TOP1 (DNA topoisomerase 1) variants and mutations
TOP1 (also known as DNA topoisomerase 1) is a human protein-coding gene encoding a DNA topoisomerase 1 protein. It relieves torsional strain in DNA by creating and resealing transient single-strand breaks during replication and transcription. Trapping of the cleavage intermediate is the mechanism exploited by topoisomerase I inhibitors such as irinotecan and topotecan in cancer therapy. This analysis covers 785 TOP1 variants and mutations. Of these, 54% have computational variant effect predictions. Disease context includes small cell lung carcinoma, breast cancer, and neoplasm. Example TOP1 variants include S2C, S2G, and S2I.
Variant analysis overview
- Gene: TOP1
- Protein: DNA topoisomerase 1
- UniProt accession: P11387
- Organism: Homo sapiens
- Variants analyzed: 785
- Variant scope: all variants
- Completed: 2026-08-22
Variant and mutation evidence
- Variant composition: 683 unspecified-consequence records; 59 missense variants; 3 frameshift variants; 28 synonymous variants; 4 stop-gained variants; 4 splice-region variants; 3 in-frame deletions; 2 substitution
- Prediction scores: 424 variants have prediction scores (54% of the analyzed set).
Clinical, disease, and population context
- Disease context: 25 disease associations are represented. Top associations: small cell lung carcinoma, breast cancer, neoplasm, non-small cell lung carcinoma, colorectal cancer, cervical cancer, ovarian cancer, triple-negative breast carcinoma, neurodegenerative disease, carcinoma, breast neoplasm, ovarian neoplasm.
Protein structure and variant hotspots
- Protein features: 1 domains; 8 post-translational modification sites.
- Structural context: 290 variants have structural context.
- PTM context: 18 variants overlap post-translational modification sites.
Data sources
Evidence in this analysis draws on EBI Proteins Variation, UniProt, gnomAD v4, EuropePMC, 3D Hotspot Analysis, Interaction Network Analysis, Protein Data Bank, AlphaFold DB, gnomAD constraint, Open Targets, MaveDB, LitVar.
Notable TOP1 variants
Examples include S2C, S2G, S2I, S2N, S2R, S2S, G3R, G3W. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.
- S2C (p.Ser2Cys), gnomAD 20-41029071-A-T, REVEL 0.17, MetaLR 0.10
- S2G (p.Ser2Gly), gnomAD 20-41029071-A-G, REVEL 0.14, MetaLR 0.09
- S2I (p.Ser2Ile), gnomAD 20-41029072-G-T, REVEL 0.17, MetaLR 0.10
- S2N (p.Ser2Asn), gnomAD 20-41029072-G-A, REVEL 0.10, MetaLR 0.09
- S2R (p.Ser2Arg), gnomAD 20-41029073-T-A, REVEL 0.22, MetaLR 0.08
- S2S (p.Ser2Ser), gnomAD 20-41029073-T-C, CADD 13.40
- G3R (p.Gly3Arg), gnomAD 20-41029074-G-A, REVEL 0.21, MetaLR 0.11
