CUL3 (Cullin-3) variants and mutations
CUL3 (also known as Cullin-3) is a human protein-coding gene encoding a cullin-3 protein. It serves as a scaffold for multiple ubiquitin-ligase complexes that control degradation of signaling and regulatory proteins, including components of the KEAP1-NRF2 pathway. Pathogenic variants can cause pseudohypoaldosteronism type II and neurodevelopmental disorders, while somatic alterations affect cancer signaling. This analysis covers 2,587 CUL3 variants and mutations. Of these, 27% have computational variant effect predictions. Disease context includes neurodevelopmental disorder with or without autism or seizures, pseudohypoaldosteronism type 2E, and neurodegenerative disease. Example CUL3 variants include S2*, S2W, and S2C.
Variant analysis overview
- Gene: CUL3
- Protein: Cullin-3
- UniProt accession: Q13618
- Organism: Homo sapiens
- Variants analyzed: 2587
- Variant scope: all variants
- Completed: 2026-08-22
Variant and mutation evidence
- Variant composition: 2,416 unspecified-consequence records; 1 stop lost; 108 synonymous variants; 44 missense variants; 4 splice-region variants; 2 in-frame deletions; 9 frameshift variants; 5 substitution
- Prediction scores: 702 variants have prediction scores (27% of the analyzed set).
Clinical, disease, and population context
- Disease context: 25 disease associations are represented. Top associations: neurodevelopmental disorder with or without autism or seizures, pseudohypoaldosteronism type 2E, neurodegenerative disease, hereditary disease, pseudohypoaldosteronism type 2A, complex neurodevelopmental disorder, pseudohypoaldosteronism type 2, schizophrenia, mathematical ability, autism spectrum disorder, papillary renal cell carcinoma, smoking initiation.
Protein structure and variant hotspots
- Protein features: 1 domains; 2 post-translational modification sites.
- Structural context: 275 variants have structural context.
- PTM context: 6 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, ClinGen, MaveDB, LitVar.
Notable CUL3 variants
Examples include S2*, S2W, S2C, N3S, S5I, S5N, S5R, K6N. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.
- S2* (p.Ser2Ter), NCI-TCGA Cosmic COSV5236, cosmic curated COSV52364, CADD 37.00, Variant assessed as somatic; high impact.
- S2W (p.Ser2Trp), Ensembl rs2106331384
- S2C (p.Ser2Cys), rs908831506, []
- N3S (p.Asn3Ser), TOPMed rs1695546313, REVEL 0.06, CADD 16.70
- S5I (p.Ser5Ile), TOPMed rs1009417526, gnomAD rs1009417526, REVEL 0.07, CADD 22.00
- S5N (p.Ser5Asn), TOPMed rs1009417526, gnomAD rs1009417526, REVEL 0.05, CADD 19.30
- S5R (p.Ser5Arg), Ensembl rs2106331366, REVEL 0.03, CADD 22.30
- K6N (p.Lys6Asn), NCI-TCGA Cosmic COSV5236, cosmic curated COSV52363, REVEL 0.04, CADD 21.50, Variant assessed as somatic; moderate impact.
