KCNJ10 (P78508) variants and mutations
KCNJ10 (also known as P78508) is a human protein-coding gene encoding an ATP-sensitive inward rectifier potassium channel 10 protein. Its annotated function is may be responsible for potassium buffering action of glial cells in the brain (By similarity). Inward rectifier potassium channels are characterized by a greater tendency to allow potassium to flow into the cell rather than out of it. It is annotated at the membrane. This analysis covers 870 KCNJ10 variants and mutations. Of these, 79% have computational variant effect predictions. Disease context includes EAST syndrome, autosomal recessive nonsyndromic hearing loss 4, and Pendred syndrome. Example KCNJ10 variants include M1V, T2M, and T2P.
Variant analysis overview
- Gene: KCNJ10
- Protein: P78508
- UniProt accession: P78508
- Organism: Homo sapiens
- Variants analyzed: 870
- Variant scope: all variants
- Completed: 2026-08-27
Variant and mutation evidence
- Variant composition: 490 unspecified-consequence records; 5 stop lost; 187 missense variants; 152 synonymous variants; 10 stop-gained variants; 17 frameshift variants; 3 in-frame insertions; 6 in-frame deletions; 2 protein altering variant; 1 splice-region variants; 4 substitution
- Prediction scores: 688 variants have prediction scores (79% of the analyzed set).
Clinical, disease, and population context
- Disease context: 25 disease associations are represented. Top associations: EAST syndrome, autosomal recessive nonsyndromic hearing loss 4, Pendred syndrome, episodic kinesigenic dyskinesia, hypercoagulability syndrome due to glycosylphosphatidylinositol deficiency, episodic kinesigenic dyskinesia 1, hereditary disease, nonsyndromic genetic hearing loss, cerebellar ataxia, Sensorineural hearing impairment, microcephaly, spastic diplegia.
Protein structure and variant hotspots
- Protein features: 2 transmembrane segments; 5 binding sites.
- Structural context: 108 variants have structural context.
Data sources
Evidence in this analysis draws on EBI Proteins Variation, UniProt, gnomAD v4, EuropePMC, Interaction Network Analysis, Protein Data Bank, AlphaFold DB, gnomAD constraint, Open Targets, ClinGen, MaveDB, LitVar.
Notable KCNJ10 variants
Examples include M1V, T2M, T2P, T2R, S3*, S3P, V4A, V4I. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.
- M1V (p.Met1Val), rs1260852127, ClinGen CA343231574, ClinVar RCV001325483, MetaLR 0.39, MetaSVM -0.22, Uncertain significance, EAST syndrome
- T2M (p.Thr2Met), TOPMed rs1378425325, gnomAD rs1378425325, AlphaMissense 0.19, MetaLR 0.34, Uncertain significance, EAST syndrome
- T2P (p.Thr2Pro), TOPMed rs981070121, CADD 22.40
- T2R (p.Thr2Arg), rs1378425325, ClinGen CA343231496, ClinVar RCV001316139, ClinVar RCV002486241, AlphaMissense 0.19, MetaLR 0.34, Uncertain significance, Autosomal recessive nonsyndromic hearing loss 4; Pendred syndrome; EAST syndrome
- S3* (p.Ser3Ter), NCI-TCGA Cosmic COSV6363, cosmic curated COSV63633, Variant assessed as somatic; high impact.
- S3P (p.Ser3Pro), 1000Genomes rs72704720, gnomAD rs72704720, CADD 23.50, PolyPhen-2 0.39
- V4A (p.Val4Ala), gnomAD rs1354455168, CADD 10.90, PolyPhen-2 0.00
- V4I (p.Val4Ile), rs144428351, ClinGen CA1193308, ClinVar RCV000500028, ClinVar RCV001865609, CADD 12.60, PolyPhen-2 0.00, Uncertain significance, not specified; EAST syndrome; Inborn genetic diseases
- V4L (p.Val4Leu), ESP rs144428351, ExAC rs144428351, TOPMed rs144428351, gnomAD rs144428351, CADD 13.00, Uncertain significance
- K6E (p.Lys6Glu), NCI-TCGA Cosmic COSV1009, cosmic curated COSV10092, Variant assessed as somatic; moderate impact.