- G3W (p.Gly3Trp), gnomAD 20-41029074-G-T, REVEL 0.20, MetaLR 0.11
- G3E (p.Gly3Glu), gnomAD 20-41029075-G-A, REVEL 0.24, MetaLR 0.09
- G3V (p.Gly3Val), gnomAD 20-41029075-G-T, REVEL 0.18, MetaLR 0.10
- G3G (p.Gly3Gly), rs1464812162, gnomAD 20-41029076-G-T, CADD 14.10
- D4E (p.Asp4Glu), ExAC rs769782931, gnomAD rs769782931, REVEL 0.18, MetaLR 0.06
- D4T (p.Asp4Thr), gnomAD 20-41029073-TG-T, CADD 32.00
- D4N (p.Asp4Asn), gnomAD 20-41029077-G-A, REVEL 0.12, MetaLR 0.10
- D4Y (p.Asp4Tyr), gnomAD 20-41029077-G-T, REVEL 0.17, MetaLR 0.10
- D4G (p.Asp4Gly), gnomAD 20-41029078-A-G, REVEL 0.15, MetaLR 0.09
- D4V (p.Asp4Val), gnomAD 20-41029078-A-T, REVEL 0.14, MetaLR 0.10
- D4D (p.Asp4Asp), gnomAD 20-41029079-C-T, CADD 14.30
- H5T (p.His5Thr), gnomAD 20-41029078-AC-A, CADD 27.20
- H5Y (p.His5Tyr), gnomAD 20-41029080-C-T, REVEL 0.03, MetaLR 0.04
- H5N (p.His5Asn), gnomAD 20-41029080-C-A, REVEL 0.04, MetaLR 0.04
- H5P (p.His5Pro), gnomAD 20-41029081-A-C, REVEL 0.06, MetaLR 0.04
- H5L (p.His5Leu), gnomAD 20-41029081-A-T, REVEL 0.07, MetaLR 0.04
- H5R (p.His5Arg), gnomAD 20-41029081-A-G, REVEL 0.03, MetaLR 0.04
- H5Q (p.His5Gln), gnomAD 20-41029082-C-A, REVEL 0.18, MetaLR 0.03
- H5H (p.His5His), gnomAD 20-41029082-C-T, CADD 12.20
- L6F (p.Leu6Phe), ExAC rs773299206, gnomAD rs773299206, REVEL 0.06, MetaLR 0.04
- L6I (p.Leu6Ile), gnomAD 20-41029083-C-A, REVEL 0.05, MetaLR 0.03
- L6V (p.Leu6Val), gnomAD 20-41029083-C-G, REVEL 0.04, MetaLR 0.03
- L6H (p.Leu6His), gnomAD 20-41029084-T-A, REVEL 0.10, MetaLR 0.04
- L6L (p.Leu6Leu), gnomAD 20-41029085-C-A, CADD 10.20
- H7R (p.His7Arg), ExAC rs771124146, gnomAD rs771124146, REVEL 0.11, MetaLR 0.07
- H7N (p.His7Asn), gnomAD 20-41029086-C-A, REVEL 0.11, MetaLR 0.08
- H7Y (p.His7Tyr), gnomAD 20-41029086-C-T, REVEL 0.15, MetaLR 0.08
- H7L (p.His7Leu), gnomAD 20-41029087-A-T, REVEL 0.15, MetaLR 0.08
- H7Q (p.His7Gln), gnomAD 20-41029088-C-G, REVEL 0.15, MetaLR 0.07
- H7H (p.His7His), gnomAD 20-41029088-C-T, CADD 13.40
- N8D (p.Asn8Asp), TOPMed rs2033080090, gnomAD rs2033080090, REVEL 0.06, MetaLR 0.07
- N8S (p.Asn8Ser), TOPMed rs2033080135, REVEL 0.09, MetaLR 0.06, Uncertain significance, not specified
- N8N (p.Asn8Asn), gnomAD 20-41029091-C-T, CADD 13.70
- N8K (p.Asn8Lys), gnomAD 20-41029091-C-A, REVEL 0.15, MetaLR 0.06
- D9N (p.Asp9Asn), Ensembl rs2122577385, REVEL 0.08, MetaLR 0.09
- D9Y (p.Asp9Tyr), gnomAD 20-41029092-G-T, REVEL 0.22, MetaLR 0.10
- D9D (p.Asp9Asp), gnomAD 20-41029094-T-C, CADD 12.10
- S10Y (p.Ser10Tyr), gnomAD 20-41029096-C-A, REVEL 0.11, MetaLR 0.10
- S10F (p.Ser10Phe), gnomAD 20-41029096-C-T, REVEL 0.12, MetaLR 0.10
- S10S (p.Ser10Ser), rs2033080186, gnomAD 20-41029097-C-T, CADD 13.50