- K6R (p.Lys6Arg), rs757582263, ClinGen CA2140349, ClinVar RCV002467086, ExAC rs757582263, REVEL 0.08, CADD 20.90, Uncertain significance, not provided
- G7D (p.Gly7Asp), TOPMed rs1695545965, REVEL 0.18, CADD 22.10
- G7V (p.Gly7Val), TOPMed rs1695545965, REVEL 0.09, CADD 21.70
- T8M (p.Thr8Met), TOPMed rs1034800855, gnomAD rs1034800855, REVEL 0.14, CADD 23.40, Uncertain significance, Pseudohypoaldosteronism type 2E; Neurodevelopmental disorder with or without aut
- T8P (p.Thr8Pro), gnomAD rs1323366532, REVEL 0.13, CADD 22.30
- G9D (p.Gly9Asp), Ensembl rs2106331339, REVEL 0.21, CADD 21.90
- G9R (p.Gly9Arg), Ensembl rs1695545721, CADD 2.45
- G9V (p.Gly9Val), Ensembl rs2106331339, REVEL 0.17, CADD 21.70
- S10G (p.Ser10Gly), TOPMed rs1695545689, REVEL 0.06, CADD 21.20
- S10R (p.Ser10Arg), Ensembl rs2106331331, REVEL 0.12, CADD 22.60, Likely benign, not provided
- R11G (p.Arg11Gly), 1000Genomes rs532085416, ExAC rs532085416, gnomAD rs532085416
- R11Q (p.Arg11Gln), Ensembl rs2106331327, REVEL 0.05, CADD 22.50, Uncertain significance, not provided
- R11W (p.Arg11Trp), 1000Genomes rs532085416, ExAC rs532085416, gnomAD rs532085416, REVEL 0.12, CADD 25.00
- D13G (p.Asp13Gly), Ensembl rs2106331319, REVEL 0.15, CADD 22.80
- D13H (p.Asp13His), rs2969802, UniProt VAR 017194, Ensembl rs2969802, AlphaMissense 0.94, MetaLR 0.26
- D13N (p.Asp13Asn), cosmic curated COSV52364, Ensembl rs2969802, REVEL 0.10, AlphaMissense 0.94
- T14A (p.Thr14Ala), gnomAD rs1449014217, REVEL 0.05, CADD 18.60
- T14N (p.Thr14Asn), ExAC rs767631760, gnomAD rs767631760, REVEL 0.08, CADD 20.20
- K15N (p.Lys15Asn), NCI-TCGA Cosmic COSV5236, cosmic curated COSV52363, NCI-TCGA Cosmic COSV5237, ExAC rs761689239, REVEL 0.07, CADD 21.60, Variant assessed as somatic; moderate impact.
- K15R (p.Lys15Arg), rs1335865703, ClinGen CA351131121, ClinVar RCV003080110, TOPMed rs1335865703, REVEL 0.08, CADD 22.30, Uncertain significance, not provided
- M16I (p.Met16Ile), NCI-TCGA TCGA novel, REVEL 0.06, CADD 22.10, Variant assessed as somatic; high impact.
- R17G (p.Arg17Gly), cosmic curated COSV99049, Ensembl rs866438129
- R17Q (p.Arg17Gln), Ensembl rs2106331302, REVEL 0.23, CADD 25.00
- R17W (p.Arg17Trp), Ensembl rs866438129, REVEL 0.47, CADD 30.00
- I18L (p.Ile18Leu), Ensembl rs2106331298
- I18M (p.Ile18Met), NCI-TCGA TCGA novel, Variant assessed as somatic; moderate impact.
- I18T (p.Ile18Thr), Ensembl rs2106331297, REVEL 0.29, CADD 26.00
- R19G (p.Arg19Gly), Ensembl rs2106331293
- R19Q (p.Arg19Gln), Ensembl rs2106331290, REVEL 0.12, CADD 23.20, Uncertain significance, not provided
- R19W (p.Arg19Trp), Ensembl rs2106331293, REVEL 0.46, CADD 29.10
- A20S (p.Ala20Ser), Ensembl rs2106331279, REVEL 0.13, CADD 23.20
- A20T (p.Ala20Thr), Ensembl rs2106331279, REVEL 0.17, CADD 23.90
- A20V (p.Ala20Val), TOPMed rs1695544914, REVEL 0.10, CADD 22.80, Uncertain significance, not provided
- F21L (p.Phe21Leu), NCI-TCGA Cosmic COSV1000, cosmic curated COSV10002, REVEL 0.11, CADD 26.40, Variant assessed as somatic; moderate impact.
