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Protein

DNA repair protein XRCC3

Gene

XRCC3

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Involved in the homologous recombination repair (HRR) pathway of double-stranded DNA, thought to repair chromosomal fragmentation, translocations and deletions. Part of the RAD21 paralog protein complex CX3 which acts in the BRCA1-BRCA2-dependent HR pathway. Upon DNA damage, CX3 acts downstream of RAD51 recruitment; the complex binds predominantly to the intersection of the four duplex arms of the Holliday junction (HJ) and to junctions of replication forks. Involved in HJ resolution and thus in processing HR intermediates late in the DNA repair process; the function may be linked to the CX3 complex and seems to involve GEN1 during mitotic cell cycle progression. Part of a PALB2-scaffolded HR complex containing BRCA2 and RAD51C and which is thought to play a role in DNA repair by HR. Plays a role in regulating mitochondrial DNA copy number under conditions of oxidative stress in the presence of RAD51 and RAD51C.4 Publications

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi107 – 114ATPSequence analysis8

GO - Molecular functioni

GO - Biological processi

  • cellular response to DNA damage stimulus Source: ProtInc
  • DNA recombination Source: ProtInc
  • DNA repair Source: BHF-UCL
  • double-strand break repair via homologous recombination Source: UniProtKB
  • interstrand cross-link repair Source: ParkinsonsUK-UCL
  • meiotic DNA recombinase assembly Source: GO_Central
  • positive regulation of mitotic cell cycle spindle assembly checkpoint Source: UniProtKB
  • reciprocal meiotic recombination Source: GO_Central
  • regulation of centrosome duplication Source: UniProtKB
  • resolution of mitotic recombination intermediates Source: UniProtKB
  • response to ionizing radiation Source: GO_Central
  • response to organic substance Source: Ensembl
  • strand invasion Source: GO_Central
  • t-circle formation Source: BHF-UCL
  • telomere maintenance via recombination Source: BHF-UCL
  • telomere maintenance via telomere trimming Source: BHF-UCL
  • telomeric loop disassembly Source: BHF-UCL

Keywordsi

Molecular functionDNA-binding
Biological processDNA damage, DNA recombination, DNA repair
LigandATP-binding, Nucleotide-binding

Enzyme and pathway databases

ReactomeiR-HSA-5685942 HDR through Homologous Recombination (HRR)
R-HSA-5693554 Resolution of D-loop Structures through Synthesis-Dependent Strand Annealing (SDSA)
R-HSA-5693568 Resolution of D-loop Structures through Holliday Junction Intermediates
R-HSA-5693579 Homologous DNA Pairing and Strand Exchange

Names & Taxonomyi

Protein namesi
Recommended name:
DNA repair protein XRCC3
Alternative name(s):
X-ray repair cross-complementing protein 3
Gene namesi
Name:XRCC3
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 14

Organism-specific databases

EuPathDBiHostDB:ENSG00000126215.13
HGNCiHGNC:12830 XRCC3
MIMi600675 gene
neXtProtiNX_O43542

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Cytoplasm, Mitochondrion, Nucleus

Pathology & Biotechi

Involvement in diseasei

Breast cancer (BC)1 Publication
Disease susceptibility is associated with variations affecting the gene represented in this entry.
Disease descriptionA common malignancy originating from breast epithelial tissue. Breast neoplasms can be distinguished by their histologic pattern. Invasive ductal carcinoma is by far the most common type. Breast cancer is etiologically and genetically heterogeneous. Important genetic factors have been indicated by familial occurrence and bilateral involvement. Mutations at more than one locus can be involved in different families or even in the same case.
See also OMIM:114480
Melanoma, cutaneous malignant 6 (CMM6)1 Publication
Disease susceptibility is associated with variations affecting the gene represented in this entry.
Disease descriptionA malignant neoplasm of melanocytes, arising de novo or from a pre-existing benign nevus, which occurs most often in the skin but also may involve other sites.
See also OMIM:613972
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_013006241T → M in CMM6; associated with disease susceptibility. 3 PublicationsCorresponds to variant dbSNP:rs861539EnsemblClinVar.1

Keywords - Diseasei

Disease mutation

Organism-specific databases

DisGeNETi7517
MalaCardsiXRCC3
MIMi114480 phenotype
613972 phenotype
OpenTargetsiENSG00000126215
PharmGKBiPA37422

Polymorphism and mutation databases

BioMutaiXRCC3

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00001229511 – 346DNA repair protein XRCC3Add BLAST346

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei1N-acetylmethionineCombined sources1

Keywords - PTMi

Acetylation

Proteomic databases

EPDiO43542
PaxDbiO43542
PeptideAtlasiO43542
PRIDEiO43542

PTM databases

iPTMnetiO43542
PhosphoSitePlusiO43542

Expressioni

Inductioni

Stress-induced increase in the mitochondrial levels is seen.1 Publication

Gene expression databases

BgeeiENSG00000126215
CleanExiHS_XRCC3
ExpressionAtlasiO43542 baseline and differential
GenevisibleiO43542 HS