- Q11R (p.Gln11Arg), gnomAD 20-41029095-TC-T, CADD 28.70
- Q11* (p.Gln11Ter), gnomAD 20-41029098-C-T, CADD 40.00
- Q11K (p.Gln11Lys), gnomAD 20-41029098-C-A, REVEL 0.08, MetaLR 0.07
- Q11L (p.Gln11Leu), gnomAD 20-41029099-A-T, REVEL 0.12, MetaLR 0.08
- Q11H (p.Gln11His), gnomAD 20-41029100-G-T, REVEL 0.13, MetaLR 0.10
- Q11Q (p.Gln11Gln), gnomAD 20-41029100-G-A, CADD 23.50
- I12M (p.Ile12Met), ExAC rs772450250, TOPMed rs772450250, gnomAD rs772450250, REVEL 0.20, MetaLR 0.10
- I12V (p.Ile12Val), TOPMed rs1035670825, gnomAD rs1035670825, REVEL 0.14, MetaLR 0.05
- I12I (p.Ile12Ile), rs772450250, gnomAD 20-41029433-C-A, CADD 21.70
- E13K (p.Glu13Lys), NCI-TCGA Cosmic COSV1007, Variant assessed as somatic; moderate impact.
- E13* (p.Glu13Ter), gnomAD 20-41029434-G-T, CADD 44.00
- E13E (p.Glu13Glu), gnomAD 20-41029436-A-G, CADD 13.30
- A14G (p.Ala14Gly), gnomAD rs1209375262
- A14V (p.Ala14Val), gnomAD rs1209375262, REVEL 0.03, MetaLR 0.04
- A14T (p.Ala14Thr), gnomAD 20-41029437-G-A, REVEL 0.02, MetaLR 0.04
- A14E (p.Ala14Glu), gnomAD 20-41029438-C-A, REVEL 0.05, MetaLR 0.04
- A14A (p.Ala14Ala), gnomAD 20-41029439-G-T, CADD 15.80
- D15V (p.Asp15Val), Ensembl rs2122578352, REVEL 0.17, MetaLR 0.02
- D15Y (p.Asp15Tyr), gnomAD rs1287804312, REVEL 0.11, MetaLR 0.05
- D15D (p.Asp15Asp), rs145138122, gnomAD 20-41029442-T-C, CADD 15.60
- F16F (p.Phe16Phe), gnomAD 20-41029445-C-T, CADD 14.40
- R17G (p.Arg17Gly), Ensembl rs2033087373, REVEL 0.18, MetaLR 0.12
- R17L (p.Arg17Leu), ExAC rs761127035, TOPMed rs761127035, gnomAD rs761127035, REVEL 0.21, MetaLR 0.09
- R17P (p.Arg17Pro), ExAC rs761127035, TOPMed rs761127035, gnomAD rs761127035
- R17Q (p.Arg17Gln), ExAC rs761127035, TOPMed rs761127035, gnomAD rs761127035, REVEL 0.16, MetaLR 0.09
- R17* (p.Arg17Ter), gnomAD 20-41029446-C-T, CADD 38.00
- R17R (p.Arg17Arg), rs2033087373, gnomAD 20-41029446-C-A, CADD 16.10
- L18L (p.Leu18Leu), rs1202822680, gnomAD 20-41029449-T-C, CADD 11.40
- L18S (p.Leu18Ser), gnomAD 20-41029450-T-C, REVEL 0.04, MetaLR 0.02
- L18F (p.Leu18Phe), gnomAD 20-41029451-G-T, REVEL 0.05, MetaLR 0.02
- N19I (p.Asn19Ile), ExAC rs764649035, TOPMed rs764649035, gnomAD rs764649035, REVEL 0.12, MetaLR 0.10
- N19D (p.Asn19Asp), gnomAD 20-41029452-A-G, REVEL 0.09, MetaLR 0.09
- N19S (p.Asn19Ser), gnomAD 20-41029453-A-G, REVEL 0.15, MetaLR 0.06
- N19N (p.Asn19Asn), gnomAD 20-41029454-T-C, CADD 17.10
- D20Y (p.Asp20Tyr), gnomAD 20-41029455-G-T, REVEL 0.16, MetaLR 0.10
- D20N (p.Asp20Asn), gnomAD 20-41029455-G-A, REVEL 0.13, MetaLR 0.08
- S21C (p.Ser21Cys), NCI-TCGA Cosmic COSV1007, TOPMed rs2033542550, Variant assessed as somatic; moderate impact.
