RNF207 (RING finger protein 207) variants and mutations
RNF207 (also known as RING finger protein 207) is a human protein-coding gene encoding a RING finger protein 207 protein. A protein involved in cardiac repolarization that may help stabilize the KCNH2/hERG channel at the membrane. It may also assist channel synthesis, folding, or export from the endoplasmic reticulum, connecting RNF207 to electrical signaling in the heart. This analysis covers 1,083 RNF207 variants and mutations. Of these, 93% have computational variant effect predictions. Disease context includes atrial fibrillation, artificial cardiac pacemaker, and hypertrophic cardiomyopathy. Example RNF207 variants include S2W, S2A, and S2*.
Variant analysis overview
- Gene: RNF207
- Protein: RING finger protein 207
- UniProt accession: Q6ZRF8
- Organism: Homo sapiens
- Variants analyzed: 1083
- Variant scope: all variants
- Completed: 2026-07-17
Variant and mutation evidence
- Variant composition: 813 unspecified-consequence records; 151 missense variants; 11 stop-gained variants; 84 synonymous variants; 14 frameshift variants; 8 in-frame deletions; 2 splice-region variants
- Prediction scores: 1,010 variants have prediction scores (93% of the analyzed set).
Clinical, disease, and population context
- Disease context: 25 disease associations are represented. Top associations: atrial fibrillation, artificial cardiac pacemaker, hypertrophic cardiomyopathy, atrioventricular block, neurodegenerative disease, heart failure, cardiac arrhythmia, Prolonged QT interval, placental retention, uterine corpus leiomyoma, benign colon neoplasm, colorectal cancer.
Protein structure and variant hotspots
- Protein features: 4 binding sites.
Data sources
Evidence in this analysis draws on EBI Proteins Variation, UniProt, gnomAD v4, EuropePMC, 3D Hotspot Analysis, Interaction Network Analysis, AlphaFold DB, gnomAD constraint, Open Targets, PharmGKB, MaveDB, LitVar.
Notable RNF207 variants
Examples include S2W, S2A, S2*, S2L, S2S, G3A, G3*, G3R. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.
- S2W (p.Ser2Trp), rs927893945, ClinGen CA17165211, ClinVar RCV004354852, TOPMed rs927893945, REVEL 0.27, CADD 29.50, Uncertain significance, not specified
- S2A (p.Ser2Ala), gnomAD 1-6206539-T-G, REVEL 0.02, CADD 22.40
- S2* (p.Ser2Ter), gnomAD 1-6206540-C-A, CADD 37.00
- S2L (p.Ser2Leu), gnomAD 1-6206540-C-T, REVEL 0.16, CADD 27.40
- S2S (p.Ser2Ser), rs540245643, gnomAD 1-6206541-G-A, CADD 4.17
- G3A (p.Gly3Ala), 1000Genomes rs758226183, ExAC rs758226183, TOPMed rs758226183, gnomAD rs758226183, REVEL 0.33, CADD 27.40
- G3* (p.Gly3Ter), gnomAD 1-6206542-G-T, CADD 37.00
- G3R (p.Gly3Arg), gnomAD 1-6206542-G-A, REVEL 0.50, CADD 29.00
