Global Variome shared LOVD
KCNV2 (potassium channel, subfamily V, member 2)
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Unique variants in the KCNV2 gene
This database is one of the
""Eye disease""
gene variant databases.
""
The variants shown are described using the NM_133497.3 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.
Reported
: The number of times this variant has been reported in the database.
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
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%)
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Column type
Example
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all entries containing 'Arg'
space
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!
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'
!=""
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!=""
all entries with this field not empty
!=""
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!="p.0"
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combination
Text
*|Ter !fs
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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
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>
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
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Matches
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all entries containing 'Asian', 'asian', including 'Caucasian', 'caucasian', etc.
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all entries containing 'Asian' but not containing 'Caucasian'
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all entries containing 'Asian' or 'African', but not containing 'Caucasian'
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all entries containing 'South Asian', but not containing 'South East Asian'
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227 entries on 3 pages. Showing entries 1 - 100.
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Effect
Reported
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
+?/.
2
-
c.(?_-214)_(1356+1)_(1357-1)del
r.(?), r.0?
Exon 1 Deletion, p.0?
-
likely pathogenic
g.(?_2717526)_(2719096_2729445)del
g.(?_2717526)_(2719096_2729445)del
KCNV2 c.(?_-214)_1356+?, Exon 1 Del, KCNV2 c.(?_-214)_1356+?, Exon 1 Deletion
-
PTCH1_000000
heterozygous, homozygous
PubMed: Georgiou 2021
-
-
Unknown
?
-
-
-
-
LOVD
+?/.
1
11i
c.(?_273)_(*64_?)del
r.?
p.(Pro92Ilefs*3)
ACMG
likely pathogenic
g.(?_2718012)_(2729791_?)del
-
c.(273_*64)del
-
KCNV2_000229
-
PubMed: Hitti-Malin 2024
,
Journal: Hitti-Malin 2024
-
-
Germline
-
-
-
-
-
Rebekkah Hitti-Malin
+?/.
1
-
c.{0}
r.0
p.0
-
likely pathogenic
g.(?_2717739)_(2729728_?)del
g.(?_2717739)_(2729728_?)del
g.2717689_2729777del
-
KCNV2_000159
-
PubMed: Ellingford 2017
-
-
Germline
-
-
-
-
-
Johan den Dunnen
./.
1
-
c.-2513636_*5469272del
r.0?
p.0?
-
pathogenic
g.204104_8198999del
g.204104_8198999del
-
-
GLDC_000111
decreased gene dosage
PubMed: DDDS 2015
,
Journal: DDDS 2015
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+/.
1
1_2
c.-147144_*77686del
r.(?)
p.?
-
pathogenic
g.2570596_2807413del
-
g.2570596_2807413del
-
KCNV2_000112
-
PubMed: Thiadens_2012
-
-
Unknown
-
-
-
-
-
LOVD
+?/.
1
-
c.-11101_1356+4531del
r.?
p.?
-
likely pathogenic
g.2706639_2723626del
g.?
KCNV2 deletion confirmed by TaqMan
-
KCNV2_000169
1 more item
PubMed: Wissinger 2008
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
1
1_2
c.-759_*57289del
r.(?)
p.?
-
likely pathogenic
g.2716981_2787016del
-
g.2716981_2787016del
-
KCNV2_000149
-
PubMed: Maggi_2021
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
1
-
c.(?_-214)_(1356+1_1357-1)del
r.spl
p.(?)
-
likely pathogenic
g.?
g.?
KCNV2, variant 1: c.1186G>T/p.G396* , variant 2 :Deletion exon 1
-
PTCH1_000000
solved, compound heterozygous
PubMed: Weisschuh 2020
-
-
Unknown
?
-
-
-
-
LOVD
?/.
1
-
c.(?_-214)_(*310_?)del
r.spl
p.(?)
-
VUS
g.?
g.?
KCNV2 nucleotide 1, protein 1:whole gene del, confirmed by TaqMan qPCR
-
PTCH1_000000
homozygous, ACMG unclassified - no access to supplementary table 2
PubMed: Hull 2020
-
-
Germline
?
-
-
-
-
LOVD
-/.
1
-
c.-64T>G
r.(?)
p.(=)
-
benign
g.2717676T>G
g.2717676T>G
-
-
KCNV2_000023
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/.
1
-
c.-42C>G
r.(?)
p.(=)
-
benign
g.2717698C>G
g.2717698C>G
-
-
KCNV2_000024
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/.
1
-
c.-15C>T
r.(?)
p.(=)
-
benign
g.2717725C>T
g.2717725C>T
KCNV2(NM_133497.4):c.-15C>T
-
KCNV2_000051
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+?/., -?/.