- S21P (p.Ser21Pro), gnomAD 20-41061396-T-C, REVEL 0.09, MetaLR 0.06
- H22R (p.His22Arg), TOPMed rs958350596, gnomAD rs958350596, REVEL 0.15, MetaLR 0.08
- H22Y (p.His22Tyr), Ensembl rs939788315, REVEL 0.19, MetaLR 0.10
- H22H (p.His22His), rs2145926971, gnomAD 20-41061401-T-C, CADD 9.26
- H22Q (p.His22Gln), gnomAD 20-41061401-T-A, REVEL 0.18, MetaLR 0.06
- K23N (p.Lys23Asn), TOPMed rs1396729231, gnomAD rs1396729231, REVEL 0.09, MetaLR 0.11
- K25R (p.Lys25Arg), TOPMed rs1302095474, gnomAD rs1302095474, REVEL 0.12, MetaLR 0.10
- D26N (p.Asp26Asn), gnomAD rs1411426916, REVEL 0.10, MetaLR 0.05
- D26Y (p.Asp26Tyr), NCI-TCGA Cosmic COSV6369, Variant assessed as somatic; moderate impact.
- D26E (p.Asp26Glu), gnomAD 20-41061413-T-G, REVEL 0.06, MetaLR 0.04
- K27E (p.Lys27Glu), TOPMed rs2033542927, REVEL 0.14, MetaLR 0.09
- p.Lys27 Asp30del, rs773155066, gnomAD 20-41061399-CATAA, CADD 20.70
- K29E (p.Lys29Glu), gnomAD 20-41061420-A-G, REVEL 0.14, MetaLR 0.10
- K29R (p.Lys29Arg), gnomAD 20-41061421-A-G, REVEL 0.12, MetaLR 0.10
- D30E (p.Asp30Glu), TOPMed rs974879110
- D30H (p.Asp30His), Ensembl rs2033542966
- D30Y (p.Asp30Tyr), NCI-TCGA TCGA novel, Variant assessed as somatic; moderate impact.
- R31* (p.Arg31Ter), NCI-TCGA Cosmic COSV1007, NCI-TCGA Cosmic COSV6369, Ensembl rs2145926983, Variant assessed as somatic; high impact.
