ARID1A (O14497) variants and mutations
ARID1A (also known as O14497) is a human protein-coding gene encoding an AT-rich interactive domain-containing protein 1A protein. It helps BAF chromatin-remodeling complexes open or reposition nucleosomes at regulatory regions and thereby control lineage-specific transcription. Somatic loss is frequent in several cancers, while germline haploinsufficiency can cause Coffin-Siris syndrome. This analysis covers 10,785 ARID1A variants and mutations. Of these, 43% have computational variant effect predictions. Disease context includes intellectual disability, autosomal dominant 14, Coffin-Siris syndrome, and urinary bladder cancer. Example ARID1A variants include M1R, M1T, and A2S.
Variant analysis overview
- Gene: ARID1A
- Protein: O14497
- UniProt accession: O14497
- Organism: Homo sapiens
- Variants analyzed: 10785
- Variant scope: all variants
- Completed: 2026-08-18
Variant and mutation evidence
- Variant composition: 10,439 unspecified-consequence records; 168 missense variants; 90 synonymous variants; 39 frameshift variants; 9 stop-gained variants; 26 in-frame deletions; 12 in-frame insertions; 2 substitution
- Prediction scores: 4,640 variants have prediction scores (43% of the analyzed set).
Clinical, disease, and population context
- Disease context: 25 disease associations are represented. Top associations: intellectual disability, autosomal dominant 14, Coffin-Siris syndrome, urinary bladder cancer, hepatocellular carcinoma, urinary bladder carcinoma, gastric adenocarcinoma, colorectal adenocarcinoma, breast adenocarcinoma, neurodegenerative disease, Coffin-Siris syndrome 1, pancreatic adenocarcinoma, esophageal cancer.
Protein structure and variant hotspots
- Protein features: 1 domains; 27 post-translational modification sites.
- Structural context: 419 variants have structural context.
- PTM context: 127 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 ARID1A variants
Examples include M1R, M1T, A2S, A2T, A2V, A2D, A2A, A3G. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.
- M1R (p.Met1Arg), rs2124739661, ClinGen CA339264178, ClinVar RCV002853169, MetaLR 0.03, MetaSVM -1.18, Uncertain significance, not provided
- M1T (p.Met1Thr), rs2124739661, ClinGen CA339264177, ClinVar RCV001564646, MetaLR 0.03, MetaSVM -1.18, Uncertain significance, not provided
- A2S (p.Ala2Ser), TOPMed rs1333869920, gnomAD rs1333869920, REVEL 0.05, MetaLR 0.02, Uncertain significance
- A2T (p.Ala2Thr), rs1333869920, ClinGen CA339264182, ClinVar RCV002288160, TOPMed rs1333869920, REVEL 0.05, MetaLR 0.01, Uncertain significance, not provided
- A2V (p.Ala2Val), Ensembl rs2124739681, REVEL 0.06, MetaLR 0.01
- A2D (p.Ala2Asp), gnomAD 1-26696408-C-A, REVEL 0.16, CADD 25.10
- A2A (p.Ala2Ala), gnomAD 1-26696409-C-A, CADD 14.20
- A3G (p.Ala3Gly), Ensembl rs2124739693, REVEL 0.06, MetaLR 0.01
- A3V (p.Ala3Val), cosmic curated COSV99081, Ensembl rs2124739693, REVEL 0.07, MetaLR 0.01
- A3P (p.Ala3Pro), gnomAD 1-26696410-G-C, REVEL 0.13, CADD 25.80
- A3T (p.Ala3Thr), gnomAD 1-26696410-G-A, REVEL 0.05, CADD 23.70
- A3S (p.Ala3Ser), gnomAD 1-26696410-G-T, REVEL 0.06, CADD 23.30
- A3E (p.Ala3Glu), gnomAD 1-26696411-C-A, REVEL 0.09, CADD 23.20
- A3A (p.Ala3Ala), rs1376608214, gnomAD 1-26696412-G-T, CADD 14.20
- Q4H (p.Gln4His), cosmic curated COSV10520, NCI-TCGA TCGA novel, TOPMed rs2080253049, REVEL 0.06, MetaLR 0.01, Variant assessed as somatic; moderate impact.