2
1
c.-2C>T
r.(=), r.(?)
p.(=)
-
likely benign, likely pathogenic
g.2717738C>T
-
c.-2C>T, KCNV2(NM_133497.4):c.-2C>T
-
KCNV2_000137
VKGL data sharing initiative Nederland
PubMed: Song-2011
-
rs75316505
CLASSIFICATION record, Unknown
-
-
-
-
-
VKGL-NL_AMC
+/., +?/.
9
-
c.0, c.0?
r.0, r.0?
p.0, p.0?
-
likely pathogenic, pathogenic
g.2580596_2817413del, g.2667638_2747340del, g.2670960_2783870del, g.2673984_2766722del, g.?
g.2580596_2817413del, g.2667638_2747340del, g.2670960_2783870del, g.2673984_2766722del, g.?
KCNV2 complete homozygous deletion, KCNV2 D4: g.2657638_2737340del, KCNV2 D5: g.2570596_2807413del,
2 more items
-
KCNV2_000171, PTCH1_000000
heterozygous, homozygous
PubMed: Georgiou 2021
,
PubMed: Grigg 2013
,
PubMed: Kiray 2020
,
PubMed: Wissinger 2011
-
-
Germline, Unknown
?, yes
-
-
-
-
LOVD
+/., +?/.
4
1
c.?
r.(?), r.?
p.?
ACMG
likely pathogenic (recessive), pathogenic
g.2667638_2747340del, g.2718169C>T, g.2718754_2718763delACCTGGTGG, g.2719299_2730682del
-
1015–1024delACCTGGTGG, C430T
-
KCNV2_000125, KCNV2_000171
ACMG PM2, PVS1
PubMed: robson-2010
,
PubMed: Weisschuh 2024
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.1A>G
r.(?)
p.(Met1?)
-
likely pathogenic
g.2717740A>G
g.2717740A>G
KCNV2 c.1A>G, p.(Met1?)
-
KCNV2_000205
heterozygous
PubMed: Georgiou 2021
-
-
Unknown
?
-
-
-
-
LOVD
+/., +?/.
2
1_1i
c.(-230_1)_(1356+1_1357-1)del
r.0?, r.?
p.(Met1?), p.0?
ACMG
likely pathogenic, pathogenic
g.(2717510_2717739)_(2719096_2729445)del
-
-
-
KCNV2_000228
no variant 2nd chromosome, case unsolved
PubMed: Hitti-Malin 2024
,
Journal: Hitti-Malin 2024
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Rebekkah Hitti-Malin
+/.
1
-
c.6_9del
r.(?)
p.(Lys3Argfs*96)
-
pathogenic (recessive)
g.2717745_2717748del
-
9:2717744TCAAA>T ENST00000382082.3:c.8_11delAACA (Lys3ArgfsTer96)
-
KCNV2_000098
-
PubMed: Carss 2017
-
-
Germline
-
-
-
-
-
LOVD
+/.
1
1
c.6_9delCAAA
r.(?)
p.(Lys3Argfs*96)
-
pathogenic
g.2717745_2717748delCAAA
-
c.6_9delCAAA
-
KCNV2_000113
normal 2nd chromosome
PubMed: Thiadens_2012
-
-
Unknown
-
-
-
-
-
LOVD
+/., +?/.
3
1
c.7A>T
r.(?)
p.(Lys3*)
-
likely pathogenic, pathogenic
g.2717746A>T
g.2717746A>T
A7T, KCNV2 A7T, Lys3Stop, KCNV2 p.Lys3X
-
KCNV2_000122
homozygous; also heterozygous for L533V,
1 more item
PubMed: robson-2010
,
PubMed: Stockman 2014
,
PubMed: Wu 2006
-
-
Germline, Unknown
?
-
-
-
-
LOVD
+/., +?/.
8
1,2
c.8_11del
r.(?)
p.(Lys3Argfs*96), p.(Lys3ArgfsTer96)
ACMG
likely pathogenic, pathogenic, pathogenic (recessive)
g.2717747_2717750del
g.2717747_2717750del
8_11delAACA, KCNV2 c.8_11del c.1381G>A, p.Lys3Argfs*96 p.Gly461Arg, KCNV2 c.8_11delAACA,
2 more items
-
KCNV2_000002
ACMG PM2, PVS1, PP5, PS4, heterozygous,
1 more item
PubMed: Gliem 2020
,
PubMed: Stockman 2014
,
PubMed: Turro 2020
,
PubMed: Weisschuh 2024
,
2 more items
-
-
Germline, Germline/De novo (untested), Unknown
?, yes
1/143 cases
-
-
-
Johan den Dunnen
+?/.
3
-
c.8_11del4
r.(?)
p.(Lys3Argfs*96)
-
likely pathogenic
g.2717747_2717750del
g.2717747_2717750del
KCNV2 c.8_11del4, p.Lys3ArgfsX96
-
KCNV2_000002
heterozygous
PubMed: Wissinger 2011
-
-
Germline
yes
-
-
-
-
LOVD
+/.
