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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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DCLRE1A
DNA cross-link repair 1A
Chromosome 10 · 10q25.3
NCBI Gene: 9937Ensembl: ENSG00000198924.9HGNC: HGNC:17660UniProt: Q6PJP8
48PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
DNA Repair
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
5'-3' DNA exonuclease activitynucleoplasmbeta-lactamase activitynucleusneurodegenerative diseasehypertensionessential hypertensionacquired thrombocytopenia
✦AI Summary

DCLRE1A encodes a DNA repair nuclease that functions as a 5'-to-3' exonuclease in multiple DNA damage repair processes 1. The protein primarily contributes to interstrand cross-link (ICL) repair, with DCLRE1A-deficient cells showing hypersensitivity to ICL-inducing agents like mitomycin C and cisplatin 1. DCLRE1A works in conjunction with XPF-ERCC1 in the 'unhooking' step of ICL repair and interacts with other genome integrity proteins including PCNA 1. Beyond canonical ICL repair, DCLRE1A protects mitochondrial homeostasis by alleviating mtDNA oxidative damage and mitochondrial dysfunction, particularly in lens epithelial cells where it prevents age-related cataract formation 2 3. The protein's expression is regulated through ubiquitin-mediated degradation by the E3 ligase SYVN1 2. Clinical significance includes associations with cancer progression, where DCLRE1A expression levels correlate with treatment response in colorectal cancer 4 and genetic variants may influence chemotherapy toxicity 5. The gene is also identified as a potential breast cancer predisposition candidate 6 and prognostic biomarker in ovarian cancer 7.

Sources cited
1
DCLRE1A functions as 5'-to-3' exonuclease in ICL repair and interacts with XPF-ERCC1
PMID: 32866775
2
DCLRE1A protects mitochondrial homeostasis and is regulated by SYVN1-mediated ubiquitination
PMID: 41421443
3
DCLRE1A prevents age-related cataract formation in lens epithelial cells
PMID: 37503815
4
DCLRE1A expression correlates with colorectal cancer progression
PMID: 31650563
5
DCLRE1A variants may influence chemotherapy toxicity
PMID: 30114658
6
DCLRE1A is a candidate breast cancer predisposition gene
PMID: 30947698
7
DCLRE1A serves as prognostic biomarker in ovarian cancer
PMID: 27806724
Disease Associationsⓘ20
neurodegenerative diseaseOpen Targets
0.34Weak
hypertensionOpen Targets
0.12Weak
essential hypertensionOpen Targets
0.09Suggestive
acquired thrombocytopeniaOpen Targets
0.08Suggestive
Abnormality of the gastrointestinal tractOpen Targets
0.08Suggestive
Increased blood pressureOpen Targets
0.07Suggestive
cardiovascular diseaseOpen Targets
0.07Suggestive
Li-Fraumeni syndromeOpen Targets
0.07Suggestive
myofibromatosis, infantile, 1Open Targets
0.06Suggestive
reticulum cell sarcomaOpen Targets
0.06Suggestive
familial atypical multiple mole melanoma syndromeOpen Targets
0.05Suggestive
Hereditary breast and ovarian cancer syndromeOpen Targets
0.05Suggestive
Ewing sarcomaOpen Targets
0.05Suggestive
response to xenobiotic stimulusOpen Targets
0.05Suggestive
NUT midline carcinomaOpen Targets
0.05Suggestive
breast carcinomaOpen Targets
0.05Suggestive
multiple fibroadenoma of the breastOpen Targets
0.05Suggestive
alveolar soft part sarcomaOpen Targets
0.05Suggestive
synovial sarcomaOpen Targets
0.05Suggestive
chondrosarcomaOpen Targets
0.05Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
ERCC4Protein interaction100%SLX4Protein interaction100%CPSF2Protein interaction100%MUS81Protein interaction100%SLX1AProtein interaction99%CPSF3Protein interaction94%
Tissue Expression6 tissues
Bone Marrow
100%
Heart
92%
Liver
84%
Brain
77%
Lung
37%
Ovary
36%
Gene Interaction Network
Click a node to explore
DCLRE1AERCC4SLX4CPSF2MUS81SLX1ACPSF3
PROTEIN STRUCTURE
Preparing viewer…
PDB5Q22 · 1.18 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.86LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.70 [0.57–0.86]
RankingsWhere DCLRE1A stands among ~20K protein-coding genes
  • #9,062of 20,598
    Most Researched48
  • #7,470of 17,882
    Most Constrained (LOEUF)0.86
Genes detectedDCLRE1A
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
DCLRE1 downregulated by SYVN1-mediated ubiquitination and degradation, weakening mitochondrial homeostasis protection in ARC formation.
PMID: 41421443
Exp Eye Res · 2026
1.00
2
The SNM1A DNA repair nuclease.
PMID: 32866775
DNA Repair (Amst) · 2020
0.90
3
The role of double-strand break repair, translesion synthesis, and interstrand crosslinks in colorectal cancer progression-clinicopathological data and survival.
PMID: 31650563
J Surg Oncol · 2020
0.80
4
DCLRE1A Contributes to DNA Damage Repair and Apoptosis in Age-Related Cataracts by Regulating the lncRNA/miRNA/mRNA Axis.
PMID: 37503815
Curr Eye Res · 2023
0.70
5
Identification of candidate cancer predisposing variants by performing whole-exome sequencing on index patients from BRCA1 and BRCA2-negative breast cancer families.
PMID: 30947698
BMC Cancer · 2019
0.60