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Protein

Protein deglycase DJ-1

Gene

Park7

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Protein deglycase that repairs methylglyoxal- and glyoxal-glycated amino acids and proteins, and releases repaired proteins and lactate or glycolate, respectively. Deglycates cysteines, arginines and lysines residues in proteins, and thus reactivates these proteins by reversing glycation by glyoxals. Acts on early glycation intermediates (hemithioacetals and aminocarbinols), preventing the formation of advanced glycation endproducts (AGE) (By similarity). Plays an important role in cell protection against oxidative stress and cell death acting as oxidative stress sensor and redox-sensitive chaperone and protease; functions probably related to its primary function (PubMed:15784737, PubMed:17015834, PubMed:20800516, PubMed:21068725). It is involved in neuroprotective mechanisms like the stabilization of NFE2L2 and PINK1 proteins, male fertility as a positive regulator of androgen signaling pathway and cell growth and transformation through, for instance, the modulation of NF-kappa-B signaling pathway (PubMed:17015834, PubMed:21097510). Its involvement in protein repair could explain other unrelated functions. Eliminates hydrogen peroxide and protects cells against hydrogen peroxide-induced cell death (PubMed:17766438). Required for correct mitochondrial morphology and function as well as for autophagy of dysfunctional mitochondria (PubMed:20186336). Plays a role in regulating expression or stability of the mitochondrial uncoupling proteins SLC25A14 and SLC25A27 in dopaminergic neurons of the substantia nigra pars compacta and attenuates the oxidative stress induced by calcium entry into the neurons via L-type channels during pacemaking (PubMed:21068725). Regulates astrocyte inflammatory responses, may modulate lipid rafts-dependent endocytosis in astrocytes and neuronal cells (PubMed:23847046, PubMed:19276172). Binds to a number of mRNAs containing multiple copies of GG or CC motifs and partially inhibits their translation but dissociates following oxidative stress (By similarity). Metal-binding protein able to bind copper as well as toxic mercury ions, enhances the cell protection mechanism against induced metal toxicity (PubMed:23792957).By similarity10 Publications

Cofactori

Note: Does not require glutathione as a cofactor, however, glycated glutathione constitutes a PARK7 substrate.By similarity

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Active sitei106NucleophileBy similarity1
Active sitei126By similarity1

GO - Molecular functioni

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Chaperone, Hydrolase, Protease

Keywords - Biological processi

Autophagy, Fertilization, Inflammatory response, Stress response

Keywords - Ligandi

Copper, RNA-binding

Protein family/group databases

MEROPSiC56.002.

Names & Taxonomyi

Protein namesi
Recommended name:
Protein deglycase DJ-1By similarity (EC:3.1.2.-By similarity, EC:3.5.1.-By similarity)
Alternative name(s):
Parkinson disease protein 7 homolog
Gene namesi
Name:Park7Imported
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 4

Organism-specific databases

MGIiMGI:2135637. Park7.

Subcellular locationi

  • Cell membrane 1 Publication; Lipid-anchor 1 Publication
  • Cytoplasm By similarity
  • Membrane raft 1 Publication
  • Nucleus By similarity
  • Mitochondrion By similarity

  • Note: Under normal conditions, located predominantly in the cytoplasm and, to a lesser extent, in the nucleus and mitochondrion. Translocates to the mitochondrion and subsequently to the nucleus in response to oxidative stress and exerts an increased cytoprotective effect against oxidative damage (By similarity). Membrane raft localization in astrocytes and neuronal cells requires palmitoylation (PubMed:23847046).By similarity1 Publication

GO - Cellular componenti

  • cell body Source: ParkinsonsUK-UCL
  • cell-cell adherens junction Source: MGI
  • chromatin Source: MGI
  • cytoplasm Source: UniProtKB
  • cytosol Source: ParkinsonsUK-UCL
  • endoplasmic reticulum Source: MGI
  • extracellular exosome Source: MGI
  • membrane raft Source: UniProtKB-SubCell
  • mitochondrial intermembrane space Source: ParkinsonsUK-UCL
  • mitochondrial matrix Source: ParkinsonsUK-UCL
  • mitochondrial respiratory chain complex I Source: Ensembl
  • mitochondrion Source: ParkinsonsUK-UCL
  • neuron projection Source: ParkinsonsUK-UCL
  • nucleus Source: UniProtKB
  • perinuclear region of cytoplasm Source: MGI
  • plasma membrane Source: UniProtKB-SubCell
  • PML body Source: MGI
  • presynapse Source: GOC
Complete GO annotation...

