Global Variome shared LOVD
CLCN1 (chloride channel, voltage-sensitive 1)
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Unique variants in the CLCN1 gene
The variants shown are described using the NM_000083.2 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.
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%)
How to query this table
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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.
366 entries on 4 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
?/?
5
_1
c.-136T>G
r.(?)
p.(=)
-
VUS
g.143013170T>G
g.143316077T>G
-
-
CLCN1_000001
1 more item
-
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/?
2
_1
c.-135T>G
r.(?)
p.(=), p.(?)
-
benign
g.143013171T>G
g.143316078T>G
-
-
CLCN1_000175
5'UTR
-
-
rs6464541
Germline
-
-
-
-
-
Johan den Dunnen
,
Birgit Neitzel
-/-, -/., -/?
22
1
c.-21C>T
r.(=), r.(?)
p.(=), p.(?)
-
benign
g.143013285C>T
g.143316192C>T
CLCN1(NM_000083.2):c.-21C>T (p.(=))
-
CLCN1_000002
VKGL data sharing initiative Nederland
-
-
rs34904831
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
Birgit Neitzel
,
Andreas Laner
,
VKGL-NL_Leiden
?/.
1
-
c.-13G>A
r.(?)
p.(=)
-
VUS
g.143013293G>A
-
CLCN1(NM_000083.2):c.-13G>A (p.(=))
-
CLCN1_000395
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/+
5
1
c.-3A>T
r.(?)
p.(=)
-
pathogenic
g.143013303A>T
g.143316210A>T
-
-
CLCN1_000003
-
George 1994, Sloan Brown 1996, Sloan Brown and George 1997
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
21i_23_
c.(2403+1_2404-1)_*39{0}
r.?
p.?
-
pathogenic (recessive)
g.(143044043_143047464)_(143049097_?)del
g.(143346950_143350371)_(143352004_?)del
-
-
CLCN1_000349
-
-
-
-
Germline
yes
-
-
-
-
Raffaella Brugnoni
?/?
1
9
c.?
r.(?)
p.(=)
-
VUS
g.?
-
1017+56G>A
-
CLCN1_000072
-
-
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
1
c.47G>A
r.(?)
p.(Trp16Ter)
-
likely pathogenic
g.143013352G>A
g.143316259G>A
-
-
CLCN1_000331
-
PubMed: Ganapathy 2019
-
rs769092535
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.53_65del
r.(?)
p.(Ser18Thrfs*55)
-
pathogenic
g.143013358_143013370del
g.143316265_143316277del
-
-
CLCN1_000272
-
-
-
-
Germline
-
-
-
-
-
Andreas Laner
-?/., ?/.
3
-
c.86A>C
r.(?)
p.(His29Pro)
-
likely benign, VUS
g.143013391A>C
g.143316298A>C
CLCN1(NM_000083.3):c.86A>C (p.H29P)
-
CLCN1_000273
3 heterozygous, no homozygous;
Clinindb (India)
, PolyPhen2: poss.damag.(PSIC: 0,15),
1 more item
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs146160029
CLASSIFICATION record, Germline
-
3/2795 individuals
-
-
-
Andreas Laner
,
VKGL-NL_Utrecht
,
Mohammed Faruq
?/?
1
1
c.86A>G
r.(?)
p.(His29Pro)
-
VUS
g.143013391A>G
g.143316298A>G
-
-
CLCN1_000179
Polyphen PSIC score 2,5 suggests probably damaging effect
-
-
-
Germline
-
-
-
-
-
Birgit Neitzel
+/+
1
1
c.98A>C
r.(?)
p.(Tyr33Cys)
-
pathogenic
g.143013403A>C
g.143316310A>C
-
-
CLCN1_000004
-
-
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.117T>G
r.(?)
p.(Asn39Lys)
-
likely benign
g.143013422T>G
-
CLCN1(NM_000083.2):c.117T>G (p.N39K)
-
CLCN1_000327
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.139C>T
r.(?)
p.(Arg47Trp)
-
VUS
g.143013444C>T
-
CLCN1(NM_000083.2):c.139C>T (p.R47W)
-
CLCN1_000324
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
+/?
1
1
c.178C>T
r.(?)
p.(Gln60*)
-
pathogenic
g.143013483C>T
g.143316390C>T
-
-
CLCN1_000182
-
-
-
-
Germline
-
-
-
-
-
Birgit Neitzel
+/+, +/., +/?, +?/.
