Legend
Please note that a short description of a certain column can be displayed when you move your mouse cursor over the column's header and hold it still. Below, a more detailed description is shown per column.
Effect : The variant's effect on the function of the gene/protein, displayed in the format 'R/C'. R is the value reported by the source (publication, submitter) and this classification may vary between records. C is the value concluded by the curator. Note that in some database the curator uses Summary records to give details on the classification of the variant.Values used: '+' indicating the variant affects function, '+?' probably affects function, '-' does not affect function, '-?' probably does not affect function, '?' effect unknown, '.' effect was not classified.
Exon : number of exon/intron containing variant; 2 = exon 2, 12i = intron 12, 2i_7i = from intron 2 to intron 7, 8i_9 = intron 8/exon 9 boundary, _1 = 5' to exon 1, 18_ = 3' of exon 18, _1_18_ = encompassing the entire 18-exon gene
DNA change (cDNA) : description of variant at DNA level, based on a coding DNA reference sequence (following HGVS recommendations); e.g. c.123C>T, c.123_145del, c.123_126dup. For deletions/duplications extending beyond the reference transcript resp. {0}/{2} is used to replace del/dup. Extent of the deletion/duplication should be specified using the genomic description (g.). "-" indicates the variant described on genomic level does not affect the coding DNA reference sequence.
RNA change : description of variant at RNA level (following HGVS recommendations).
r.123c>u
r.? = unknown
r.(?) = RNA not analysed but probably transcribed copy of DNA variant
r.spl? = RNA not analysed but variant probably affects splicing
r.(spl?) = RNA not analysed but variant may affect splicing
r.0? = change expected to abolish transcription
Protein : description of variant at protein level (following HGVS recommendations).
p.(Arg345Pro) = change predicted from DNA (RNA not analysed)
p.Arg345Pro = change derived from RNA analysis
p.? = unknown effect
p.0? = probably no protein produced
Allele : On which allele is the variant located? Does not necessarily imply inheritance! 'Paternal' (confirmed or inferred), 'Maternal' (confirmed or inferred), 'Parent #1' or #2 for compound heterozygosity without having screened the parents, 'Unknown' for heterozygosity without having screened the parents, 'Both' for homozygozity.
Classification method : The method used for the clinical classification of this variant.
All options:
ACMG
ACGS
EAHAD-CFDB
ENIGMA
IARC
InSiGHT
kConFab
other
Clinical classification : Clinical classification of variant, preferably based on standardised criteria (e.g. ACMG), directed on the clinical consequences as published/submitted, indicated using an enriched system including inheritance: e.g. pathogenic, pathogenic (dominant), pathogenic (recessive), pathogenic (!), pathogenic (maternal), pathogenic (paternal). Standard inheritance is covered by dominant/recessive, imprinting by maternal/paternal. A '!' warns for exceptional circumstances to be explained in the 'Remarks' field (low penetrance, variants pathogenic in heterozygous state only, hypomorphic/hypermorphic variants, protective variants, etc.). Non-disease consequences (e.g. drug metabolism (pharmacogenetics), risk factor, blood group, tasting bitter) are indicated using additions to the benign classification; benign (dominant), benign (recessive), benign (!), etc. The value 'association' is used for variants associated with a phenotype and 'NA' for variants from in vitro/in silico records. NOTE: classification may differ from the opinion of the curator as given in a variant SUMMARY-record or the 'Functional effect concluded'). NOTE: pathogenic/likely pathogenic should go together with "variant (probably) affects function" In ClassFunctional.
All options:
pathogenic
pathogenic (dominant)
pathogenic (recessive)
pathogenic (!)
pathogenic (maternal)
pathogenic (paternal)
likely pathogenic
likely pathogenic (dominant)
likely pathogenic (recessive)
likely pathogenic (!)
likely pathogenic (maternal)
likely pathogenic (paternal)
VUS
VUS (!)
likely benign
likely benign (dominant)
likely benign (recessive)
likely benign (!)
likely benign (maternal)
likely benign (paternal)
benign
benign (dominant)
benign (recessive)
benign (!)
