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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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SHLD3
shieldin complex subunit 3
Chromosome 5 · 5q12.3
NCBI Gene: 112441434Ensembl: ENSG00000253251.4HGNC: HGNC:53826UniProt: Q6ZNX1
8PubMed Papers
15Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
DNA Repair
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingpositive regulation of isotype switchingnegative regulation of double-strand break repair via homologous recombinationpositive regulation of double-strand break repair via nonhomologous end joiningdiverticular diseaseIncisional herniastenosing tenosynovitisglomerulonephritis
✦AI Summary

SHLD3 is a core subunit of the shieldin complex, which protects DNA double-strand breaks (DSBs) to promote non-homologous end joining (NHEJ) repair 1. As a component of the four-subunit shieldin complex (REV7, SHLD1, SHLD2, and SHLD3), SHLD3 functions downstream of 53BP1 and RIF1 during G1 and S phase to suppress DNA end resection and inhibit homologous recombination 1. SHLD3 recruits REV7 through specific structural interactions involving its REV7-binding domain, with both the N-terminal loop and C-terminal α-helix essential for high-affinity binding 2. The shieldin complex localizes to DSB sites in a 53BP1-dependent manner, where SHLD2's OB-fold domains bind single-stranded DNA to physically shield broken DNA ends from nucleolytic degradation 1. SHLD3 also mediates conformational heterodimerization of REV7, creating a closed/open REV7 dimer critical for complex assembly and NHEJ efficiency 3. The shieldin complex regulates immunoglobulin class-switch recombination and promotes PARP inhibitor sensitivity in BRCA1-deficient cells by preventing excessive resection 1. TRIP13 AAA+ ATPase can disassemble shieldin through ATP-dependent remodeling of the SHLD3-REV7 interface, providing a mechanism for dynamic control of DNA repair pathway choice 4. Dysregulation of SHLD3 function is associated with PARP inhibitor resistance in homologous recombination-deficient tumors.

Sources cited
1
SHLD3 is a core shieldin complex subunit that protects DNA ends to suppress resection and promote NHEJ; loss impairs class switching and causes hyper-resection
PMID: 30022168
2
SHLD3 recruits REV7 through a ladle-shaped REV7-binding domain using N-terminal loop and C-terminal α-helix for high-affinity binding
PMID: 31796627
3
SHLD3 mediates conformational heterodimerization of REV7 critical for shieldin assembly and NHEJ efficiency
PMID: 32332881
4
TRIP13 ATPase remodels SHLD3-REV7 interface to disassemble shieldin and regulate DNA repair pathway choice
PMID: 33597306
5
SHLD3 interaction with MAD2L2/REV7 requires proper dimerization and is essential for shieldin activity in DNA repair
PMID: 34521823
6
Shieldin complex containing SHLD3 recruits CST-Polα machinery to control DSB resection and PARP inhibitor sensitivity in BRCA1-deficient cells
PMID: 30022158
7
CHAMP1 binding to REV7 reduces shieldin complex levels, promoting HR repair and PARP inhibitor resistance
PMID: 36044844
8
Shieldin containing SHLD3 functions as the ultimate effector of 53BP1-dependent DNA end protection
PMID: 30948458
Disease Associationsⓘ15
diverticular diseaseOpen Targets
0.08Suggestive
Incisional herniaOpen Targets
0.04Suggestive
stenosing tenosynovitisOpen Targets
0.03Suggestive
glomerulonephritisOpen Targets
0.02Suggestive
food allergyOpen Targets
0.02Suggestive
Abnormality of the skeletal systemOpen Targets
0.02Suggestive
Inguinal herniaOpen Targets
0.02Suggestive
B-cell non-Hodgkins lymphomaOpen Targets
0.00Suggestive
neoplasmOpen Targets
0.00Suggestive
prostate adenocarcinomaOpen Targets
0.00Suggestive
papillary thyroid carcinomaOpen Targets
0.00Suggestive
cancerOpen Targets
0.00Suggestive
paracoccidioidomycosisOpen Targets
0.00Suggestive
gastric cancerOpen Targets
0.00Suggestive
ovarian serous cystadenocarcinomaOpen Targets
0.00Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
TP53BP1Protein interaction96%SHLD2Protein interaction92%TRIP13Protein interaction92%SHLD1Protein interaction78%MAD2L2Protein interaction57%RIF1Shared pathway35%
Tissue Expression6 tissues
Bone Marrow
100%
Liver
14%
Heart
13%
Ovary
9%
Lung
9%
Brain
8%
Gene Interaction Network
Click a node to explore
SHLD3TP53BP1SHLD2TRIP13SHLD1MAD2L2RIF1
PROTEIN STRUCTURE
Preparing viewer…
PDB6M7B · 1.77 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.02LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.66 [0.44–1.02]
RankingsWhere SHLD3 stands among ~20K protein-coding genes
  • #17,706of 20,598
    Most Researched8
  • #9,998of 17,882
    Most Constrained (LOEUF)1.02
Genes detectedSHLD3
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
MAD2L2 dimerization and TRIP13 control shieldin activity in DNA repair.
PMID: 34521823
Nat Commun · 2021
1.00
2
The shieldin complex mediates 53BP1-dependent DNA repair.
PMID: 30022168
Nature · 2018
0.90
3
CHAMP1 binds to REV7/FANCV and promotes homologous recombination repair.
PMID: 36044844
Cell Rep · 2022
0.80
4
53BP1-RIF1-shieldin counteracts DSB resection through CST- and Polα-dependent fill-in.
PMID: 30022158
Nature · 2018
0.70
5
Shieldin - the protector of DNA ends.
PMID: 30948458
EMBO Rep · 2019
0.60