- Q4K (p.Gln4Lys), Ensembl rs2124739705, REVEL 0.06, MetaLR 0.01
- Q4L (p.Gln4Leu), Ensembl rs2124739709, REVEL 0.04, MetaLR 0.01
- Q4R (p.Gln4Arg), Ensembl rs2124739709, REVEL 0.06, MetaLR 0.01
- Q4E (p.Gln4Glu), gnomAD 1-26696413-C-G, REVEL 0.08, CADD 22.80
- Q4* (p.Gln4Ter), gnomAD 1-26696413-C-T, CADD 36.00
- Q4P (p.Gln4Pro), gnomAD 1-26696414-A-C, REVEL 0.15, CADD 23.50
- Q4Q (p.Gln4Gln), gnomAD 1-26696415-G-A, CADD 12.10
- V5A (p.Val5Ala), cosmic curated COSV10520, Ensembl rs2124739727, REVEL 0.04, MetaLR 0.01
- V5D (p.Val5Asp), Ensembl rs2124739727, REVEL 0.11, MetaLR 0.01
- V5G (p.Val5Gly), Ensembl rs2124739727, REVEL 0.09, MetaLR 0.01
- V5I (p.Val5Ile), rs960279959, ClinGen CA19640271, ClinVar RCV003858460, TOPMed rs960279959, REVEL 0.04, MetaLR 0.01, Uncertain significance, not provided
- V5L (p.Val5Leu), TOPMed rs960279959, gnomAD rs960279959, REVEL 0.03, MetaLR 0.01, Uncertain significance
- V5P (p.Val5Pro), gnomAD 1-26696411-CGCAGG, CADD 26.60
- V5F (p.Val5Phe), gnomAD 1-26696416-G-T, REVEL 0.09, MetaLR 0.01
- V5V (p.Val5Val), rs1417092122, gnomAD 1-26696418-C-A, CADD 12.70
- A6?, NCI-TCGA TCGA novel, Variant assessed as somatic; high impact.
- A6D (p.Ala6Asp), Ensembl rs2124739740, REVEL 0.14, MetaLR 0.01
- A6G (p.Ala6Gly), Ensembl rs2124739740, MetaLR 0.01, MetaSVM -0.72
- A6T (p.Ala6Thr), gnomAD 1-26696419-G-A, REVEL 0.07, MetaLR 0.01
- A6P (p.Ala6Pro), gnomAD 1-26696419-G-C, REVEL 0.10, MetaLR 0.02
- A6S (p.Ala6Ser), gnomAD 1-26696419-G-T, REVEL 0.04, MetaLR 0.01
- A6V (p.Ala6Val), gnomAD 1-26696420-C-T, REVEL 0.04, MetaLR 0.01
- A6A (p.Ala6Ala), gnomAD 1-26696421-C-T, CADD 14.40
- P7L (p.Pro7Leu), Ensembl rs2124739753, REVEL 0.04, MetaLR 0.00
- P7S (p.Pro7Ser), ExAC rs762713857, TOPMed rs762713857, REVEL 0.07, MetaLR 0.00
- P7A (p.Pro7Ala), gnomAD 1-26696417-TCGCCC, CADD 26.50
- P7T (p.Pro7Thr), gnomAD 1-26696418-CGCCCC, CADD 26.70
- P7H (p.Pro7His), gnomAD 1-26696423-C-A, REVEL 0.05, MetaLR 0.01
- P7P (p.Pro7Pro), gnomAD 1-26696424-C-G, CADD 13.90
- A8D (p.Ala8Asp), Ensembl rs2124739772, REVEL 0.10, MetaLR 0.01
- A8G (p.Ala8Gly), cosmic curated COSV10885, Ensembl rs2124739772, REVEL 0.10, MetaLR 0.01
- A8P (p.Ala8Pro), gnomAD rs1296885198, REVEL 0.14, MetaLR 0.01, Uncertain significance
- A8S (p.Ala8Ser), gnomAD rs1296885198, REVEL 0.09, MetaLR 0.01, Uncertain significance