benign (maternal)
benign (paternal)
conflicting
association
NA
DNA change (genomic) (hg19) : HGVS description of variant at DNA level, based on the genomic (chromosomal) DNA reference sequence; e.g. g.12345678C>T, g.12345679del, g.12345678_12345890dup
DNA change (hg38) : HGVS description of variant at DNA level, based on the hg38 genomic (chromosomal) eference sequence; e.g. g.12345678C>T, g.12345679del, g.12345678_12345890dup
Published as : listed only when different from "DNA change"; variant as reported originally (e.g. 521delT). Variants seen in animal models, tested in vitro, predicted from RNA analysis, etc. are described between brackets like c.(456C>G)
ISCN : description of the variant according to ISCN nomenclature
DB-ID : database ID of variant, grouping multiple observations of the same variant together, starting with the HGNC gene symbol, followed by an underscore (_) and a six digit number (e.g. DMD_012345). _000000 is used for variants where DNA was not analysed (change predicted from RNA analysis), variants seen in animal models or variants not seen in humans but functionally tested in vitro
Variant remarks : remarks regarding variant described, e.g. germline mosaicism in mother, 345 kb deletion, muscle RNA analysed, not in 200 control chromosomes tested, on founder haplotype, etc.
Reference : publication describing the variant submitted, incl. links to OMIM, PubMed or other source, e.g. "den Dunnen ASHG2003 P2346"
ClinVar ID : ID of variant in ClinVar database
dbSNP ID : the dbSNP ID
Origin : Origin of variant/record: Germline = in all cells, De novo = in all cells, but not in either parent, Germline/De novo (untested) = in all cells, parents not tested (use only when De novo is likely, e.g. isolated/sporadic cases with dominant disease), Somatic = present in a subset of cells, but not in either parent, Uniparental disomy = from parental disomy (maternal or paternal), CLASSIFICATION record = submitter only sharing variant classification (note another report may share Individual data), SUMMARY record = master summary record from curator (may link to another database), In vitro (cloned) = data resulting from in vitro functional assays, animal model = data from animal model, Artefact = false positive variant call, DUPLICATE record = variant already described on another chromosome (e.g. unbalanced translocation, duplicating transposition, 2nd fusion transcript, etc.)
All options:
Germline
De novo
Germline/De novo (untested)
Somatic
Uniparental disomy
Uniparental disomy, maternal allele
Uniparental disomy, paternal allele
CLASSIFICATION record
SUMMARY record
In vitro (cloned)
In silico
animal model
Artefact
DUPLICATE record
Unknown
Not applicable
Segregation : Indicates whether the variant segregates with the phenotype (yes), does not segregate with the phenotype (no) or segregation is unknown (?)
All options:
? = unknown
yes = segregates with phenotype
no = does not segregate with phenotype
- = not applicable
Frequency : frequency in which the variant was found; e.g 5/760 chromosomes (in 5 of 760 chromosomes tested), 1/33 patients (in 1 of 33 patients analysed in study), 0.05 controls (in 5% of control cases tested)
Re-site : restriction enzyme recognition site created (+) or destroyed (-); e.g. BglII+;BamHI-
VIP : variant VIP-status was requested for matchmaking - need collaboration(s) to crack the case - please contact the submitter/curator.
NOTE: to get VIP status ask the curator.
Methylation : result of methylation test; GOM (gain of methylation), LOM (loss of methylation), 30% (30% methylated). NOTE: when several tests were done mention the method as well (e.g. MS-PCR 75%)
Effect
Exon
DNA change (cDNA)
RNA change
Protein
Classification method
Clinical classification
DNA change (genomic) (hg19)
DNA change (hg38)
Published as
ISCN
DB-ID
Variant remarks
Reference
ClinVar ID
dbSNP ID
Origin
Segregation
Frequency
Re-site
VIP
Methylation
Owner
./.
-
c.-982063_*517476del
r.0?
p.0?
-
pathogenic
g.35304067_36866011del
-
-
-
CLIC6_000001
decreased gene dosage
PubMed: DDDS 2015 , Journal: DDDS 2015
-
-
De novo
-
-
-
-
-
Johan den Dunnen
-/.
-
c.-685C>T
r.(?)
p.(=)
-
benign
g.35884633G>A
g.34512335G>A
-
-
KCNE1_000099
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/.
-
c.-577C>T
r.(?)
p.(=)
-
benign
g.35884525G>A
g.34512227G>A
-
-
KCNE1_000098
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/.
-
c.-253A>G
r.(?)
p.(=)
-
likely benign
g.35883490T>C
g.34511192T>C
-
-
KCNE1_000097
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/.
-
c.-252G>A
r.(?)
p.(=)
-
benign
g.35883489C>T
g.34511191C>T
-
-
KCNE1_000084
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/.