- P22L (p.Pro22Leu), rs867746441, ClinGen CA66591319, ClinVar RCV003402484, Ensembl rs867746441, REVEL 0.17, CADD 24.30, Uncertain significance, CUL3-related disorder
- P22R (p.Pro22Arg), Ensembl rs867746441, NCI-TCGA TCGA novel, REVEL 0.20, CADD 24.30, Uncertain significance
- M23I (p.Met23Ile), Ensembl rs1574693229, REVEL 0.33, CADD 22.40
- M23K (p.Met23Lys), Ensembl rs2106305253, REVEL 0.47, CADD 25.20
- M23L (p.Met23Leu), Ensembl rs2106305263, REVEL 0.30, CADD 23.00
- M23R (p.Met23Arg), Ensembl rs2106305253
- M23T (p.Met23Thr), Ensembl rs2106305253
- T24A (p.Thr24Ala), gnomAD rs1694762152, REVEL 0.21, CADD 21.80
- T24I (p.Thr24Ile), Ensembl rs2106305230, REVEL 0.33, CADD 23.90
- T24N (p.Thr24Asn), Ensembl rs2106305230, REVEL 0.19, CADD 21.90
- T24S (p.Thr24Ser), gnomAD rs1694762152, REVEL 0.18, CADD 19.00
- M25I (p.Met25Ile), Ensembl rs2106305193, cosmic curated COSV52367
- M25K (p.Met25Lys), gnomAD rs1382194775, Uncertain significance
- M25R (p.Met25Arg), gnomAD rs1382194775, Uncertain significance
- M25T (p.Met25Thr), rs1382194775, ClinGen CA351131040, ClinVar RCV001847461, gnomAD rs1382194775, AlphaMissense 0.99, MetaLR 0.28, Uncertain significance, not provided
- D26E (p.Asp26Glu), Ensembl rs2106305178
- D26H (p.Asp26His), Ensembl rs2106305184, REVEL 0.53, CADD 27.30, Uncertain significance, not provided
- D26N (p.Asp26Asn), Ensembl rs2106305184, REVEL 0.38, CADD 27.30, Uncertain significance
- D26Y (p.Asp26Tyr), rs2106305184, ClinGen CA351131033, NCI-TCGA Cosmic COSV5236, cosmic curated COSV52368, REVEL 0.56, CADD 27.90, Uncertain significance, not provided
- E27* (p.Glu27Ter), Ensembl rs2106305165, CADD 36.00
- E27K (p.Glu27Lys), cosmic curated COSV52366, Ensembl rs2106305165, REVEL 0.56, CADD 25.00
- E27Q (p.Glu27Gln), Ensembl rs2106305165
- K28I (p.Lys28Ile), Ensembl rs2106305157
- Y29* (p.Tyr29Ter), ExAC rs769172836, TOPMed rs769172836
- Y29C (p.Tyr29Cys), Ensembl rs1694761916
- Y29F (p.Tyr29Phe), Ensembl rs1694761916, REVEL 0.32, CADD 22.80
- Y29H (p.Tyr29His), NCI-TCGA Cosmic COSV5236, cosmic curated COSV52368, Variant assessed as somatic; moderate impact.
- V30E (p.Val30Glu), Ensembl rs2106305104, REVEL 0.78, CADD 24.90
- V30G (p.Val30Gly), Ensembl rs2106305104
- V30I (p.Val30Ile), Ensembl rs1694761642
- V30L (p.Val30Leu), NCI-TCGA Cosmic COSV5236, cosmic curated COSV52369, Ensembl rs1694761642, REVEL 0.43, CADD 23.20, Variant assessed as somatic; moderate impact.
- N31H (p.Asn31His), NCI-TCGA Cosmic COSV5237, cosmic curated COSV52370, Variant assessed as somatic; moderate impact.
- N31I (p.Asn31Ile), Ensembl rs2106305076
- N31Y (p.Asn31Tyr), Ensembl rs2106305092
- S32I (p.Ser32Ile), Ensembl rs1694761235
- S32N (p.Ser32Asn), Ensembl rs1694761235
- S32R (p.Ser32Arg), Ensembl rs2106305054, REVEL 0.14, CADD 22.50
- S32T (p.Ser32Thr), Ensembl rs1694761235
- S32A (p.Ser32Ala), rs780471397, []
- I33F (p.Ile33Phe), Ensembl rs2106305043
- W34* (p.Trp34Ter), Ensembl rs2106305030
- W34C (p.Trp34Cys), Ensembl rs2106305030
- W34L (p.Trp34Leu), Ensembl rs2106305033
- W34S (p.Trp34Ser), Ensembl rs2106305033
- D35E (p.Asp35Glu), gnomAD rs1165128140, REVEL 0.32, CADD 14.20
- D35G (p.Asp35Gly), ExAC rs749470638, gnomAD rs749470638, REVEL 0.29, CADD 23.00
- D35H (p.Asp35His), Ensembl rs2106305023
- D35N (p.Asp35Asn), Ensembl rs2106305023
- D35Y (p.Asp35Tyr), Ensembl rs2106305023
- L36F (p.Leu36Phe), Ensembl rs2106304995
- L36I (p.Leu36Ile), Ensembl rs2106304995, Uncertain significance, Pseudohypoaldosteronism type 2E
- L36P (p.Leu36Pro), Ensembl rs2106304991
- L36V (p.Leu36Val), Ensembl rs2106304995
- L37M (p.Leu37Met), Ensembl rs2106304974
- L37P (p.Leu37Pro), Ensembl rs2106304961
- L37Q (p.Leu37Gln), Ensembl rs2106304961
- L37V (p.Leu37Val), Ensembl rs2106304974
- N39H (p.Asn39His), rs2469185181, ClinGen CA351130939, ClinVar RCV003087072, Uncertain significance, not provided
- N39M (p.Asn39Met), NCI-TCGA TCGA novel, Variant assessed as somatic; high impact.
