Global Variome shared LOVD
KCNJ2 (potassium inwardly-rectifying channel, subfa...)
LOVD v.3.0 Build 30b [
Current LOVD status
]
Register as submitter
|
Log in
Curator:
Global Variome, with Curator vacancy
View all genes
View KCNJ2 gene homepage
View graphs about the KCNJ2 gene database
Create a new gene entry
View all transcripts
View all transcripts of gene KCNJ2
Create a new transcript information entry
View all variants
View all variants affecting transcripts
View unique variants in gene KCNJ2
View all variants in gene KCNJ2
Full data view for gene KCNJ2
Create a new data submission
View active genomic custom columns
Enable more genomic custom columns
View all individuals
View all individuals with variants in gene KCNJ2
Create a new data submission
View active custom columns
Enable more custom columns
View all diseases
View all diseases associated with gene KCNJ2
Create a new disease information entry
View available phenotype columns
View all screenings
View all screenings for gene KCNJ2
Create a new data submission
View active custom columns
Enable more custom columns
Submit new data
All variants in the KCNJ2 gene
The variants shown are described using the NM_000891.2 transcript reference sequence.
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%)
How to query this table
All list views have search fields which can be used to search data. You can search for a complete word or you can search for a part of a search term. If you enclose two or more words in double quotes, LOVD will search for the combination of those words only exactly in the order you specify. Note that search terms are case-insensitive and that wildcards such as * are treated as normal text! For all options, like "and", "or", and "not" searches, or searching for prefixes or suffixes, see the table below.
Operator
Column type
Example
Matches
Text
Arg
all entries containing 'Arg'
space
Text
Arg Ser
all entries containing 'Arg' and 'Ser'
|
Text
Arg|Ser
all entries containing 'Arg' or 'Ser'
!
Text
!fs
all entries not containing 'fs'
^
Text
^p.(Arg
all entries beginning with 'p.(Arg'
$
Text
Ser)$
all entries ending with 'Ser)'
=""
Text
=""
all entries with this field empty
=""
Text
="p.0"
all entries exactly matching 'p.0'
!=""
Text
!=""
all entries with this field not empty
!=""
Text
!="p.0"
all entries not exactly matching 'p.0?'
combination
Text
*|Ter !fs
all entries containing '*' or 'Ter' but not containing 'fs'
Date
2020
all entries matching the year 2020
|
Date
2020-03|2020-04
all entries matching March or April, 2020
!
Date
!2020-03
all entries not matching March, 2020
<
Date
<2020
all entries before the year 2020
<=
Date
<=2020-06
all entries in or before June, 2020
>
Date
>2020-06
all entries after June, 2020
>=
Date
>=2020-06-15
all entries on or after June 15th, 2020
combination
Date
2019|2020 <2020-03
all entries in 2019 or 2020, and before March, 2020
Numeric
23
all entries exactly matching 23
|
Numeric
23|24
all entries exactly matching 23 or 24
!
Numeric
!23
all entries not exactly matching 23
<
Numeric
<23
all entries lower than 23
<=
Numeric
<=23
all entries lower than, or equal to, 23
>
Numeric
>23
all entries higher than 23
>=
Numeric
>=23
all entries higher than, or equal to, 23
combination
Numeric
>=20 <30 !23
all entries with values from 20 to 29, but not equal to 23
Some more advanced examples:
Example
Matches
Asian
all entries containing 'Asian', 'asian', including 'Caucasian', 'caucasian', etc.
Asian !Caucasian
all entries containing 'Asian' but not containing 'Caucasian'
Asian|African !Caucasian
all entries containing 'Asian' or 'African', but not containing 'Caucasian'
"South Asian"
all entries containing 'South Asian', but not containing 'South East Asian'
To sort on a certain column, click on the column header or on the arrows. If that column is already selected to sort on, the sort order will be swapped. The column currently sorted on has a darker blue background color than the other columns. The up and down arrows next to the column name indicate the current sorting direction. When sorting on any field other than the default, LOVD will sort secondarily on the default sort column.
192 entries on 2 pages. Showing entries 1 - 100.
10 per page
25 per page
50 per page
100 per page
Legend
How to query
« First
Prev
1
2
Next
Last »
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.-158_-157insGTAGTCAGA
r.(?)
p.(=)
-
likely benign
g.68171023_68171024insGTAGTCAGA
g.70174882_70174883insGTAGTCAGA
-
-
KCNJ2_000078
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/.
-
c.-2C>T
r.(?)
p.(=)
-
likely benign
g.68171179C>T
g.70175038C>T
KCNJ2(NM_000891.3):c.-2C>T
-
KCNJ2_000079
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/?