-
c.-51+4G>A
r.spl?
p.?
-
likely benign
g.35830948C>T
-
KCNE1(NM_000219.4):c.-51+4G>A (p.?)
-
KCNE1_000158
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/?
4
c.-3del
r.(=)
p.(=)
-
VUS
g.35821935del
g.34449637del
-
-
KCNE1_000149
-
MORL Deafness Variation Database , PubMed: Duzkale 2013
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
?/.
-
c.-3del
r.(?)
p.(=)
-
VUS
g.35821935del
-
-
-
KCNE1_000149
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
-
c.4A>T
r.(?)
p.(Ile2Phe)
ACMG
VUS
g.35821929T>A
g.34449631T>A
-
-
KCNE1_000151
-
PubMed: Isbister 2020
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
-
c.8T>C
r.(?)
p.(Leu3Pro)
-
likely benign
g.35821925A>G
g.34449627A>G
-
-
KCNE1_000108
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/+
4
c.9_12del
r.(?)
p.(Ser4Thrfs*5)
-
pathogenic
g.35821923_35821926del
g.34449625_34449628del
-
-
KCNE1_000148
-
MORL Deafness Variation Database , PubMed: Kapplinger 2009
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
+/+
4
c.12dup
r.(?)
p.(Asn5*)
-
pathogenic
g.35821921dup
g.34449623dup
-
-
KCNE1_000147
-
MORL Deafness Variation Database , PubMed: Kapplinger 2009
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
-?/-?
4
c.18C>G
r.(=)
p.(=)
-
likely benign
g.35821915G>C
g.34449617G>C
-
-
KCNE1_000146
-
MORL Deafness Variation Database , PubMed: Duzkale 2013
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
+/+
4
c.20C>T
r.(?)
p.(Thr7Ile)
-
pathogenic
g.35821913G>A
g.34449615G>A
-
-
KCNE1_000145
-
MORL Deafness Variation Database , PubMed: Tranebjærg 1993 , PubMed: Schulze-Bahr 1997
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
+/+
4
c.23C>T
r.(?)
p.(Ala8Val)
-
pathogenic
g.35821910G>A
g.34449612G>A
-
-
KCNE1_000144
-
MORL Deafness Variation Database , PubMed: Ohno 2007 , PubMed: Priori 2013 , PubMed: Kapplinger 2009 , PubMed: Amendola 2015
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
?/.
-
c.23C>T
r.(?)
p.(Ala8Val)
-
VUS
g.35821910G>A
-
KCNE1(NM_001127670.1):c.23C>T (p.A8V)
-
KCNE1_000144
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/.
-
c.29C>T
r.(?)
p.(Thr10Met)
-
likely benign
g.35821904G>A
g.34449606G>A
KCNE1(NM_001127670.1):c.29C>T (p.T10M), KCNE1(NM_001127670.4):c.29C>T (p.T10M)
-
KCNE1_000096
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/+
4
c.29C>T
r.(?)
p.(Thr10Met)
-
pathogenic
g.35821904G>A
g.34449606G>A
-
-
KCNE1_000096
-
MORL Deafness Variation Database , PubMed: Priori 2013 , PubMed: Kapplinger 2009 , PubMed: Ng 2013 , PubMed: Amendola 2015
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
?/.
-
c.29C>T
r.(?)
p.(Thr10Met)
-
VUS
g.35821904G>A
g.34449606G>A
-
-
KCNE1_000096
conflicting interpretations of pathogenicity; 4 heterozygous, no homozygous; Clinindb (India)
PubMed: Narang 2020 , Journal: Narang 2020
-
rs144917638
Germline
-
4/2793 individuals
-
-
-
Mohammed Faruq
?/.
-
c.29C>T
r.(?)
p.(Thr10Met)
-
VUS
g.35821904G>A
g.34449606G>A
KCNE1(NM_001127670.1):c.29C>T (p.T10M), KCNE1(NM_001127670.4):c.29C>T (p.T10M)
-
KCNE1_000096
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
?/.
-
c.29C>T
r.(?)
p.(Thr10Met)
-
VUS
g.35821904G>A
-
KCNE1(NM_001127670.1):c.29C>T (p.T10M), KCNE1(NM_001127670.4):c.29C>T (p.T10M)
-
KCNE1_000096
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/.