- G3V (p.Gly3Val), gnomAD 1-6206543-G-T, REVEL 0.49, CADD 29.30
- G3G (p.Gly3Gly), gnomAD 1-6206544-A-G, CADD 14.40
- A4G (p.Ala4Gly), ExAC rs763869142, gnomAD rs763869142, REVEL 0.06, CADD 19.80
- A4T (p.Ala4Thr), TOPMed rs1243619480, gnomAD rs1243619480, REVEL 0.06, CADD 24.40
- A4S (p.Ala4Ser), gnomAD 1-6206545-G-T, REVEL 0.08, CADD 22.80
- A4A (p.Ala4Ala), rs1667908966, gnomAD 1-6206547-T-C, CADD 12.20
- I5L (p.Ile5Leu), gnomAD 1-6206548-A-C, REVEL 0.10, CADD 22.70
- I5T (p.Ile5Thr), gnomAD 1-6206549-T-C, REVEL 0.10, CADD 26.80
- I5I (p.Ile5Ile), gnomAD 1-6206550-C-A, CADD 13.20
- I5M (p.Ile5Met), gnomAD 1-6206550-C-G, REVEL 0.10, CADD 24.10
- F6L (p.Phe6Leu), ExAC rs751553548, gnomAD rs751553548, REVEL 0.16, CADD 23.20, Uncertain significance, not specified
- F6V (p.Phe6Val), gnomAD 1-6206551-T-G, REVEL 0.19, CADD 28.20
- F6F (p.Phe6Phe), gnomAD 1-6206553-C-T, CADD 9.99
- G7R (p.Gly7Arg), ExAC rs757231400, TOPMed rs757231400, gnomAD rs757231400, REVEL 0.02, CADD 9.27
- G7W (p.Gly7Trp), ExAC rs757231400, TOPMed rs757231400, gnomAD rs757231400, REVEL 0.09, CADD 13.30
- G7G (p.Gly7Gly), gnomAD 1-6206556-G-T, CADD 9.07
- P8L (p.Pro8Leu), TOPMed rs1238732878, gnomAD rs1238732878, REVEL 0.08, CADD 22.40
- P8S (p.Pro8Ser), TOPMed rs910748567, gnomAD rs910748567, REVEL 0.01, CADD 12.90
- P8T (p.Pro8Thr), gnomAD 1-6206557-C-A, REVEL 0.04, CADD 16.00
- P8R (p.Pro8Arg), gnomAD 1-6206558-C-G, REVEL 0.10, CADD 23.30
- P8H (p.Pro8His), gnomAD 1-6206558-C-A, REVEL 0.09, CADD 23.40
- P8P (p.Pro8Pro), gnomAD 1-6206559-C-A, CADD 8.85
- L9Q (p.Leu9Gln), rs369840055, ESP rs369840055, ExAC rs369840055, TOPMed rs369840055, REVEL 0.09, CADD 22.90, Variant assessed as somatic; moderate impact.
- L9W (p.Leu9Trp), rs1313319721, gnomAD 1-6206556-GC-G, CADD 20.20
- L9L (p.Leu9Leu), rs780923710, gnomAD 1-6206560-C-T, CADD 10.90
- L9H (p.Leu9His), gnomAD 1-6206560-C-CA, CADD 24.60
- L9P (p.Leu9Pro), gnomAD 1-6206561-T-C, REVEL 0.09, CADD 16.80
- E10G (p.Glu10Gly), Ensembl rs2100918315, REVEL 0.03, CADD 23.00
- p.Glu10 Leu15del, rs1208851659, gnomAD 1-6206557-CCCCTGG, CADD 18.80
- E10K (p.Glu10Lys), gnomAD 1-6206563-G-A, REVEL 0.06, CADD 24.10
- E10* (p.Glu10Ter), gnomAD 1-6206563-G-T, CADD 41.00
- E10V (p.Glu10Val), gnomAD 1-6206564-A-T, REVEL 0.10, CADD 24.20
- E10E (p.Glu10Glu), gnomAD 1-6206565-G-A, CADD 11.20
- E10D (p.Glu10Asp), gnomAD 1-6206565-G-T, REVEL 0.01, CADD 19.10
- G11S (p.Gly11Ser), ExAC rs754991187, TOPMed rs754991187, gnomAD rs754991187, REVEL 0.05, CADD 20.50
- G11A (p.Gly11Ala), gnomAD 1-6206564-AG-A, CADD 24.70
- G11C (p.Gly11Cys), gnomAD 1-6206566-G-T, REVEL 0.07, CADD 25.70
- G11V (p.Gly11Val), gnomAD 1-6206567-G-T, REVEL 0.05, CADD 22.60