2
c.283-_294+del
r.(?)
p.(Ser95_Phe98del)
-
pathogenic
g.?
-
-
-
KCNJ2_000019
M1, buried residue, scatterd cytoplasmic pattern (degraede proteins or channel mistrafficking)
PubMed: Plaster 2001
-
-
Germline
-
1/16 ATS families
-
-
-
Ikuko Takeda
+/?
2
c.940-_945+del
r.(?)
p.(Ser314_Tyr315del)
-
pathogenic
g.?
-
-
-
KCNJ2_000059
G-loop in cytoplasmic, scatterd cytoplasmic pattern (degraede proteins or channel mistrafficking), golgi export motif
PubMed: Yoon 2006
PubMed: Plaster 2001
-
-
De novo
-
1/16 ATS families
-
-
-
Ikuko Takeda
?/.
-
c.22C>T
r.(?)
p.(Arg8Cys)
-
VUS
g.68171202C>T
-
KCNJ2(NM_000891.2):c.22C>T (p.(Arg8Cys))
-
KCNJ2_000110
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
-
c.97G>C
r.(?)
p.(Gly33Arg)
-
VUS
g.68171277G>C
-
KCNJ2(NM_000891.2):c.97G>C (p.G33R)
-
KCNJ2_000108
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
?/?
2
c.118C>T
r.(?)
p.(Arg40*)
-
VUS
g.68171298C>T
g.70175157C>T
-
-
KCNJ2_000002
cytoplamic, N-terminus
PubMed: Kuramoto 2012
-
-
Unknown
-
-
-
-
-
Ikuko Takeda
?/.
-
c.128G>A
r.(?)
p.(Cys43Tyr)
-
VUS
g.68171308G>A
-
KCNJ2(NM_000891.3):c.128G>A (p.C43Y)
-
KCNJ2_000112
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
-
c.146A>G
r.(?)
p.(Lys49Arg)
-
VUS
g.68171326A>G
g.70175185A>G
-
-
KCNJ2_000080
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/?
2
c.161G>T
r.(?)
p.(Cys54Phe)
-
pathogenic
g.68171341G>T
g.70175200G>T
-
-
KCNJ2_000003
cytoplasmic
PubMed: Bendahhou 2007
-
-
Germline
-
-
-
-
-
Ikuko Takeda
-/.
-
c.174C>T
r.(?)
p.(Phe58=)
-
benign
g.68171354C>T
g.70175213C>T
KCNJ2(NM_000891.3):c.174C>T (p.F58=)
-
KCNJ2_000072
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/?
2
c.199C>T
r.(?)
p.(Arg67Trp)
-
pathogenic
g.68171379C>T
g.70175238C>T
-
-
KCNJ2_000004
PIP2 binding, cytoplasmic
PubMed: Andelfinger 2002
-
-
Germline
-
41/77 in the family
-
-
-
Ikuko Takeda
+/?
2
c.199C>T
r.(?)
p.(Arg67Trp)
-
pathogenic
g.68171379C>T
g.70175238C>T
-
-
KCNJ2_000004
KJ-01, PIP2 binding, cytoplasmic
PubMed: Haruna 2007
-
-
Germline
-
-
-
-
-
Ikuko Takeda
+/?
2
c.199C>T
r.(?)
p.(Arg67Trp)
-
pathogenic
g.68171379C>T
g.70175238C>T
-
-
KCNJ2_000004
PIP2 binding, cytoplasmic
PubMed: Donaldson 2003
-
-
De novo
-
2/17 ATS probands
-
-
-
Ikuko Takeda
+/?
2
c.199C>T
r.(?)
p.(Arg67Trp)
-
pathogenic
g.68171379C>T
g.70175238C>T
-
-
KCNJ2_000004
PIP2 binding, cytoplasmic
PubMed: Donaldson 2003
-
-
Unknown
-
2/17 ATS probands
-
-
-
Ikuko Takeda
+/?
2
c.199C>T
r.(?)
p.(Arg67Trp)
-
pathogenic
g.68171379C>T
g.70175238C>T
-
-
KCNJ2_000004
PIP2 binding, cytoplasmic
PubMed: Davies 2005
-
-
De novo
-
2/>140 periodic paralysis
-
-
-
Ikuko Takeda
+/?
2
c.199C>T
r.(?)
p.(Arg67Trp)
-
pathogenic
g.68171379C>T
g.70175238C>T
-
-
KCNJ2_000004
PIP2 binding, cytoplasmic
PubMed: Davies 2005
-
-
Germline
-
2/>140 periodic paralysis
-
-
-
Ikuko Takeda
+/?