-
c.30G>A
r.(?)
p.(Thr10=)
-
benign
g.35821903C>T
g.34449605C>T
KCNE1(NM_000219.3):c.30G>A (p.T10=)
-
KCNE1_000107
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/.
-
c.30G>A
r.(?)
p.(Thr10=)
-
likely benign
g.35821903C>T
-
KCNE1(NM_000219.3):c.30G>A (p.T10=)
-
KCNE1_000107
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-?/-?
4
c.30G>T
r.(=)
p.(=)
-
likely benign
g.35821903C>A
g.34449605C>A
-
-
KCNE1_000143
-
MORL Deafness Variation Database
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
+/+
4
c.47T>C
r.(?)
p.(Leu16Pro)
-
pathogenic
g.35821886A>G
g.34449588A>G
-
-
KCNE1_000142
-
MORL Deafness Variation Database , PubMed: Yoshinaga 2014
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
?/.
-
c.49T>C
r.(?)
p.(Trp17Arg)
-
VUS
g.35821884A>G
g.34449586A>G
KCNE1(NM_001127670.1):c.49T>C (p.W17R)
-
KCNE1_000095
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/+
4
c.50G>A
r.(?)
p.(Trp17*)
-
pathogenic
g.35821883C>T
g.34449585C>T
-
-
KCNE1_000141
-
MORL Deafness Variation Database , PubMed: Kapplinger 2009
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
+/+
4
c.59C>T
r.(?)
p.(Thr20Ile)
-
pathogenic
g.35821874G>A
g.34449576G>A
-
-
KCNE1_000140
-
MORL Deafness Variation Database , PubMed: Millat 2009
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
+/+
4
c.74G>T
r.(?)
p.(Gly25Val)
-
pathogenic
g.35821859C>A
g.34449561C>A
-
-
KCNE1_000139
-
MORL Deafness Variation Database , PubMed: Olesen 2012
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
+/+
4
c.83C>T
r.(?)
p.(Ser28Leu)
-
pathogenic
g.35821850G>A
g.34449552G>A
-
-
KCNE1_000138
-
MORL Deafness Variation Database , PubMed: Obeyesekere 2012 , PubMed: Kapplinger 2009 , PubMed: Shim 2005
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
-/.
-
c.84G>A
r.(?)
p.(Ser28=)
-
benign
g.35821849C>T
g.34449551C>T
KCNE1(NM_000219.3):c.84G>A (p.S28=), KCNE1(NM_001127670.1):c.84G>A (p.S28=), LOC102723475(NM_001330065.1):c.93G>A (p.S31=)
-
KCNE1_000083
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/.
-
c.84G>A
r.(?)
p.(Ser28=)
-
likely benign
g.35821849C>T
g.34449551C>T
KCNE1(NM_000219.3):c.84G>A (p.S28=), KCNE1(NM_001127670.1):c.84G>A (p.S28=), LOC102723475(NM_001330065.1):c.93G>A (p.S31=)
-
KCNE1_000083
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
-
c.84G>A
r.(?)
p.(Ser28=)
-
likely benign
g.35821849C>T
g.34449551C>T
KCNE1(NM_000219.3):c.84G>A (p.S28=), KCNE1(NM_001127670.1):c.84G>A (p.S28=), LOC102723475(NM_001330065.1):c.93G>A (p.S31=)
-
KCNE1_000083
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-/.
-
c.84G>A
r.(?)
p.(Ser28=)
-
benign
g.35821849C>T
g.34449551C>T
KCNE1(NM_000219.3):c.84G>A (p.S28=), KCNE1(NM_001127670.1):c.84G>A (p.S28=), LOC102723475(NM_001330065.1):c.93G>A (p.S31=)
-
KCNE1_000083
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/.
-
c.85G>A
r.(?)
p.(Gly29Ser)
-
likely benign
g.35821848C>T
g.34449550C>T
-
-
KCNE1_000094
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/+
4
c.94C>T
r.(?)
p.(Arg32Cys)
-
pathogenic
g.35821839G>A
g.34449541G>A
-
-
KCNE1_000137
-
MORL Deafness Variation Database , PubMed: McGorrian 2013
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
?/.
-
c.94C>T
r.(?)
p.(Arg32Cys)
-
VUS
g.35821839G>A
-
KCNE1(NM_001127670.1):c.94C>T (p.R32C)
-
KCNE1_000137
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+?/+?