- A40G (p.Ala40Gly), Ensembl rs2106304940
- A40P (p.Ala40Pro), ExAC rs780471397, gnomAD rs780471397
- A40S (p.Ala40Ser), ExAC rs780471397, gnomAD rs780471397, REVEL 0.50, CADD 25.30
- A40T (p.Ala40Thr), ExAC rs780471397, gnomAD rs780471397
- A40V (p.Ala40Val), NCI-TCGA TCGA novel, Ensembl rs2106304940, Variant assessed as somatic; moderate impact.
- I41N (p.Ile41Asn), Ensembl rs1694760468, Uncertain significance, not provided
- I41S (p.Ile41Ser), Ensembl rs1694760468
- I41V (p.Ile41Val), ExAC rs756230201, gnomAD rs756230201, REVEL 0.44, CADD 25.40, Uncertain significance, Pseudohypoaldosteronism type 2E; Neurodevelopmental disorder with or without aut
- Q42* (p.Gln42Ter), NCI-TCGA Cosmic COSV1000, NCI-TCGA Cosmic COSV5236, cosmic curated COSV52367, Ensembl rs2106304909, Variant assessed as somatic; high impact.
- Q42E (p.Gln42Glu), NCI-TCGA Cosmic COSV1000, cosmic curated COSV10002, NCI-TCGA Cosmic COSV5236, Ensembl rs2106304909, REVEL 0.36, CADD 23.80, Variant assessed as somatic; moderate impact.
- Q42H (p.Gln42His), Ensembl rs1694760276
- Q42L (p.Gln42Leu), TOPMed rs1305579931
- Q42R (p.Gln42Arg), TOPMed rs1305579931
- E43D (p.Glu43Asp), Ensembl rs2106304880, CADD 1.84
- E43K (p.Glu43Lys), NCI-TCGA Cosmic COSV5237, cosmic curated COSV52370, Ensembl rs2106304888, Variant assessed as somatic; moderate impact.
- E43Q (p.Glu43Gln), Ensembl rs2106304888
- I44M (p.Ile44Met), gnomAD rs1212518379, REVEL 0.44, CADD 23.90
- I44V (p.Ile44Val), Ensembl rs2106304873
- Q45* (p.Gln45Ter), Ensembl rs2106304858
- Q45E (p.Gln45Glu), Ensembl rs2106304858
- Q45H (p.Gln45His), Ensembl rs2106304847
- Q45K (p.Gln45Lys), Ensembl rs2106304858
- R46C (p.Arg46Cys), ExAC rs745998120, gnomAD rs745998120, REVEL 0.29, CADD 31.00
- R46G (p.Arg46Gly), ExAC rs745998120, gnomAD rs745998120
- R46H (p.Arg46His), rs190453078, ClinGen CA2140260, ClinVar RCV002598952, ClinVar RCV005028243, REVEL 0.24, CADD 24.40, Conflicting interpretations, not specified; Pseudohypoaldosteronism type 2E; Neurodevelopmental disorder with
- R46L (p.Arg46Leu), 1000Genomes rs190453078, ExAC rs190453078, gnomAD rs190453078, Likely benign
- R46P (p.Arg46Pro), 1000Genomes rs190453078, ExAC rs190453078, gnomAD rs190453078, Likely benign
- R46S (p.Arg46Ser), ExAC rs745998120, gnomAD rs745998120, REVEL 0.23, CADD 23.30
- K47E (p.Lys47Glu), Ensembl rs2106304815
- K47M (p.Lys47Met), Ensembl rs2106304808
- K47N (p.Lys47Asn), Ensembl rs2106304797
- N48H (p.Asn48His), NCI-TCGA Cosmic COSV5236, Variant assessed as somatic; moderate impact.
- N48I (p.Asn48Ile), Ensembl rs2106304790
- N48K (p.Asn48Lys), Ensembl rs2106304784
- N48Y (p.Asn48Tyr), NCI-TCGA Cosmic COSV5236, cosmic curated COSV52366, Variant assessed as somatic; moderate impact.