- R31Q (p.Arg31Gln), ExAC rs779948444, gnomAD rs779948444, REVEL 0.10, MetaLR 0.03
- E32K (p.Glu32Lys), gnomAD rs1311452696, REVEL 0.18, MetaLR 0.11
- E32G (p.Glu32Gly), gnomAD 20-41061430-A-G, REVEL 0.18, MetaLR 0.11
- H33R (p.His33Arg), TOPMed rs1166021854, REVEL 0.12, MetaLR 0.08
- H33Y (p.His33Tyr), TOPMed rs1364036505, gnomAD rs1364036505, REVEL 0.12, MetaLR 0.07
- R34Q (p.Arg34Gln), TOPMed rs2033543288, REVEL 0.10, MetaLR 0.04
- R34W (p.Arg34Trp), TOPMed rs1319354641, gnomAD rs1319354641, REVEL 0.15, MetaLR 0.07, Uncertain significance, not specified
- R34L (p.Arg34Leu), gnomAD 20-41061436-G-T, REVEL 0.08, MetaLR 0.05
- H35H (p.His35His), rs2033543328, gnomAD 20-41061440-C-T, CADD 9.07
- K36R (p.Lys36Arg), gnomAD rs1246537609, REVEL 0.11, MetaLR 0.11
- E37D (p.Glu37Asp), Ensembl rs2145926997
- E37V (p.Glu37Val), TOPMed rs1363354119, gnomAD rs1363354119, REVEL 0.10, MetaLR 0.05
- H38N (p.His38Asn), gnomAD rs1273361617, REVEL 0.08, MetaLR 0.08
- H38R (p.His38Arg), gnomAD 20-41061448-A-G, REVEL 0.15, MetaLR 0.07
- K39N (p.Lys39Asn), Ensembl rs2145927003
- K40E (p.Lys40Glu), gnomAD rs1348183473, REVEL 0.24, MetaLR 0.10
- K40R (p.Lys40Arg), TOPMed rs1457507702, gnomAD rs1457507702, REVEL 0.16, MetaLR 0.08
- K40del (p.Lys40del), gnomAD 20-41061449-CAAG-, CADD 19.50
- K40K (p.Lys40Lys), gnomAD 20-41061455-G-A, CADD 9.47
- E41del (p.Glu41del), gnomAD 20-41061453-AAGG-, CADD 20.10
- E41E (p.Glu41Glu), gnomAD 20-41061458-G-A, CADD 1.78
- K42N (p.Lys42Asn), NCI-TCGA Cosmic COSV6369, Variant assessed as somatic; moderate impact.
- D43E (p.Asp43Glu), Ensembl rs2145927007, REVEL 0.20, MetaLR 0.04
- R44L (p.Arg44Leu), TOPMed rs1316906747, gnomAD rs1316906747, REVEL 0.20, MetaLR 0.11
- R44Q (p.Arg44Gln), TOPMed rs1316906747, gnomAD rs1316906747, REVEL 0.17, MetaLR 0.11
- R44W (p.Arg44Trp), rs751834546, NCI-TCGA Cosmic COSV6369, ExAC rs751834546, TOPMed rs751834546, REVEL 0.17, MetaLR 0.11, Variant assessed as somatic; moderate impact.
- R44R (p.Arg44Arg), gnomAD 20-41061465-C-A, CADD 13.10
- E45V (p.Glu45Val), TOPMed rs2033543873, gnomAD rs2033543873
- E45K (p.Glu45Lys), gnomAD 20-41061468-G-A, REVEL 0.11, MetaLR 0.10
- E45E (p.Glu45Glu), gnomAD 20-41061470-A-G, CADD 8.47
- K46K (p.Lys46Lys), rs2145927024, gnomAD 20-41061473-G-A, CADD 8.62
- S47C (p.Ser47Cys), gnomAD rs1196598053, REVEL 0.10, MetaLR 0.06
- S47Y (p.Ser47Tyr), gnomAD rs1196598053
- S47F (p.Ser47Phe), gnomAD 20-41061475-C-T, REVEL 0.11, MetaLR 0.04
- S47S (p.Ser47Ser), gnomAD 20-41061476-C-T, CADD 8.59
- K48E (p.Lys48Glu), gnomAD 20-41061477-A-G, REVEL 0.22, MetaLR 0.10
- K48M (p.Lys48Met), gnomAD 20-41061478-A-T, REVEL 0.24, MetaLR 0.11
- K48K (p.Lys48Lys), rs35905808, gnomAD 20-41061479-G-A, CADD 8.14
- H49Y (p.His49Tyr), gnomAD 20-41061480-C-T, REVEL 0.07, MetaLR 0.04
- H49N (p.His49Asn), gnomAD 20-41061480-C-A, REVEL 0.10, MetaLR 0.04
- H49R (p.His49Arg), gnomAD 20-41061481-A-G, REVEL 0.09, MetaLR 0.04
- H49H (p.His49His), rs1181161878, gnomAD 20-41061482-T-C, CADD 8.08
- S50S (p.Ser50Ser), rs748506884, gnomAD 20-41061485-C-T, CADD 12.70
- N51D (p.Asn51Asp), Ensembl rs2033544203
- N51K (p.Asn51Lys), TOPMed rs933474316
- N51S (p.Asn51Ser), gnomAD 20-41061487-A-G, REVEL 0.10, MetaLR 0.08
- S52N (p.Ser52Asn), Ensembl rs2145927039
- S52S (p.Ser52Ser), gnomAD 20-41076171-T-C, CADD 19.40
- E53G (p.Glu53Gly), NCI-TCGA Cosmic COSV6369, Variant assessed as somatic; moderate impact.
