Kahle (2010).
Parkinson's disease-associated DJ-1 modulates innate immunity signaling in Caenorhabditis elegans.
  J Neural Transm, 117, 599-604.  
20842103 F.Kamp, N.Exner, A.K.Lutz, N.Wender, J.Hegermann, B.Brunner, B.Nuscher, T.Bartels, A.Giese, K.Beyer, S.Eimer, K.F.Winklhofer, and C.Haass (2010).
Inhibition of mitochondrial fusion by α-synuclein is rescued by PINK1, Parkin and DJ-1.
  EMBO J, 29, 3571-3589.  
20124404 F.Kthiri, H.T.Le, V.Gautier, T.Caldas, A.Malki, A.Landoulsi, C.Bohn, P.Bouloc, and G.Richarme (2010).
Protein aggregation in a mutant deficient in yajL, the bacterial homolog of the Parkinsonism-associated protein DJ-1.
  J Biol Chem, 285, 10328-10336.  
20186336 G.Krebiehl, S.Ruckerbauer, L.F.Burbulla, N.Kieper, B.Maurer, J.Waak, H.Wolburg, Z.Gizatullina, F.N.Gellerich, D.Woitalla, O.Riess, P.J.Kahle, T.Proikas-Cezanne, and R.Krüger (2010).
Reduced basal autophagy and impaired mitochondrial dynamics due to loss of Parkinson's disease-associated protein DJ-1.
  PLoS One, 5, e9367.  
20945983 G.Lopez, and E.Sidransky (2010).
Autosomal recessive mutations in the development of Parkinson's disease.
  Biomark Med, 4, 713-721.  
20043722 G.Xiromerisiou, E.Dardiotis, V.Tsimourtou, P.M.Kountra, K.N.Paterakis, E.Z.Kapsalaki, K.N.Fountas, and G.M.Hadjigeorgiou (2010).
Genetic basis of Parkinson disease.
  Neurosurg Focus, 28, E7.  
20133695 H.Aleyasin, M.W.Rousseaux, P.C.Marcogliese, S.J.Hewitt, I.Irrcher, A.P.Joselin, M.Parsanejad, R.H.Kim, P.Rizzu, S.M.Callaghan, R.S.Slack, T.W.Mak, and D.S.Park (2010).
DJ-1 protects the nigrostriatal axis from the neurotoxin MPTP by modulation of the AKT pathway.
  Proc Natl Acad Sci U S A, 107, 3186-3191.  
20639397 I.Irrcher, H.Aleyasin, E.L.Seifert, S.J.Hewitt, S.Chhabra, M.Phillips, A.K.Lutz, M.W.Rousseaux, L.Bevilacqua, A.Jahani-Asl, S.Callaghan, J.G.MacLaurin, K.F.Winklhofer, P.Rizzu, P.Rippstein, R.H.Kim, C.X.Chen, E.A.Fon, R.S.Slack, M.E.Harper, H.M.McBride, T.W.Mak, and D.S.Park (2010).
Loss of the Parkinson's disease-linked gene DJ-1 perturbs mitochondrial dynamics.
  Hum Mol Genet, 19, 3734-3746.  
  20398440 I.P.de Castro, L.M.Martins, and R.Tufi (2010).
Mitochondrial quality control and neurological disease: an emerging connection.
  Expert Rev Mol Med, 12, e12.  
20127688 J.A.Opsahl, L.V.Hjørnevik, V.H.Bull, L.Fismen, A.K.Frøyset, D.Gromyko, T.Solstad, and K.E.Fladmark (2010).
Increased interaction between DJ-1 and the Mi-2/ nucleosome remodelling and deacetylase complex during cellular stress.
  Proteomics, 10, 1494-1504.  
20304780 J.Chen, L.Li, and L.S.Chin (2010).
Parkinson disease protein DJ-1 converts from a zymogen to a protease by carboxyl-terminal cleavage.
  Hum Mol Genet, 19, 2395-2408.  
20955928 J.Hardy (2010).
Genetic analysis of pathways to Parkinson disease.
  Neuron, 68, 201-206.  
21068725 J.N.Guzman, J.Sanchez-Padilla, D.Wokosin, J.Kondapalli, E.Ilijic, P.T.Schumacker, and D.J.Surmeier (2010).
Oxidant stress evoked by pacemaking in dopaminergic neurons is attenuated by DJ-1.
  Nature, 468, 696-700.  
21091313 K.M.Prakash, and E.K.Tan (2010).
Development of Parkinson's disease biomarkers.
  Expert Rev Neurother, 10, 1811-1825.  
20817635 L.F.Burbulla, C.Schelling, H.Kato, D.Rapaport, D.Woitalla, C.Schiesling, C.Schulte, M.Sharma, T.Illig, P.Bauer, S.Jung, A.Nordheim, L.Schöls, O.Riess, and R.Krüger (2010).
Dissecting the role of the mitochondrial chaperone mortalin in Parkinson's disease: functional impact of disease-related variants on mitochondrial homeostasis.
  Hum Mol Genet, 19, 4437-4452.  
20735469 L.F.Burbulla, G.Krebiehl, and R.Krüger (2010).
Balance is the challenge--the impact of mitochondrial dynamics in Parkinson's disease.