- K6N (p.Lys6Asn), rs1280213852, NCI-TCGA Cosmic COSV6363, cosmic curated COSV63632, TOPMed rs1280213852, CADD 22.40, PolyPhen-2 0.01, Uncertain significance, Autosomal recessive nonsyndromic hearing loss 4; EAST syndrome
- V7A (p.Val7Ala), rs1440235554, ClinGen CA343231285, ClinVar RCV001338938, TOPMed rs1440235554, CADD 26.90, PolyPhen-2 0.94, Uncertain significance, EAST syndrome
- V7E (p.Val7Glu), TOPMed rs1440235554, gnomAD rs1440235554, Uncertain significance
- V7G (p.Val7Gly), cosmic curated COSV63635
- Y8C (p.Tyr8Cys), ExAC rs765568974, gnomAD rs765568974, CADD 21.50, PolyPhen-2 0.00
- Y9H (p.Tyr9His), rs2525431806, ClinGen CA343231187, ClinVar RCV002570850, CADD 18.80, PolyPhen-2 0.00, Uncertain significance, EAST syndrome
- S10N (p.Ser10Asn), Ensembl rs1557968217
- S10T (p.Ser10Thr), Ensembl rs1557968217
- Q11K (p.Gln11Lys), rs2525431789, ClinGen CA343231108, ClinVar RCV003034550, CADD 25.70, PolyPhen-2 0.86, Uncertain significance, EAST syndrome
- Q11L (p.Gln11Leu), cosmic curated COSV10529
- Q11R (p.Gln11Arg), TOPMed rs1648635539, CADD 25.30, PolyPhen-2 0.90
- T12N (p.Thr12Asn), rs2101925216, ClinGen CA343231016, ClinVar RCV001371655, Ensembl rs2101925216, AlphaMissense 0.27, MetaLR 0.56, Uncertain significance, EAST syndrome
- T13I (p.Thr13Ile), Ensembl rs1648635350, AlphaMissense 0.32, MetaLR 0.31
- T13S (p.Thr13Ser), rs1648635350, ClinGen CA343230960, ClinVar RCV003617365, AlphaMissense 0.32, MetaLR 0.31, Uncertain significance, EAST syndrome
- Q14R (p.Gln14Arg), rs776766074, Uncertain significance
- T15P (p.Thr15Pro), gnomAD rs1350872638
- T15R (p.Thr15Arg), TOPMed rs1010999260, CADD 21.00, PolyPhen-2 0.28
- E16D (p.Glu16Asp), NCI-TCGA Cosmic COSV6363, cosmic curated COSV63634, Variant assessed as somatic; moderate impact.
- E16Q (p.Glu16Gln), NCI-TCGA Cosmic COSV6363, cosmic curated COSV63633, Variant assessed as somatic; moderate impact.
- S17N (p.Ser17Asn), NCI-TCGA Cosmic COSV1009, cosmic curated COSV10092, NCI-TCGA Cosmic COSV6363, CADD 17.10, PolyPhen-2 0.07, Variant assessed as somatic; moderate impact.
- S17T (p.Ser17Thr), NCI-TCGA Cosmic COSV1009, NCI-TCGA Cosmic COSV6363, cosmic curated COSV63633, Variant assessed as somatic; moderate impact.
- R18Q (p.Arg18Gln), rs115466046, ClinGen CA288914, cosmic curated COSV99058, ClinVar RCV000117319, CADD 18.70, PolyPhen-2 0.06, Conflicting interpretations, Inborn genetic diseases; not provided; not specified
- R18W (p.Arg18Trp), rs138457635, ClinGen CA315261, cosmic curated COSV10092, ClinVar RCV000464419, CADD 27.70, PolyPhen-2 0.64, Conflicting interpretations, Inborn genetic diseases; not provided; Autosomal recessive nonsyndromic hearing
- P19S (p.Pro19Ser), NCI-TCGA Cosmic COSV1009, cosmic curated COSV10092, Variant assessed as somatic; moderate impact.
- L20P (p.Leu20Pro), gnomAD rs1170775164
- L20R (p.Leu20Arg), gnomAD rs1170775164, CADD 25.40, PolyPhen-2 0.99
- M21I (p.Met21Ile), rs746292052, ExAC rs746292052, gnomAD rs746292052, ClinGen CA1193302, CADD 18.10, PolyPhen-2 0.00, Uncertain significance, Autosomal recessive nonsyndromic hearing loss 4; EAST syndrome
- M21T (p.Met21Thr), rs878854483, ClinGen CA10581732, ClinVar RCV000229107, ClinVar RCV002365189, CADD 20.70, PolyPhen-2 0.00, Uncertain significance, Inborn genetic diseases; EAST syndrome
- G22A (p.Gly22Ala), NCI-TCGA Cosmic COSV6363, cosmic curated COSV63635, Variant assessed as somatic; moderate impact.
- G22D (p.Gly22Asp), TOPMed rs906427765, Uncertain significance, EAST syndrome
- G22R (p.Gly22Arg), gnomAD rs1212431708, CADD 22.00
- G22S (p.Gly22Ser), cosmic curated COSV63634
- P23S (p.Pro23Ser), rs1648632903, ClinGen CA343230611, ClinVar RCV003044952, gnomAD rs1648632903, AlphaMissense 0.09, MetaLR 0.18, Uncertain significance, EAST syndrome
- P23T (p.Pro23Thr), rs1648632903, ClinGen CA343230615, ClinVar RCV002295984, AlphaMissense 0.09, MetaLR 0.18, Uncertain significance, EAST syndrome
- G24E (p.Gly24Glu), gnomAD rs267598119, CADD 14.40, PolyPhen-2 0.01
- G24R (p.Gly24Arg), NCI-TCGA TCGA novel, CADD 18.00, PolyPhen-2 0.00, Variant assessed as somatic; moderate impact.
- I25M (p.Ile25Met), NCI-TCGA TCGA novel, Variant assessed as somatic; moderate impact.
- R26* (p.Arg26Ter), rs138943405, ClinGen CA207249, cosmic curated COSV63632, ClinVar RCV000193637, CADD 36.00, Pathogenic
- R26Q (p.Arg26Gln), cosmic curated COSV10821, TOPMed rs369062738, gnomAD rs369062738, CADD 20.50, PolyPhen-2 0.07
- R27Q (p.Arg27Gln), rs776766074, ClinGen CA1193299, ClinVar RCV001304284, ClinVar RCV002539549, CADD 19.70, PolyPhen-2 0.06, Uncertain significance, EAST syndrome; Inborn genetic diseases; Autosomal recessive nonsyndromic hearing
- R27W (p.Arg27Trp), rs769464065, ClinGen CA1193300, NCI-TCGA Cosmic COSV6363, cosmic curated COSV63634, CADD 23.70, PolyPhen-2 0.94, Uncertain significance, Pendred syndrome; Autosomal recessive nonsyndromic hearing loss 4; EAST syndrome
- R28Q (p.Arg28Gln), rs766560083, ClinGen CA31471500, cosmic curated COSV10467, ClinVar RCV001341861, CADD 23.20, PolyPhen-2 0.88, Uncertain significance, Autosomal recessive nonsyndromic hearing loss 4; EAST syndrome; Pendred syndrome
- R28W (p.Arg28Trp), rs780870986, NCI-TCGA Cosmic COSV6363, cosmic curated COSV63633, ExAC rs780870986, CADD 24.60, PolyPhen-2 0.97, Uncertain significance, Inborn genetic diseases
- R29K (p.Arg29Lys), NCI-TCGA Cosmic COSV6363, cosmic curated COSV63632, Variant assessed as somatic; moderate impact.
- V30F (p.Val30Phe), ExAC rs754483304, gnomAD rs754483304, CADD 22.10, PolyPhen-2 0.86
- V30G (p.Val30Gly), Ensembl rs1571265894
- V30I (p.Val30Ile), ExAC rs754483304, gnomAD rs754483304, CADD 17.10, PolyPhen-2 0.04
- T32K (p.Thr32Lys), rs1648631972, cosmic curated COSV63633, ClinVar RCV004588656, AlphaMissense 0.49, MetaLR 0.75, Uncertain significance, not provided
- T32R (p.Thr32Arg), Ensembl rs1648631972
- K33N (p.Lys33Asn), cosmic curated COSV10092
- D34H (p.Asp34His), cosmic curated COSV10092
- D34Y (p.Asp34Tyr), NCI-TCGA Cosmic COSV1009, Variant assessed as somatic; moderate impact.
- R36C (p.Arg36Cys), rs1256202581, ClinGen CA343230271, NCI-TCGA Cosmic COSV6363, cosmic curated COSV63632, CADD 28.80, PolyPhen-2 0.98, Uncertain significance, EAST syndrome
- R36H (p.Arg36His), rs779913708, ClinGen CA1193296, cosmic curated COSV10529, ClinVar RCV001361621, CADD 18.80, PolyPhen-2 0.03, Uncertain significance, EAST syndrome; Autosomal recessive nonsyndromic hearing loss 4
- R36L (p.Arg36Leu), ExAC rs779913708, TOPMed rs779913708, gnomAD rs779913708, CADD 25.60, PolyPhen-2 0.91, Uncertain significance
- S37K (p.Ser37Lys), cosmic curated COSV10970
- S37N (p.Ser37Asn), NCI-TCGA TCGA novel, Variant assessed as somatic; moderate impact.
- N38D (p.Asn38Asp), rs1085307764, ClinGen CA343230218, ClinVar RCV000489850, Ensembl rs1085307764, AlphaMissense 0.85, MetaLR 0.94, Uncertain significance, not provided
- V39M (p.Val39Met), rs1274663711, ClinGen CA343230174, cosmic curated COSV63633, ClinVar RCV001822117, CADD 23.40, PolyPhen-2 1.00, Conflicting interpretations, EAST syndrome; Inborn genetic diseases; not provided
- R40K (p.Arg40Lys), cosmic curated COSV63634
- M41I (p.Met41Ile), NCI-TCGA Cosmic COSV6363, cosmic curated COSV63634, CADD 16.30, PolyPhen-2 0.00, Variant assessed as somatic; moderate impact.
- M41V (p.Met41Val), TOPMed rs1427151114, Uncertain significance, Autosomal recessive nonsyndromic hearing loss 4; EAST syndrome
- E42K (p.Glu42Lys), rs794727265, ClinGen CA241456, cosmic curated COSV63633, ClinVar RCV000175716, CADD 23.30, PolyPhen-2 0.53, Uncertain significance, not provided; EAST syndrome
- E42Q (p.Glu42Gln), TOPMed rs794727265, Uncertain significance
- H43R (p.His43Arg), rs779913708, []
- I44M (p.Ile44Met), Ensembl rs1648631047
- I44V (p.Ile44Val), rs1331687844, ClinGen CA343230061, ClinVar RCV002701104, ClinVar RCV002710072, CADD 15.20, PolyPhen-2 0.02, Uncertain significance, not provided; Inborn genetic diseases; EAST syndrome
- A45D (p.Ala45Asp), NCI-TCGA Cosmic COSV1009, cosmic curated COSV10092, NCI-TCGA Cosmic COSV6363, Ensembl rs1648630918, Variant assessed as somatic; moderate impact.
- A45T (p.Ala45Thr), rs750246232, ClinGen CA1193292, ClinVar RCV000558426, ClinVar RCV001574872, CADD 15.90, PolyPhen-2 0.00, Uncertain significance, not provided; KCNJ10-related disorder; Inborn genetic diseases
- A45V (p.Ala45Val), cosmic curated COSV63635
- D46N (p.Asp46Asn), rs141553756, ClinGen CA1193291, cosmic curated COSV10092, ClinVar RCV000374787, CADD 22.90, PolyPhen-2 0.51, Conflicting interpretations, Inborn genetic diseases; not specified; not provided
- R48C (p.Arg48Cys), rs754056344, ClinGen CA1193289, ClinVar RCV001238790, ClinVar RCV001568647, CADD 23.90, PolyPhen-2 0.76, Uncertain significance, EAST syndrome; not provided
- R48H (p.Arg48His), rs201907875, ClinGen CA1193288, ClinVar RCV003117002, 1000Genomes rs201907875, CADD 17.60, PolyPhen-2 0.38, Uncertain significance, EAST syndrome
- R48P (p.Arg48Pro), rs201907875, ClinGen CA343229948, ClinVar RCV000824215, 1000Genomes rs201907875, CADD 19.80, PolyPhen-2 0.41, Uncertain significance, EAST syndrome
- F49V (p.Phe49Val), Ensembl rs1648630415, CADD 21.00, PolyPhen-2 0.05
- L50F (p.Leu50Phe), rs773510302, ClinGen CA1193287, ClinVar RCV000646753, ClinVar RCV000725019, CADD 23.70, PolyPhen-2 1.00, Uncertain significance, EAST syndrome; Pendred syndrome; Autosomal recessive nonsyndromic hearing loss 4
- L50P (p.Leu50Pro), Ensembl rs1130181
- Y51H (p.Tyr51His), rs2525431163, ClinGen CA343229917, ClinVar RCV003065207, Uncertain significance, EAST syndrome
- L52F (p.Leu52Phe), gnomAD rs1238558136
- K53E (p.Lys53Glu), rs2101925129, ClinGen CA343229870, ClinVar RCV001951735, Ensembl rs2101925129, AlphaMissense 0.70, MetaLR 0.66, Uncertain significance, EAST syndrome
- L55M (p.Leu55Met), TOPMed rs1432570156, CADD 22.80, PolyPhen-2 0.99
- W56* (p.Trp56Ter), NCI-TCGA Cosmic COSV6363, cosmic curated COSV63632, Variant assessed as somatic; high impact.
- T57I (p.Thr57Ile), rs2525431132, ClinGen CA343229787, ClinVar RCV003985003, Likely pathogenic, EAST syndrome
- T58A (p.Thr58Ala), NCI-TCGA Cosmic COSV6363, cosmic curated COSV63633, Variant assessed as somatic; moderate impact.
- T58I (p.Thr58Ile), rs768028468, ClinGen CA1193285, ClinVar RCV003618180, ExAC rs768028468, CADD 26.40, PolyPhen-2 1.00, Uncertain significance, EAST syndrome
- F59L (p.Phe59Leu), NCI-TCGA Cosmic COSV6363, cosmic curated COSV63634, Variant assessed as somatic; moderate impact.
- F59V (p.Phe59Val), gnomAD rs1648629710, CADD 18.70, PolyPhen-2 0.03
- F59S (p.Phe59Ser), rs768028468, []
- I60T (p.Ile60Thr), rs759993423, ClinGen CA315278, cosmic curated COSV63633, ClinVar RCV000187814, CADD 25.40, PolyPhen-2 0.52, Conflicting interpretations, Inborn genetic diseases; not provided; Autosomal recessive nonsyndromic hearing
- I60V (p.Ile60Val), rs1648629565, ClinGen CA343229735, ClinVar RCV001895948, ClinVar RCV002482657, CADD 15.10, PolyPhen-2 0.02, Uncertain significance, Pendred syndrome; EAST syndrome; Autosomal recessive nonsyndromic hearing loss 4
- D61G (p.Asp61Gly), NCI-TCGA Cosmic COSV6363, cosmic curated COSV63633, Variant assessed as somatic; moderate impact.
- M62I (p.Met62Ile), cosmic curated COSV63634
- Q63P (p.Gln63Pro), NCI-TCGA Cosmic COSV1009, cosmic curated COSV10092, TOPMed rs1648629284, Uncertain significance, Inborn genetic diseases; EAST syndrome; Autosomal recessive nonsyndromic hearing
- R65C (p.Arg65Cys), rs387906834, ClinGen CA129068, NCI-TCGA Cosmic COSV6363, cosmic curated COSV63633, CADD 28.40, PolyPhen-2 1.00, Likely pathogenic, EAST syndrome
- R65H (p.Arg65His), rs137853066, NCI-TCGA Cosmic COSV1044, NCI-TCGA Cosmic COSV6363, cosmic curated COSV63635, CADD 27.20, PolyPhen-2 1.00, Uncertain significance, Inborn genetic diseases
- R65L (p.Arg65Leu), cosmic curated COSV10442
- R65P (p.Arg65Pro), rs137853066, ClinGen CA118807, ClinVar RCV000007888, ClinVar RCV001003572, CADD 27.60, PolyPhen-2 1.00, Pathogenic, Microcephaly; Renal tubular dysfunction; Bilateral sensorineural hearing impairm
- R65S (p.Arg65Ser), ExAC rs387906834, TOPMed rs387906834, gnomAD rs387906834, CADD 25.80, Likely pathogenic, in SESAMES
- Y66C (p.Tyr66Cys), cosmic curated COSV10529, ExAC rs776212962, gnomAD rs776212962, CADD 27.10, PolyPhen-2 0.99
- K67N (p.Lys67Asn), gnomAD 1-160042332-C-A, MetaLR 0.74, MetaSVM 0.32
- K67K (p.Lys67Lys), gnomAD 1-160042332-C-T, CADD 10.50
- L68P (p.Leu68Pro), UniProt VAR 072746, Pathogenic, in SESAMES
- L70F (p.Leu70Phe), rs1648628910, ClinGen CA343229572, NCI-TCGA Cosmic COSV6363, cosmic curated COSV63634, AlphaMissense 0.36, MetaLR 0.84, Uncertain significance, EAST syndrome
- L70L (p.Leu70Leu), rs768340401, gnomAD 1-160042323-G-A, CADD 8.33
- F71del (p.Phe71del), gnomAD 1-160042318-GAGA-, CADD 19.20
- S72F (p.Ser72Phe), ExAC rs746513174, gnomAD rs746513174, CADD 23.60, PolyPhen-2 0.83
- S72S (p.Ser72Ser), gnomAD 1-160042317-A-G, CADD 2.35
- A73T (p.Ala73Thr), rs779548724, ClinGen CA1193279, ClinVar RCV001234667, ExAC rs779548724, AlphaMissense 0.13, MetaLR 0.76, Uncertain significance, EAST syndrome
- A73V (p.Ala73Val), rs758441824, NCI-TCGA Cosmic COSV6363, cosmic curated COSV63632, ExAC rs758441824, CADD 24.00, PolyPhen-2 0.75, Uncertain significance, EAST syndrome
- A73A (p.Ala73Ala), rs144495959, gnomAD 1-160042314-C-T, CADD 0.86
- T74I (p.Thr74Ile), rs796052604, ClinGen CA315263, cosmic curated COSV63633, ClinVar RCV000187806, CADD 26.10, PolyPhen-2 1.00, Conflicting interpretations, not provided; EAST syndrome
- T74T (p.Thr74Thr), rs778972804, gnomAD 1-160042311-G-A, CADD 9.37
- F75L (p.Phe75Leu), rs757159382, ClinGen CA31471247, ClinVar RCV000023176, ExAC rs757159382, CADD 22.90, PolyPhen-2 1.00, Pathogenic, EAST syndrome
- F75V (p.Phe75Val), NCI-TCGA TCGA novel, Variant assessed as somatic; moderate impact., in SESAMES
- p.Phe75 Thr78del, gnomAD 1-160042299-TGTGC, CADD 19.20
- F75F (p.Phe75Phe), rs757159382, gnomAD 1-160042308-A-G, CADD 8.91
- A76A (p.Ala76Ala), rs1249044917, gnomAD 1-160042305-T-C, CADD 7.08
- A76T (p.Ala76Thr), gnomAD 1-160042307-C-T, MetaLR 0.51, MetaSVM -0.13
- G77D (p.Gly77Asp), NCI-TCGA Cosmic COSV1009, cosmic curated COSV10092, Variant assessed as somatic; moderate impact., in SESAMES
- G77R (p.Gly77Arg), rs137853072, ClinGen CA118814, ClinVar RCV000007894, UniProt VAR 063060, AlphaMissense 0.99, MetaLR 0.90, Pathogenic, EAST syndrome
- G77V (p.Gly77Val), ExAC rs753667101, gnomAD rs753667101, CADD 18.90, PolyPhen-2 0.07
- T78P (p.Thr78Pro), gnomAD 1-160042234-TCCAG, CADD 29.10
- T78T (p.Thr78Thr), rs748359138, gnomAD 1-160042299-T-C, CADD 9.78
- W79* (p.Trp79Ter), rs2525430986, ClinGen CA343229348, ClinVar RCV003618900, Pathogenic
- W79R (p.Trp79Arg), rs2101925083, ClinGen CA343229356, ClinVar RCV001891352, Ensembl rs2101925083, AlphaMissense 1.00, MetaLR 0.95, Uncertain significance, EAST syndrome
- F80L (p.Phe80Leu), ESP rs377638115, ExAC rs377638115, TOPMed rs377638115, gnomAD rs377638115, CADD 22.90, PolyPhen-2 0.96, Uncertain significance, not provided; EAST syndrome
- F80V (p.Phe80Val), rs377638115, ClinGen CA1193271, ClinVar RCV001987506, ClinVar RCV002275297, CADD 27.20, PolyPhen-2 0.99, Uncertain significance, not provided; EAST syndrome
- F80F (p.Phe80Phe), rs774758209, gnomAD 1-160042293-G-A, CADD 10.20
- L81F (p.Leu81Phe), rs939420346, ClinGen CA31471198, ClinVar RCV001202418, ClinVar RCV002451416, CADD 19.60, PolyPhen-2 0.01, Uncertain significance, Autosomal recessive nonsyndromic hearing loss 4; Pendred syndrome; EAST syndrome
- L81L (p.Leu81Leu), rs766863064, gnomAD 1-160042290-G-T, CADD 7.40
- F82S (p.Phe82Ser), ESP rs373270208, ExAC rs373270208, gnomAD rs373270208, CADD 29.00, PolyPhen-2 1.00
- G83A (p.Gly83Ala), rs17853258, ClinGen CA1193266, ClinVar RCV001922147, ClinVar RCV002425172, AlphaMissense 0.63, MetaLR 0.82, Uncertain significance, Inborn genetic diseases; Pendred syndrome; EAST syndrome
- G83V (p.Gly83Val), rs17853258, ESP rs17853258, ExAC rs17853258, TOPMed rs17853258, AlphaMissense 0.63, MetaLR 0.82, Uncertain significance
- G83G (p.Gly83Gly), rs768195772, gnomAD 1-160042284-G-A, CADD 0.58
- V84A (p.Val84Ala), ExAC rs775164061
- V84M (p.Val84Met), rs140646329, ClinGen CA315265, cosmic curated COSV10092, ClinVar RCV000187807, CADD 23.30, PolyPhen-2 0.99, Uncertain significance, Inborn genetic diseases; EAST syndrome; Autosomal recessive nonsyndromic hearing
- V84V (p.Val84Val), rs981173172, gnomAD 1-160042281-C-G, CADD 5.22
- V85M (p.Val85Met), gnomAD 1-160042280-C-T, MetaLR 0.63, MetaSVM 0.24
- V85L (p.Val85Leu), gnomAD 1-160042280-C-G, MetaLR 0.27, MetaSVM -0.61
- W86* (p.Trp86Ter), gnomAD rs1287232738, CADD 36.00
- Y87C (p.Tyr87Cys), cosmic curated COSV63633
- L88L (p.Leu88Leu), gnomAD 1-160042269-C-G, CADD 8.06
- V89I (p.Val89Ile), rs2525430906, ClinGen CA343229106, ClinVar RCV003055339, CADD 15.20, PolyPhen-2 0.04, Uncertain significance, EAST syndrome
- V89A (p.Val89Ala), gnomAD 1-160042267-A-G, MetaLR 0.78, MetaSVM 0.65
- A90V (p.Ala90Val), rs771759286, ExAC rs771759286, gnomAD rs771759286, CADD 25.90, PolyPhen-2 0.99, Variant assessed as somatic; moderate impact.