2
c.199C>T
r.(?)
p.(Arg67Trp)
-
pathogenic
g.68171379C>T
g.70175238C>T
-
-
KCNJ2_000004
PIP2 binding, cytoplasmic
PubMed: Kimura 2012
-
-
Unknown
-
-
-
-
-
Ikuko Takeda
+/.
-
c.199C>T
r.(?)
p.(Arg67Trp)
-
pathogenic
g.68171379C>T
-
KCNJ2(NM_000891.2):c.199C>T (p.(Arg67Trp))
-
KCNJ2_000004
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+?/?
2
c.200G>A
r.(?)
p.(Arg67Gln)
-
likely pathogenic
g.68171380G>A
g.70175239G>A
-
-
KCNJ2_000005
PIP2 binding, no dominant negative effect when co-expressed with WT, cytoplasmic / *Frequency: patients with a suspected clinical diagnosis of congenital long QT syndrome (LQTS)
PubMed: Eckhardt 2007
-
-
Unknown
-
1/541 patients*
-
-
-
Ikuko Takeda
+/?
2
c.200G>A
r.(?)
p.(Arg67Gln)
-
pathogenic
g.68171380G>A
g.70175239G>A
-
-
KCNJ2_000005
Kindred 323, PIP2 binding, trafficking defect, dominant negative effect
PubMed: Haruna 2007
-
-
Germline
-
-
-
-
-
Ikuko Takeda
+?/.
-
c.200G>A
r.(?)
p.(Arg67Gln)
-
likely pathogenic
g.68171380G>A
g.70175239G>A
-
-
KCNJ2_000005
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/?
2
c.202T>G
r.(?)
p.(Tyr68Asp)
-
likely pathogenic
g.68171382T>G
g.70175241T>G
-
-
KCNJ2_000006
cytoplasmic, slide helix
PubMed: Davies 2005
-
-
Germline
-
1/>140 periodic paralysis
-
-
-
Ikuko Takeda
?/.
-
c.208G>T
r.(?)
p.(Ala70Ser)
-
VUS
g.68171388G>T
g.70175247G>T
KCNJ2(NM_000891.2):c.208G>T (p.(Ala70Ser)), KCNJ2(NM_000891.3):c.208G>T (p.A70S)
-
KCNJ2_000073
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
-
c.208G>T
r.(?)
p.(Ala70Ser)
-
VUS
g.68171388G>T
-
KCNJ2(NM_000891.2):c.208G>T (p.(Ala70Ser)), KCNJ2(NM_000891.3):c.208G>T (p.A70S)
-
KCNJ2_000073
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/?
2
c.211G>A
r.(?)
p.(Asp71Asn)
-
VUS
g.68171391G>A
g.70175250G>A
-
-
KCNJ2_000007
cytoplasmic, slide helix
PubMed: Donaldson 2003
-
-
De novo
-
1/17 ATS probands
-
-
-
Ikuko Takeda
+/?
2
c.211G>T
r.(?)
p.(Asp71Tyr)
-
pathogenic
g.68171391G>T
g.70175250G>T
-
-
KCNJ2_000008
cytoplasmic, slide helix
PubMed: Marrus 2011
-
-
Unknown
-
-
-
-
-
Ikuko Takeda
+/?
2
c.212A>T
r.(?)
p.(Asp71Val)
-
pathogenic
g.68171392A>T
g.70175251A>T
-
-
KCNJ2_000009
cytoplasmic, slide helix, dominant negative
PubMed: Plaster 2001
-
-
Germline
-
1/16 ATS families
-
-
-
Ikuko Takeda
?/.
-
c.213C>A
r.(?)
p.(Asp71Glu)
-
VUS
g.68171393C>A
g.70175252C>A
KCNJ2(NM_000891.3):c.213C>A (p.D71E)
-
KCNJ2_000081
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/.
-
c.213C>T
r.(?)
p.(Asp71=)
-
benign
g.68171393C>T
-
KCNJ2(NM_000891.2):c.213C>T (p.D71=), KCNJ2(NM_000891.3):c.213C>T (p.D71=)
-
KCNJ2_000099
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/.
-
c.213C>T
r.(?)
p.(Asp71=)
-
likely benign
g.68171393C>T
-
KCNJ2(NM_000891.2):c.213C>T (p.D71=), KCNJ2(NM_000891.3):c.213C>T (p.D71=)
-
KCNJ2_000099
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/?