  Eur J Clin Invest, 40, 1048-1060.  
20377612 L.Gan, D.A.Johnson, and J.A.Johnson (2010).
Keap1-Nrf2 activation in the presence and absence of DJ-1.
  Eur J Neurosci, 31, 967-977.  
21258649 L.J.Martin (2010).
Mitochondrial and Cell Death Mechanisms in Neurodegenerative Diseases.
  Pharmaceuticals (Basel), 3, 839-915.  
20938043 L.M.Bekris, I.F.Mata, and C.P.Zabetian (2010).
The genetics of Parkinson disease.
  J Geriatr Psychiatry Neurol, 23, 228-242.  
20457924 L.Y.Hao, B.I.Giasson, and N.M.Bonini (2010).
DJ-1 is critical for mitochondrial function and rescues PINK1 loss of function.
  Proc Natl Acad Sci U S A, 107, 9747-9752.  
20172943 M.Natale, D.Bonino, P.Consoli, T.Alberio, R.G.Ravid, M.Fasano, and E.M.Bucci (2010).
A meta-analysis of two-dimensional electrophoresis pattern of the Parkinson's disease-related protein DJ-1.
  Bioinformatics, 26, 946-952.  
20811350 M.P.Horowitz, and J.T.Greenamyre (2010).
Gene-environment interactions in Parkinson's disease: the importance of animal modeling.
  Clin Pharmacol Ther, 88, 467-474.  
20421364 M.R.Cookson, and O.Bandmann (2010).
Parkinson's disease: insights from pathways.
  Hum Mol Genet, 19, R21-R27.  
20187230 M.R.Cookson (2010).
DJ-1, PINK1, and their effects on mitochondrial pathways.
  Mov Disord, 25, S44-S48.  
20696314 M.R.Cookson (2010).
Unravelling the role of defective genes.
  Prog Brain Res, 183, 43-57.  
19925845 M.Westerlund, B.Hoffer, and L.Olson (2010).
Parkinson's disease: Exit toxins, enter genetics.
  Prog Neurobiol, 90, 146-156.  
20698836 N.Chang, L.Li, R.Hu, Y.Shan, B.Liu, L.Li, H.Wang, H.Feng, D.Wang, C.Cheung, M.Liao, and Q.Wan (2010).
Differential regulation of NMDA receptor function by DJ-1 and PINK1.
  Aging Cell, 9, 837-850.  
20036196 R.Bandopadhyay, and J.de Belleroche (2010).
Pathogenesis of Parkinson's disease: emerging role of molecular chaperones.
  Trends Mol Med, 16, 27-36.  
20938049 S.Ishikawa, T.Taira, K.Takahashi-Niki, T.Niki, H.Ariga, and S.M.Iguchi-Ariga (2010).
Human DJ-1-specific transcriptional activation of tyrosine hydroxylase gene.
  J Biol Chem, 285, 39718-39731.  
19951033 S.Rosales-Corral, R.J.Reiter, D.X.Tan, G.G.Ortiz, and G.Lopez-Armas (2010).
Functional aspects of redox control during neuroinflammation.
  Antioxid Redox Signal, 13, 193-247.  
20221655 S.Surendran, and S.Rajasankar (2010).
Parkinson's disease: oxidative stress and therapeutic approaches.
  Neurol Sci, 31, 531-540.  
20202083 S.Yamashita, A.Mori, E.Kimura, S.Mita, Y.Maeda, T.Hirano, and M.Uchino (2010).
DJ-1 forms complexes with mutant SOD1 and ameliorates its toxicity.
  J Neurochem, 113, 860-870.  
20836127 T.Alberio, M.Colapinto, M.Natale, R.Ravizza, M.B.Gariboldi, E.M.Bucci, L.Lopiano, and M.Fasano (2010).
Changes in the two-dimensional electrophoresis pattern of the Parkinson's disease related protein DJ-1 in human SH-SY5Y neuroblastoma cells after dopamine treatment.
  IUBMB Life, 62, 688-692.  
20014057 T.Inoue, M.Kitagawa, T.Tanaka, S.Nakagawa, and T.Koyama (2010).
Depression and major depressive disorder in patients with Parkinson's disease.
  Mov Disord, 25, 44-49.  
20014998 U.Saeed, A.Ray, R.K.Valli, A.M.Kumar, and V.Ravindranath (2010).
DJ-1 loss by glutaredoxin but not glutathione depletion triggers Daxx translocation and cell death.
  Antioxid Redox Signal, 13, 127-144.  
20053987 V.Loeb, E.Yakunin, A.Saada, and R.Sharon (2010).
The transgenic overexpression of alpha-synuclein and not its related pathology associates with complex I inhibition.
  J Biol Chem, 285, 7334-7343.  
20580911 V.Ruipérez, F.Darios, and B.Davletov (2010).
Alpha-synuclein, lipids and Parkinson's disease.
  Prog Lipid Res, 49, 420-428.  
19492085 A.Sandebring, K.J.Thomas, A.Beilina, M.van der Brug, M.M.Cleland, R.Ahmad, D.W.Miller, I.Zambrano, R.F.Cowburn, H.Behbahani, A.Cedazo-Mínguez, and M.R.Cookson (2009).
