Parkinson Disease Protein 7 (PARK7)

DJ1; Autosomal Recessive,Early Onset

SPN	N329
HGNC	16369
MGI	2135637
PubMed	21633165
RGD	621808
UniProt	Q99497
Wiki	PARK7

PARK7 belongs to the peptidase C56 family of proteins. It acts as a positive regulator of androgen receptor-dependent transcription. It may also function as a redox-sensitive chaperone, as a sensor for oxidative stress, and it apparently protects neurons against oxidative stress and cell death.

Northern blot analysis revealed that DJ1 is ubiquitously expressed as a 1.0-kb transcript. Western blot analysis and immunofluorescence showed that the DJ1 protein is present in both nuclei and cytoplasm of HeLa cells.A search of the GenBank protein database revealed that DJ1 has approximately 40% identity to the 198-amino acid protein product of the E. coli thiazole monophosphate biosynthesis (ThiJ) gene. A homolog also exists in the nematode C. elegans.

Reference

"DJ-1, a novel oncogene which transforms mouse NIH3T3 cells in cooperation with ras."
Biochem. Biophys. Res. Commun. 231:509-513(1997) [PubMed] [Europe PMC] [Abstract]

"Complete sequencing and characterization of 21,243 full-length human cDNAs."
Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]

"The DNA sequence and biological annotation of human chromosome 1."
Nature 441:315-321(2006) [PubMed] [Europe PMC] [Abstract]

"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]

"Molecular cloning of human and mouse DJ-1 genes and identification of Sp1-dependent activation of the human DJ-1 promoter."
Gene 263:285-292(2001) [PubMed] [Europe PMC] [Abstract]

, Submitted (DEC-2008) to UniProtKB

"DJ-1 positively regulates the androgen receptor by impairing the binding of PIASx alpha to the receptor."
J. Biol. Chem. 276:37556-37563(2001) [PubMed] [Europe PMC] [Abstract]

"L166P mutant DJ-1, causative for recessive Parkinson's disease, is degraded through the ubiquitin-proteasome system."
J. Biol. Chem. 278:36588-36595(2003) [PubMed] [Europe PMC] [Abstract]

"A missense mutation (L166P) in DJ-1, linked to familial Parkinson's disease, confers reduced protein stability and impairs homo-oligomerization."
J. Neurochem. 87:1558-1567(2003) [PubMed] [Europe PMC] [Abstract]

"DJBP: a novel DJ-1-binding protein, negatively regulates the androgen receptor by recruiting histone deacetylase complex, and DJ-1 antagonizes this inhibition by abrogation of this complex."
Mol. Cancer Res. 1:247-261(2003) [PubMed] [Europe PMC] [Abstract]

"Immunocytochemical localization of DJ-1 in human male reproductive tissue."
Mol. Reprod. Dev. 66:391-397(2003) [PubMed] [Europe PMC] [Abstract]

"DJ-1 colocalizes with tau inclusions: a link between parkinsonism and dementia."
Ann. Neurol. 55:113-118(2004) [PubMed] [Europe PMC] [Abstract]

"The expression of DJ-1 (PARK7) in normal human CNS and idiopathic Parkinson's disease."
Brain 127:420-430(2004) [PubMed] [Europe PMC] [Abstract]

"DJ-1 has a role in antioxidative stress to prevent cell death."
EMBO Rep. 5:213-218(2004) [PubMed] [Europe PMC] [Abstract]

"DJ-1 is a redox-dependent molecular chaperone that inhibits alpha-synuclein aggregate formation."
PLoS Biol. 2:1-10(2004) [PubMed] [Europe PMC] [Abstract]

"Immunoaffinity profiling of tyrosine phosphorylation in cancer cells."
Nat. Biotechnol. 23:94-101(2005) [PubMed] [Europe PMC] [Abstract]

"Proper SUMO-1 conjugation is essential to DJ-1 to exert its full activities."
Cell Death Differ. 13:96-108(2006) [PubMed] [Europe PMC] [Abstract]

"DJ-1 interacts with HIPK1 and affects H2O2-induced cell death."
Free Radic. Res. 40:155-165(2006) [PubMed] [Europe PMC] [Abstract]

"DJ-1, a cancer- and Parkinson's disease-associated protein, stabilizes the antioxidant transcriptional master regulator Nrf2."
Proc. Natl. Acad. Sci. U.S.A. 103:15091-15096(2006) [PubMed] [Europe PMC] [Abstract]

"RNA binding activity of the recessive parkinsonism protein DJ-1 supports involvement in multiple cellular pathways."
Proc. Natl. Acad. Sci. U.S.A. 105:10244-10249(2008) [PubMed] [Europe PMC] [Abstract]

"Parkin, PINK1, and DJ-1 form a ubiquitin E3 ligase complex promoting unfolded protein degradation."
J. Clin. Invest. 119:650-660(2009) [PubMed] [Europe PMC] [Abstract]