2
-
c.8_11delAACA
r.(?)
p.(Lys3Argfs*96)
-
pathogenic
g.2717747_2717750del
g.2717747_2717750del
KCNV2 c.8_11delAACA, p.(Lys3Argfs*96)
-
KCNV2_000002
heterozygous, homozygous
PubMed: Georgiou 2021
-
-
Unknown
?
-
-
-
-
LOVD
+?/.
2
-
c.19_1356+9571delinsCATTTG
r.(?)
p.(Arg7Hisfs*57), p.?
-
likely pathogenic
g.2717758_2728666delinsCATTTG
g.2717758_2728666delinsCATTTG
KCNV2 D1: c.19_1356+9571delinsCATTTG, p.Arg7HisfsX57
-
KCNV2_000172
homozygous
PubMed: Wissinger 2011
-
-
Germline
yes
-
-
-
-
LOVD
-?/.
1
-
c.58G>A
r.(?)
p.(Glu20Lys)
-
likely benign
g.2717797G>A
-
KCNV2(NM_133497.4):c.58G>A (p.E20K)
-
KCNV2_000161
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
1
-
c.76C>T
r.(?)
p.(His26Tyr)
-
VUS
g.2717815C>T
g.2717815C>T
KCNV2(NM_133497.3):c.76C>T (p.H26Y)
-
KCNV2_000052
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/., +?/., -/., -?/.
4
-
c.80G>A
r.(?)
p.(Arg27His)
-
benign, likely benign, likely pathogenic, pathogenic
g.2717819G>A
g.2717819G>A
c.80G>A, p.(Arg27His), KCNV2 c.80G>A, KCNV2(NM_133497.4):c.80G>A (p.R27H)
-
KCNV2_000072
classification based on frequency in 305 unrelated individuals, complex homozygous,
2 more items
PubMed: Fujinami 2013
,
PubMed: Le 2019
,
PubMed: Wang 2019
-
-
CLASSIFICATION record, Germline
?, yes
frequency 0.022
-
-
-
Global Variome, with Curator vacancy
,
VKGL-NL_AMC
?/.
1
-
c.95C>G
r.(?)
p.(Ser32Cys)
-
VUS
g.2717834C>G
-
KCNV2(NM_133497.3):c.95C>G (p.S32C)
-
KCNV2_000162
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.130C>T
r.(?)
p.(His44Tyr)
-
likely benign
g.2717869C>T
g.2717869C>T
KCNV2(NM_133497.3):c.130C>T (p.H44Y)
-
KCNV2_000053
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/.
1
-
c.132C>T
r.(?)
p.(His44=)
-
benign
g.2717871C>T
g.2717871C>T
KCNV2(NM_133497.4):c.132C>T (p.H44=)
-
KCNV2_000073
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
1
1
c.133G>A
r.(?)
p.(Gly45Ser)
-
VUS
g.2717872G>A
-
c.133G>A
-
KCNV2_000160
-
PubMed: Borràs 2013
-
-
Germline
no
Novel
-
-
-
LOVD
+?/.
1
-
c.137G>A
r.(?)
p.(Trp46*)
-
likely pathogenic
g.2717876G>A
g.2717876G>A
KCNV2 p.W46X:c.137G>A
-
KCNV2_000173
compound heterozygous
PubMed: Lenis 2013
-
-
Unknown
?
-
-
-
-
LOVD
?/.
1
-
c.142G>A
r.(?)
p.(Glu48Lys)
-
VUS
g.2717881G>A
g.2717881G>A
KCNV2(NM_133497.4):c.142G>A (p.E48K)
-
KCNV2_000074
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/.
1
-
c.144G>A
r.(?)
p.(Glu48=)
-
likely benign
g.2717883G>A
-
KCNV2(NM_133497.4):c.144G>A (p.E48=)
-
KCNV2_000163
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+?/., ?/.
4
1
c.159C>G
r.(?)
p.(Tyr53*), p.Tyr53*
-
likely pathogenic, VUS
g.2717898C>G
g.2717898C>G
c.159C>G, KCNV2 c.159C>G (p.Y53X), KCNV2 c.159C>G, p.Tyr53*
-
KCNV2_000120
homozygous
PubMed: Abdelkader 2020
,
PubMed: Abu-Safieh-2013
,
PubMed: Khan 2012
-
-
Germline
yes
-
-
-
-
LOVD
+/., ?/.
2
1
c.162C>A
r.(?)
p.(Tyr54*), p.(Tyr54Ter)
-
pathogenic, VUS
g.2717901C>A
g.2717901C>A
c.162C>A
-
KCNV2_000026
VKGL data sharing initiative Nederland
PubMed: Littink 2010
-
-
CLASSIFICATION record, Unknown
-
-
-
-
-
VKGL-NL_Nijmegen
-/.