4
c.95G>A
r.(?)
p.(Arg32His)
-
likely pathogenic
g.35821838C>T
g.34449540C>T
-
-
KCNE1_000136
-
MORL Deafness Variation Database , PubMed: Berge 2008 , PubMed: Kapplinger 2009 , PubMed: Westenskow 2004 , PubMed: Splawski 2000
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
+?/.
-
c.95G>A
r.(?)
p.(Arg32His)
-
likely pathogenic
g.35821838C>T
g.34449540C>T
-
-
KCNE1_000136
variants reported seperately, unknown if mono-allelic or bi-allelic
PubMed: Retterer 2016
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
?/.
-
c.106C>T
r.(?)
p.(Arg36Cys)
-
VUS
g.35821827G>A
g.34449529G>A
NM_001127670:c.C106T
-
KCNE1_000071
-
PubMed: Lopes 2013 , Journal: Lopes 2013
-
-
Germline
-
1/223 cases HCM
-
-
-
Johan den Dunnen
?/.
-
c.106C>T
r.(?)
p.(Arg36Cys)
-
VUS
g.35821827G>A
g.34449529G>A
KCNE1(NM_001127670.1):c.106C>T (p.R36C)
-
KCNE1_000071
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
-
c.107G>A
r.(?)
p.(Arg36His)
-
likely benign
g.35821826C>T
g.34449528C>T
-
-
KCNE1_000106
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/+
4
c.107G>A
r.(?)
p.(Arg36His)
-
pathogenic
g.35821826C>T
g.34449528C>T
-
-
KCNE1_000106
-
MORL Deafness Variation Database , PubMed: Napolitano 2005
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
?/?
4
c.109A>G
r.(?)
p.(Ser37Gly)
-
VUS
g.35821824T>C
g.34449526T>C
-
-
KCNE1_000135
-
MORL Deafness Variation Database , PubMed: Ng 2013
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
-/.
-
c.111C>T
r.(?)
p.(Ser37=)
-
benign
g.35821822G>A
g.34449524G>A
KCNE1(NM_001127670.1):c.111C>T (p.S37=)
-
KCNE1_000093
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/-
4
c.112A>G
r.(?)
p.(Ser38Gly)
-
benign
g.35821821T>C
g.34449523T>C
112G>A (G38S)
-
KCNE1_000076
-
PubMed: Ackerman 2003
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/-
4
c.112A>G
r.(?)
p.(Ser38Gly)
-
benign
g.35821821T>C
g.34449523T>C
113G>A (G38S)
-
KCNE1_000076
-
PubMed: Ackerman 2003
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/-
4
c.112A>G
r.(?)
p.(Ser38Gly)
-
benign
g.35821821T>C
g.34449523T>C
114G>A (G38S)
-
KCNE1_000076
-
PubMed: Ackerman 2003
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/-
4
c.112A>G
r.(?)
p.(Ser38Gly)
-
benign
g.35821821T>C
g.34449523T>C
115G>A (G38S)
-
KCNE1_000076
-
PubMed: Ackerman 2003
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
-
c.112A>G
r.(?)
p.(Ser38Gly)
-
benign
g.35821821T>C
g.34449523T>C
KCNE1(NM_000219.3):c.112A>G (p.S38G), KCNE1(NM_000219.6):c.112A>G (p.S38G), LOC102723475(NM_001330065.1):c.121A>G (p.S41G)
-
KCNE1_000076
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/.
-
c.112A>G
r.(?)
p.(Ser38Gly)
-
benign
g.35821821T>C
g.34449523T>C
KCNE1(NM_000219.3):c.112A>G (p.S38G), KCNE1(NM_000219.6):c.112A>G (p.S38G), LOC102723475(NM_001330065.1):c.121A>G (p.S41G)
-
KCNE1_000076
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-/.
-
c.112A>G
r.(?)
p.(Ser38Gly)
-
benign
g.35821821T>C
g.34449523T>C
KCNE1(NM_000219.3):c.112A>G (p.S38G), KCNE1(NM_000219.6):c.112A>G (p.S38G), LOC102723475(NM_001330065.1):c.121A>G (p.S41G)
-
KCNE1_000076
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/.