"Mitochondrial localization of DJ-1 leads to enhanced neuroprotection."
J. Neurosci. Res. 87:123-129(2009) [PubMed] [Europe PMC] [Abstract]

"Parkinson disease protein DJ-1 converts from a zymogen to a protease by carboxyl-terminal cleavage."
Hum. Mol. Genet. 19:2395-2408(2010) [PubMed] [Europe PMC] [Abstract]

"Initial characterization of the human central proteome."
BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract]

"DJ-1 enhances cell survival through the binding of cezanne, a negative regulator of NF-{kappa}B."
J. Biol. Chem. 286:4098-4106(2011) [PubMed] [Europe PMC] [Abstract]

"Human DJ-1 and its homologs are novel glyoxalases."
Hum. Mol. Genet. 21:3215-3225(2012) [PubMed] [Europe PMC] [Abstract]

"DJ-1 associates with lipid rafts by palmitoylation and regulates lipid rafts-dependent endocytosis in astrocytes."
Hum. Mol. Genet. 22:4805-4817(2013) [PubMed] [Europe PMC] [Abstract]

"Parkinson disease protein DJ-1 binds metals and protects against metal-induced cytotoxicity."
J. Biol. Chem. 288:22809-22820(2013) [PubMed] [Europe PMC] [Abstract]

"Parkinsonism-associated protein DJ-1/Park7 is a major protein deglycase that repairs methylglyoxal- and glyoxal-glycated cysteine, arginine and lysine residues."
J. Biol. Chem. 290:1885-1897(2015) [PubMed] [Europe PMC] [Abstract]

"An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."
J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]

"Crystal structure of DJ-1/RS and implication on familial Parkinson's disease."
FEBS Lett. 549:171-175(2003) [PubMed] [Europe PMC] [Abstract]

"Crystal structure of human DJ-1, a protein associated with early onset Parkinson's disease."
J. Biol. Chem. 278:31372-31379(2003) [PubMed] [Europe PMC] [Abstract]

"The crystal structure of DJ-1, a protein related to male fertility and Parkinson's disease."
J. Biol. Chem. 278:31380-31384(2003) [PubMed] [Europe PMC] [Abstract]

"Crystal structures of human DJ-1 and Escherichia coli Hsp31, which share an evolutionarily conserved domain."
J. Biol. Chem. 278:44552-44559(2003) [PubMed] [Europe PMC] [Abstract]

"The 1.1-A resolution crystal structure of DJ-1, the protein mutated in autosomal recessive early onset Parkinson's disease."
Proc. Natl. Acad. Sci. U.S.A. 100:9256-9261(2003) [PubMed] [Europe PMC] [Abstract]

"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(2004) [PubMed] [Europe PMC] [Abstract]

"The role of pathogenic DJ-1 mutations in Parkinson's disease."
Ann. Neurol. 54:283-286(2003) [PubMed] [Europe PMC] [Abstract]

"Mutations in the DJ-1 gene associated with autosomal recessive early-onset Parkinsonism."
Science 299:256-259(2003) [PubMed] [Europe PMC] [Abstract]

"The R98Q variation in DJ-1 represents a rare polymorphism."
Ann. Neurol. 55:145-146(2004) [PubMed] [Europe PMC] [Abstract]

"Novel homozygous p.E64D mutation in DJ1 in early onset Parkinson disease (PARK7)."
Hum. Mutat. 24:321-329(2004) [PubMed] [Europe PMC] [Abstract]

"Differential effects of Parkinson's disease-associated mutations on stability and folding of DJ-1."
J. Biol. Chem. 279:6943-6951(2004) [PubMed] [Europe PMC] [Abstract]

"Analysis of an early-onset Parkinson's disease cohort for DJ-1 mutations."
Mov. Disord. 19:796-800(2004) [PubMed] [Europe PMC] [Abstract]

"DJ-1 (PARK7) mutations are less frequent than Parkin (PARK2) mutations in early-onset Parkinson disease."
Neurology 62:389-394(2004) [PubMed] [Europe PMC] [Abstract]

"DJ-1 mutations and parkinsonism-dementia-amyotrophic lateral sclerosis complex."
Ann. Neurol. 58:803-807(2005) [PubMed] [Europe PMC] [Abstract]

"Association of PINK1 and DJ-1 confers digenic inheritance of early-onset Parkinson's disease."
Hum. Mol. Genet. 15:1816-1825(2006) [PubMed] [Europe PMC] [Abstract]

"Parkin-mediated K63-linked polyubiquitination targets misfolded DJ-1 to aggresomes via binding to HDAC6."
J. Cell Biol. 178:1025-1038(2007) [PubMed] [Europe PMC] [Abstract]

"Reduced basal autophagy and impaired mitochondrial dynamics due to loss of Parkinson's disease-associated protein DJ-1."
PLoS ONE 5:E9367-E9367(2010) [PubMed] [Europe PMC] [Abstract]