Mitochondrial alterations in PINK1 deficient cells are influenced by calcineurin-dependent dephosphorylation of dynamin-related protein 1.
  PLoS One, 4, e5701.  
18842101 B.Lu, and H.Vogel (2009).
Drosophila models of neurodegenerative diseases.
  Annu Rev Pathol, 4, 315-342.  
19416715 D.J.Fernandez-Ayala, A.Sanz, S.Vartiainen, K.K.Kemppainen, M.Babusiak, E.Mustalahti, R.Costa, T.Tuomela, M.Zeviani, J.Chung, K.M.O'Dell, P.Rustin, and H.T.Jacobs (2009).
Expression of the Ciona intestinalis alternative oxidase (AOX) in Drosophila complements defects in mitochondrial oxidative phosphorylation.
  Cell Metab, 9, 449-460.  
18715146 D.W.Ethell, and Q.Fei (2009).
Parkinson-linked genes and toxins that affect neuronal cell death through the Bcl-2 family.
  Antioxid Redox Signal, 11, 529-540.  
18711745 E.Junn, W.H.Jang, X.Zhao, B.S.Jeong, and M.M.Mouradian (2009).
Mitochondrial localization of DJ-1 leads to enhanced neuroprotection.
  J Neurosci Res, 87, 123-129.  
19564950 E.Seppet, M.Gruno, A.Peetsalu, Z.Gizatullina, H.P.Nguyen, S.Vielhaber, M.H.Wussling, S.Trumbeckaite, O.Arandarcikaite, D.Jerzembeck, M.Sonnabend, K.Jegorov, S.Zierz, F.Striggow, and F.N.Gellerich (2009).
Mitochondria and energetic depression in cell pathophysiology.
  Int J Mol Sci, 10, 2252-2303.  
19306499 H.Li, and M.Guo (2009).
Protein degradation in Parkinson disease revisited: it's complex.
  J Clin Invest, 119, 442-445.  
19124468 J.Blackinton, M.Lakshminarasimhan, K.J.Thomas, R.Ahmad, E.Greggio, A.S.Raza, M.R.Cookson, and M.A.Wilson (2009).
Formation of a stabilized cysteine sulfinic acid is critical for the mitochondrial function of the parkinsonism protein DJ-1.
  J Biol Chem, 284, 6476-6485.
PDB codes: 3ezg 3f71
19146923 J.Blackinton, R.Kumaran, M.P.van der Brug, R.Ahmad, L.Olson, D.Galter, A.Lees, R.Bandopadhyay, and M.R.Cookson (2009).
Post-transcriptional regulation of mRNA associated with DJ-1 in sporadic Parkinson disease.
  Neurosci Lett, 452, 8.  
19815446 J.E.Ahlskog (2009).
Parkin and PINK1 parkinsonism may represent nigral mitochondrial cytopathies distinct from Lewy body Parkinson's disease.
  Parkinsonism Relat Disord, 15, 721-727.  
19243238 J.M.Tan, E.S.Wong, and K.L.Lim (2009).
Protein misfolding and aggregation in Parkinson's disease.
  Antioxid Redox Signal, 11, 2119-2134.  
19293155 J.Waak, S.S.Weber, K.Görner, C.Schall, H.Ichijo, T.Stehle, and P.J.Kahle (2009).
Oxidizable residues mediating protein stability and cytoprotective interaction of DJ-1 with apoptosis signal-regulating kinase 1.
  J Biol Chem, 284, 14245-14257.  
19448646 J.Wang, J.Wang, H.R.Zhang, H.J.Shi, D.Ma, H.X.Zhao, B.Lin, and R.S.Li (2009).
Proteomic analysis of seminal plasma from asthenozoospermia patients reveals proteins that affect oxidative stress responses and semen quality.
  Asian J Androl, 11, 484-491.  
19500376 K.A.Malkus, E.Tsika, and H.Ischiropoulos (2009).
Oxidative modifications, mitochondrial dysfunction, and impaired protein degradation in Parkinson's disease: how neurons are lost in the Bermuda triangle.
  Mol Neurodegener, 4, 24.  
19657588 L.De Mena, E.Coto, E.Sánchez-Ferrero, R.Ribacoba, L.M.Guisasola, C.Salvador, M.Blázquez, and V.Alvarez (2009).
Mutational screening of the mortalin gene (HSPA9) in Parkinson's disease.
  J Neural Transm, 116, 1289-1293.  
19029246 L.M.Griffiths, D.Swartzlander, K.L.Meadows, K.D.Wilkinson, A.H.Corbett, and P.W.Doetsch (2009).
Dynamic compartmentalization of base excision repair proteins in response to nuclear and mitochondrial oxidative stress.
  Mol Cell Biol, 29, 794-807.  
19203217 L.Yang, K.Zhao, N.Y.Calingasan, G.Luo, H.H.Szeto, and M.F.Beal (2009).
Mitochondria targeted peptides protect against 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine neurotoxicity.
  Antioxid Redox Signal, 11, 2095-2104.  