- A8T (p.Ala8Thr), rs1296885198, ClinGen CA339264219, ClinVar RCV001774362, ClinVar RCV005415619, REVEL 0.11, MetaLR 0.01, Uncertain significance, not provided; Intellectual disability, autosomal dominant 14
- A8R (p.Ala8Arg), rs1557568922, gnomAD 1-26696419-G-GC, CADD 25.70
- p.Ala8 Pro17del, gnomAD 1-26696421-CCCCGC, CADD 19.50
- A8V (p.Ala8Val), gnomAD 1-26696426-C-T, REVEL 0.11, MetaLR 0.00
- A8A (p.Ala8Ala), rs1339353653, gnomAD 1-26696427-C-A, CADD 14.20
- A9D (p.Ala9Asp), TOPMed rs1271598449, gnomAD rs1271598449, REVEL 0.04, MetaLR 0.01
- A9P (p.Ala9Pro), gnomAD rs1400046660, REVEL 0.04, MetaLR 0.01
- A9S (p.Ala9Ser), gnomAD rs1400046660, REVEL 0.05, MetaLR 0.00
- A9T (p.Ala9Thr), gnomAD rs1400046660, REVEL 0.05, MetaLR 0.01
- A9V (p.Ala9Val), TOPMed rs1271598449, gnomAD rs1271598449, REVEL 0.04, MetaLR 0.01
- A9A (p.Ala9Ala), gnomAD 1-26696430-C-G, CADD 14.50
- A10del (p.Ala10del), rs1557568946, gnomAD 1-26696422-CCCG-C, CADD 19.10
- p.Ala10dup, rs1557568946, gnomAD 1-26696422-C-CCCG, CADD 18.90
- A10L (p.Ala10Leu), gnomAD 1-26696422-CCCGCC, CADD 26.50
- A10Q (p.Ala10Gln), gnomAD 1-26696429-CCG-C, CADD 25.70
- A10P (p.Ala10Pro), gnomAD 1-26696430-CG-C, CADD 25.60
- A10S (p.Ala10Ser), gnomAD 1-26696431-G-T, REVEL 0.03, MetaLR 0.01
- A10T (p.Ala10Thr), gnomAD 1-26696431-G-A, REVEL 0.07, MetaLR 0.00
- A10D (p.Ala10Asp), gnomAD 1-26696432-C-A, REVEL 0.07, MetaLR 0.01
- A10G (p.Ala10Gly), gnomAD 1-26696432-C-G, REVEL 0.05, MetaLR 0.01
- A10V (p.Ala10Val), gnomAD 1-26696432-C-T, REVEL 0.06, MetaLR 0.01
- A10A (p.Ala10Ala), gnomAD 1-26696433-C-A, CADD 14.50
- S11G (p.Ser11Gly), TOPMed rs1234998421, gnomAD rs1234998421, REVEL 0.08, MetaLR 0.01
- S11T (p.Ser11Thr), Ensembl rs2124739811, REVEL 0.13, MetaLR 0.00
- S11A (p.Ser11Ala), rs797045262, gnomAD 1-26696421-CCCCGC, CADD 26.70
- S11L (p.Ser11Leu), gnomAD 1-26696422-CCCGCC, CADD 26.60
- p.Ser11 Ser12del, gnomAD 1-26696432-CCAGCA, CADD 19.80
- S11C (p.Ser11Cys), gnomAD 1-26696434-A-T, REVEL 0.07, MetaLR 0.01
- S11I (p.Ser11Ile), gnomAD 1-26696434-AGCAGC, CADD 26.50
- S11N (p.Ser11Asn), gnomAD 1-26696435-G-A, REVEL 0.10, MetaLR 0.01
- S11R (p.Ser11Arg), gnomAD 1-26696436-C-A, REVEL 0.07, MetaLR 0.01
- S11S (p.Ser11Ser), rs2124739815, gnomAD 1-26696436-C-T, CADD 14.40
- S12G (p.Ser12Gly), TOPMed rs2080253464, REVEL 0.13, MetaLR 0.01
- S12I (p.Ser12Ile), TOPMed rs1342376116, gnomAD rs1342376116, REVEL 0.07, MetaLR 0.01
- S12N (p.Ser12Asn), TOPMed rs1342376116, gnomAD rs1342376116, REVEL 0.09, MetaLR 0.00