-
c.112A>G
r.(?)
p.(Ser38Gly)
-
benign
g.35821821T>C
g.34449523T>C
KCNE1(NM_000219.3):c.112A>G (p.S38G), KCNE1(NM_000219.6):c.112A>G (p.S38G), LOC102723475(NM_001330065.1):c.121A>G (p.S41G)
-
KCNE1_000076
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/-
4
c.115G>A
r.(?)
p.(Asp39Asn)
-
benign
g.35821818C>T
g.34449520C>T
-
-
KCNE1_000134
-
MORL Deafness Variation Database , PubMed: Abitbol 1999
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
-?/.
-
c.117C>T
r.(?)
p.(Asp39=)
-
likely benign
g.35821816G>A
g.34449518G>A
-
-
KCNE1_000092
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/.
-
c.129G>A
r.(?)
p.(Glu43=)
-
likely benign
g.35821804C>T
g.34449506C>T
-
-
KCNE1_000105
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/.
-
c.133del
r.(?)
p.(Leu45Serfs*19)
-
likely pathogenic
g.35821802del
-
KCNE1(NM_000219.3):c.133delC (p.L45Sfs*19)
-
KCNE1_000154
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
?/.
-
c.139G>A
r.(?)
p.(Val47Ile)
-
VUS
g.35821794C>T
g.34449496C>T
KCNE1(NM_001127670.1):c.139G>A (p.V47I)
-
KCNE1_000091
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
-
c.139G>A
r.(?)
p.(Val47Ile)
-
likely benign
g.35821794C>T
-
KCNE1(NM_001127670.1):c.139G>A (p.V47I)
-
KCNE1_000091
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
-
c.139G>A
r.(?)
p.(Val47Ile)
-
VUS
g.35821794C>T
g.34449496C>T
-
-
KCNE1_000091
-
PubMed: Eisenberger 2018
-
rs199473353
Germline
-
-
-
-
-
Johan den Dunnen
+/+
4
c.139G>T
r.(?)
p.(Val47Phe)
-
pathogenic
g.35821794C>A
g.34449496C>A
-
-
KCNE1_000133
-
MORL Deafness Variation Database , PubMed: Bianchi 1999
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
?/.
-
c.142C>T
r.(?)
p.(Leu48Phe)
-
VUS
g.35821791G>A
g.34449493G>A
KCNE1(NM_000219.3):c.142C>T (p.L48F), KCNE1(NM_000219.6):c.142C>T (p.(Leu48Phe), p.L48F), KCNE1(NM_001127670.1):c.142C>T (p.L48F)
-
KCNE1_000082
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
?/.
-
c.142C>T
r.(?)
p.(Leu48Phe)
-
VUS
g.35821791G>A
-
KCNE1(NM_000219.3):c.142C>T (p.L48F), KCNE1(NM_000219.6):c.142C>T (p.(Leu48Phe), p.L48F), KCNE1(NM_001127670.1):c.142C>T (p.L48F)
-
KCNE1_000082
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
-
c.142C>T
r.(?)
p.(Leu48Phe)
-
VUS
g.35821791G>A
-
KCNE1(NM_000219.3):c.142C>T (p.L48F), KCNE1(NM_000219.6):c.142C>T (p.(Leu48Phe), p.L48F), KCNE1(NM_001127670.1):c.142C>T (p.L48F)
-
KCNE1_000082
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
?/.
-
c.142C>T
r.(?)
p.(Leu48Phe)
-
VUS
g.35821791G>A
-
KCNE1(NM_000219.3):c.142C>T (p.L48F), KCNE1(NM_000219.6):c.142C>T (p.(Leu48Phe), p.L48F), KCNE1(NM_001127670.1):c.142C>T (p.L48F)
-
KCNE1_000082
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
-
c.142C>T
r.(?)
p.(Leu48Phe)
-
VUS
g.35821791G>A
-
KCNE1(NM_000219.3):c.142C>T (p.L48F), KCNE1(NM_000219.6):c.142C>T (p.(Leu48Phe), p.L48F), KCNE1(NM_001127670.1):c.142C>T (p.L48F)
-
KCNE1_000082
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/+
4
c.147G>A
r.(?)
p.(Met49Ile)
-
pathogenic
g.35821786C>T
g.34449488C>T
-
-
KCNE1_000132
-
MORL Deafness Variation Database , PubMed: Lieve 2013
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
?/.