19904590 M.Marella, B.B.Seo, T.Yagi, and A.Matsuno-Yagi (2009).
Parkinson's disease and mitochondrial complex I: a perspective on the Ndi1 therapy.
  J Bioenerg Biomembr, 41, 493-497.  
19142195 M.Sakurai, T.Kawamura, H.Nishimura, H.Suzuki, F.Tezuka, and K.Abe (2009).
Induction of Parkinson disease-related proteins in motor neurons after transient spinal cord ischemia in rabbits.
  J Cereb Blood Flow Metab, 29, 752-758.  
18712292 N.Lev, D.Ickowicz, Y.Barhum, E.Melamed, and D.Offen (2009).
DJ-1 Changes in G93A-SOD1 Transgenic Mice: Implications for Oxidative Stress in ALS.
  J Mol Neurosci, 38, 94.  
18974921 N.Lev, D.Ickowicz, Y.Barhum, S.Lev, E.Melamed, and D.Offen (2009).
DJ-1 protects against dopamine toxicity.
  J Neural Transm, 116, 151-160.  
19686841 P.J.Kahle, J.Waak, and T.Gasser (2009).
DJ-1 and prevention of oxidative stress in Parkinson's disease and other age-related disorders.
  Free Radic Biol Med, 47, 1354-1361.  
19059336 R.Banerjee, A.A.Starkov, M.F.Beal, and B.Thomas (2009).
Mitochondrial dysfunction in the limelight of Parkinson's disease pathogenesis.
  Biochim Biophys Acta, 1792, 651-663.  
19425177 R.González-Polo, M.Niso-Santano, J.M.Morán, M.A.Ortiz-Ortiz, J.M.Bravo-San Pedro, G.Soler, and J.M.Fuentes (2009).
Silencing DJ-1 reveals its contribution in paraquat-induced autophagy.
  J Neurochem, 109, 889-898.  
19825160 R.J.Keyser, L.van der Merwe, M.Venter, C.Kinnear, L.Warnich, J.Carr, and S.Bardien (2009).
Identification of a novel functional deletion variant in the 5'-UTR of the DJ-1 gene.
  BMC Med Genet, 10, 105.  
19703902 S.Ishikawa, T.Taira, T.Niki, K.Takahashi-Niki, C.Maita, H.Maita, H.Ariga, and S.M.Iguchi-Ariga (2009).
Oxidative status of DJ-1-dependent activation of dopamine synthesis through interaction of tyrosine hydroxylase and 4-dihydroxy-L-phenylalanine (L-DOPA) decarboxylase with DJ-1.
  J Biol Chem, 284, 28832-28844.  
18647263 S.J.Mullett, R.L.Hamilton, and D.A.Hinkle (2009).
DJ-1 immunoreactivity in human brain astrocytes is dependent on infarct presence and infarct age.
  Neuropathology, 29, 125-131.  
19144925 S.Vasseur, S.Afzal, J.Tardivel-Lacombe, D.S.Park, J.L.Iovanna, and T.W.Mak (2009).
DJ-1/PARK7 is an important mediator of hypoxia-induced cellular responses.
  Proc Natl Acad Sci U S A, 106, 1111-1116.  
19332121 V.S.Van Laar, A.J.Mishizen, M.Cascio, and T.G.Hastings (2009).
Proteomic identification of dopamine-conjugated proteins from isolated rat brain mitochondria and SH-SY5Y cells.
  Neurobiol Dis, 34, 487-500.  
19242547 W.Liu, C.Vives-Bauza, R.Acín-Peréz-, A.Yamamoto, Y.Tan, Y.Li, J.Magrané, M.A.Stavarache, S.Shaffer, S.Chang, M.G.Kaplitt, X.Y.Huang, M.F.Beal, G.Manfredi, and C.Li (2009).
PINK1 defect causes mitochondrial dysfunction, proteasomal deficit and alpha-synuclein aggregation in cell culture models of Parkinson's disease.
  PLoS ONE, 4, e4597.  
19680148 Y.Yang, and B.Lu (2009).
Mitochondrial morphogenesis, distribution, and Parkinson disease: insights from PINK1.
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19422283 Z.Yao, and N.W.Wood (2009).
Cell death pathways in Parkinson's disease: role of mitochondria.
  Antioxid Redox Signal, 11, 2135-2149.  
18570440 A.C.Witt, M.Lakshminarasimhan, B.C.Remington, S.Hasim, E.Pozharski, and M.A.Wilson (2008).
Cysteine pKa depression by a protonated glutamic acid in human DJ-1.
  Biochemistry, 47, 7430-7440.
PDB codes: 2or3 3cy6 3cyf 3cz9 3cza
18093566 A.H.Schapira (2008).
Mitochondria in the aetiology and pathogenesis of Parkinson's disease.
  Lancet Neurol, 7, 97.  
18512864 A.K.Reeve, K.J.Krishnan, and D.M.Turnbull (2008).
Age related mitochondrial degenerative disorders in humans.
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18560593 A.Wood-Kaczmar, S.Gandhi, Z.Yao, A.S.Abramov, E.A.Miljan, G.Keen, L.Stanyer, I.Hargreaves, K.Klupsch, E.Deas, J.Downward, L.Mansfield, P.Jat, J.Taylor, S.Heales, M.R.Duchen, D.Latchman, S.J.Tabrizi, and N.W.Wood (2008).