- S12R (p.Ser12Arg), TOPMed rs2080253464, REVEL 0.08, MetaLR 0.01
- S12T (p.Ser12Thr), TOPMed rs1342376116, gnomAD rs1342376116, REVEL 0.09, MetaLR 0.00
- p.Ser12dup, gnomAD 1-26696432-C-CCAG, CADD 19.60
- S12S (p.Ser12Ser), rs112262001, gnomAD 1-26696439-C-T, CADD 14.40
- L13V (p.Leu13Val), TOPMed rs1343731961, gnomAD rs1343731961, REVEL 0.07, MetaLR 0.01
- L13L (p.Leu13Leu), rs1343731961, gnomAD 1-26696440-C-T, CADD 12.40
- L13M (p.Leu13Met), gnomAD 1-26696440-C-A, REVEL 0.09, MetaLR 0.01
- L13R (p.Leu13Arg), gnomAD 1-26696441-T-G, REVEL 0.12, MetaLR 0.01
- L13Q (p.Leu13Gln), gnomAD 1-26696441-T-A, REVEL 0.14, MetaLR 0.01
- L13P (p.Leu13Pro), gnomAD 1-26696441-T-C, REVEL 0.12, MetaLR 0.01
- G14D (p.Gly14Asp), gnomAD rs1278981030, REVEL 0.14, MetaLR 0.01
- G14S (p.Gly14Ser), cosmic curated COSV10965, TOPMed rs1270640436, REVEL 0.13, MetaLR 0.00
- G14A (p.Gly14Ala), gnomAD 1-26696441-TG-T, CADD 24.50
- G14R (p.Gly14Arg), gnomAD 1-26696443-G-C, REVEL 0.13, MetaLR 0.01
- G14C (p.Gly14Cys), gnomAD 1-26696443-G-T, REVEL 0.14, MetaLR 0.01
- G14V (p.Gly14Val), gnomAD 1-26696444-G-T, REVEL 0.13, MetaLR 0.00
- G14G (p.Gly14Gly), rs2124739865, gnomAD 1-26696445-C-A, CADD 13.90
- N15H (p.Asn15His), Ensembl rs2124739869, MetaLR 0.00, MetaSVM -0.66
- N15A (p.Asn15Ala), gnomAD 1-26696442-GGGCAA, CADD 27.20
- N15D (p.Asn15Asp), gnomAD 1-26696446-A-G, REVEL 0.09, MetaLR 0.00
- N15Y (p.Asn15Tyr), gnomAD 1-26696446-A-T, REVEL 0.10, MetaLR 0.00
- N15S (p.Asn15Ser), gnomAD 1-26696447-A-G, REVEL 0.12, MetaLR 0.00
- N15K (p.Asn15Lys), gnomAD 1-26696448-C-A, REVEL 0.05, MetaLR 0.00
- N15N (p.Asn15Asn), gnomAD 1-26696448-C-T, CADD 13.60
- P16A (p.Pro16Ala), TOPMed rs1349772934, gnomAD rs1349772934, REVEL 0.05, MetaLR 0.01
- P16L (p.Pro16Leu), TOPMed rs2080253801, REVEL 0.09, MetaLR 0.00
- P16S (p.Pro16Ser), cosmic curated COSV61383, TOPMed rs1349772934, gnomAD rs1349772934, REVEL 0.05, MetaLR 0.01
- P16T (p.Pro16Thr), TOPMed rs1349772934, gnomAD rs1349772934, REVEL 0.07, MetaLR 0.01
- P16R (p.Pro16Arg), gnomAD 1-26696447-AC-A, CADD 25.60
- P16Q (p.Pro16Gln), gnomAD 1-26696450-C-A, REVEL 0.09, MetaLR 0.01
- P16P (p.Pro16Pro), gnomAD 1-26696451-G-T, CADD 12.90
- P17A (p.Pro17Ala), Ensembl rs2124739909, REVEL 0.10, MetaLR 0.01
- P17L (p.Pro17Leu), NCI-TCGA TCGA novel, REVEL 0.09, MetaLR 0.01, Variant assessed as somatic; moderate impact.
