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25 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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HLTF
helicase like transcription factor
Chromosome 3 · 3q24
NCBI Gene: 6596Ensembl: ENSG00000071794.16HGNC: HGNC:11099UniProt: A0A0C4DGA6
163PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
DNA Repair
RESEARCH IMPACT
Trending
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein localization to chromatinprotein K63-linked ubiquitinationsingle-stranded DNA bindingRNA bindingatrial fibrillationatrial flutteralcohol drinkingneurodegenerative disease
✦AI Summary

HLTF (helicase-like transcription factor) is a multifunctional DNA-dependent ATPase and E3 ubiquitin-protein ligase that maintains genome stability through several mechanisms. As an ATP-dependent dsDNA translocase (lacking classical helicase activity), HLTF catalyzes replication fork reversal by unwinding and reannealing nascent and parental DNA strands, facilitating error-free postreplication repair via template switching 1. HLTF catalyzes Lys-63-linked polyubiquitination of monoubiquitinated PCNA at Lys-164 in response to genotoxic stress. Additionally, HLTF resolves G-quadruplex structures in cooperation with MSH2, suppressing G4 accumulation and replication stress while restraining DNA synthesis when G4s are stabilized 2. HLTF contributes to nucleotide excision repair and interacts with mismatch repair proteins MSH2 3. In disease contexts, excessive HLTF accumulation at stalled replication forks promotes MRE11-dependent fork degradation; the deubiquitinase USP37 counteracts HLTF to protect fork stability and promote BRCA1-deficient cell survival, making HLTF relevant to PARP inhibitor resistance 4. In glioblastoma, HLTF stabilization via the LINC01088/USP7 axis prevents ferroptosis and promotes malignancy 5. HLTF also functions in double-strand break repair outcomes 6 and serves as a context-dependent repressor of prime editing efficiency 7.

Sources cited
1
HLTF is an ATP-dependent DNA translocase that mediates replication fork reversal
PMID: 39089193
2
HLTF resolves G-quadruplex structures and suppresses G4 accumulation in cooperation with MSH2
PMID: 39142279
3
HLTF interacts with MSH2, a mismatch repair protein
PMID: 35784486
4
USP37 counteracts HLTF to protect replication fork stability and overcome PARP inhibitor resistance in BRCA1-deficient cells
PMID: 40548939
5
HLTF stabilization via LINC01088/USP7 axis prevents ferroptosis in glioblastoma
PMID: 40000422
6
HLTF has functions in Cas9-induced double-strand break repair
PMID: 41037600
7
HLTF acts as a context-dependent repressor of prime editing efficiency
PMID: 38608704
Disease Associationsⓘ20
atrial fibrillationOpen Targets
0.46Moderate
atrial flutterOpen Targets
0.31Weak
alcohol drinkingOpen Targets
0.29Weak
neurodegenerative diseaseOpen Targets
0.14Weak
neoplasmOpen Targets
0.11Weak
hepatocellular carcinomaOpen Targets
0.10Suggestive
glioblastoma multiformeOpen Targets
0.07Suggestive
gliomaOpen Targets
0.07Suggestive
malignant colon neoplasmOpen Targets
0.07Suggestive
colonic neoplasmOpen Targets
0.07Suggestive
non-small cell lung carcinomaOpen Targets
0.07Suggestive
adenomaOpen Targets
0.05Suggestive
papillary carcinomaOpen Targets
0.05Suggestive
tropical spastic paraparesisOpen Targets
0.04Suggestive
polycythemia veraOpen Targets
0.03Suggestive
cancerOpen Targets
0.03Suggestive
squamous cell carcinomaOpen Targets
0.03Suggestive
Alzheimer disease type 1Open Targets
0.03Suggestive
intestinal neoplasmOpen Targets
0.03Suggestive
exudative vitreoretinopathy 2, X-linkedOpen Targets
0.03Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
PRIMPOLProtein interaction100%RAD52Protein interaction100%WRNProtein interaction100%FANCMProtein interaction100%RAD51Protein interaction100%EXO1Protein interaction92%
Tissue Expression6 tissues
Heart
100%
Bone Marrow
87%
Brain
80%
Ovary
50%
Liver
32%
Lung
18%
Gene Interaction Network
Click a node to explore
HLTFPRIMPOLRAD52WRNFANCMRAD51EXO1
PROTEIN STRUCTURE
Preparing viewer…
PDB4XZF · 1.38 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.48LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF1.13 [0.88–1.48]
RankingsWhere HLTF stands among ~20K protein-coding genes
  • #2,754of 20,598
    Most Researched163 · top quartile
  • #15,060of 17,882
    Most Constrained (LOEUF)1.48
Genes detectedHLTF
Sources retrieved25 papers
Response time—
📄 Sources
25▼
1
Mechanisms and regulation of replication fork reversal.
PMID: 39089193
DNA Repair (Amst) · 2024
1.00
2
HLTF resolves G4s and promotes G4-induced replication fork slowing to maintain genome stability.
PMID: 39142279
Mol Cell · 2024
0.90
3
Profound synthetic lethality between SMARCAL1 and FANCM.
PMID: 39510066
Mol Cell · 2024
0.80
4
Acetyltransferase NAT10 inhibits T-cell immunity and promotes nasopharyngeal carcinoma progression through DDX5/HMGB1 axis.
PMID: 39939141
J Immunother Cancer · 2025
0.72
5
A comprehensive genetic catalog of human double-strand break repair.
PMID: 41037600
Science · 2025
0.70