21
1i
c.180+3A>T
r.(?), r.(spl?), r.spl, r.spl?
p.(?), p.?
ACMG
likely pathogenic, pathogenic, pathogenic (recessive)
g.143013488A>T
g.143316395A>T
IVS1+3A>T
-
CLCN1_000005
VKGL data sharing initiative Nederland,
1 more item
Ginjaar, unpublished, Sun 2001,
PubMed: Brugnoni 2022
,
Journal: Brugnoni 2022
,
1 more item
-
rs202217420
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
Ieke Ginjaar
,
Birgit Neitzel
,
Andreas Laner
,
VKGL-NL_Nijmegen
?/.
1
-
c.181-44T>C
r.(=)
p.(=)
-
VUS
g.143016804T>C
g.143319711T>C
-
-
CLCN1_000264
-
-
-
-
Germline
-
-
-
-
-
Andreas Laner
?/.
1
-
c.181-43A>C
r.(=)
p.(=)
-
VUS
g.143016805A>C
g.143319712A>C
-
-
CLCN1_000265
-
-
-
-
Germline
-
-
-
-
-
Andreas Laner
+/+
2
2
c.202C>T
r.(?)
p.(Gln68*)
-
pathogenic
g.143016869C>T
g.143319776C>T
-
-
CLCN1_000167
-
Zhang 1996
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/+, +/.
4
2
c.220C>T
r.(?)
p.(Gln74*)
ACMG
pathogenic, pathogenic (recessive)
g.143016887C>T
g.143319794C>T
-
-
CLCN1_000006
ACMG: PVS1, PM3, PM2_SUP
Mailander 1996, PMID: 8571958, 24349310
VCV000462829.2
rs1554434400
Germline
yes
-
-
-
-
Johan den Dunnen
,
Andreas Laner
?/.
1
-
c.240G>A
r.(?)
p.(=)
-
VUS
g.143016907G>A
-
-
-
CLCN1_000387
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
-
c.243_249del
r.(?)
p.(Lys81Asnfs*28)
-
pathogenic
g.143016910_143016916del
g.143319817_143319823del
-
-
CLCN1_000266
-
-
-
rs140311
Germline
-
-
-
-
-
Andreas Laner
+?/.
1
2
c.244A>G
r.(?)
p.(Thr82Ala)
-
likely pathogenic
g.143016911A>G
g.143319818A>G
-
-
CLCN1_000283
-
-
-
-
Germline
-
-
-
-
-
Raffaella Brugnoni
-/-, -/., -/?
102
2
c.261C>T
r.(?)
p.(=), p.(Thr87=), p.(Thr87Thr)
-
benign
g.143016928C>T
g.143319835C>T
CLCN1(NM_000083.3):c.261C>T (p.T87=), Thr87Thr
-
CLCN1_000007
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
Birgit Neitzel
,
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
+?/.
1
-
c.264G>A
r.(?)
p.(Val88=)
-
likely pathogenic
g.143016931G>A
g.143319838G>A
-
-
CLCN1_000300
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
-
c.301+1G>C
r.spl?
p.(=)
-
VUS
g.143016969G>C
g.143319876G>C
-
-
CLCN1_000267
-
-
-
-
Germline
-
-
-
-
-
Andreas Laner
-/-, -/., -/?
24
2i
c.301+27T>A
r.(=), r.(?)
p.(=), p.(?)
-
benign
g.143016995T>A
g.143319902T>A
CLCN1(NM_000083.3):c.301+27T>A
-
CLCN1_000008
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
Birgit Neitzel
,
VKGL-NL_Utrecht
-?/.
1
-
c.302-79_302-78del
r.(=)
p.(=)
-
likely benign
g.143017678_143017679del
-
CLCN1(NM_000083.3):c.302-79_302-78delCT
-
CLCN1_000336
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-/.
1
-
c.302-4C>T
r.spl?
p.?
-
benign
g.143017753C>T
g.143320660C>T
-
-
CLCN1_000301
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/+, +/.
11
2i
c.302-2A>C
r.spl, r.spl?
p.?
-
pathogenic
g.143017755A>C
g.143320662A>C
-
-
CLCN1_000010
VKGL data sharing initiative Nederland
Ginjaar, unpublished, Laboratory for Diagnostic Genome Analyses
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
Ieke Ginjaar
,
VKGL-NL_Nijmegen
+/+, +/.
13
2i
c.302-1G>A
r.spl, r.spl?
p.?