PINK1 is necessary for long term survival and mitochondrial function in human dopaminergic neurons.
  PLoS ONE, 3, e2455.  
18707151 C.Fu, J.Hu, T.Liu, T.Ago, J.Sadoshima, and H.Li (2008).
Quantitative analysis of redox-sensitive proteome with DIGE and ICAT.
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18978800 C.Henchcliffe, and M.F.Beal (2008).
Mitochondrial biology and oxidative stress in Parkinson disease pathogenesis.
  Nat Clin Pract Neurol, 4, 600-609.  
18162323 C.Maita, S.Tsuji, I.Yabe, S.Hamada, A.Ogata, H.Maita, S.M.Iguchi-Ariga, H.Sasaki, and H.Ariga (2008).
Secretion of DJ-1 into the serum of patients with Parkinson's disease.
  Neurosci Lett, 431, 86-89.  
18822273 C.P.Ramsey, and B.I.Giasson (2008).
The E163K DJ-1 mutant shows specific antioxidant deficiency.
  Brain Res, 1239, 1.  
18447897 C.Schiesling, N.Kieper, K.Seidel, and R.Krüger (2008).
Review: Familial Parkinson's disease--genetics, clinical phenotype and neuropathology in relation to the common sporadic form of the disease.
  Neuropathol Appl Neurobiol, 34, 255-271.  
18601999 C.Terracciano, A.Nogalska, W.K.Engel, S.Wojcik, and V.Askanas (2008).
In inclusion-body myositis muscle fibers Parkinson-associated DJ-1 is increased and oxidized.
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19076434 C.Zhou, Y.Huang, and S.Przedborski (2008).
Oxidative stress in Parkinson's disease: a mechanism of pathogenic and therapeutic significance.
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17882163 D.Yanagisawa, Y.Kitamura, M.Inden, K.Takata, T.Taniguchi, S.Morikawa, M.Morita, T.Inubushi, I.Tooyama, T.Taira, S.M.Iguchi-Ariga, A.Akaike, and H.Ariga (2008).
DJ-1 protects against neurodegeneration caused by focal cerebral ischemia and reperfusion in rats.
  J Cereb Blood Flow Metab, 28, 563-578.  
18368143 G.B.Irvine, O.M.El-Agnaf, G.M.Shankar, and D.M.Walsh (2008).
Protein aggregation in the brain: the molecular basis for Alzheimer's and Parkinson's diseases.
  Mol Med, 14, 451-464.  
18436956 G.Malgieri, and D.Eliezer (2008).
Structural effects of Parkinson's disease linked DJ-1 mutations.
  Protein Sci, 17, 855-868.  
  18700027 H.L.Huang, C.M.Cendan, C.Roza, K.Okuse, R.Cramer, J.F.Timms, and J.N.Wood (2008).
Proteomic profiling of neuromas reveals alterations in protein composition and local protein synthesis in hyper-excitable nerves.
  Mol Pain, 4, 33.  
18286226 H.Latsoudis, C.Spanaki, G.Chlouverakis, and A.Plaitakis (2008).
Mitochondrial DNA polymorphisms and haplogroups in Parkinson's disease and control individuals with a similar genetic background.
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18687903 H.Plun-Favreau, and J.Hardy (2008).
PINK1 in mitochondrial function.
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19021753 J.C.Fitzgerald, and H.Plun-Favreau (2008).
Emerging pathways in genetic Parkinson's disease: autosomal-recessive genes in Parkinson's disease--a common pathway?
  FEBS J, 275, 5758-5766.  
18309325 J.S.Mo, M.Y.Kim, E.J.Ann, J.A.Hong, and H.S.Park (2008).
DJ-1 modulates UV-induced oxidative stress signaling through the suppression of MEKK1 and cell death.
  Cell Death Differ, 15, 1030-1041.  
18245082 L.Devi, V.Raghavendran, B.M.Prabhu, N.G.Avadhani, and H.K.Anandatheerthavarada (2008).
Mitochondrial import and accumulation of alpha-synuclein impair complex I in human dopaminergic neuronal cultures and Parkinson disease brain.
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18181649 M.Lakshminarasimhan, M.T.Maldonado, W.Zhou, A.L.Fink, and M.A.Wilson (2008).
Structural impact of three Parkinsonism-associated missense mutations on human DJ-1.
  Biochemistry, 47, 1381-1392.
PDB codes: 2rk3 2rk4 2rk6 3b36 3b38 3b3a
19081372 M.P.Mattson, M.Gleichmann, and A.Cheng (2008).
Mitochondria in neuroplasticity and neurological disorders.
  Neuron, 60, 748-766.  
18626009 M.P.van der Brug, J.Blackinton, J.Chandran, L.Y.Hao, A.Lal, K.Mazan-Mamczarz, J.Martindale, C.Xie, R.Ahmad, K.J.Thomas, A.Beilina, J.R.Gibbs, J.Ding, A.J.Myers, M.Zhan, H.Cai, N.M.Bonini, M.Gorospe, and M.R.Cookson (2008).