2
c.220A>G
r.(?)
p.(Thr74Ala)
-
pathogenic
g.68171400A>G
g.70175259A>G
-
-
KCNJ2_000010
slide helix, channel gating, PIP2 sensitivity
PubMed: Ballester 2006
-
-
Germline
-
-
-
-
-
Ikuko Takeda
+/?
2
c.223A>G
r.(?)
p.(Thr75Ala)
-
pathogenic
g.68171403A>G
g.70175262A>G
-
-
KCNJ2_000011
cytoplasmic, slide helix, dominant negative effect
PubMed: Fodstad 2004
-
-
Germline
-
1/188 LQT patients
-
-
-
Ikuko Takeda
+/?
2
c.224C>G
r.(?)
p.(Thr75Arg)
-
pathogenic
g.68171404C>G
g.70175263C>G
-
-
KCNJ2_000012
cytoplasmic, slide helix
PubMed: Yoon 2006
-
-
Unknown
-
-
-
-
-
Ikuko Takeda
+/?
2
c.224C>G
r.(?)
p.(Thr75Arg)
-
pathogenic
g.68171404C>G
g.70175263C>G
-
-
KCNJ2_000012
cytoplasmic, slide helix
PubMed: Donaldson 2003
-
-
De novo
-
1/17 ATS probands
-
-
-
Ikuko Takeda
+/?
2
c.224C>G
r.(?)
p.(Thr75Arg)
-
pathogenic
g.68171404C>G
g.70175263C>G
-
-
KCNJ2_000012
cytoplasmic, slide helix
PubMed: Lu 2006
-
-
Germline
-
-
-
-
-
Ikuko Takeda
+/.
-
c.224C>G
r.(?)
p.(Thr75Arg)
-
pathogenic
g.68171404C>G
g.70175263C>G
-
-
KCNJ2_000012
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/?
2
c.224C>T
r.(?)
p.(Thy75Met)
-
pathogenic
g.68171404C>T
g.70175263C>T
-
-
KCNJ2_000013
cytoplasmic region, slide helix, N-terminus, defective tafficking, no dominant negative effect
PubMed: Tani 2007
-
-
Germline
-
-
-
-
-
Ikuko Takeda
+/?
2
c.224C>T
r.(?)
p.(Thy75Met)
-
pathogenic
g.68171404C>T
g.70175263C>T
-
-
KCNJ2_000013
cytoplasmic region, slide helix, N-terminus, dominant negative effect, normal trafficking / *Frequency: patients with a suspected clinical diagnosis of congenital long QT syndrome (LQTS)
PubMed: Eckhardt 2007
-
-
Unknown
-
1/541 patients*
-
-
-
Ikuko Takeda
+/?
2
c.224C>T
r.(?)
p.(Thy75Met)
-
pathogenic
g.68171404C>T
g.70175263C>T
-
-
KCNJ2_000013
cytoplasmic, slide helix, dominant negative effect
PubMed: Davies 2005
-
-
Unknown
-
1/>140 periodic paralysis
-
-
-
Ikuko Takeda
+/.
-
c.224C>T
r.(?)
p.(Thr75Met)
-
pathogenic
g.68171404C>T
g.70175263C>T
KCNJ2(NM_000891.3):c.224C>T (p.T75M)
-
KCNJ2_000013
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
-
c.230T>A
r.(?)
p.(Val77Glu)
-
likely pathogenic (dominant)
g.68171410T>A
g.70175269T>A
-
-
KCNJ2_000095
de novo in patient
Handklo-Jamal, submitted 2019
-
-
De novo
yes
-
-
-
-
Shimrit Oz
?/?
2
c.232G>T
r.(?)
p.(Asp78Tyr)
-
VUS
g.68171412G>T
g.70175271G>T
-
-
KCNJ2_000014
cytoplasmic, slide helix
PubMed: Yoon 2006
-
-
Unknown
-
-
-
-
-
Ikuko Takeda
+/?
2
c.233A>G
r.(?)
p.(Asp78Gly)
-
pathogenic
g.68171413A>G
g.70175272A>G
-
-
KCNJ2_000015
cytoplasmic, slide helix
PubMed: Davies 2005
-
-
Unknown
-
1/>140 periodic paralysis
-
-
-
Ikuko Takeda
+/?
2
c.244C>T
r.(?)
p.(Arg82Trp)
-
pathogenic
g.68171424C>T
g.70175283C>T
-
-
KCNJ2_000016
M1 / *Frequency: patients with a suspected clinical diagnosis of congenital long QT syndrome (LQTS)
PubMed: Eckhardt 2007
-
-
Unknown
-
1/541 patients*
-
-
-
Ikuko Takeda
+/?