Keywords - Cellular componenti

Cell membrane, Cytoplasm, Membrane, Mitochondrion, Nucleus

Pathology & Biotechi

Disruption phenotypei

Increased sensitivity of embryonic cortical neurons to oxidative stress. Age-dependent increase in mitochondrial hydrogen peroxide production and reduced mitochondrial aconitase activity. Down-regulation of Slc25a14 and Slc25a27, compromised calcium-induced uncoupling and increased oxidation of mitochondrial matrix proteins specifically in the dopaminergic neurons of the substantia nigra pars compacta. Reduced N2el2 protein expression. Impaired mitochondrial function and morphology with reduced autophagy leading to accumulation of defective mitochondria. Targeted knockouts in astrocytes exhibit augmented LPS-induced CRK/p38 phosphorylation and signaling, they don't stimulate TLR4 endocytosis upon LPS stimulation.6 Publications

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi46C → A: Sulfinic acid detected following treatment with hydrogen peroxide. 1 Publication1
Mutagenesisi53C → A: Sulfinic acid detected following treatment with hydrogen peroxide. 1 Publication1
Mutagenesisi106C → A: No sulfinic acid detected following treatment with hydrogen peroxide. 1 Publication1

Keywords - Diseasei

Tumor suppressor

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
PropeptideiPRO_0000405560? – 189Removed in mature form
Initiator methionineiRemovedBy similarity
ChainiPRO_00001578502 – ?Protein deglycase DJ-1

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei2N-acetylalanineBy similarity1
Lipidationi46S-palmitoyl cysteineBy similarity1
Lipidationi53S-palmitoyl cysteineBy similarity1
Modified residuei67PhosphotyrosineBy similarity1
Modified residuei106Cysteine sulfinic acid (-SO2H); alternateBy similarity1
Lipidationi106S-palmitoyl cysteine; alternateBy similarity1
Cross-linki130Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO)By similarity
Modified residuei148N6-acetyllysineCombined sources1
Modified residuei182N6-succinyllysineCombined sources1

Post-translational modificationi

Sumoylated on Lys-130 by PIAS2 or PIAS4; which is essential for cell-growth promoting activity and transforming activity.By similarity
Undergoes cleavage of a C-terminal peptide and subsequent activation of protease activity in response to oxidative stress.By similarity

Keywords - PTMi

Acetylation, Isopeptide bond, Lipoprotein, Oxidation, Palmitate, Phosphoprotein, Ubl conjugation, Zymogen

Proteomic databases

EPDiQ99LX0.
MaxQBiQ99LX0.
PaxDbiQ99LX0.
PeptideAtlasiQ99LX0.
PRIDEiQ99LX0.
TopDownProteomicsiQ99LX0.

2D gel databases

REPRODUCTION-2DPAGEQ99LX0.
UCD-2DPAGEQ99LX0.

PTM databases

iPTMnetiQ99LX0.
PhosphoSitePlusiQ99LX0.
SwissPalmiQ99LX0.

Expressioni

Tissue specificityi

Expressed in erythroblasts and in mature red blood cells from peripheral blood (at protein level).1 Publication

Developmental stagei

Expression increases during erythroid development (at protein level).1 Publication

Inductioni

By hydrogen peroxide.1 Publication

Gene expression databases

BgeeiENSMUSG00000028964.
ExpressionAtlasiQ99LX0. baseline and differential.
GenevisibleiQ99LX0. MM.

Interactioni

Subunit structurei

Homodimer. Binds EFCAB6/DJBP and PIAS2. Part of a ternary complex containing PARK7, EFCAB6/DJBP and AR. Binds to HIPK1 (By similarity). Interacts (via N-terminus) with OTUD7B (PubMed:21097510). Interacts with BBS1, CLCF1 and MTERF (PubMed:21097510).By similarity1 Publication

GO - Molecular functioni

Protein-protein interaction databases

BioGridi208257. 14 interactors.
IntActiQ99LX0. 8 interactors.
MINTiMINT-1869265.
STRINGi10090.ENSMUSP00000030805.

Structurei

3D structure databases

ProteinModelPortaliQ99LX0.
SMRiQ99LX0.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the peptidase C56 family.Curated

Phylogenomic databases

eggNOGiKOG2764. Eukaryota.
COG0693. LUCA.
GeneTreeiENSGT00390000001231.
HOGENOMiHOG000063194.
HOVERGENiHBG053511.
InParanoidiQ99LX0.
KOiK05687.
OMAiTCFEFAL.
OrthoDBiEOG091G12NS.
PhylomeDBiQ99LX0.
TreeFamiTF300119.

Family and domain databases

Gene3Di3.40.50.880. 1 hit.
InterProiIPR029062. Class_I_gatase-like.
IPR002818. DJ-1/PfpI.
IPR006287. DJ1.
[Graphical view]
PfamiPF01965. DJ-1_PfpI. 1 hit.
[Graphical view]
SUPFAMiSSF52317. SSF52317. 1 hit.
TIGRFAMsiTIGR01383. not_thiJ. 1 hit.