Organism-specific databases

HPAiHPA062422
HPA068953

Interactioni

Subunit structurei

Interacts with RAD51C and RAD51. Part of the CX3 complex consisting of RAD51C and XRCC3; the complex has a ring-like structure arranged into a flat disc around a central channel; CX3 can interact with RAD51 in vitro. Forms a complex with FANCD2, BRCA2 and phosphorylated FANCG. Interacts with SWSAP1 and ZSWIM7; involved in homologous recombination repair. Interacts directly with PALB2 which may serve as a scaffold for a HR complex containing PALB2, BRCA2, RAD51C, RAD51 and XRCC3.6 Publications

Binary interactionsi

Show more details

Protein-protein interaction databases

BioGridi113351, 163 interactors
CORUMiO43542
DIPiDIP-42016N
IntActiO43542, 137 interactors
MINTiO43542
STRINGi9606.ENSP00000343392

Structurei

3D structure databases

ProteinModelPortaliO43542
SMRiO43542
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the RecA family. RAD51 subfamily.Curated

Phylogenomic databases

eggNOGiKOG1564 Eukaryota
COG0468 LUCA
GeneTreeiENSGT00860000133731
HOGENOMiHOG000239162
HOVERGENiHBG054179
InParanoidiO43542
KOiK10880
OMAiLCINQVT
OrthoDBiEOG091G0SMJ
PhylomeDBiO43542
TreeFamiTF101203

Family and domain databases

CDDicd01123 Rad51_DMC1_radA, 1 hit
InterProiView protein in InterPro
IPR013632 DNA_recomb/repair_Rad51_C
IPR016467 DNA_recomb/repair_RecA-like
IPR027417 P-loop_NTPase
IPR033925 Rad51_DMC1_RadA
IPR020588 RecA_ATP-bd
PfamiView protein in Pfam
PF08423 Rad51, 1 hit
PIRSFiPIRSF005856 Rad51, 1 hit
SUPFAMiSSF52540 SSF52540, 1 hit
PROSITEiView protein in PROSITE
PS50162 RECA_2, 1 hit

Sequencei

Sequence statusi: Complete.

O43542-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MDLDLLDLNP RIIAAIKKAK LKSVKEVLHF SGPDLKRLTN LSSPEVWHLL
60 70 80 90 100
RTASLHLRGS SILTALQLHQ QKERFPTQHQ RLSLGCPVLD ALLRGGLPLD
110 120 130 140 150
GITELAGRSS AGKTQLALQL CLAVQFPRQH GGLEAGAVYI CTEDAFPHKR
160 170 180 190 200
LQQLMAQQPR LRTDVPGELL QKLRFGSQIF IEHVADVDTL LECVNKKVPV
210 220 230 240 250
LLSRGMARLV VIDSVAAPFR CEFDSQASAP RARHLQSLGA TLRELSSAFQ
260 270 280 290 300
SPVLCINQVT EAMEEQGAAH GPLGFWDERV SPALGITWAN QLLVRLLADR
310 320 330 340
LREEEAALGC PARTLRVLSA PHLPPSSCSY TISAEGVRGT PGTQSH
Length:346
Mass (Da):37,850
Last modified:June 1, 1998 - v1
Checksum:i4DDF3B163C3B3332
GO

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_02040594R → H1 PublicationCorresponds to variant dbSNP:rs3212057Ensembl.1
Natural variantiVAR_013006241T → M in CMM6; associated with disease susceptibility. 3 PublicationsCorresponds to variant dbSNP:rs861539EnsemblClinVar.1
Natural variantiVAR_029295271G → R. Corresponds to variant dbSNP:rs28903080Ensembl.1
Natural variantiVAR_029296302R → H. Corresponds to variant dbSNP:rs28903081Ensembl.1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF035586 mRNA Translation: AAC05368.1
AF037222 Genomic DNA Translation: AAC04805.1
AF508041 Genomic DNA Translation: AAM23015.1
BC001036 mRNA Translation: AAH01036.1
BC002949 mRNA Translation: AAH02949.1
BC011725 mRNA Translation: AAH11725.1
CCDSiCCDS9984.1
RefSeqiNP_001093588.1, NM_001100118.1
NP_001093589.1, NM_001100119.1
NP_005423.1, NM_005432.3
XP_005268103.1, XM_005268046.2
XP_011535440.1, XM_011537138.2
UniGeneiHs.592325
Hs.733412

Genome annotation databases

EnsembliENST00000352127; ENSP00000343392; ENSG00000126215
ENST00000553264; ENSP00000451974; ENSG00000126215
ENST00000554913; ENSP00000451362; ENSG00000126215
ENST00000555055; ENSP00000452598; ENSG00000126215
GeneIDi7517
KEGGihsa:7517

Keywords - Coding sequence diversityi

Polymorphism

Similar proteinsi

Entry informationi

Entry nameiXRCC3_HUMAN
AccessioniPrimary (citable) accession number: O43542
Secondary accession number(s): O43568, Q9BU18
Entry historyiIntegrated into UniProtKB/Swiss-Prot: April 3, 2002
Last sequence update: June 1, 1998
Last modified: May 23, 2018
This is version 160 of the entry and version 1 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. Human chromosome 14
    Human chromosome 14: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. SIMILARITY comments
    Index of protein domains and families

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