-
c.148G>A
r.(?)
p.(Val50Ile)
-
VUS
g.35821785C>T
-
KCNE1(NM_001127670.1):c.148G>A (p.V50I)
-
KCNE1_000152
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/+
4
c.152_153delinsAT
r.(?)
p.(Leu51His)
-
pathogenic
g.35821780_35821781delinsAT
g.34449482_34449483delinsAT
-
-
KCNE1_000131
-
MORL Deafness Variation Database , PubMed: Bianchi 1999
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
+/+
4
c.154G>A
r.(?)
p.(Gly52Arg)
-
pathogenic
g.35821779C>T
g.34449481C>T
-
-
KCNE1_000130
-
MORL Deafness Variation Database , PubMed: Ma 2003 , PubMed: Harmer 2010
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
-/-
4
c.155G>C
r.(?)
p.(Gly52Ala)
-
benign
g.35821778C>G
g.34449480C>G
-
-
KCNE1_000075
-
PubMed: Ackerman 2003
-
-
Germline
-
1/305 controls
-
-
-
Johan den Dunnen
+/+
4
c.158T>C
r.(?)
p.(Phe53Ser)
-
pathogenic
g.35821775A>G
g.34449477A>G
-
-
KCNE1_000129
-
MORL Deafness Variation Database , PubMed: Napolitano 2005
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
+?/+?
4
c.160T>G
r.(?)
p.(Phe54Val)
-
likely pathogenic
g.35821773A>C
g.34449475A>C
-
-
KCNE1_000128
-
MORL Deafness Variation Database , PubMed: Liu 2013
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
-/.
-
c.162C>T
r.(?)
p.(Phe54=)
-
benign
g.35821771G>A
g.34449473G>A
KCNE1(NM_000219.3):c.162C>T (p.F54=)
-
KCNE1_000081
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/.
-
c.162C>T
r.(?)
p.(Phe54=)
-
likely benign
g.35821771G>A
g.34449473G>A
KCNE1(NM_000219.3):c.162C>T (p.F54=)
-
KCNE1_000081
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-?/.
-
c.162C>T
r.(?)
p.(Phe54=)
-
likely benign
g.35821771G>A
g.34449473G>A
KCNE1(NM_000219.3):c.162C>T (p.F54=)
-
KCNE1_000081
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/.
-
c.163G>A
r.(?)
p.(Gly55Ser)
-
likely benign
g.35821770C>T
g.34449472C>T
-
-
KCNE1_000104
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/+
4
c.163G>A
r.(?)
p.(Gly55Ser)
-
pathogenic
g.35821770C>T
g.34449472C>T
-
-
KCNE1_000104
-
MORL Deafness Variation Database , PubMed: Kapplinger 2009 , PubMed: Duzkale 2013
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
?/.
-
c.163G>A
r.(?)
p.(Gly55Ser)
-
VUS
g.35821770C>T
g.34449472C>T
-
-
KCNE1_000104
3 heterozygous, no homozygous; Clinindb (India)
PubMed: Narang 2020 , Journal: Narang 2020
-
rs199473644
Germline
-
3/2795 individuals
-
-
-
Mohammed Faruq
+/+
4
c.172A>C
r.(?)
p.(Thr58Pro)
-
pathogenic
g.35821761T>G
g.34449463T>G
-
-
KCNE1_000127
-
MORL Deafness Variation Database , PubMed: Tyson 1997 , PubMed: Kapplinger 2009
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
+/+
4
c.172_177delinsCCCCCT
r.(?)
p.(Thr58_Leu59delinsProPro)
-
pathogenic
g.35821756_35821761delinsAGGGGG
g.34449458_34449463delinsAGGGGG
-
-
KCNE1_000125
-
MORL Deafness Variation Database , PubMed: Tranebjærg 1993 , PubMed: Tyson 1997 , PubMed: Huang 2001
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
-/.
-
c.174C>G
r.(?)
p.(Thr58=)
-
benign
g.35821759G>C
g.34449461G>C
KCNE1(NM_000219.3):c.174C>G (p.T58=), KCNE1(NM_001127670.1):c.174C>G (p.T58=)
-
KCNE1_000090
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/.
-
c.174C>G
r.(?)
p.(Thr58=)
-
likely benign
g.35821759G>C
g.34449461G>C
KCNE1(NM_000219.3):c.174C>G (p.T58=), KCNE1(NM_001127670.1):c.174C>G (p.T58=)
-
KCNE1_000090
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-?/-?
4
c.174C>G
r.(=)
p.(=)
-
likely benign
g.35821759G>C
g.34449461G>C
-
-
KCNE1_000090
-
MORL Deafness Variation Database , PubMed: Duzkale 2013
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
-?/.
