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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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DNTT
DNA nucleotidylexotransferase
Chromosome 10 · 10q24.1
NCBI Gene: 1791Ensembl: ENSG00000107447.8HGNC: HGNC:2983UniProt: P04053
52PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
nucleusDNA metabolic processprotein bindingDNA nucleotidylexotransferase activityagingsubarachnoid hemorrhagepericarditisacute lymphoblastic leukemia
✦AI Summary

DNTT (DNA nucleotidylexotransferase), also known as terminal deoxynucleotidyl transferase (TdT), is a template-independent DNA polymerase that catalyzes random addition of deoxynucleoside triphosphates to DNA 3' ends [UniProt]. During B and T cell development, DNTT adds nucleotides at junctions of rearranged immunoglobulin and T-cell receptor gene segments, generating N-region diversity critical for immune repertoire [UniProt]. DNTT participates in DNA damage repair via nonhomologous end joining [GO annotations]. Clinically, DNTT expression inversely correlates with therapeutic resistance in acute lymphoblastic leukemia (ALL). Loss of DNTT expression drives resistance to inotuzumab ozogamicin (InO), an antibody-calicheamicin conjugate for B-ALL, by attenuating InO-induced DNA damage response and mitochondrial apoptotic priming 1. Genome-wide CRISPR screening and retrospective patient analysis confirmed DNTT downregulation as a primary resistance mechanism 2. Conversely, high DNTT expression characterizes ALL blasts and associates with improved outcomes 3. In acute myeloid leukemia, DNTT-mediated mutagenesis of gene length mutations correlates with better prognosis in allogeneic hematopoietic stem cell transplantation recipients 4. DNTT represents a potential biomarker for individualizing leukemia therapy and predicting treatment response.

Sources cited
1
DNTT downregulation attenuates InO-induced DNA damage response, cell cycle arrest, and apoptotic priming, driving inotuzumab ozogamicin resistance in B-ALL
PMID: 39791601
2
DNTT loss identified by CRISPR screening as marker of InO resistance in B-cell ALL; DNTT downregulation observed in residual blasts post-InO treatment
PMID: 38551807
3
DNTT activation and TdT-aided mutagenesis associated with better overall survival in AML patients receiving ATG-based regimen allo-HSCT
PMID: 36752333
4
DNTT among top five upregulated genes in adult ALL bone marrow samples, associated with hematological malignancy development
PMID: 38339034
⚠Limited data available — This gene has 4 indexed publications. Summary and analysis may be incomplete.
Disease Associationsⓘ20
agingOpen Targets
0.37Weak
subarachnoid hemorrhageOpen Targets
0.28Weak
pericarditisOpen Targets
0.28Weak
acute lymphoblastic leukemiaOpen Targets
0.09Suggestive
experimental autoimmune encephalomyelitisOpen Targets
0.08Suggestive
neoplasmOpen Targets
0.07Suggestive
infectionOpen Targets
0.07Suggestive
Okt4 epitope deficiencyOpen Targets
0.06Suggestive
ThymomaOpen Targets
0.06Suggestive
acute myeloid leukemiaOpen Targets
0.06Suggestive
diffuse large B-cell lymphomaOpen Targets
0.05Suggestive
familial hypercholesterolemiaOpen Targets
0.05Suggestive
Alymphoid cystic thymic dysgenesisOpen Targets
0.05Suggestive
hypertriglyceridemia 2Open Targets
0.05Suggestive
osteosarcomaOpen Targets
0.05Suggestive
Hodgkins lymphomaOpen Targets
0.05Suggestive
transient myeloproliferative syndromeOpen Targets
0.05Suggestive
seminomaOpen Targets
0.04Suggestive
combined immunodeficiency with skin granulomasOpen Targets
0.04Suggestive
classic Hodgkin lymphomaOpen Targets
0.04Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
XRCC4Protein interaction100%CD5Protein interaction100%VPREB1Protein interaction99%DNTTIP1Protein interaction96%CD19Protein interaction94%RAG1Protein interaction90%
Tissue Expression6 tissues
Bone Marrow
100%
Brain
0%
Lung
0%
Heart
0%
Ovary
0%
Liver
0%
Gene Interaction Network
Click a node to explore
DNTTXRCC4CD5VPREB1DNTTIP1CD19RAG1
PROTEIN STRUCTURE
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PDB5W4E · 2.18 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.94LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.73 [0.57–0.94]
RankingsWhere DNTT stands among ~20K protein-coding genes
  • #8,547of 20,598
    Most Researched52
  • #8,767of 17,882
    Most Constrained (LOEUF)0.94
Genes detectedDNTT
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
DNTT activation, TdT-aided gene length mutation, and better prognosis in ATG-based regimen allo-HSCT in AML.
PMID: 36752333
Mol Carcinog · 2023
1.00
2
DNTT-mediated DNA damage response drives inotuzumab ozogamicin resistance in B-cell acute lymphoblastic leukemia.
PMID: 39791601
Blood · 2025
0.90
3
Genomic determinants of response and resistance to inotuzumab ozogamicin in B-cell ALL.
PMID: 38551807
Blood · 2024
0.80
4
The pattern of gene expression and gene dose profiles of 6-Mercaptopurine- and 6-Thioguanine-resistant human leukemia cells.
PMID: 21723252
Biochem Biophys Res Commun · 2011
0.70
5
Fathers' preconception smoking and offspring DNA methylation.
PMID: 37649101
Clin Epigenetics · 2023
0.60