2
c.244C>T
r.(?)
p.(Arg82Trp)
-
pathogenic
g.68171424C>T
g.70175283C>T
-
-
KCNJ2_000016
M1 / *Frequency: Catecholaminergic polymorphic ventricular tachycardia patients
PubMed: Tester 2006
-
-
Unknown
-
2/541 patients*
-
-
-
Ikuko Takeda
+/?
2
c.244C>T
r.(?)
p.(Arg82Trp)
-
pathogenic
g.68171424C>T
g.70175283C>T
-
-
KCNJ2_000016
M1 / *Frequency: Catecholaminergic polymorphic ventricular tachycardia patients
PubMed: Tester 2006
-
-
Unknown
-
2/541 patients*
-
-
-
Ikuko Takeda
+/?
2
c.244C>T
r.(?)
p.(Arg82Trp)
-
pathogenic
g.68171424C>T
g.70175283C>T
-
-
KCNJ2_000016
M1
PubMed: Kimura 2012
-
-
Unknown
-
-
-
-
-
Ikuko Takeda
+/?
2
c.244C>T
r.(?)
p.(Arg82Trp)
-
pathogenic
g.68171424C>T
g.70175283C>T
-
-
KCNJ2_000016
M1, KCNH2 P1093L
PubMed: Kuramoto 2012
-
-
Unknown
-
-
-
-
-
Ikuko Takeda
+/.
-
c.244C>T
r.(?)
p.(Arg82Trp)
-
pathogenic
g.68171424C>T
g.70175283C>T
-
-
KCNJ2_000016
2 heterozygous, no homozygous;
Clinindb (India)
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs199473373
Germline
-
2/2795 individuals
-
-
-
Mohammed Faruq
+?/.
-
c.244C>T
r.(?)
p.(Arg82Trp)
-
likely pathogenic
g.68171424C>T
g.70175283C>T
-
-
KCNJ2_000016
variant definitively linked to disease
Fusco 2042, submitted
67568
rs199473373
Germline
-
-
-
-
-
Carmela Fusco
+/?
2
c.245G>A
r.(?)
p.(Arg82Gln)
-
pathogenic
g.68171425G>A
g.70175284G>A
-
-
KCNJ2_000017
M1
PubMed: Davies 2005
-
-
Germline
-
1/>140 periodic paralysis
-
-
-
Ikuko Takeda
+/?
2
c.245G>A
r.(?)
p.(Arg82Gln)
-
pathogenic
g.68171425G>A
g.70175284G>A
-
-
KCNJ2_000017
M1
PubMed: Kimura 2012
-
-
Unknown
-
-
-
-
-
Ikuko Takeda
+/.
-
c.245G>A
r.(?)
p.(Arg82Gln)
ACMG
pathogenic (dominant)
g.68171425G>A
-
-
-
KCNJ2_000017
ACMG: PS3, PS4, PM2_SUP, PP1
PMID: 22806368, 16217063, 23644778, 22589293, 24861851, 23867365, 23516313, 16217063, 22589293
-
-
Germline
?
-
-
-
-
Andreas Laner
+/.
-
c.245G>A
r.(?)
p.(Arg82Gln)
-
pathogenic
g.68171425G>A
-
KCNJ2(NM_000891.3):c.245G>A (p.R82Q)
-
KCNJ2_000017
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
-
c.248G>T
r.(?)
p.(Trp83Leu)
-
VUS
g.68171428G>T
g.70175287G>T
KCNJ2(NM_000891.3):c.248G>T (p.W83L)
-
KCNJ2_000074
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/?
2
c.277G>A
r.(?)
p.(Ile93Val)
-
pathogenic
g.68171457G>A
g.70175316G>A
-
-
KCNJ2_000018
M1
PubMed: Xia 2005
-
-
Germline
-
1/30 AF families
-
-
-
Ikuko Takeda
?/.
-
c.277G>A
r.(?)
p.(Val93Ile)
-
VUS
g.68171457G>A
g.70175316G>A
-
-
KCNJ2_000018
conflicting interpretations of pathogenicity; 9 heterozygous, no homozygous;
Clinindb (India)
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs147750704
Germline
-
9/2795 individuals
-
-
-
Mohammed Faruq
-?/.
-
c.277G>A
r.(?)
p.(Val93Ile)
-
likely benign
g.68171457G>A
-
KCNJ2(NM_000891.2):c.277G>A (p.(Val93Ile))
-
KCNJ2_000018
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+?/?