- G11D (p.Gly11Asp), gnomAD 1-6206567-G-A, REVEL 0.03, CADD 19.30
- G11G (p.Gly11Gly), gnomAD 1-6206568-C-A, CADD 11.00
- P12T (p.Pro12Thr), Ensembl rs1571195708
- P12A (p.Pro12Ala), gnomAD 1-6206569-C-G, REVEL 0.03, CADD 12.00
- P12S (p.Pro12Ser), gnomAD 1-6206569-C-T, REVEL 0.03, CADD 14.30
- P12R (p.Pro12Arg), gnomAD 1-6206570-C-G, REVEL 0.09, CADD 9.19
- P12P (p.Pro12Pro), gnomAD 1-6206571-G-T, CADD 6.83
- S13N (p.Ser13Asn), 1000Genomes rs201053260, ESP rs201053260, ExAC rs201053260, TOPMed rs201053260, REVEL 0.02, CADD 9.03
- S13I (p.Ser13Ile), gnomAD 1-6206573-G-T, REVEL 0.03, CADD 10.60
- S13R (p.Ser13Arg), gnomAD 1-6206574-C-A, REVEL 0.01, CADD 0.57
- S14Y (p.Ser14Tyr), gnomAD 1-6206576-C-A, REVEL 0.03, CADD 19.80
- S14S (p.Ser14Ser), gnomAD 1-6206577-C-T, CADD 9.94
- L15W (p.Leu15Trp), gnomAD 1-6206575-TC-T, CADD 22.90
- L15M (p.Leu15Met), gnomAD 1-6206578-C-A, REVEL 0.08, CADD 21.80
- L15L (p.Leu15Leu), gnomAD 1-6206580-G-T, CADD 10.20
- D16G (p.Asp16Gly), TOPMed rs1667910591, REVEL 0.20, CADD 24.20
- D16Y (p.Asp16Tyr), TOPMed rs1184064725, REVEL 0.31, CADD 27.10
- D16V (p.Asp16Val), gnomAD 1-6206582-A-T, REVEL 0.25, CADD 22.70
- D16D (p.Asp16Asp), gnomAD 1-6206583-T-C, CADD 8.26
- D16E (p.Asp16Glu), gnomAD 1-6206583-T-A, REVEL 0.04, CADD 9.36
- A17T (p.Ala17Thr), 1000Genomes rs142728754, ESP rs142728754, ExAC rs142728754, TOPMed rs142728754, REVEL 0.07, CADD 6.58, Uncertain significance, not specified
- A17P (p.Ala17Pro), gnomAD 1-6206584-G-C, REVEL 0.02, CADD 7.82
- A17D (p.Ala17Asp), gnomAD 1-6206585-C-A, REVEL 0.06, CADD 12.90
- A17V (p.Ala17Val), gnomAD 1-6206585-C-T, REVEL 0.06, CADD 13.40
- A17A (p.Ala17Ala), rs773727266, gnomAD 1-6206586-C-T, CADD 4.02
- P18R (p.Pro18Arg), Ensembl rs1557581067, REVEL 0.05, CADD 14.00
- P18S (p.Pro18Ser), Ensembl rs1667911130, REVEL 0.01, CADD 3.05
- P18P (p.Pro18Pro), rs747273582, gnomAD 1-6206589-G-A, CADD 3.61
- S19N (p.Ser19Asn), ExAC rs771503338, TOPMed rs771503338, gnomAD rs771503338, REVEL 0.02, CADD 15.50
- S19A (p.Ser19Ala), rs1557581081, gnomAD 1-6206588-CG-C, CADD 8.22
- S19R (p.Ser19Arg), gnomAD 1-6206590-A-C, REVEL 0.03, CADD 22.60
- S19I (p.Ser19Ile), gnomAD 1-6206591-G-T, REVEL 0.08, CADD 22.70
- S19S (p.Ser19Ser), gnomAD 1-6206592-C-T, CADD 10.50
- I20T (p.Ile20Thr), TOPMed rs1667911742, gnomAD rs1667911742, REVEL 0.05, CADD 17.60
- I20V (p.Ile20Val), gnomAD rs1362375438, REVEL 0.06, CADD 13.60
- I20I (p.Ile20Ile), rs1667911847, gnomAD 1-6206595-C-T, CADD 8.30
- H21Y (p.His21Tyr), gnomAD 1-6206596-C-T, REVEL 0.23, CADD 24.50
- H21N (p.His21Asn), gnomAD 1-6206596-C-A, REVEL 0.13, CADD 22.70
- H21Q (p.His21Gln), gnomAD 1-6206598-C-A, REVEL 0.13, CADD 23.90
- H21H (p.His21His), gnomAD 1-6206598-C-T, CADD 11.20