RNA binding activity of the recessive parkinsonism protein DJ-1 supports involvement in multiple cellular pathways.
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18680555 M.Vila, D.Ramonet, and C.Perier (2008).
Mitochondrial alterations in Parkinson's disease: new clues.
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18440504 N.B.Cole, D.Dieuliis, P.Leo, D.C.Mitchell, and R.L.Nussbaum (2008).
Mitochondrial translocation of alpha-synuclein is promoted by intracellular acidification.
  Exp Cell Res, 314, 2076-2089.  
18566920 P.H.Reddy (2008).
Mitochondrial medicine for aging and neurodegenerative diseases.
  Neuromolecular Med, 10, 291-315.  
17996022 R.E.Burke (2008).
Programmed cell death and new discoveries in the genetics of parkinsonism.
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18787878 S.Biskup, M.Gerlach, A.Kupsch, H.Reichmann, P.Riederer, P.Vieregge, U.Wüllner, and T.Gasser (2008).
Genes associated with Parkinson syndrome.
  J Neurol, 255, 8.  
18333761 S.DiMauro, and E.A.Schon (2008).
Mitochondrial disorders in the nervous system.
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18395015 S.R.Danielson, and J.K.Andersen (2008).
Oxidative and nitrative protein modifications in Parkinson's disease.
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18158863 S.Rosner, N.Giladi, and A.Orr-Urtreger (2008).
Advances in the genetics of Parkinson's disease.
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18922803 S.S.Cha, H.I.Jung, H.Jeon, Y.J.An, I.K.Kim, S.Yun, H.J.Ahn, K.C.Chung, S.H.Lee, P.G.Suh, and S.O.Kang (2008).
Crystal structure of filamentous aggregates of human DJ-1 formed in an inorganic phosphate-dependent manner.
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PDB code: 3bwe
17680806 V.Bogaerts, J.Theuns, and C.van Broeckhoven (2008).
Genetic findings in Parkinson's disease and translation into treatment: a leading role for mitochondria?
  Genes Brain Behav, 7, 129-151.  
18426756 Y.Mizuno, N.Hattori, S.Kubo, S.Sato, K.Nishioka, T.Hatano, H.Tomiyama, M.Funayama, Y.Machida, and H.Mochizuki (2008).
Progress in the pathogenesis and genetics of Parkinson's disease.
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  17449461 A.B.Manning-Bog, and J.W.Langston (2007).
Model fusion, the next phase in developing animal models for Parkinson's disease.
  Neurotox Res, 11, 219-240.  
17537576 A.C.Belin, B.F.Björk, M.Westerlund, D.Galter, O.Sydow, C.Lind, K.Pernold, L.Rosvall, A.Håkansson, B.Winblad, H.Nissbrandt, C.Graff, and L.Olson (2007).
Association study of two genetic variants in mitochondrial transcription factor A (TFAM) in Alzheimer's and Parkinson's disease.
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17620882 C.Klein, and K.Lohmann-Hedrich (2007).
Impact of recent genetic findings in Parkinson's disease.
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17610816 D.Qu, J.Rashidian, M.P.Mount, H.Aleyasin, M.Parsanejad, A.Lira, E.Haque, Y.Zhang, S.Callaghan, M.Daigle, M.W.Rousseaux, R.S.Slack, P.R.Albert, I.Vincent, J.M.Woulfe, and D.S.Park (2007).
Role of Cdk5-mediated phosphorylation of Prx2 in MPTP toxicity and Parkinson's disease.
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17766438 E.Andres-Mateos, C.Perier, L.Zhang, B.Blanchard-Fillion, T.M.Greco, B.Thomas, H.S.Ko, M.Sasaki, H.Ischiropoulos, S.Przedborski, T.M.Dawson, and V.L.Dawson (2007).
DJ-1 gene deletion reveals that DJ-1 is an atypical peroxiredoxin-like peroxidase.
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18067416 E.Greggio, and A.Singleton (2007).
Kinase signaling pathways as potential targets in the treatment of Parkinson's disease.
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17385668 E.K.Tan, and L.M.Skipper (2007).
Pathogenic mutations in Parkinson disease.
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18003894 H.Aleyasin, M.W.Rousseaux, M.Phillips, R.H.Kim, R.J.Bland, S.Callaghan, R.S.Slack, M.J.During, T.W.Mak, and D.S.Park (2007).
The Parkinson's disease gene DJ-1 is also a key regulator of stroke-induced damage.
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17465880 H.Q.Wang, and R.Takahashi (2007).
Expanding insights on the involvement of endoplasmic reticulum stress in Parkinson's disease.
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17535435 H.Yamaguchi, and J.Shen (2007).
Absence of dopaminergic neuronal degeneration and oxidative damage in aged DJ-1-deficient mice.
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Selective enrichment of DJ-1 protein in primate striatal neuronal processes: implications for Parkinson's disease.
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17299411 J.C.Paterna, A.Leng, E.Weber, J.Feldon, and H.Büeler (2007).
DJ-1 and Parkin modulate dopamine-dependent behavior and inhibit MPTP-induced nigral dopamine neuron loss in mice.
