PARK7

PARK7
Available structures PDB Ortholog search: PDBe RCSB List of PDB id codes 1J42, 1P5F, 1PDV, 1PDW, 1PE0, 1Q2U, 1SOA, 1UCF, 2OR3, 2R1T, 2R1U, 2R1V, 2RK3, 2RK4, 2RK6, 3B36, 3B38, 3B3A, 3BWE, 3CY6, 3CYF, 3CZ9, 3CZA, 3EZG, 3F71, 3SF8, 4BTE, 4MNT, 4MTC, 4N0M, 4N12, 4OGF, 4OQ4, 4P2G, 4P34, 4P35, 4P36, 4ZGG, 4RKW, 4RKY, 4S0Z
Identifiers
Aliases	PARK7, DJ-1, DJ1, HEL-S-67p, Parkinsonism associated deglycase
External IDs	MGI: 2135637 HomoloGene: 38295 GeneCards: PARK7
Gene ontology Molecular function • receptor binding • repressing transcription factor binding • cytokine binding • core promoter binding • cupric ion binding • transcription factor binding • L-dopa decarboxylase activator activity • peptidase activity • kinase binding • tyrosine 3-monooxygenase activator activity • enzyme binding • mRNA binding • protein homodimerization activity • scaffold protein binding • small protein activating enzyme binding • single-stranded DNA binding • ubiquitin-like protein conjugating enzyme binding • protein binding • peroxiredoxin activity • double-stranded DNA binding • copper ion binding • mercury ion binding • transcription coactivator activity • oxidoreductase activity, acting on peroxide as acceptor • androgen receptor binding • superoxide dismutase copper chaperone activity • RNA binding • identical protein binding • ubiquitin-specific protease binding • glyoxalase (glycolic acid-forming) activity • glyoxalase III activity • hydrolase activity • cuprous ion binding • cadherin binding involved in cell-cell adhesion Cellular component • cytoplasm • cytosol • membrane • mitochondrial intermembrane space • mitochondrion • neuron projection • nucleus • mitochondrial respiratory chain complex I • endoplasmic reticulum • membrane raft • chromatin • extracellular exosome • plasma membrane • axon • mitochondrial matrix • cell body • PML body • presynapse • perinuclear region of cytoplasm • cell-cell adherens junction Biological process • negative regulation of neuron apoptotic process • positive regulation of transcription regulatory region DNA binding • negative regulation of protein sumoylation • negative regulation of cysteine-type endopeptidase activity involved in apoptotic signaling pathway • negative regulation of protein binding • synaptic transmission, dopaminergic • positive regulation of interleukin-8 production • negative regulation of proteasomal ubiquitin-dependent protein catabolic process • Ras protein signal transduction • cellular response to glyoxal • negative regulation of protein acetylation • positive regulation of reactive oxygen species biosynthetic process • positive regulation of oxidative phosphorylation uncoupler activity • positive regulation of protein kinase B signaling • negative regulation of protein catabolic process • adult locomotory behavior • regulation of neuron apoptotic process • negative regulation of extrinsic apoptotic signaling pathway • negative regulation of cell death • glycolate biosynthetic process • response to oxidative stress • positive regulation of protein homodimerization activity • negative regulation of gene expression • single fertilization • positive regulation of peptidyl-serine phosphorylation • positive regulation of mitochondrial electron transport, NADH to ubiquinone • negative regulation of hydrogen peroxide-induced neuron intrinsic apoptotic signaling pathway • negative regulation of oxidative stress-induced cell death • inflammatory response • negative regulation of oxidative stress-induced neuron intrinsic apoptotic signaling pathway • regulation of fibril organization • negative regulation of neuron death • negative regulation of protein phosphorylation • negative regulation of endoplasmic reticulum stress-induced intrinsic apoptotic signaling pathway • negative regulation of protein kinase activity • negative regulation of oxidative stress-induced neuron death • membrane depolarization • protein stabilization • mitochondrion organization • positive regulation of sequence-specific DNA binding transcription factor activity • negative regulation of death-inducing signaling complex assembly • negative regulation of apoptotic process • proteolysis • negative regulation of protein K48-linked deubiquitination • enzyme active site formation via L-cysteine sulfinic acid • dopamine uptake involved in synaptic transmission • positive regulation of tyrosine 3-monooxygenase activity • positive regulation of L-dopa decarboxylase activity • negative regulation of hydrogen peroxide-induced neuron death • regulation of inflammatory response • regulation of androgen receptor signaling pathway • negative regulation of hydrogen peroxide-induced cell death • positive regulation of mitophagy • response to hydrogen peroxide • detoxification of copper ion • positive regulation of superoxide dismutase activity • positive regulation of dopamine biosynthetic process • positive regulation of protein localization to nucleus • glyoxal catabolic process • positive regulation of L-dopa biosynthetic process • activation of protein kinase B activity • membrane hyperpolarization • detoxification of mercury ion • negative regulation of TRAIL-activated apoptotic signaling pathway • cellular oxidant detoxification • negative regulation of ubiquitin-specific protease activity • regulation of TRAIL receptor biosynthetic process • autophagy • cellular response to reactive oxygen species • regulation of mitochondrial membrane potential • negative regulation of protein ubiquitination • hydrogen peroxide metabolic process • methylglyoxal catabolic process to D-lactate via S-lactoyl-glutathione • positive regulation of gene expression • negative regulation of protein export from nucleus • negative regulation of ubiquitin-protein transferase activity • positive regulation of oxidative stress-induced intrinsic apoptotic signaling pathway • cellular response to oxidative stress • positive regulation of androgen receptor activity • positive regulation of pyrroline-5-carboxylate reductase activity • response to drug • positive regulation of transcription from RNA polymerase II promoter • cellular response to hydrogen peroxide • cell-cell adhesion • lactate biosynthetic process • protein deglycosylation • protein repair Sources:Amigo / QuickGO
RNA expression pattern
More reference expression data
Orthologs
Species	Human	Mouse
Entrez	11315	57320
Ensembl	ENSG00000116288	ENSMUSG00000028964
UniProt	Q99497	Q99LX0
RefSeq (mRNA)	NM_001123377 NM_007262	NM_020569
RefSeq (protein)	NP_001116849.1 NP_009193.2	NP_065594.2
Location (UCSC)	Chr 1: 7.95 – 7.99 Mb	Chr 4: 150.9 – 150.91 Mb
PubMed search	[1]	[2]
Wikidata