- P22A (p.Pro22Ala), gnomAD rs1361903684, REVEL 0.60, CADD 25.20
- P22L (p.Pro22Leu), gnomAD rs1667912066, REVEL 0.55, CADD 26.20
- P22S (p.Pro22Ser), gnomAD rs1361903684, REVEL 0.57, CADD 26.00
- P22P (p.Pro22Pro), gnomAD 1-6206601-G-T, CADD 11.60
- L23M (p.Leu23Met), gnomAD rs1433959860
- L23P (p.Leu23Pro), TOPMed rs1421131881, gnomAD rs1421131881, REVEL 0.92, CADD 31.00
- L23L (p.Leu23Leu), gnomAD 1-6206602-C-T, CADD 14.10
- V24L (p.Val24Leu), gnomAD 1-6206605-G-T, REVEL 0.42, CADD 22.30
- V24V (p.Val24Val), rs776958302, gnomAD 1-6206607-G-A, CADD 14.40
- C25Y (p.Cys25Tyr), rs373315370, ClinGen CA557639, ClinVar RCV004446604, ESP rs373315370, REVEL 0.99, CADD 30.00, Uncertain significance, not specified
- C25F (p.Cys25Phe), gnomAD 1-6206609-G-T, REVEL 0.99, CADD 31.00
- C25C (p.Cys25Cys), rs764692485, gnomAD 1-6206610-C-T, CADD 14.60
- P26L (p.Pro26Leu), ExAC rs775187088, TOPMed rs775187088, gnomAD rs775187088, REVEL 0.25, CADD 21.20
- P26Q (p.Pro26Gln), ExAC rs775187088, TOPMed rs775187088, gnomAD rs775187088, REVEL 0.48, CADD 23.40
- P26T (p.Pro26Thr), gnomAD 1-6206611-C-A, REVEL 0.38, CADD 20.90
- P26P (p.Pro26Pro), rs762578598, gnomAD 1-6206613-G-T, CADD 12.20
- L27P (p.Leu27Pro), ExAC rs751372134, TOPMed rs751372134, gnomAD rs751372134, REVEL 0.87, CADD 29.60
- L27L (p.Leu27Leu), rs763676774, gnomAD 1-6206614-C-T, CADD 14.80
- C28S (p.Cys28Ser), Ensembl rs1667913345
- C28F (p.Cys28Phe), gnomAD 1-6206618-G-T, REVEL 0.98, CADD 32.00
- C28C (p.Cys28Cys), rs1241111602, gnomAD 1-6206619-C-T, CADD 15.60
- p.His29 Cys63del, gnomAD 1-6206607-GTGCCCG, CADD 22.60
- H29Y (p.His29Tyr), gnomAD 1-6206620-C-T, REVEL 0.57, CADD 26.20
- H29N (p.His29Asn), gnomAD 1-6206620-C-A, REVEL 0.30, CADD 22.50
- H29H (p.His29His), rs756962071, gnomAD 1-6206622-C-T, CADD 13.10
- H29Q (p.His29Gln), gnomAD 1-6206622-C-A, REVEL 0.31, CADD 22.40
- V30L (p.Val30Leu), ExAC rs767540321, TOPMed rs767540321, gnomAD rs767540321, REVEL 0.21, CADD 16.70
- V30M (p.Val30Met), ExAC rs767540321, TOPMed rs767540321, gnomAD rs767540321, REVEL 0.28, CADD 21.90
- V30G (p.Val30Gly), gnomAD 1-6206622-C-CG, CADD 25.40
- V30V (p.Val30Val), gnomAD 1-6206625-G-T, CADD 6.63
- Q31E (p.Gln31Glu), TOPMed rs1223768788, gnomAD rs1223768788, REVEL 0.38, CADD 23.10
- Q31* (p.Gln31Ter), gnomAD 1-6206626-C-T, CADD 36.00
- Q31H (p.Gln31His), gnomAD 1-6206628-G-T, REVEL 0.46, CADD 23.80
- Y32P (p.Tyr32Pro), gnomAD 1-6206627-AGTACGA, CADD 29.50
- Y32H (p.Tyr32His), gnomAD 1-6206629-T-C, REVEL 0.75, CADD 29.10
- Y32* (p.Tyr32Ter), gnomAD 1-6206631-C-G, CADD 34.00
- Y32Y (p.Tyr32Tyr), gnomAD 1-6206631-C-T, CADD 9.82
- E33K (p.Glu33Lys), NCI-TCGA Cosmic COSV6500, REVEL 0.32, CADD 23.60, Variant assessed as somatic; moderate impact.