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17579517 J.W.Pridgeon, J.A.Olzmann, L.S.Chin, and L.Li (2007).
PINK1 Protects against Oxidative Stress by Phosphorylating Mitochondrial Chaperone TRAP1.
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17652776 K.Beyer (2007).
Mechanistic aspects of Parkinson's disease: alpha-synuclein and the biomembrane.
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17623781 K.Nakamura, and R.H.Edwards (2007).
Physiology versus pathology in Parkinson's disease.
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  17593875 L.J.Martin (2007).
Transgenic mice with human mutant genes causing Parkinson's disease and amyotrophic lateral sclerosis provide common insight into mechanisms of motor neuron selective vulnerability to degeneration.
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17388981 M.C.Shih, A.C.Felicio, C.de Oliveira Godeiro-Junior, P.de Carvalho Aguiar, L.A.de Andrade, H.B.Ferraz, and R.A.Bressan (2007).
Molecular imaging in hereditary forms of parkinsonism.
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18052765 M.Di Napoli, I.M.Shah, and D.A.Stewart (2007).
Molecular pathways and genetic aspects of Parkinson's disease: from bench to bedside.
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Genetics of Parkinson's disease and parkinsonism.
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18615181 N.Inamdar, D.Arulmozhi, A.Tandon, and S.Bodhankar (2007).
Parkinson's disease: genetics and beyond.
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18091429 N.Pankratz, and T.Foroud (2007).
Genetics of Parkinson disease.
  Genet Med, 9, 801-811.  
17680003 S.Przedborski (2007).
Peroxiredoxin-2 links Cdk5 to neurodegeneration.
  Nat Med, 13, 907-909.  
18175395 T.Gasser (2007).
Update on the genetics of Parkinson's disease.
  Mov Disord, 22, S343-S350.  
17257049 Y.Wei, D.Ringe, M.A.Wilson, and M.J.Ondrechen (2007).
Identification of functional subclasses in the DJ-1 superfamily proteins.
  PLoS Comput Biol, 3, e10.  
16836655 A.Abeliovich, and M.Flint Beal (2006).
Parkinsonism genes: culprits and clues.
  J Neurochem, 99, 1062-1072.  
16390825 A.Sekito, S.Koide-Yoshida, T.Niki, T.Taira, S.M.Iguchi-Ariga, and H.Ariga (2006).
DJ-1 interacts with HIPK1 and affects H2O2-induced cell death.
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16932540 C.Klein, and M.G.Schlossmacher (2006).
The genetics of Parkinson disease: Implications for neurological care.
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20396491 C.S.Lee, S.J.Chung, and J.S.Kim (2006).
Syndromic approach to Parkinson's disease: role of functional imaging.
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16815218 D.A.Butterfield, H.M.Abdul, S.Newman, and T.Reed (2006).
Redox proteomics in some age-related neurodegenerative disorders or models thereof.
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16796586 D.Gosal, O.A.Ross, and M.Toft (2006).
Parkinson's disease: the genetics of a heterogeneous disorder.
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16418269 G.Hao, B.Derakhshan, L.Shi, F.Campagne, and S.S.Gross (2006).
SNOSID, a proteomic method for identification of cysteine S-nitrosylation sites in complex protein mixtures.
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16517609 J.Choi, M.C.Sullards, J.A.Olzmann, H.D.Rees, S.T.Weintraub, D.E.Bostwick, M.Gearing, A.I.Levey, L.S.Chin, and L.Li (2006).
Oxidative damage of DJ-1 is linked to sporadic Parkinson and Alzheimer diseases.
  J Biol Chem, 281, 10816-10824.  
17068789 J.Hardy, H.Cai, M.R.Cookson, K.Gwinn-Hardy, and A.Singleton (2006).
Genetics of Parkinson's disease and parkinsonism.
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16805775 J.Q.Kwong, M.F.Beal, and G.Manfredi (2006).
The role of mitochondria in inherited neurodegenerative diseases.
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17146283 L.J.Martin (2006).
Mitochondriopathy in Parkinson disease and amyotrophic lateral sclerosis.
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16894167 M.C.Meulener, K.Xu, L.Thomson, L.Thompson, H.Ischiropoulos, and N.M.Bonini (2006).
Mutational analysis of DJ-1 in Drosophila implicates functional inactivation by oxidative damage and aging.
  Proc Natl Acad Sci U S A, 103, 12517-12522.  
16570276 M.Pardo, A.García, B.Thomas, A.Piñeiro, A.Akoulitchev, R.A.Dwek, and N.Zitzmann (2006).
The characterization of the invasion phenotype of uveal melanoma tumour cells shows the presence of MUC18 and HMG-1 metastasis markers and leads to the identification of DJ-1 as a potential serum biomarker.
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17051205 M.T.Lin, and M.F.Beal (2006).
Mitochondrial dysfunction and oxidative stress in neurodegenerative diseases.
  Nature, 443, 787-795.  
17034344 N.Lev, D.Ickowicz, Y.Barhum, N.Blondheim, E.Melamed, and D.Offen (2006).
Experimental encephalomyelitis induces changes in DJ-1: implications for oxidative stress in multiple sclerosis.