1
-
c.168G>A
r.(?)
p.(Glu56=)
-
benign
g.2717907G>A
g.2717907G>A
KCNV2(NM_133497.4):c.168G>A (p.E56=)
-
KCNV2_000054
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/.
1
-
c.183C>G
r.(?)
p.(Gly61=)
-
benign
g.2717922C>G
g.2717922C>G
-
-
KCNV2_000027
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
-
c.183_185del
r.(?)
p.(Glu64del)
ACMG
VUS
g.2717922_2717924del
g.2717922_2717924del
KCNV2:NM_133497 c.183_185del, p.E64del
-
KCNV2_000146
heterozygous, individual solved, variant non-causal
PubMed: Rodriguez-Munoz 2020
-
-
Germline
?
-
-
-
-
LOVD
?/.
1
-
c.193G>A
r.(?)
p.(Asp65Asn)
-
VUS
g.2717932G>A
g.2717932G>A
KCNV2(NM_133497.3):c.193G>A (p.D65N)
-
KCNV2_000075
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/., +?/.
3
1
c.200G>A
r.(?)
p.(Trp67*), p.(Trp67Ter)
ACMG
likely pathogenic, pathogenic
g.2717939G>A
g.2717939G>A
KCNV2 c.200G>A(;)1348T>G, V1: c.200G>A, (p.Trp67Ter), KCNV2 c.G200A (p.W67X),
1 more item
-
KCNV2_000157
alleles in cis or trans; heterozygous, heterozygous
PubMed: Chen 2021
,
PubMed: Chen 2021
,
PubMed: Kutsuma 2019
-
-
Germline, Germline/De novo (untested), Unknown
?, yes
Taiwan Biobank: 0; GnomAD_exome_East: 0; GnomAD_All: 0.00000398
-
-
-
LOVD
-?/.
1
-
c.207C>T
r.(?)
p.(Asp69=)
-
likely benign
g.2717946C>T
g.2717946C>T
KCNV2(NM_133497.3):c.207C>T (p.D69=)
-
KCNV2_000055
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/.
1
-
c.217G>T
r.(?)
p.(Glu73*)
-
likely pathogenic
g.2717956G>T
g.2717956G>T
KCNV2 c.217G>T, p.E73X
-
KCNV2_000174
heterozygous
PubMed: Wissinger 2008
-
-
Germline
yes
-
-
-
-
LOVD
?/.
1
-
c.221A>G
r.(?)
p.(Glu74Gly)
-
VUS
g.2717960A>G
g.2717960A>G
KCNV2(NM_133497.3):c.221A>G (p.E74G)
-
KCNV2_000056
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
-
c.224_230delACCAGCA
r.(?)
p.(Asp75Glyfs*23)
-
pathogenic
g.2717963_2717969del
g.2717963_2717969del
KCNV2 c.224_230delACCAGCA, p.(Asp75Glyfs*23)
-
KCNV2_000206
homozygous
PubMed: Georgiou 2021
-
-
Unknown
?
-
-
-
-
LOVD
+?/.
3
-
c.226C>T
r.(?)
p.(Gln76*)
-
likely pathogenic
g.2717965C>T
g.2717965C>T
KCNV2 c.226C>T, p.Q76X
-
KCNV2_000175
homozygous
PubMed: Wissinger 2008
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
2
-
c.238G>T
r.(?)
p.(Glu80*)
-
likely pathogenic
g.2717977G>T
g.2717977G>T
KCNV2 c.238G > T*: p.Glu80Stop
-
KCNV2_000176
homozygous
PubMed: Vincent 2013
-
-
Germline
yes
-
-
-
-
LOVD
+?/., ?/.
2
1
c.240G>T
r.(?)
p.(Glu80Asp)
-
likely pathogenic, VUS
g.2717979G>T
g.2717979G>T
c.240G>T, KCNV2 c.240 G>T (p.E80D)
-
KCNV2_000121
homozygous
PubMed: Abu-Safieh-2013
,
PubMed: Khan 2012
-
-
Germline
yes
-
-
-
-
LOVD
-/.
1
-
c.249C>T
r.(?)
p.(Thr83=)
-
benign
g.2717988C>T
g.2717988C>T
KCNV2(NM_133497.4):c.249C>T (p.T83=)
-
KCNV2_000076
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/.
2
-
c.258C>G
r.(?)
p.(Pro86=)
-
likely benign
g.2717997C>G
g.2717997C>G
KCNV2(NM_133497.3):c.258C>G (p.P86=), KCNV2(NM_133497.4):c.258C>G (p.P86=)
-
KCNV2_000091
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
?/.
1
-
c.259_266dup
r.(?)
p.(Ser90ArgfsTer13)
-
VUS
g.2717998_2718005dup
g.2717998_2718005dup
254_255insGCCCGAGG
-
KCNV2_000107
-
PubMed: Wang 2015
-
-
Germline
-
-
-
-
-
LOVD
+/.
1
1
c.263G>A
r.(?)
p.(Gly88Asp)
-
pathogenic
g.2718002G>A
-
c.263G>A
-
KCNV2_000114
normal 2nd chromosome
PubMed: Thiadens_2012
-
-
Unknown
-
-
-
-
-
LOVD
-?/.
1
-
c.291C>T
r.(?)
p.(Ser97=)
-
likely benign
g.2718030C>T
g.2718030C>T
KCNV2(NM_133497.3):c.291C>T (p.S97=)
-
KCNV2_000057
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
2
-
c.291_292ins[105_223;TCACCACCACCTT;231_291]
r.(?)
p.(Thr98fs)
-
pathogenic (recessive)
g.2718030_2718031ins[2717844_2717962;TCACCACCACCTT;2717970_2718030]
g.2718030_2718031ins[2717844_2717962;TCACCACCACCTT;2717970_2718030]
chr9:2717844-2718028dup;222_232delinsGGTCACCACCACCTTGG
-
KCNV2_000097
delins combined with duplication including delins
PubMed: Carss 2017
-
-
Germline, Uniparental disomy, maternal allele
-
-
-
-
-
Johan den Dunnen
-?/.
2
-
c.312T>C
r.(?)
p.(Gly104=)
-
likely benign
g.2718051T>C
g.2718051T>C
KCNV2(NM_133497.3):c.312T>C (p.G104=), KCNV2(NM_133497.4):c.312T>C (p.G104=)
-
KCNV2_000058
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
+/.
1
-
c.323_329del
r.(?)
p.(Tyr108TrpfsTer14)
-
pathogenic
g.2718062_2718068del
g.2718062_2718068del
-
-
KCNV2_000010
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/., +?/.
2
1
c.323_329del7
r.(?)
p.(Tyr108Trpfs*14)
-
likely pathogenic, pathogenic
g.2718062_2718068del, g.2718062_2718068del7
g.2718062_2718068del
c.323_329del7, KCNV2 c.323_329del7, p.Tyr108TrpfsX14
-
KCNV2_000010, KCNV2_000115
heterozygous
PubMed: Thiadens_2012
,
PubMed: Wissinger 2011
-
-
Germline, Unknown
yes
-
-
-
-
LOVD
+/., +?/.
5
1
c.325C>T
r.(?)
p.(Gln109*)
-
likely pathogenic, pathogenic
g.2718064C>T
g.2718064C>T
Allele 1 c.325C>T p.(Gln109*), Allele 2 c.325C>T p.(Gln109*), C325T, KCNV2 C325T, Gln109Stop,
1 more item
-
KCNV2_000123
homozygous
PubMed: Georgiou 2021
,
PubMed: Khan 2019
,
PubMed: robson-2010
,
PubMed: Wu 2006
-
-
Germline, Germline/De novo (untested), Unknown
?
-
-
-
-
LOVD
-?/.
1
-
c.328C>T
r.(?)
p.(Leu110=)
-
likely benign
g.2718067C>T
g.2718067C>T
KCNV2(NM_133497.3):c.328C>T (p.L110=)
-
KCNV2_000077
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/.
1
-
c.336C>T
r.(?)
p.(Tyr112=)
-
benign
g.2718075C>T
g.2718075C>T
KCNV2(NM_133497.4):c.336C>T (p.Y112=)
-
KCNV2_000078
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/., +?/.
6
1
c.339C>A
r.(?)
p.(Cys113*), p.(Cys113Ter)
-
likely pathogenic, pathogenic
g.2718078C>A
g.2718078C>A
c.339C>A, KCNV2 c.339C>A, p.(Cys113*), KCNV2 c.339C>A, p.Cys113X,
1 more item
-
KCNV2_000059
heterozygous, normal 2nd chromosome, VKGL data sharing initiative Nederland
PubMed: Georgiou 2021
,
PubMed: Thiadens_2012
,
PubMed: Zobor 2012
-
-
CLASSIFICATION record, Germline, Unknown
?, yes
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
+/.
2
-
c.357dup
r.(?)
p.(Lys120Glnfs*252)
ACMG
pathogenic
g.2718096dup
g.2718096dup
KCNV2 c.357dup, p.(Lys120Glnfs*252)
-
KCNV2_000138
single heterozygous variant (recessive)
PubMed: Jespersgaar 2019
-
-
Germline
?
-
-
-
-
LOVD
+?/.
5
-
c.357_358insC
r.(?)
p.(Lys120Glnfs*252)
-
likely pathogenic
g.2718096dup
g.2718096dup
KCNV2 c.357_358insC, p.K120fsX371
-
KCNV2_000138
heterozygous, homozygous
PubMed: Wissinger 2008
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
1
1
c.368T>G
r.(?)
p.(Leu123Arg)
-
likely pathogenic (recessive)
g.2718107T>G
-
c.368T>G
-
KCNV2_000155
-
PubMed: Liu-2020
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/.
2
1
c.377T>A
r.(?)
p.(Leu126Gln)
-
likely pathogenic, pathogenic
g.2718116T>A
g.2718116T>A
KCNV2 T377A/?, Leu126Gln, T377A
-
KCNV2_000124
2nd variant unknown, single heterozygous
PubMed: robson-2010
,
PubMed: Wu 2006
-
-
Germline, Unknown
?
-
-
-
-
LOVD
+/.
2
1
c.411_414del
r.(?)
p.(Leu138Alafs*72)
ACMG
pathogenic
g.2718149_2718152del, g.2718150_2718153del
g.2718150_2718153del
c.410_413delGCCT
-
KCNV2_000041
-
Sharon, submitted,
PubMed: Sharon 2019
-
-
Germline
-
1/2420 IRD families
-
-
-
Global Variome, with Curator vacancy
,
Dror Sharon
+?/.
2
-
c.411_414delCCTG
r.(?)
p.(Leu138Alafs*72)
-
likely pathogenic
g.2718150_2718153del
g.2718150_2718153del
KCNV2 c.411-414delCCTG, p.Leu138Alafs*70
-
KCNV2_000041
homozygous
PubMed: Zelinger 2013
-
-
Germline
yes
-
-
-
-
LOVD
+/.
1
-
c.417C>A
r.(?)
p.(Cys139*)
-
pathogenic
g.2718156C>A
g.2718156C>A
KCNV2 c.417C>A, p.(Cys139*)
-
KCNV2_000207
heterozygous
PubMed: Georgiou 2021
-
-
Unknown
?
-
-
-
-
LOVD
+/., +?/., ?/.
55
1, Glu143X
c.427G>T
r.(?)
p.(Glu143*), p.(Glu143Ter), p.Glu143*
-
likely pathogenic, pathogenic, VUS
g.2718166G>T
g.2718166G>T
Allele 1 c.427G>T (p.Glu143*), Allele 2 c.427G>T (p.Glu143*), c.427G>T, G427T,
7 more items
-
KCNV2_000029
heterozygous, homozygous, VKGL data sharing initiative Nederland
PubMed: Abdelkader 2020
,
PubMed: Abu-Safieh-2013
,
PubMed: Georgiou 2021
,
PubMed: Khan 2012
,
6 more items
-
-
CLASSIFICATION record, Germline, Germline/De novo (untested), Unknown
?, yes
-
-
-
-
VKGL-NL_Nijmegen
+/., +?/.
4
1
c.433C>T
r.(?)
p.(Gln145*), p.(Gln145Ter)
ACMG
likely pathogenic, pathogenic
g.2718172C>T
g.2718172C>T
KCNV2 c.433C>T, p.(Gln145*), KCNV2 C430T, Gin145Stop
-
KCNV2_000177
heterozygous, heterozygous, D147F in phase
PubMed: Georgiou 2021
,
PubMed: Hitti-Malin 2024
,
Journal: Hitti-Malin 2024
,
PubMed: Wu 2006
-
-
Germline, Unknown
?
-
-
-
-
Rebekkah Hitti-Malin
+?/.
3
-
c.434_*30+154del
r.(?)
p.(Gln145_Asn545delinsLeuCysValVal)
-
likely pathogenic
g.2718173_2729911del
g.2718173_2729911del
KCNV2 D2: c.434_*30+154del, p.Glu145LeufsX4
-
KCNV2_000178
heterozygous
PubMed: Wissinger 2011
-
-
Germline
yes
-
-
-
-
LOVD
?/.
1
-
c.441C>A
r.(?)
p.(Asp147Glu)
ACMG
VUS
g.2718180C>A
g.2718180C>A
KCNV2:NM_133497 c.C441A, p.D147E
-
KCNV2_000147
heterozygous, individual solved, variant non-causal
PubMed: Rodriguez-Munoz 2020
-
-
Germline
?
-
-
-
-
LOVD
+?/.
2
-
c.442G>A
r.(?)
p.(Glu148Lys)
-
likely pathogenic
g.2718181G>A
g.2718181G>A
KCNV2 c.442G>A, p.(Glu148Lys),
1 more item
-
KCNV2_000150
heterozygous, possibly solved, compound heterozygous
PubMed: Georgiou 2021
,
PubMed: Weisschuh 2020
-
-
Unknown
?
-
-
-
-
LOVD
+/., +?/.
17
-
c.442G>T
r.(?)
p.(Glu148*), p.(Glu148Ter)
-
likely pathogenic, pathogenic
g.2718181G>T
g.2718181G>T
KCNV2 c.442G>T, E148X, KCNV2 c.442G>T, p.(Glu148*), KCNV2 c.442G>T, p.E148X,
3 more items
-
KCNV2_000006
heterozygous, homozygous, solved, homozygous, VKGL data sharing initiative Nederland
PubMed: BenSalah 2008
,
PubMed: Georgiou 2021
,
PubMed: Stone 2017
,
PubMed: Weisschuh 2020
,
3 more items
-
-
CLASSIFICATION record, Germline, Unknown
?, yes
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Nijmegen
,
VKGL-NL_AMC
+/.
1
-
c.445_446delTA
r.(?)
p.(Tyr149Leufs*222)
-
pathogenic
g.2718184_2718185del
g.2718184_2718185del
KCNV2 c.445_446delTA, p.(Tyr149Leufs*222)
-
KCNV2_000208
homozygous
PubMed: Georgiou 2021
-
-
Unknown
?
-
-
-
-
LOVD
+?/.
2
-
c.451T>C
r.(?)
p.(Phe151Leu), p.(Phe151Val)
-
likely pathogenic
g.2718190T>C
g.2718190T>C
KCNV2 c.451T>C, p.(Phe151Leu), KCNV2 c.451T>C, p.(Phe151Val)
-
KCNV2_000209
heterozygous
PubMed: Georgiou 2021
-
-
Unknown
?
-
-
-
-
LOVD
+/.
2
1
c.451T>G
r.(?)
p.(Phe151Val)
-
pathogenic
g.2718190T>G
-
T451G
-
KCNV2_000126
2nd variant unknown
PubMed: robson-2010
-
-
Germline
-
-
-
-
-
LOVD
+?/.
1
-
c.451_453del
r.(?)
p.(Phe151del)
-
likely pathogenic
g.2718190_2718192del
g.2718190_2718192del
KCNV2 c.447_449del3, p.Phe150del
-
KCNV2_000170
1 more item
PubMed: Zobor 2012
-
-
Germline
yes
-
-
-
-
LOVD
-/.
1
-
c.453C>T
r.(?)
p.(Phe151=)
-
benign
g.2718192C>T
g.2718192C>T
KCNV2(NM_133497.4):c.453C>T (p.F151=)
-
KCNV2_000060
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+?/.
1
-
c.454G>A
r.(?)
p.(Asp152Asn)
-
likely pathogenic
g.2718193G>A
g.2718193G>A
-
-
KCNV2_000104
-
PubMed: Oishi 2016
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/.
4
1
c.455A>G
r.(?)
p.(Asp152Gly)
ACMG
likely pathogenic
g.2718194A>G
g.2718194A>G
KCNV2 c.455A>G, p.(Asp152Gly)
-
KCNV2_000042
homozygous
Sharon, submitted,
PubMed: Georgiou 2021
,
PubMed: Sharon 2019
-
-
Germline, Unknown
?
1/2420 IRD families
-
-
-
Global Variome, with Curator vacancy
,
Dror Sharon
+/.
3
-
c.457C>G
r.(?)
(p.Arg153Gly)
-
pathogenic
g.2718196C>G
g.2718196C>G
KCNV2 c.457C>G, (p.Arg153Gly)
-
KCNV2_000210
heterozygous
PubMed: Esteves-Leandro 2021
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
1
-
c.460_461insCG
r.(?)
p.(Asp154Alafs*58)
-
likely pathogenic
g.2718198_2718199dup
g.2718198_2718199dup
KCNV2 c.460_461insCG: p.Asp154Ala fsx58
-
KCNV2_000179
homozygous
PubMed: Vincent 2013
-
-
Germline
yes
-
-
-
-
LOVD
?/.
1
1
c.465G>A
r.(=)
p.(=)
-
VUS
g.2718204G>A
-
c.465G>A
-
KCNV2_000151
-
PubMed: Agange-2017
-
-
Germline
-
-
-
-
-
LOVD
+?/.
2
-
c.473T>G
r.(?)
p.(Phe158Cys)
-
likely pathogenic
g.2718212T>G
g.2718212T>G
KCNV2 c.473T>G, p.(Phe158Cys), KCNV2 c.473T>G, p.Phe158Cys
-
KCNV2_000180
heterozygous
PubMed: Georgiou 2021
,
PubMed: Thiagalingam 2007
-
-
Unknown
?
-
-
-
-
LOVD
+?/.
1
-
c.484T>A
r.(?)
p.(Tyr162Asn)
-
likely pathogenic
g.2718223T>A
g.2718223T>A
KCNV2 c.484T>A, p.(Tyr162Asn)
-
KCNV2_000211
heterozygous
PubMed: Georgiou 2021
-
-
Unknown
?
-
-
-
-
LOVD
+?/.
1
-
c.491T>C
r.(?)
p.(Phe164Ser)
-
likely pathogenic
g.2718230T>C
g.2718230T>C
KCNV2 c.491T>C, p.Phe164Ser
-
KCNV2_000181
heterozygous
PubMed: Wissinger 2011
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
1
-
c.494A>G
r.(?)
p.(Tyr165Cys)
-
likely pathogenic
g.2718233A>G
g.2718233A>G
KCNV2 c.494A>G, p.(Tyr165Cys)
-
KCNV2_000212
heterozygous
PubMed: Georgiou 2021
-
-
Unknown
?