- E33Q (p.Glu33Gln), NCI-TCGA Cosmic COSV6500, REVEL 0.28, CADD 22.80, Variant assessed as somatic; moderate impact.
- E33* (p.Glu33Ter), gnomAD 1-6206632-G-T, CADD 36.00
- E33D (p.Glu33Asp), gnomAD 1-6206634-G-T, REVEL 0.35, CADD 23.60
- E33E (p.Glu33Glu), rs750298125, gnomAD 1-6206634-G-A, CADD 13.80
- R34H (p.Arg34His), rs140205685, ClinGen CA557648, ClinVar RCV004093754, ESP rs140205685, REVEL 0.13, CADD 20.40, Uncertain significance, not specified
- R34G (p.Arg34Gly), gnomAD 1-6206635-C-G, REVEL 0.26, CADD 22.20
- R34C (p.Arg34Cys), gnomAD 1-6206635-C-T, REVEL 0.23, CADD 23.20
- R34L (p.Arg34Leu), gnomAD 1-6206636-G-T, REVEL 0.24, CADD 22.40
- R34R (p.Arg34Arg), gnomAD 1-6206637-C-T, CADD 14.40
- P35L (p.Pro35Leu), TOPMed rs919519328, REVEL 0.83, CADD 24.10, Uncertain significance, not specified
- P35T (p.Pro35Thr), gnomAD 1-6206638-C-A, REVEL 0.90, CADD 26.70
- P35S (p.Pro35Ser), gnomAD 1-6206638-C-T, REVEL 0.90, CADD 27.50
- P35R (p.Pro35Arg), gnomAD 1-6206639-C-G, REVEL 0.90, CADD 26.30
- P35P (p.Pro35Pro), rs1176613154, gnomAD 1-6206640-G-A, CADD 12.50
- C36R (p.Cys36Arg), ExAC rs779124390, gnomAD rs779124390, REVEL 0.72, CADD 26.10
- C36Y (p.Cys36Tyr), TOPMed rs1667914410, gnomAD rs1667914410, REVEL 0.76, CADD 30.00
- C36L (p.Cys36Leu), gnomAD 1-6206640-G-GT, CADD 32.00
- C36F (p.Cys36Phe), gnomAD 1-6206642-G-T, REVEL 0.75, CADD 31.00
- C36C (p.Cys36Cys), gnomAD 1-6206643-T-C, CADD 15.70
- L37P (p.Leu37Pro), ExAC rs748183755, gnomAD rs748183755, REVEL 0.90, CADD 32.00
- L37L (p.Leu37Leu), gnomAD 1-6206646-T-C, CADD 14.40
- L38M (p.Leu38Met), gnomAD 1-6206647-C-A, REVEL 0.66, CADD 25.60
- L38Q (p.Leu38Gln), gnomAD 1-6206648-T-A, REVEL 0.93, CADD 32.00
- L38L (p.Leu38Leu), gnomAD 1-6206649-G-C, CADD 13.20
- D39E (p.Asp39Glu), rs1044246467, ClinGen CA17165357, ClinVar RCV004446592, TOPMed rs1044246467, REVEL 0.47, CADD 25.50, Uncertain significance, not specified
- D39G (p.Asp39Gly), gnomAD rs1159049015, REVEL 0.80, CADD 27.90
- D39N (p.Asp39Asn), gnomAD 1-6206650-G-A, REVEL 0.50, CADD 27.10
- D39Y (p.Asp39Tyr), gnomAD 1-6206650-G-T, REVEL 0.84, CADD 32.00
- D39D (p.Asp39Asp), gnomAD 1-6206652-C-T, CADD 14.70
- C40Y (p.Cys40Tyr), gnomAD 1-6206654-G-A, REVEL 0.98, CADD 31.00