- P17S (p.Pro17Ser), Ensembl rs2124739909, REVEL 0.10, MetaLR 0.01
- P17T (p.Pro17Thr), cosmic curated COSV61388, Ensembl rs2124739909, REVEL 0.09, MetaLR 0.01
- P17R (p.Pro17Arg), gnomAD 1-26696450-CG-C, CADD 23.50
- P17Q (p.Pro17Gln), gnomAD 1-26696453-C-A, REVEL 0.09, MetaLR 0.01
- P17P (p.Pro17Pro), rs2080253851, gnomAD 1-26696454-G-A, CADD 14.90
- P18A (p.Pro18Ala), gnomAD rs1262356135, MetaLR 0.00, MetaSVM -0.85
- P18S (p.Pro18Ser), gnomAD rs1262356135, REVEL 0.15, MetaLR 0.01
- P18T (p.Pro18Thr), gnomAD rs1262356135, REVEL 0.12, MetaLR 0.01
- P18C (p.Pro18Cys), gnomAD 1-26696453-C-CTT, CADD 27.60
- P18R (p.Pro18Arg), gnomAD 1-26696456-C-G, REVEL 0.09, MetaLR 0.01
- P18Q (p.Pro18Gln), gnomAD 1-26696456-C-A, REVEL 0.10, MetaLR 0.01
- P18L (p.Pro18Leu), gnomAD 1-26696456-C-T, REVEL 0.10, MetaLR 0.01
- P18P (p.Pro18Pro), rs1488028736, gnomAD 1-26696457-G-A, CADD 14.90
- P19A (p.Pro19Ala), rs2080253926, ClinGen CA339264291, ClinVar RCV001090915, Ensembl rs2080253926, AlphaMissense 0.10, MetaLR 0.00, Uncertain significance, not provided
- P19L (p.Pro19Leu), Ensembl rs2124739935, REVEL 0.07, MetaLR 0.00
- P19Q (p.Pro19Gln), Ensembl rs2124739935, REVEL 0.05, MetaLR 0.00
- P19S (p.Pro19Ser), Ensembl rs2080253926, REVEL 0.07, AlphaMissense 0.10, Uncertain significance
- p.Pro19 Pro21del, gnomAD 1-26696448-CCCGCC, CADD 20.10
- P19T (p.Pro19Thr), gnomAD 1-26696458-C-A, REVEL 0.05, MetaLR 0.00
- P19R (p.Pro19Arg), gnomAD 1-26696459-C-G, REVEL 0.06, MetaLR 0.00
- P19P (p.Pro19Pro), rs1193944607, gnomAD 1-26696460-G-C, CADD 14.60
- P20L (p.Pro20Leu), gnomAD rs2080253986, REVEL 0.03, MetaLR 0.01
- P20Q (p.Pro20Gln), gnomAD rs2080253986, REVEL 0.03, MetaLR 0.01
- P20S (p.Pro20Ser), Ensembl rs2124739947, REVEL 0.10, MetaLR 0.01
- P20A (p.Pro20Ala), gnomAD 1-26696421-C-CCCC, CADD 25.30
- p.Pro20 Pro21del, rs748085214, gnomAD 1-26696448-CCCGCC, CADD 20.20
- P20R (p.Pro20Arg), gnomAD 1-26696459-CG-C, CADD 26.90
- P20T (p.Pro20Thr), gnomAD 1-26696461-C-A, REVEL 0.10, MetaLR 0.01
- P20P (p.Pro20Pro), rs2124739963, gnomAD 1-26696463-G-A, CADD 14.80
- P21H (p.Pro21His), Ensembl rs2124739978, REVEL 0.06, MetaLR 0.02
- P21S (p.Pro21Ser), gnomAD rs2080254035, REVEL 0.01, MetaLR 0.01
- p.Pro21dup, rs748085214, gnomAD 1-26696448-C-CCCG, CADD 19.30
- P21del (p.Pro21del), rs748085214, gnomAD 1-26696448-CCCG-C, CADD 15.90
- P21L (p.Pro21Leu), gnomAD 1-26696463-GCC-G, CADD 26.30
- P21T (p.Pro21Thr), gnomAD 1-26696464-C-A, REVEL 0.04, MetaLR 0.01
- P21P (p.Pro21Pro), gnomAD 1-26696466-C-G, CADD 14.70
- S22* (p.Ser22Ter), NCI-TCGA TCGA novel, CADD 36.00, Variant assessed as somatic; high impact.