Sequencei

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

Q99LX0-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MASKRALVIL AKGAEEMETV IPVDVMRRAG IKVTVAGLAG KDPVQCSRDV
60 70 80 90 100
MICPDTSLED AKTQGPYDVV VLPGGNLGAQ NLSESPMVKE ILKEQESRKG
110 120 130 140 150
LIAAICAGPT ALLAHEVGFG CKVTTHPLAK DKMMNGSHYS YSESRVEKDG
160 170 180
LILTSRGPGT SFEFALAIVE ALVGKDMANQ VKAPLVLKD
Length:189
Mass (Da):20,021
Last modified:June 1, 2001 - v1
Checksum:i877C825CCA07468F
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti127P → T in BAE40278 (PubMed:16141072).Curated1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AB015652 mRNA. Translation: BAA29063.2.
AK146368 mRNA. Translation: BAE27118.1.
AK153948 mRNA. Translation: BAE32271.1.
AK168341 mRNA. Translation: BAE40278.1.
AL607084 Genomic DNA. Translation: CAM19230.1.
BC002187 mRNA. Translation: AAH02187.1.
CCDSiCCDS18975.1.
RefSeqiNP_065594.2. NM_020569.3.
UniGeneiMm.277349.

Genome annotation databases

EnsembliENSMUST00000030805; ENSMUSP00000030805; ENSMUSG00000028964.
ENSMUST00000105673; ENSMUSP00000101298; ENSMUSG00000028964.
ENSMUST00000105674; ENSMUSP00000101299; ENSMUSG00000028964.
ENSMUST00000105675; ENSMUSP00000101300; ENSMUSG00000028964.
GeneIDi57320.
KEGGimmu:57320.
UCSCiuc008vxz.2. mouse.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AB015652 mRNA. Translation: BAA29063.2.
AK146368 mRNA. Translation: BAE27118.1.
AK153948 mRNA. Translation: BAE32271.1.
AK168341 mRNA. Translation: BAE40278.1.
AL607084 Genomic DNA. Translation: CAM19230.1.
BC002187 mRNA. Translation: AAH02187.1.
CCDSiCCDS18975.1.
RefSeqiNP_065594.2. NM_020569.3.
UniGeneiMm.277349.

3D structure databases

ProteinModelPortaliQ99LX0.
SMRiQ99LX0.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi208257. 14 interactors.
IntActiQ99LX0. 8 interactors.
MINTiMINT-1869265.
STRINGi10090.ENSMUSP00000030805.

Protein family/group databases

MEROPSiC56.002.

PTM databases

iPTMnetiQ99LX0.
PhosphoSitePlusiQ99LX0.
SwissPalmiQ99LX0.

2D gel databases

REPRODUCTION-2DPAGEQ99LX0.
UCD-2DPAGEQ99LX0.

Proteomic databases

EPDiQ99LX0.
MaxQBiQ99LX0.
PaxDbiQ99LX0.
PeptideAtlasiQ99LX0.
PRIDEiQ99LX0.
TopDownProteomicsiQ99LX0.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000030805; ENSMUSP00000030805; ENSMUSG00000028964.
ENSMUST00000105673; ENSMUSP00000101298; ENSMUSG00000028964.
ENSMUST00000105674; ENSMUSP00000101299; ENSMUSG00000028964.
ENSMUST00000105675; ENSMUSP00000101300; ENSMUSG00000028964.
GeneIDi57320.
KEGGimmu:57320.
UCSCiuc008vxz.2. mouse.

Organism-specific databases

CTDi11315.
MGIiMGI:2135637. Park7.

Phylogenomic databases

eggNOGiKOG2764. Eukaryota.
COG0693. LUCA.
GeneTreeiENSGT00390000001231.
HOGENOMiHOG000063194.
HOVERGENiHBG053511.
InParanoidiQ99LX0.
KOiK05687.
OMAiTCFEFAL.
OrthoDBiEOG091G12NS.
PhylomeDBiQ99LX0.
TreeFamiTF300119.

Miscellaneous databases

PROiQ99LX0.
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000028964.
ExpressionAtlasiQ99LX0. baseline and differential.
GenevisibleiQ99LX0. MM.

Family and domain databases

Gene3Di3.40.50.880. 1 hit.
InterProiIPR029062. Class_I_gatase-like.
IPR002818. DJ-1/PfpI.
IPR006287. DJ1.
[Graphical view]
PfamiPF01965. DJ-1_PfpI. 1 hit.
[Graphical view]
SUPFAMiSSF52317. SSF52317. 1 hit.
TIGRFAMsiTIGR01383. not_thiJ. 1 hit.
ProtoNetiSearch...

Entry informationi

Entry nameiPARK7_MOUSE
AccessioniPrimary (citable) accession number: Q99LX0
Secondary accession number(s): O88306, Q3THB9, Q3U509
Entry historyi
Integrated into UniProtKB/Swiss-Prot: December 7, 2004
Last sequence update: June 1, 2001
Last modified: November 30, 2016
This is version 127 of the entry and version 1 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Caution

Glyoxylase activity previously reported reflects in fact its deglycase activity (PubMed:22523093).By similarity1 Publication

Keywords - Technical termi

Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  2. Peptidase families
    Classification of peptidase families and list of entries
  3. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.