2
c.281T>C
r.(?)
p.(Leu94Pro)
-
likely pathogenic
g.68171461T>C
g.70175320T>C
-
-
KCNJ2_000001
-
-
-
-
Germline
-
-
-
-
-
Ikuko Takeda
+/?
2
c.301T>C
r.(?)
p.(Cys101Arg)
-
pathogenic
g.68171481T>C
g.70175340T>C
-
-
KCNJ2_000020
M1, lipid-facing
PubMed: Chun 2004
-
-
Unknown
-
-
-
-
-
Ikuko Takeda
+/?
2
c.301T>C
r.(?)
p.(Cys101Arg)
-
pathogenic
g.68171481T>C
g.70175340T>C
-
-
KCNJ2_000020
M1, lipid-facing
PubMed: Zhang 2005
PubMed: Ballester 2006
-
-
Unknown
-
-
-
-
-
Ikuko Takeda
+?/?
2
c.368T>G
r.(?)
p.(Val123Gly)
-
likely pathogenic
g.68171548T>G
g.70175407T>G
-
-
KCNJ2_000021
pore region
PubMed: Davies 2005
-
-
Unknown
-
1/>140 periodic paralysis
-
-
-
Ikuko Takeda
+/?
2
c.407C>T
r.(?)
p.(Ser136Phe)
-
pathogenic
g.68171587C>T
g.70175446C>T
-
-
KCNJ2_000022
pore helix
PubMed: Plaster 2001
-
-
Unknown
-
1/16 ATS families
-
-
-
Ikuko Takeda
?/.
-
c.424A>G
r.(?)
p.(Thr142Ala)
-
VUS
g.68171604A>G
-
-
-
KCNJ2_000102
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/?
2
c.430G>A
r.(?)
p.(Gly144Ser)
-
pathogenic
g.68171610G>A
g.70175469G>A
-
-
KCNJ2_000023
G-loop in cytoplasmic, pore region, selectivity filter residues GYG
PubMed: Plaster 2001
-
-
De novo
-
1/16 ATS families
-
-
-
Ikuko Takeda
+/?
2
c.430G>A
r.(?)
p.(Gly144Ser)
-
pathogenic
g.68171610G>A
g.70175469G>A
-
-
KCNJ2_000023
G-loop in cytoplasmic, pore region, selectivity filter residues GYG, heterozygous for a KCNQ1 mutation (c.1022C>T
PubMed: Haruna 2007
-
-
Germline
-
-
-
-
-
Ikuko Takeda
+?/.
2
c.430G>A
r.(?)
p.(Gly144Ser)
ACMG
pathogenic (dominant)
g.68171610G>A
g.70175469G>A
-
-
KCNJ2_000023
ACMG: PS4_MOD, PM5, PP3_MOD, PS2_SUP, PS3_SUP, PM2_SUP, PP2
PMID: 11371347, 12163457, 12909315, 14522976, 22002906
VCV000067573.6
-
Germline
?
-
LanerMGZ
-
-
Andreas Laner
+/?
2
c.431G>A
r.(?)
p.(Gly144Asp)
-
pathogenic
g.68171611G>A
g.70175470G>A
-
-
KCNJ2_000024
G-loop in cytoplasmic, pore region, selectivity filter residues GYG
PubMed: Kimura 2012
-
-
Unknown
-
-
-
-
-
Ikuko Takeda
+/?
2
c.431G>A
r.(?)
p.(Gly144Asp)
-
pathogenic
g.68171611G>A
g.70175470G>A
-
-
KCNJ2_000024
G-loop in cytoplasmic, pore region, selectivity filter residues GYG
PubMed: Lim 2010
-
-
De novo
-
-
-
-
-
Ikuko Takeda
+/?
2
c.431G>C
r.(?)
p.(Gly144Ala)
-
pathogenic
g.68171611G>C
g.70175470G>C
-
-
KCNJ2_000025
G-loop in cytoplasmic, pore region, selectivity filter residues GYG
PubMed: Ballester 2006
-
-
Germline
-
-
-
-
-
Ikuko Takeda
+/?
2
c.436G>A
r.(?)
p.(Gly146Ser)
-
pathogenic
g.68171616G>A
g.70175475G>A
-
-
KCNJ2_000026
G-loop in cytoplasmic, pore region, selectivity filter residues GYG, trafficking defect, dominat negative effect
PubMed: Haruna 2007
-
-
De novo
-
-
-
-
-
Ikuko Takeda
?/?
2
c.437G>A
r.(?)
p.(Gly146Asp)
-
VUS
g.68171617G>A
g.70175476G>A
-
-
KCNJ2_000027
G-loop in cytoplasmic, pore region, selectivity filter residues GYG
PubMed: Yoon 2006
-
-
Germline
-
-
-
-
-
Ikuko Takeda
?/?
2
c.437G>A
r.(?)
p.(Gly146Asp)
-
VUS
g.68171617G>A
g.70175476G>A
-
-
KCNJ2_000027
G-loop in cytoplasmic, pore region, selectivity filter residues GYG
PubMed: Donaldson 2003
-
-
Germline
-
1/17 ATS probands
-
-
-
Ikuko Takeda
?/?
2
c.437G>C
r.(?)
p.(Gly146Ala)
-
VUS
g.68171617G>C
g.70175476G>C
-
-
KCNJ2_000028
G-loop in cytoplasmic, pore region, selectivity filter residues GYG, trafficking defect, dominat negative effect
PubMed: Kim 2009
-
-
De novo
-
-
-
-
-
Ikuko Takeda
+?/?
2
c.461G>A
r.(?)
p.(Cys154Tyr)
-
likely pathogenic
g.68171641G>A
g.70175500G>A
-
-
KCNJ2_000029
M2, C154 and C122 critical for proper channel folding
PubMed: Sacconi 2009
-
-
Germline
-
-
-
-
-
Ikuko Takeda
+?/?
2
c.461G>T
r.(?)
p.(Cys154Phe)
-
likely pathogenic
g.68171641G>T
g.70175500G>T
-
-
KCNJ2_000030
M2, C154 and C122 critical for proper channel folding
PubMed: Bendahhou 2005
-
-
Unknown
-
-
-
-
-
Ikuko Takeda
?/.
-
c.476T>C
r.(?)
p.(Phe159Ser)
-
VUS
g.68171656T>C
g.70175515T>C
KCNJ2(NM_000891.3):c.476T>C (p.(Phe159Ser))
-
KCNJ2_000089
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
-
c.476T>C
r.(?)
p.(Phe159Ser)
-
VUS
g.68171656T>C
-
KCNJ2(NM_000891.3):c.476T>C (p.(Phe159Ser))
-
KCNJ2_000089
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/?
2
c.488_493del
r.(?)
p.(Phe163_Gln164del)
-
pathogenic
g.68171668_68171673del
g.70175527_70175532del
-
-
KCNJ2_000031
M2, 164; buried residue
PubMed: Fodstad 2004
-
-
De novo
-
1/188 LQT patients
-
-
-
Ikuko Takeda
-?/.
-
c.498C>T
r.(?)
p.(Ile166=)
-
likely benign
g.68171678C>T
-
KCNJ2(NM_000891.2):c.498C>T (p.I166=)
-
KCNJ2_000103
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/?
2
c.514G>A
r.(?)
p.(Asp172Asn)
-
pathogenic
g.68171694G>A
g.70175553G>A
-
-
KCNJ2_000032
M2, pore-lining, control of polyamine-mediated channel gating, rectification
PubMed: Priori 2005
PubMed: El Harchi 2009
-
-
Germline
-
-
-
-
-
Ikuko Takeda
?/.
2
c.532G>A
r.(?)
p.(Ala178Thr)
-
VUS
g.68171712G>A
g.70175571G>A
-
-
KCNJ2_000070
-
PubMed: Allegue 2015
,
Journal: Allegue 2015
-
-
Germline
yes
-
-
-
-
Anna Iglesias
+/?
2
c.557C>T
r.(?)
p.(Pro186Leu)
-
pathogenic
g.68171737C>T
g.70175596C>T
-
-
KCNJ2_000033
cytoplasmic, PKKR motif (186-189) implicated in binding PIP2
PubMed: Tristani 2002
-
-
Unknown
-
-
-
-
-
Ikuko Takeda
?/?
2
c.566G>T
r.(?)
p.(Arg189Ile)
-
VUS
g.68171746G>T
g.70175605G>T
-
-
KCNJ2_000034
cytoplasmic, PKKR motif, PIP2 binding
PubMed: Donaldson, 2003
-
-
Germline
-
1/17 ATS probands
-
-
-
Ikuko Takeda
+/?
2
c.574A>G
r.(?)
p.(Thr192Ala)
-
pathogenic
g.68171754A>G
g.70175613A>G
-
-
KCNJ2_000035
phosphatidylinositol-4,5-bisphosphate (PIP2)–binding domain
PubMed: Ai 2002
-
-
Germline
-
-
-
-
-
Ikuko Takeda
+/?
2
c.574A>G
r.(?)
p.(Thr192Ala)
-
pathogenic
g.68171754A>G
g.70175613A>G
-
-
KCNJ2_000035
phosphatidylinositol-4,5-bisphosphate (PIP2)–binding domain
PubMed: Haruna 2007
-
-
Germline
-
-
-
-
-
Ikuko Takeda
+/?
2
c.574A>G
r.(?)
p.(Thr192Ala)
-
pathogenic
g.68171754A>G
g.70175613A>G
-
-
KCNJ2_000035
phosphatidylinositol-4,5-bisphosphate (PIP2)–binding domain
PubMed: Nagase 2007
-
-
Unknown
-
-
-
-
-
Ikuko Takeda
+/?
2
c.574A>G
r.(?)
p.(Thr192Ala)
-
pathogenic
g.68171754A>G
g.70175613A>G
-
-
KCNJ2_000035
phosphatidylinositol-4,5-bisphosphate (PIP2)–binding domain
PubMed: Nagashima 2010
-
-
Unknown
-
-
-
-
-
Ikuko Takeda
?/?
2
c.575C>T
r.(?)
p.(Thr192Ile)
-
VUS
g.68171755C>T
g.70175614C>T
-
-
KCNJ2_000036
phosphatidylinositol-4,5-bisphosphate (PIP2)–binding domain
PubMed: Chan 2010
-
-
Germline
-
-
-
-
-
Ikuko Takeda
-?/.
-
c.597C>T
r.(?)
p.(Ala199=)
-
likely benign
g.68171777C>T
g.70175636C>T
-
-
KCNJ2_000090
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/.
-
c.602T>A
r.(?)
p.(Ile201Asn)
-
likely pathogenic
g.68171782T>A
-
KCNJ2(NM_000891.3):c.602T>A (p.I201N)
-
KCNJ2_000100
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
-
c.616G>A
r.(?)
p.(Gly206Ser)
-
VUS
g.68171796G>A
g.70175655G>A
-
-
KCNJ2_000082
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
-
c.631A>C
r.(?)
p.(Met211Leu)
-
VUS
g.68171811A>C
g.70175670A>C
KCNJ2(NM_000891.3):c.631A>C (p.M211L)
-
KCNJ2_000091
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+/?
2
c.644G>A
r.(?)
p.(Gly215Asp)
-
pathogenic
g.68171824G>A
g.70175683G>A
-
-
KCNJ2_000037
C-terminus, essential for the aassembly of Kir2.1
PubMed: Hosaka 2003
-
-
Unknown
-
-
-
-
-
Ikuko Takeda
+/?
2
c.644G>A
r.(?)
p.(Gly215Asp)
-
pathogenic
g.68171824G>A
g.70175683G>A
-
-
KCNJ2_000037
C-terminus, essential for the aassembly of Kir2.1
PubMed: Haruna 2007
-
-
Unknown
-
-
-
-
-
Ikuko Takeda
+/?
2
c.644G>A
r.(?)
p.(Gly215Asp)
-
pathogenic
g.68171824G>A
g.70175683G>A
-
-
KCNJ2_000037
C-terminus, essential for the aassembly of Kir2.1
PubMed: Lim 2010
-
-
Germline
-
-
-
-
-
Ikuko Takeda
+/?
2
c.646A>C
r.(?)
p.(Asn216His)
-
pathogenic
g.68171826A>C
g.70175685A>C
-
-
KCNJ2_000038
CD loop of cytosolic domain, affect the sensitivity to cholesterol
PubMed: Tristani 2002
-
-
Unknown
-
-
-
-
-
Ikuko Takeda
+?/.
-
c.647A>G
r.(?)
p.(Asn216Ser)
-
likely pathogenic
g.68171827A>G
-
KCNJ2(NM_000891.3):c.647A>G (p.N216S)
-
KCNJ2_000106
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+/?
2
c.650T>C
r.(?)
p.(Leu217Pro)
-
pathogenic
g.68171830T>C
g.70175689T>C
-
-
KCNJ2_000039
cytoplasmic
PubMed: Davies 2005
-
-
Unknown
-
1/>140 periodic paralysis
-
-
-
Ikuko Takeda
10 per page
25 per page
50 per page
100 per page
Legend
How to query
« First
Prev
1
2
Next
Last »
Screenscraping/webscraping (downloading large amounts of data using scripts) is strictly prohibited.
Use our
APIs
to retrieve data.
Powered by
LOVD v.3.0
Build 30b
LOVD software ©2004-2024
Leiden University Medical Center
Database contents © by their respective submitters and curators