-
-
-
-
LOVD
?/.
1
1
c.502G>A
r.(?)
p.(Gly168Arg)
-
VUS
g.2718241G>A
g.2718241G>A
G502A
-
KCNV2_000109
-
PubMed: Katagiri 2014
-
-
Germline
-
-
-
-
-
LOVD
+?/.
1
1
c.520dupG
r.(?)
p.(Asp174Glyfs*198)
-
likely pathogenic
g.2718259dup
g.2718259dup
KCNV2 c.520dupG (p.D174GfsX198)
-
KCNV2_000182
heterozygous
PubMed: Kutsuma 2019
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
4
-
c.529T>C
r.(?)
p.(Cys177Arg)
-
likely pathogenic
g.2718268T>C
g.2718268T>C
KCNV2 c.529T>C
-
KCNV2_000105
compound heterozygous
PubMed: Fujinami 2013
,
PubMed: Oishi 2016
-
-
Germline
yes
-
-
-
-
LOVD
+?/., ?/.
2
-
c.530G>C
r.(?)
(p.Cys177Ser), p.(Cys177Ser)
-
likely pathogenic, VUS
g.2718269G>C
g.2718269G>C
KCNV2 c.530G>C, (p.Cys177Ser)
-
KCNV2_000164
homozygous, VKGL data sharing initiative Nederland
PubMed: Abdelkader 2020
-
-
CLASSIFICATION record, Germline
yes
-
-
-
-
VKGL-NL_Nijmegen
+?/.
1
-
c.531T>A
r.(?)
p.(Cys177*)
-
likely pathogenic
g.2718270T>A
g.2718270T>A
KCNV2 p.C177X: c.531T>A
-
KCNV2_000183
compound heterozygous
PubMed: Lenis 2013
-
-
Unknown
?
-
-
-
-
LOVD
+?/.
1
-
c.533C>T
r.(?)
p.(Pro178Leu)
-
likely pathogenic
g.2718272C>T
g.2718272C>T
KCNV2 c.533C>T, p.Pro178Leu
-
KCNV2_000184
heterozygous
PubMed: Thiagalingam 2007
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
3
1
c.550G>A
r.(?)
p.(Glu184Lys)
-
likely pathogenic
g.2718289G>A
g.2718289G>A
KCNV2 c.550G>A, p.(Glu184Lys), KCNV2 c.550G>A, p.E184K,
1 more item
-
KCNV2_000143
compound heterozygous, heterozygous
PubMed: Georgiou 2021
,
PubMed: Martin Merida 2019
,
PubMed: Wissinger 2008
-
-
Germline, Unknown
?, yes
-
-
-
-
LOVD
+?/.
1
-
c.551A>T
r.(?)
p.(Glu184Val)
-
likely pathogenic
g.2718290A>T
g.2718290A>T
KCNV2 c.551A>T, p.E184V
-
KCNV2_000185
heterozygous
PubMed: Wissinger 2008
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
1
1
c.554T>G
r.(?)
p.(Leu185Arg)
ACMG
likely pathogenic
g.2718293T>G
g.2718293T>G
-
-
KCNV2_000230
-
PubMed: Hitti-Malin 2024
,
Journal: Hitti-Malin 2024
-
-
Germline
-
-
-
-
-
Rebekkah Hitti-Malin
+/.
1
1
c.556_571del16
r.(?)
p.(Tyr187Serfs*19)
-
pathogenic
g.2718295_2718310del16
-
c.556_571del16
-
KCNV2_000116
-
PubMed: Thiadens_2012
-
-
Unknown
-
-
-
-
-
LOVD
+/., +?/., ?/.
7
-
c.562T>A
r.(?)
p.(Trp188Arg)
-
likely pathogenic, pathogenic (recessive), VUS
g.2718301T>A
g.2718301T>A
9:2718301T>A ENST00000382082.3:c.562T>A (Trp188Arg), KCNV2 c.562T > A, p.(Trp188Arg).,
3 more items
-
KCNV2_000099
heterozygous, homozygous, homozygous, ACMG unclassified - no access to supplementary table 2
PubMed: Carss 2017
,
PubMed: Georgiou 2021
,
PubMed: Hull 2020
,
PubMed: Kiray 2020
,
PubMed: Turro 2020
-
-
Germline, Germline/De novo (untested), Unknown
?, yes
-
-
-
-
LOVD
+?/., ?/.
2
-
c.562T>C
r.(?)
p.(Trp188Arg)
-
likely pathogenic (recessive), VUS
g.2718301T>C
g.2718301T>C
-
-
KCNV2_000031
VKGL data sharing initiative Nederland
PubMed: Taylor 2017
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
VKGL-NL_Nijmegen
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