- S22L (p.Ser22Leu), cosmic curated COSV10520, TOPMed rs1377110828, gnomAD rs1377110828, REVEL 0.12, MetaLR 0.03
- S22P (p.Ser22Pro), Ensembl rs2124739988, REVEL 0.10, MetaLR 0.03
- S22T (p.Ser22Thr), Ensembl rs2124739988
- S22W (p.Ser22Trp), TOPMed rs1377110828, gnomAD rs1377110828, REVEL 0.18, MetaLR 0.03
- S22R (p.Ser22Arg), gnomAD 1-26696462-CG-C, CADD 27.00
- S22G (p.Ser22Gly), gnomAD 1-26696465-CCT-C, CADD 26.10
- S22A (p.Ser22Ala), gnomAD 1-26696467-T-G, REVEL 0.12, MetaLR 0.02
- S22S (p.Ser22Ser), rs2124740000, gnomAD 1-26696469-G-A, CADD 14.60
- E23* (p.Glu23Ter), rs1557569082, ClinGen CA339264314, ClinVar RCV000722667, ClinVar RCV004723138, CADD 36.00, Likely pathogenic
- E23G (p.Glu23Gly), gnomAD rs2080254177, REVEL 0.08, MetaLR 0.01
- E23K (p.Glu23Lys), Ensembl rs1557569082, REVEL 0.16, MetaLR 0.02, Likely pathogenic
- E23Q (p.Glu23Gln), Ensembl rs1557569082, REVEL 0.20, MetaLR 0.02, Likely pathogenic
- E23D (p.Glu23Asp), gnomAD 1-26696472-G-T, REVEL 0.12, MetaLR 0.02
- E23E (p.Glu23Glu), rs1477132345, gnomAD 1-26696472-G-A, CADD 13.30
- L24P (p.Leu24Pro), TOPMed rs2080254294, REVEL 0.10, MetaLR 0.03
- L24Q (p.Leu24Gln), TOPMed rs2080254294, REVEL 0.08, MetaLR 0.03
- L24R (p.Leu24Arg), TOPMed rs2080254294
- L24V (p.Leu24Val), TOPMed rs910020727, gnomAD rs910020727, MetaLR 0.01, MetaSVM -0.98
- L24M (p.Leu24Met), gnomAD 1-26696473-C-A, REVEL 0.18, MetaLR 0.03
- L24L (p.Leu24Leu), rs910020727, gnomAD 1-26696473-C-T, CADD 13.40
- K25E (p.Lys25Glu), Ensembl rs2124740037, REVEL 0.07, MetaLR 0.01
- p.Lys25delinsThrTer, gnomAD 1-26696476-A-ACTT, CADD 29.50
- K25R (p.Lys25Arg), gnomAD 1-26696477-A-G, REVEL 0.06, MetaLR 0.01
- K25M (p.Lys25Met), gnomAD 1-26696477-A-T, REVEL 0.07, MetaLR 0.02
- K25T (p.Lys25Thr), gnomAD 1-26696477-A-C, REVEL 0.09, MetaLR 0.01
- K25N (p.Lys25Asn), gnomAD 1-26696478-G-C, REVEL 0.09, MetaLR 0.01
- K25K (p.Lys25Lys), rs942482061, gnomAD 1-26696478-G-A, CADD 13.80
Public ARID1A analysis runs
- ARID1A analysis run — ARID1A (10,785 variants) — completed 2026-08-18