- C40F (p.Cys40Phe), gnomAD 1-6206654-G-T, REVEL 0.98, CADD 32.00
- C40C (p.Cys40Cys), gnomAD 1-6206655-T-C, CADD 14.00
- F41L (p.Phe41Leu), ExAC rs778104467, gnomAD rs778104467, REVEL 0.44, CADD 23.50
- F41S (p.Phe41Ser), ExAC rs758702002, TOPMed rs758702002, gnomAD rs758702002, REVEL 0.42, CADD 25.50
- H42H (p.His42His), rs1667914993, gnomAD 1-6206661-C-T, CADD 11.20
- D43H (p.Asp43His), NCI-TCGA Cosmic COSV6500, Variant assessed as somatic; moderate impact.
- D43N (p.Asp43Asn), TOPMed rs929520404, REVEL 0.18, CADD 20.70
- D43Y (p.Asp43Tyr), TOPMed rs929520404, REVEL 0.44, CADD 25.50
- D43del (p.Asp43del), gnomAD 1-6206659-CACG-C, CADD 21.90
- D43D (p.Asp43Asp), rs1166216424, gnomAD 1-6206664-C-T, CADD 13.60
- D43E (p.Asp43Glu), gnomAD 1-6206664-C-A, REVEL 0.20, CADD 16.40
- F44L (p.Phe44Leu), gnomAD rs1350024016, REVEL 0.73, CADD 31.00
- F44F (p.Phe44Phe), gnomAD 1-6206667-C-T, CADD 13.40
- C45G (p.Cys45Gly), TOPMed rs902689014, gnomAD rs902689014, REVEL 0.96, CADD 28.90
- C45Y (p.Cys45Tyr), rs1312663755, ClinGen CA338068519, ClinVar RCV004446595, TOPMed rs1312663755, REVEL 0.96, CADD 28.30, Uncertain significance, not specified
- C45R (p.Cys45Arg), gnomAD 1-6206668-T-C, REVEL 0.96, CADD 29.60
- C45S (p.Cys45Ser), gnomAD 1-6206668-T-A, REVEL 0.96, CADD 27.80
- C45F (p.Cys45Phe), gnomAD 1-6206669-G-T, REVEL 0.97, CADD 28.70
- A46T (p.Ala46Thr), rs139333535, ClinGen CA557653, ClinVar RCV004253678, ESP rs139333535, REVEL 0.33, CADD 19.50, Uncertain significance, not specified
- A46V (p.Ala46Val), rs1212873448, TOPMed rs1212873448, REVEL 0.66, CADD 24.40, Variant assessed as somatic; moderate impact.
- A46S (p.Ala46Ser), gnomAD 1-6206671-G-T, REVEL 0.40, CADD 18.90
- A46A (p.Ala46Ala), gnomAD 1-6206673-C-T, CADD 12.80
- G47D (p.Gly47Asp), NCI-TCGA Cosmic COSV6500, REVEL 0.39, CADD 19.90, Variant assessed as somatic; moderate impact.
- G47S (p.Gly47Ser), TOPMed rs1238612497, gnomAD rs1238612497, REVEL 0.17, CADD 18.10
- G47C (p.Gly47Cys), gnomAD 1-6206674-G-T, REVEL 0.45, CADD 25.90
- G47V (p.Gly47Val), gnomAD 1-6206675-G-T, REVEL 0.48, CADD 22.90
Public RNF207 analysis runs
- RNF207 analysis run — RNF207 (1,083 variants) — completed 2026-07-17