-
pathogenic
g.143017756G>A
g.143320663G>A
-
-
CLCN1_000009
-
Ginjaar, unpublished, Laboratory for Diagnostic Genome Analyses,
1 more item
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Ieke Ginjaar
?/.
1
-
c.305G>T
r.(?)
p.(Cys102Phe)
-
VUS
g.143017760G>T
-
CLCN1(NM_000083.2):c.305G>T (p.C102F)
-
CLCN1_000328
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/+, +/., +?/., ?/.
10
3
c.313C>T
r.(?)
p.(Arg105Cys), p.Arg105Cys
ACMG
likely pathogenic, pathogenic, pathogenic (recessive), VUS
g.143017768C>T
g.143320675C>T
C313T, R105C
-
CLCN1_000011
1 more item
Ginjaar, unpublished, Meyer-Kleine 1995,
PubMed: Brugnoni 2022
,
Journal: Brugnoni 2022
,
2 more items
-
rs201509501
Germline
-
-
-
-
-
Johan den Dunnen
,
Ieke Ginjaar
,
Andreas Laner
,
Raffaella Brugnoni
-?/., ?/?
2
3
c.314G>A
r.(?)
p.(Arg105His)
-
likely benign, VUS
g.143017769G>A
g.143320676G>A
-
-
CLCN1_000183
VKGL data sharing initiative Nederland,
1 more item
-
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Birgit Neitzel
,
VKGL-NL_Nijmegen
-/.
1
-
c.316C>G
r.(?)
p.(Leu106Val)
-
benign
g.143017771C>G
g.143320678C>G
CLCN1(NM_000083.2):c.316C>G (p.L106V)
-
CLCN1_000311
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/.
2
-
c.352=
r.(=)
p.(Trp118=)
-
benign
g.143017807G>T
g.143320714G>T
CLCN1(NM_000083.3):c.352G>T (p.G118W)
-
CLCN1_000012
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
,
VKGL-NL_Utrecht
-/-, -/?
18
3
c.352G>T
r.(?)
p.(Gly118Trp), p.(Trp118Gly)
-
benign
g.143017807G>T
g.143320714G>T
-
-
CLCN1_000012
1 more item
-
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Birgit Neitzel
+/.
1
-
c.373G>A
r.(?)
p.(Gly125Arg)
-
pathogenic
g.143017828G>A
g.143320735G>A
-
-
CLCN1_000268
prob. path.
-
-
-
Germline
-
-
-
-
-
Andreas Laner
+/+
1
3
c.382A>G
r.(?)
p.(Met128Val)
-
pathogenic
g.143017837A>G
g.143320744A>G
-
-
CLCN1_000013
-
Colding-Jörgensen 2003
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/+, +/.
3
3
c.385G>A
r.(?)
p.(Ala129Thr)
-
pathogenic
g.143017840G>A
g.143320747G>A
-
-
CLCN1_000014
-
Ginjaar, unpublished
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Ieke Ginjaar
+/+
1
3
c.394A>T
r.(?)
p.(Ser132Cys)
-
pathogenic
g.143017849A>T
g.143320756A>T
-
-
CLCN1_000015
-
Wu 2002
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.399G>A
r.(?)
p.(Trp133*)
-
pathogenic
g.143017854G>A
g.143320761G>A
-
-
CLCN1_000269
-
-
-
-
Germline
-
-
-
-
-
Andreas Laner
?/.
1
-
c.399G>C
r.(?)
p.(Trp133Cys)
-
VUS
g.143017854G>C
-
CLCN1(NM_000083.2):c.399G>C (p.(Trp133Cys))
-
CLCN1_000390
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/+, +/.
4
3
c.407A>G
r.(?)
p.(Asp136Gly)
-
pathogenic
g.143017862A>G
g.143320769A>G
-
-
CLCN1_000016
-
Heine 1994, Heine 1994; Meyer-Kleine 1995; Mailander 1996 ref func:Fahlke 1995/ Pusch 1996
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Andreas Laner
+?/., ?/?
2
3
c.409T>G
r.(?)
p.(Tyr137As), p.(Tyr137Asp)
-
likely pathogenic, VUS
g.143017864T>G
g.143320771T>G
-
-
CLCN1_000184
combination of variants not reported, unclassified variant
PubMed: Topf 2020
-
-
Germline
-
1/1001 cases
-
-
-
Johan den Dunnen
,
Birgit Neitzel
+/+
2
3
c.411C>G
r.(?)
p.(Tyr137*)
-
pathogenic
g.143017866C>G
g.143320773C>G
-
-
CLCN1_000017
-
-
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
1
-
c.433+72C>G
r.(=)
p.(=)
-
benign
g.143017960C>G
-
CLCN1(NM_000083.3):c.433+72C>G
-
CLCN1_000353
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
?/.