-
c.174C>G
r.(?)
p.(Thr58=)
-
likely benign
g.35821759G>C
-
KCNE1(NM_000219.3):c.174C>G (p.T58=), KCNE1(NM_001127670.1):c.174C>G (p.T58=)
-
KCNE1_000090
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/+
4
c.176T>C
r.(?)
p.(Leu59Pro)
-
pathogenic
g.35821757A>G
g.34449459A>G
-
-
KCNE1_000126
-
MORL Deafness Variation Database , PubMed: Kapplinger 2009
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
+/+
4
c.179G>A
r.(?)
p.(Gly60Asp)
-
pathogenic
g.35821754C>T
g.34449456C>T
-
-
KCNE1_000124
-
MORL Deafness Variation Database , PubMed: Olesen 2012
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
+/+
4
c.199C>T
r.(?)
p.(Arg67Cys)
-
pathogenic
g.35821734G>A
g.34449436G>A
-
-
KCNE1_000123
-
MORL Deafness Variation Database , PubMed: Kapplinger 2009 , PubMed: Duzkale 2013
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
?/.
-
c.200G>A
r.(?)
p.(Arg67His)
-
VUS
g.35821733C>T
g.34449435C>T
KCNE1(NM_000219.3):c.200G>A (p.R67H)
-
KCNE1_000080
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/+
4
c.200G>A
r.(?)
p.(Arg67His)
-
pathogenic
g.35821733C>T
g.34449435C>T
-
-
KCNE1_000080
-
MORL Deafness Variation Database , PubMed: Priori 2013 , PubMed: Kapplinger 2009 , PubMed: Amendola 2015
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
?/?
4
c.202T>A
r.(?)
p.(Ser68Thr)
-
VUS
g.35821731A>T
g.34449433A>T
-
-
KCNE1_000122
-
MORL Deafness Variation Database , PubMed: Abitbol 1999
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
-/-
4
c.206A>G
r.(?)
p.(Lys69Arg)
-
benign
g.35821727T>C
g.34449429T>C
-
-
KCNE1_000074
-
PubMed: Ackerman 2003
-
-
Germline
-
1/305 controls
-
-
-
Johan den Dunnen
-?/-?
4
c.206A>G
r.(?)
p.(Lys69Arg)
-
likely benign
g.35821727T>C
g.34449429T>C
-
-
KCNE1_000074
-
MORL Deafness Variation Database , PubMed: Ackerman 2003 , PubMed: Ng 2013
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
?/.
-
c.206A>G
r.(?)
p.(Lys69Arg)
-
VUS
g.35821727T>C
-
KCNE1(NM_000219.4):c.206A>G (p.(Lys69Arg)), KCNE1(NM_001127670.1):c.206A>G (p.K69R)
-
KCNE1_000074
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/.
-
c.206A>G
r.(?)
p.(Lys69Arg)
-
likely benign
g.35821727T>C
-
KCNE1(NM_000219.4):c.206A>G (p.(Lys69Arg)), KCNE1(NM_001127670.1):c.206A>G (p.K69R)
-
KCNE1_000074
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/+
4
c.209A>T
r.(?)
p.(Lys70Met)
-
pathogenic
g.35821724T>A
g.34449426T>A
-
-
KCNE1_000121
-
MORL Deafness Variation Database , PubMed: Kapplinger 2009
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
+/+
4
c.210G>C
r.(?)
p.(Lys70Asn)
-
pathogenic
g.35821723C>G
g.34449425C>G
-
-
KCNE1_000120
-
MORL Deafness Variation Database , PubMed: Lai 2005
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
-/-
4
c.220T>G
r.(?)
p.(Ser74Ala)
-
benign
g.35821713A>C
g.34449415A>C
-
-
KCNE1_000119
-
MORL Deafness Variation Database , PubMed: Abitbol 1999
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
+/+
4
c.221C>T
r.(?)
p.(Ser74Leu)
-
pathogenic
g.35821712G>A
g.34449414G>A
-
-
KCNE1_000118
-
MORL Deafness Variation Database , PubMed: Alders 1993 , PubMed: Splawski 1997 , PubMed: Westenskow 2004 , PubMed: Harmer 2010
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
-?/.
-
c.222G>A
r.(?)
p.(=)
-
likely benign
g.35821711C>T
-
KCNE1(NM_001127670.1):c.222G>A (p.S74=)
-
KCNE1_000157
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC