KCNH3 — 126 ClinVar variants
All ClinVar germline KCNH3 variants across every classification, each assembled from ClinVar, gnomAD, AlphaMissense, conservation and more. 112 flagged for review →
6 Benign · 6 Likely benign · 114 Uncertain significance
Full KCNH3 gene report on Sugi Atlas → Gene function, pathways, protein structure, constraint & interactions⚑ Evidence disagreements 112
Variants where the independent computational evidence doesn't line up with the clinical picture — a QC signal for a second look, not a reclassification.
Predictors lean, ClinVar uncertain 101
ClinVar classifies these uncertain (VUS), but the independent predictors unanimously lean one way — a triage signal for review, not a reclassification.
- KCNH3 p.Ala1030Thr Uncertain significance Single submitter
- KCNH3 p.Ala158Thr Uncertain significance Single submitter
- KCNH3 p.Ala209Val Uncertain significance Single submitter
- KCNH3 p.Ala3Val Uncertain significance Single submitter
- KCNH3 p.Ala637Val Uncertain significance Single submitter
- KCNH3 p.Ala683Val Uncertain significance Single submitter
- KCNH3 p.Ala799Thr Uncertain significance Single submitter
- KCNH3 p.Ala929Val Uncertain significance Single submitter
- KCNH3 p.Ala953Gly Uncertain significance Single submitter
- KCNH3 p.Arg101Trp Uncertain significance Single submitter
- KCNH3 p.Arg153Cys Uncertain significance Single submitter
- KCNH3 p.Arg157Gln Uncertain significance Single submitter
- KCNH3 p.Arg258His Uncertain significance Single submitter
- KCNH3 p.Arg338His Uncertain significance Single submitter
- KCNH3 p.Arg344Cys Uncertain significance Single submitter
- KCNH3 p.Arg348Gln Uncertain significance Single submitter
- KCNH3 p.Arg415Trp Uncertain significance Single submitter
- KCNH3 p.Arg588His Uncertain significance Single submitter
- KCNH3 p.Arg651Gln Uncertain significance Single submitter
- KCNH3 p.Arg697Gln Uncertain significance Single submitter
- KCNH3 p.Arg771His Uncertain significance Single submitter
- KCNH3 p.Arg772Gln Uncertain significance Single submitter
- KCNH3 p.Arg776Trp Uncertain significance Single submitter
- KCNH3 p.Arg778His Uncertain significance Single submitter
- KCNH3 p.Arg802Trp Uncertain significance Single submitter
- KCNH3 p.Arg80His Uncertain significance Single submitter
- KCNH3 p.Arg842Cys Uncertain significance Single submitter
- KCNH3 p.Arg842His Uncertain significance Single submitter
- KCNH3 p.Arg842Leu Uncertain significance Single submitter
- KCNH3 p.Arg84His Uncertain significance Single submitter
- KCNH3 p.Arg882Gln Uncertain significance Single submitter
- KCNH3 p.Arg920Trp Uncertain significance Single submitter
- KCNH3 p.Arg989Gln Uncertain significance Single submitter
- KCNH3 p.Asn550Ser Uncertain significance Single submitter
- KCNH3 p.Asp143Asn Uncertain significance Single submitter
- KCNH3 p.Asp523His Uncertain significance Single submitter
- KCNH3 p.Asp747Ala Uncertain significance Single submitter
- KCNH3 p.Gln936Arg Uncertain significance Single submitter
- KCNH3 p.Glu1022Gly Uncertain significance Single submitter
- KCNH3 p.Glu390Lys Uncertain significance Single submitter
- KCNH3 p.Glu556Lys Uncertain significance Single submitter
- KCNH3 p.Glu653Lys Uncertain significance Single submitter
- KCNH3 p.Glu897Gln Uncertain significance Single submitter
- KCNH3 p.Gly1006Arg Uncertain significance Single submitter
- KCNH3 p.Gly1047Arg Uncertain significance Single submitter
- KCNH3 p.Gly1049Glu Uncertain significance Single submitter
- KCNH3 p.Gly1050Ser Uncertain significance Single submitter
- KCNH3 p.Gly162Ala Uncertain significance Single submitter
- KCNH3 p.Gly162Ser Uncertain significance Single submitter
- KCNH3 p.Gly162Val Uncertain significance Single submitter
- KCNH3 p.Gly187Asp Uncertain significance Single submitter
- KCNH3 p.Gly259Ser Uncertain significance Single submitter
- KCNH3 p.Gly419Ala Uncertain significance Single submitter
- KCNH3 p.Gly709Ala Uncertain significance Single submitter
- KCNH3 p.Gly833Ala Uncertain significance Single submitter
- KCNH3 p.Gly861Arg Uncertain significance Single submitter
- KCNH3 p.Gly861Ser Uncertain significance Single submitter
- KCNH3 p.Gly933Arg Uncertain significance Single submitter
- KCNH3 p.Gly933Glu Uncertain significance Single submitter
- KCNH3 p.His1058Pro Uncertain significance Single submitter
- KCNH3 p.His963Tyr Uncertain significance Single submitter
- KCNH3 p.Leu7Arg Uncertain significance Single submitter
- KCNH3 p.Leu935Gln Uncertain significance Single submitter
- KCNH3 p.Lys137Arg Uncertain significance Single submitter
- KCNH3 p.Met812Leu Uncertain significance Single submitter
- KCNH3 p.Pro1020Leu Uncertain significance Single submitter
- KCNH3 p.Pro1029Ser Uncertain significance Single submitter
- KCNH3 p.Pro185Arg Uncertain significance Single submitter
- KCNH3 p.Pro214Ser Uncertain significance Single submitter
- KCNH3 p.Pro600Ser Uncertain significance Single submitter
- KCNH3 p.Pro749Leu Uncertain significance Single submitter
- KCNH3 p.Pro775His Uncertain significance Single submitter
- KCNH3 p.Pro777Arg Uncertain significance Single submitter
- KCNH3 p.Pro801Gln Uncertain significance Single submitter
- KCNH3 p.Pro926Leu Uncertain significance Single submitter
- KCNH3 p.Pro926Thr Uncertain significance Single submitter
- KCNH3 p.Pro928Ser Uncertain significance Single submitter
- KCNH3 p.Ser1034Asn Uncertain significance Single submitter
- KCNH3 p.Ser134Ile Uncertain significance Single submitter
- KCNH3 p.Ser432Asn Uncertain significance Single submitter
- KCNH3 p.Ser450Arg Uncertain significance Single submitter
- KCNH3 p.Ser462Asn Uncertain significance Single submitter
- KCNH3 p.Ser693Asn Uncertain significance Single submitter
- KCNH3 p.Ser710Cys Uncertain significance Single submitter
- KCNH3 p.Ser834Leu Uncertain significance Single submitter
- KCNH3 p.Ser851Arg Uncertain significance Single submitter
- KCNH3 p.Ser860Arg Uncertain significance Single submitter
- KCNH3 p.Thr13Ala Uncertain significance Single submitter
- KCNH3 p.Thr25Met Uncertain significance Single submitter
- KCNH3 p.Thr408Ser Uncertain significance Single submitter
- KCNH3 p.Thr518Met Uncertain significance Single submitter
- KCNH3 p.Thr740Met Uncertain significance Single submitter
- KCNH3 p.Thr995Ile Uncertain significance Single submitter
- KCNH3 p.Val1083Ile Uncertain significance Single submitter
- KCNH3 p.Val173Met Uncertain significance Single submitter
- KCNH3 p.Val195Met Uncertain significance Single submitter
- KCNH3 p.Val246Met Uncertain significance Single submitter
- KCNH3 p.Val366Met Uncertain significance Single submitter
- KCNH3 p.Val413Leu Uncertain significance Single submitter
- KCNH3 p.Val662Met Uncertain significance Single submitter
- KCNH3 p.Val669Ile Uncertain significance Single submitter
Predictors split 11
The independent predictors disagree with each other — read with care.
- KCNH3 p.Ala357Thr Uncertain significance Single submitter
- KCNH3 p.Ala66Asp Uncertain significance Single submitter
- KCNH3 p.Arg903Cys Uncertain significance Single submitter
- KCNH3 p.Asp523Glu Uncertain significance Single submitter
- KCNH3 p.Gly179Arg Uncertain significance Single submitter
- KCNH3 p.Gly194Arg Uncertain significance Single submitter
- KCNH3 p.Ile396Thr Uncertain significance Single submitter
- KCNH3 p.Ile488Leu Uncertain significance Single submitter
- KCNH3 p.Leu370Ile Uncertain significance Single submitter
- KCNH3 p.Tyr541Cys Uncertain significance Single submitter
- KCNH3 p.Val285Met Uncertain significance Single submitter
All KCNH3 variants
Benign 6
Likely benign 6
- p.Ala371= Single submitter
- p.Arg159Gly Single submitter
- p.Arg384Gln Single submitter
- p.Arg914Gly Single submitter
- p.Val377Ile Single submitter
- p.Val843Met Single submitter
Uncertain significance 114
- p.Ala1030Thr Single submitter ⚠
- p.Ala158Thr Single submitter ⚠
- p.Ala209Val Single submitter ⚠
- p.Ala357Thr Single submitter ⚠
- p.Ala3Val Single submitter ⚠
- p.Ala637Val Single submitter ⚠
- p.Ala66Asp Single submitter ⚠
- p.Ala683Val Single submitter ⚠
- p.Ala799Thr Single submitter ⚠
- p.Ala929Val Single submitter ⚠
- p.Ala953Gly Single submitter ⚠
- p.Arg101Trp Single submitter ⚠
- p.Arg153Cys Single submitter ⚠
- p.Arg157Gln Single submitter ⚠
- p.Arg258His Single submitter ⚠
- p.Arg338His Single submitter ⚠
- p.Arg344Cys Single submitter ⚠
- p.Arg348Gln Single submitter ⚠
- p.Arg415Trp Single submitter ⚠
- p.Arg588His Single submitter ⚠
- p.Arg651Gln Single submitter ⚠
- p.Arg697Gln Single submitter ⚠
- p.Arg771His Single submitter ⚠
- p.Arg772Gln Single submitter ⚠
- p.Arg776Trp Single submitter ⚠
- p.Arg778His Single submitter ⚠
- p.Arg802Trp Single submitter ⚠
- p.Arg80His Single submitter ⚠
- p.Arg842Cys Single submitter ⚠
- p.Arg842His Single submitter ⚠
- p.Arg842Leu Single submitter ⚠
- p.Arg84His Single submitter ⚠
- p.Arg882Gln Single submitter ⚠
- p.Arg903Cys Single submitter ⚠
- p.Arg920Trp Single submitter ⚠
- p.Arg989Gln Single submitter ⚠
- p.Asn550Ser Single submitter ⚠
- p.Asp143Asn Single submitter ⚠
- p.Asp523Glu Single submitter ⚠
- p.Asp523His Single submitter ⚠
- p.Asp747Ala Single submitter ⚠
- p.Gln936Arg Single submitter ⚠
- p.Glu1022Gly Single submitter ⚠
- p.Glu390Lys Single submitter ⚠
- p.Glu556Lys Single submitter ⚠
- p.Glu653Lys Single submitter ⚠
- p.Glu897Gln Single submitter ⚠
- p.Gly1006Arg Single submitter ⚠
- p.Gly1047Arg Single submitter ⚠
- p.Gly1049Glu Single submitter ⚠
- p.Gly1050Ser Single submitter ⚠
- p.Gly162Ala Single submitter ⚠
- p.Gly162Ser Single submitter ⚠
- p.Gly162Val Single submitter ⚠
- p.Gly179Arg Single submitter ⚠
- p.Gly187Asp Single submitter ⚠
- p.Gly194Arg Single submitter ⚠
- p.Gly259Ser Single submitter ⚠
- p.Gly419Ala Single submitter ⚠
- p.Gly709Ala Single submitter ⚠
- p.Gly833Ala Single submitter ⚠
- p.Gly861Arg Single submitter ⚠
- p.Gly861Ser Single submitter ⚠
- p.Gly933Arg Single submitter ⚠
- p.Gly933Glu Single submitter ⚠
- p.His1058Pro Single submitter ⚠
- p.His963Tyr Single submitter ⚠
- p.Ile396Thr Single submitter ⚠
- p.Ile488Leu Single submitter ⚠
- p.Leu370Ile Single submitter ⚠
- p.Leu7Arg Single submitter ⚠
- p.Leu935Gln Single submitter ⚠
- p.Lys137Arg Single submitter ⚠
- p.Met812Leu Single submitter ⚠
- p.Pro1020Leu Single submitter ⚠
- p.Pro1029Ser Single submitter ⚠
- p.Pro185Arg Single submitter ⚠
- p.Pro214Ser Single submitter ⚠
- p.Pro600Ser Single submitter ⚠
- p.Pro749Leu Single submitter ⚠
- p.Pro775His Single submitter ⚠
- p.Pro777Arg Single submitter ⚠
- p.Pro801Gln Single submitter ⚠
- p.Pro926Leu Single submitter ⚠
- p.Pro926Thr Single submitter ⚠
- p.Pro928Ser Single submitter ⚠
- p.Ser1034Asn Single submitter ⚠
- p.Ser134Ile Single submitter ⚠
- p.Ser432Asn Single submitter ⚠
- p.Ser450Arg Single submitter ⚠
- p.Ser462Asn Single submitter ⚠
- p.Ser693Asn Single submitter ⚠
- p.Ser710Cys Single submitter ⚠
- p.Ser834Leu Single submitter ⚠
- p.Ser851Arg Single submitter ⚠
- p.Ser860Arg Single submitter ⚠
- p.Thr13Ala Single submitter ⚠
- p.Thr25Met Single submitter ⚠
- p.Thr408Ser Single submitter ⚠
- p.Thr518Met Single submitter ⚠
- p.Thr740Met Single submitter ⚠
- p.Thr995Ile Single submitter ⚠
- p.Trp814Arg Single submitter
- p.Tyr541Cys Single submitter ⚠
- p.Val1083Ile Single submitter ⚠
- p.Val173Met Single submitter ⚠
- p.Val195Met Single submitter ⚠
- p.Val246Met Single submitter ⚠
- p.Val285Met Single submitter ⚠
- p.Val358Ala Single submitter
- p.Val366Met Single submitter ⚠
- p.Val413Leu Single submitter ⚠
- p.Val662Met Single submitter ⚠
- p.Val669Ile Single submitter ⚠