  Antioxid Redox Signal, 8, 1987-1995.  
16978147 O.A.Buneeva, and A.E.Medvedev (2006).
Ubiquitin-protein ligase parkin and its role in the development of Parkinson's disease.
  Biochemistry (Mosc), 71, 851-860.  
16495942 P.M.Abou-Sleiman, M.M.Muqit, and N.W.Wood (2006).
Expanding insights of mitochondrial dysfunction in Parkinson's disease.
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16998822 S.Biskup, and D.J.Moore (2006).
Detrimental deletions: mitochondria, aging and Parkinson's disease.
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16615060 S.Kubo, N.Hattori, and Y.Mizuno (2006).
Recessive Parkinson's disease.
  Mov Disord, 21, 885-893.  
17038803 T.Yanagida, K.Takata, M.Inden, Y.Kitamura, T.Taniguchi, K.Yoshimoto, T.Taira, and H.Ariga (2006).
Distribution of DJ-1, Parkinson's disease-related protein PARK7, and its alteration in 6-hydroxydopamine-treated hemiparkinsonian rat brain.
  J Pharmacol Sci, 102, 243-247.  
15976810 Y.Shinbo, T.Niki, T.Taira, H.Ooe, K.Takahashi-Niki, C.Maita, C.Seino, S.M.Iguchi-Ariga, and H.Ariga (2006).
Proper SUMO-1 conjugation is essential to DJ-1 to exert its full activities.
  Cell Death Differ, 13, 96.  
15824318 A.Beilina, M.Van Der Brug, R.Ahmad, S.Kesavapany, D.W.Miller, G.A.Petsko, and M.R.Cookson (2005).
Mutations in PTEN-induced putative kinase 1 associated with recessive parkinsonism have differential effects on protein stability.
  Proc Natl Acad Sci U S A, 102, 5703-5708.  
15743667 B.W.Gibson (2005).
The human mitochondrial proteome: oxidative stress, protein modifications and oxidative phosphorylation.
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16275903 C.Li, and M.F.Beal (2005).
Leucine-rich repeat kinase 2: a new player with a familiar theme for Parkinson's disease pathogenesis.
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16022590 D.J.Moore, A.B.West, V.L.Dawson, and T.M.Dawson (2005).
Molecular pathophysiology of Parkinson's disease.
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15983381 E.Junn, H.Taniguchi, B.S.Jeong, X.Zhao, H.Ichijo, and M.M.Mouradian (2005).
Interaction of DJ-1 with Daxx inhibits apoptosis signal-regulating kinase 1 activity and cell death.
  Proc Natl Acad Sci U S A, 102, 9691-9696.  
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Roles of Drosophila DJ-1 in survival of dopaminergic neurons and oxidative stress.
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15755676 H.F.Poon, M.Frasier, N.Shreve, V.Calabrese, B.Wolozin, and D.A.Butterfield (2005).
Mitochondrial associated metabolic proteins are selectively oxidized in A30P alpha-synuclein transgenic mice--a model of familial Parkinson's disease.
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Association of DJ-1 with chaperones and enhanced association and colocalization with mitochondrial Hsp70 by oxidative stress.
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DJ-1 is present in a large molecular complex in human brain tissue and interacts with alpha-synuclein.
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Mitochondria take center stage in aging and neurodegeneration.
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16227987 M.J.LaVoie, B.L.Ostaszewski, A.Weihofen, M.G.Schlossmacher, and D.J.Selkoe (2005).
Dopamine covalently modifies and functionally inactivates parkin.
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16139213 M.Meulener, A.J.Whitworth, C.E.Armstrong-Gold, P.Rizzu, P.Heutink, P.D.Wes, L.J.Pallanck, and N.M.Bonini (2005).
Drosophila DJ-1 mutants are selectively sensitive to environmental toxins associated with Parkinson's disease.
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The biochemistry of Parkinson's disease.
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DJ-1, a novel regulator of the tumor suppressor PTEN.
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Genetics of Parkinson's disease.
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16155123 Y.Yang, S.Gehrke, M.E.Haque, Y.Imai, J.Kosek, L.Yang, M.F.Beal, I.Nishimura, K.Wakamatsu, S.Ito, R.Takahashi, and B.Lu (2005).
Inactivation of Drosophila DJ-1 leads to impairments of oxidative stress response and phosphatidylinositol 3-kinase/Akt signaling.
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PINK, PANK, or PARK? A clinicians' guide to familial parkinsonism.
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How many pathways are there to nigral death?
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Mitochondria and dopamine: new insights into recessive parkinsonism.
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15547643 M.R.Cookson (2004).
Molecules that cause or prevent Parkinson's disease.
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15502874 S.Shendelman, A.Jonason, C.Martinat, T.Leete, and A.Abeliovich (2004).
DJ-1 is a redox-dependent molecular chaperone that inhibits alpha-synuclein aggregate formation.
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The most recent references are shown first. Citation data come partly from CiteXplore and partly from an automated harvesting procedure. Note that this is likely to be only a partial list as not all journals are covered by either method. However, we are continually building up the citation data so more and more references will be included with time. Where a reference describes a PDB structure, the PDB codes are shown on the right.