- E53* (p.Glu53Ter), gnomAD 20-41076172-G-T, CADD 41.00
- E53E (p.Glu53Glu), rs773155637, gnomAD 20-41076174-A-G, CADD 13.00
- H54R (p.His54Arg), gnomAD rs1357991289, REVEL 0.04, MetaLR 0.06
- D56A (p.Asp56Ala), TOPMed rs2033725364, gnomAD rs2033725364, REVEL 0.17, MetaLR 0.08
- D56H (p.Asp56His), Ensembl rs2033725321
- S57C (p.Ser57Cys), ExAC rs762757738, gnomAD rs762757738, REVEL 0.11, MetaLR 0.10
- S57T (p.Ser57Thr), gnomAD 20-41076184-T-A, REVEL 0.04, MetaLR 0.07
- S57Y (p.Ser57Tyr), gnomAD 20-41076185-C-A, REVEL 0.10, MetaLR 0.09
- S57S (p.Ser57Ser), rs766516532, gnomAD 20-41076186-T-G, CADD 13.40
- H61Q (p.His61Gln), NCI-TCGA Cosmic COSV6369, REVEL 0.08, MetaLR 0.05, Variant assessed as somatic; moderate impact.
- E65* (p.Glu65Ter), Ensembl rs2033725583
- E65D (p.Glu65Asp), NCI-TCGA TCGA novel, Variant assessed as somatic; moderate impact.
- T67A (p.Thr67Ala), TOPMed rs2033725622
- T67P (p.Thr67Pro), TOPMed rs2033725622, REVEL 0.12, MetaLR 0.06
- T67S (p.Thr67Ser), Ensembl rs2145935018
- K68Q (p.Lys68Gln), Ensembl rs2033725685
- H69P (p.His69Pro), ExAC rs751566633, TOPMed rs751566633, gnomAD rs751566633, REVEL 0.06, MetaLR 0.05
- H69Q (p.His69Gln), ExAC rs755282743, gnomAD rs755282743, REVEL 0.11, MetaLR 0.04
- H69R (p.His69Arg), ExAC rs751566633, TOPMed rs751566633, gnomAD rs751566633, REVEL 0.05, MetaLR 0.05
- D71E (p.Asp71Glu), Ensembl rs2145935029
- D71Y (p.Asp71Tyr), NCI-TCGA Cosmic COSV6369, Variant assessed as somatic; moderate impact.
- G72E (p.Gly72Glu), NCI-TCGA Cosmic COSV1007, Variant assessed as somatic; moderate impact.
- S73N (p.Ser73Asn), TOPMed rs2033725761
- S73R (p.Ser73Arg), ExAC rs767844946, gnomAD rs767844946
- S74* (p.Ser74Ter), NCI-TCGA Cosmic COSV6369, CADD 36.00, Variant assessed as somatic; high impact.
- S74P (p.Ser74Pro), TOPMed rs2033725844, REVEL 0.10, MetaLR 0.07
- S74T (p.Ser74Thr), TOPMed rs2033725844
- H77N (p.His77Asn), Ensembl rs2145935038, REVEL 0.06, MetaLR 0.04
- H77Y (p.His77Tyr), Ensembl rs2145935038
Public TOP1 analysis runs
- TOP1 analysis run — TOP1 (785 variants) — completed 2026-08-22