Protein deglycase DJ-1, also known as Parkinson disease protein 7, is a protein which in humans is encoded by the PARK7 gene.^[3]

Structure

Gene

The gene PARK7, also known as DJ-1, encodes a protein of the peptidase C56 family. The human gene PARK7 has 8 Exons and locates at chromosome band 1p36.23.^[3]

Protein

The human protein deglycase DJ-1 is 20 kDa in size and composed of 189 amino acids with seven β-strands and nine α-helices in total and is present as a dimer.^[4][5][6] It belongs to the peptidase C56 family of proteins.

The protein structures of human protein DJ-1, Escherichia coli chaperone Hsp31 and an Archaea protease are evolutionarily conserved.^[7]

Function

Under an oxidative condition, protein deglycase DJ-1 inhibits the aggregation of α-synuclein via its chaperone activity,^[8][9] thus functions as a redox-sensitive chaperone and as a sensor for oxidative stress. Accordingly, DJ-1 apparently protects neurons against oxidative stress and cell death.^[3] In parallel, Protein DJ-1 acts as a positive regulator of androgen receptor-dependent transcription.

Pyrroloquinoline quinone (PQQ) has been shown to reduce the self-oxidation of the DJ-1 protein, an early step in the onset of some forms of Parkinson's disease.^[10]

Clinical significance

Defects in this gene are the cause of autosomal recessive early-onset Parkinson's disease 7.^[3][11]

Interactions

PARK7 has been shown to interact with:

• CASK,^[12]
• EFCAB6,^[13] and
• PIAS2.^[14]

See also

• Parkinson's disease

References

Further reading

PDB gallery
1j42: Crystal Structure of Human DJ-1  1p5f: Crystal Structure of Human DJ-1  1pdv: Crystal structure of human DJ-1, P 31 2 1 space group  1pdw: Crystal structure of human DJ-1, P 1 21 1 space group  1pe0: Crystal structure of the K130R mutant of human DJ-1  1ps4: crystal structure of DJ-1  1q2u: Crystal structure of DJ-1/RS and implication on familial Parkinson's disease  1soa: Human DJ-1 with sulfinic acid  1ucf: The Crystal Structure of DJ-1, a Protein Related to Male Fertility and Parkinson's Disease  2or3: Pre-oxidation Complex of Human DJ-1

This article incorporates text from the United States National Library of Medicine, which is in the public domain.