- N49I (p.Asn49Ile), Ensembl rs2106304773
- N49K (p.Asn49Lys), Ensembl rs2106304768
- N49S (p.Asn49Ser), Ensembl rs2106304773
- N49T (p.Asn49Thr), Ensembl rs2106304773
- S50G (p.Ser50Gly), Ensembl rs2106304761
- S50I (p.Ser50Ile), cosmic curated COSV52367, Ensembl rs2106304752
- S50N (p.Ser50Asn), NCI-TCGA Cosmic COSV5236, NCI-TCGA Cosmic COSV5237, cosmic curated COSV52370, Ensembl rs2106304752, Variant assessed as somatic; moderate impact.
- S50R (p.Ser50Arg), Ensembl rs2106304741
- S50T (p.Ser50Thr), Ensembl rs2106304752
- G51C (p.Gly51Cys), Ensembl rs2106304724
- G51R (p.Gly51Arg), Ensembl rs2106304724
- G51S (p.Gly51Ser), Ensembl rs2106304724, REVEL 0.47, CADD 24.40
- L52F (p.Leu52Phe), NCI-TCGA Cosmic COSV5236, Ensembl rs2106304711, Variant assessed as somatic; moderate impact.
- L52H (p.Leu52His), Ensembl rs2106304708
- L52I (p.Leu52Ile), Ensembl rs2106304711
- L52V (p.Leu52Val), NCI-TCGA Cosmic COSV5236, cosmic curated COSV52368, Variant assessed as somatic; moderate impact.
- S53C (p.Ser53Cys), Ensembl rs2106304699
- S53G (p.Ser53Gly), Ensembl rs2106304699
- S53I (p.Ser53Ile), NCI-TCGA Cosmic COSV1000, cosmic curated COSV10002, Ensembl rs2106304690, Variant assessed as somatic; moderate impact.
- S53R (p.Ser53Arg), Ensembl rs2106304679, NCI-TCGA Cosmic COSV5236, cosmic curated COSV52366, Variant assessed as somatic; moderate impact.
- S53T (p.Ser53Thr), Ensembl rs2106304690
- F54C (p.Phe54Cys), Ensembl rs2106304673
- E55* (p.Glu55Ter), ExAC rs758208528, gnomAD rs758208528, CADD 36.00
- E55D (p.Glu55Asp), Ensembl rs2106304650
- E55K (p.Glu55Lys), ExAC rs758208528, gnomAD rs758208528
- E55Q (p.Glu55Gln), cosmic curated COSV52372, ExAC rs758208528, gnomAD rs758208528
- E55V (p.Glu55Val), Ensembl rs2106304658
- E56* (p.Glu56Ter), Ensembl rs2106304646
- E56D (p.Glu56Asp), cosmic curated COSV52370, 1000Genomes rs558688318, ExAC rs558688318, gnomAD rs558688318, Likely benign
- E56K (p.Glu56Lys), Ensembl rs2106304646
- E56Q (p.Glu56Gln), Ensembl rs2106304646
- E56V (p.Glu56Val), Ensembl rs2106304641
- L57F (p.Leu57Phe), Ensembl rs2106304624
- L57H (p.Leu57His), Ensembl rs2106304617
- L57V (p.Leu57Val), Ensembl rs2106304624
- Y58* (p.Tyr58Ter), Ensembl rs2106304571
- Y58C (p.Tyr58Cys), rs1553535841, ClinGen CA351130799, ClinVar RCV000677281, ClinVar RCV000987041, REVEL 0.87, CADD 29.20, Pathogenic/Likely pathogenic, not provided; Autosomal dominant pseudohypoaldosteronism type 1
- Y58F (p.Tyr58Phe), Ensembl rs1553535841, Pathogenic
- Y58H (p.Tyr58His), Ensembl rs2106304594
- Y58N (p.Tyr58Asn), Ensembl rs2106304594
- Y58S (p.Tyr58Ser), Ensembl rs1553535841, Pathogenic
- R59G (p.Arg59Gly), NCI-TCGA TCGA novel, Variant assessed as somatic; moderate impact.
- R59I (p.Arg59Ile), Ensembl rs2106304562
- R59K (p.Arg59Lys), Ensembl rs2106304562
Public CUL3 analysis runs
- CUL3 analysis run — CUL3 (2,587 variants) — completed 2026-08-22