- V91V (p.Val91Val), rs745340219, gnomAD 1-160042260-C-T, CADD 8.01
- V91L (p.Val91Leu), gnomAD 1-160042262-C-G, MetaLR 0.23, MetaSVM -0.82
- A92S (p.Ala92Ser), rs1314505994, ClinGen CA343229039, ClinVar RCV000499500, ClinVar RCV001368931, CADD 13.50, PolyPhen-2 0.14, Uncertain significance, not specified; EAST syndrome; not provided
- A92T (p.Ala92Thr), NCI-TCGA Cosmic COSV1009, cosmic curated COSV10092, Variant assessed as somatic; moderate impact.
- A92V (p.Ala92Val), cosmic curated COSV63633, gnomAD rs1228532296, CADD 14.50, PolyPhen-2 0.00
- H93L (p.His93Leu), rs1377862008, ClinGen CA343229004, ClinVar RCV001348491, gnomAD rs1377862008, AlphaMissense 0.55, MetaLR 0.75, Uncertain significance, EAST syndrome
- H93R (p.His93Arg), rs1377862008, ClinGen CA343229007, ClinVar RCV001328935, ClinVar RCV004770059, AlphaMissense 0.55, MetaLR 0.75, Uncertain significance, not provided; EAST syndrome
- G94E (p.Gly94Glu), cosmic curated COSV10467
- G94G (p.Gly94Gly), rs1436802011, gnomAD 1-160042251-C-T, CADD 7.50
- G94R (p.Gly94Arg), gnomAD 1-160042253-C-T, MetaLR 0.93, MetaSVM 1.05
- D95A (p.Asp95Ala), gnomAD rs1281903569, CADD 27.80, PolyPhen-2 1.00, Uncertain significance, EAST syndrome
- D95E (p.Asp95Glu), gnomAD 1-160042248-G-T, MetaLR 0.95, MetaSVM 1.09
- L96L (p.Leu96Leu), gnomAD 1-160042245-C-G, CADD 7.51
- L97Q (p.Leu97Gln), rs1401791648, ClinGen CA343228923, ClinVar RCV000646756, TOPMed rs1401791648, CADD 17.90, PolyPhen-2 0.05, Uncertain significance, EAST syndrome
- L97L (p.Leu97Leu), gnomAD 1-160042244-G-A, CADD 7.71
- E98K (p.Glu98Lys), rs200054482, ClinGen CA343228911, ClinVar RCV001222843, 1000Genomes rs200054482, CADD 21.60, PolyPhen-2 0.27, Uncertain significance, EAST syndrome
- E98Q (p.Glu98Gln), rs200054482, NCI-TCGA Cosmic COSV1009, cosmic curated COSV10092, 1000Genomes rs200054482, CADD 20.20, Uncertain significance
- L99M (p.Leu99Met), TOPMed rs1648626270, CADD 18.40, PolyPhen-2 0.04
- L99P (p.Leu99Pro), rs540341763, ClinGen CA1193261, ClinVar RCV000259824, ClinVar RCV000354704, CADD 19.90, PolyPhen-2 0.01, Conflicting interpretations, EAST syndrome; not provided; Autosomal recessive nonsyndromic hearing loss 4
- L99L (p.Leu99Leu), rs1417569647, gnomAD 1-160042236-C-G, CADD 7.20
- L99V (p.Leu99Val), gnomAD 1-160042238-G-C, MetaLR 0.40, MetaSVM -0.63
- D100A (p.Asp100Ala), Ensembl rs1571265721
- D100E (p.Asp100Glu), rs139069413, ClinGen CA1193259, cosmic curated COSV10467, ClinVar RCV000503569, CADD 0.00, PolyPhen-2 0.00, Conflicting interpretations, Inborn genetic diseases; not specified; not provided
- D100D (p.Asp100Asp), rs139069413, gnomAD 1-160042233-G-A, CADD 0.16
- D100G (p.Asp100Gly), gnomAD 1-160042234-T-C, MetaLR 0.40, MetaSVM -0.68
Public KCNJ10 analysis runs
- KCNJ10 analysis run — KCNJ10 (870 variants) — completed 2026-08-27