1
3i
c.434-2_435dup
r.spl?
p.?
-
VUS
g.143018456_143018459dup
g.143321363_143321366dup
434-5_434-4insGCA
-
CLCN1_000281
-
-
-
-
Unknown
?
-
-
-
-
Carmen Palma
?/?
1
3i
c.435-91C>T
r.(?)
p.(?)
-
VUS
g.143018368C>T
g.143321275C>T
-
-
CLCN1_000186
1 more item
-
-
-
Germline
-
-
-
-
-
Birgit Neitzel
+/+
1
4
c.449A>G
r.(?)
p.(Tyr150Cys)
-
pathogenic
g.143018473A>G
g.143321380A>G
-
-
CLCN1_000018
-
Mailander 1996 ref func:Wollnik 1997
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., +?/.
2
-
c.450C>A
r.(?)
p.(Tyr150*)
ACMG
likely pathogenic, pathogenic
g.143018474C>A
g.143321381C>A
CLCN1(NM_000083.3):c.450C>A (p.Y150*)
-
CLCN1_000325
ACMG grading: PVS1,PM2 24y old male, severe Myotonia, positive family history of Myotonia,
1 more item
-
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Andreas Laner
,
VKGL-NL_Utrecht
-/., -/?
2
4
c.450C>T
r.(?)
p.(=), p.(Tyr150Tyr)
-
benign
g.143018474C>T
g.143321381C>T
CLCN1(NM_000083.3):c.450C>T (p.Y150=)
-
CLCN1_000187
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Birgit Neitzel
,
VKGL-NL_Utrecht
-/., ?/?
3
4
c.461A>G
r.(?)
p.(Gln154Arg)
-
benign, VUS
g.143018485A>G
g.143321392A>G
-
-
CLCN1_000019
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record, Germline
-
0/200
-
-
-
Johan den Dunnen
,
Fernando Morales
,
VKGL-NL_Nijmegen
+/.
2
4
c.478C>T
r.(?)
p.(Gln160*)
-
pathogenic
g.143018502C>T
g.143321409C>T
-
-
CLCN1_000271
-
-
-
rs80411
Germline
-
-
-
-
-
Andreas Laner
,
Raffaella Brugnoni
+/?, +?/.
3
4
c.480G>C
r.(?)
p.(Gln160His)
-
likely pathogenic, pathogenic
g.143018504G>C
g.143321411G>C
-
-
CLCN1_000189
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Birgit Neitzel
,
VKGL-NL_Nijmegen
+/+
1
4
c.481T>G
r.(?)
p.(Phe161Val)
-
pathogenic
g.143018505T>G
g.143321412T>G
-
-
CLCN1_000020
-
Plassart-Schiess 1998
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/?
1
4
c.490T>C
r.(?)
p.(Trp164Arg)
-
VUS
g.143018514T>C
g.143321421T>C
-
-
CLCN1_000190
unclassified variant
-
-
-
Germline
-
-
-
-
-
Birgit Neitzel
+/+
1
4
c.494T>G
r.(?)
p.(Val165Gly)
-
pathogenic
g.143018518T>G
g.143321425T>G
-
-
CLCN1_000021
-
Meyer-Kleine 1995 ref func:Zhang 2000a
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/+, +/., +/?, +?/+, +?/., ?/.
46
4
c.501C>G
r.(?)
p.(Phe167Leu), p.Phe167Leu
ACMG
likely pathogenic, pathogenic, pathogenic (recessive), VUS
g.143018525C>G
g.143321432C>G
C501G, F167L
-
CLCN1_000022
no variant 2nd chromosome,
2 more items
George 1994; Meyer-Kleine 1995 ref func:Zhang 2000a, Ginjaar, unpublished,
PubMed: Maggi 2017
,
3 more items
-
rs149729531
Germline, Germline/De novo (untested)
yes
-
-
-
-
Johan den Dunnen
,
Ieke Ginjaar
,
Birgit Neitzel
,
Andreas Laner
,
Carmen Palma
,
Raffaella Brugnoni
,
Anaïs Begemann
,
Helen Latsoudis
-?/.
1
-
c.531C>T
r.(?)
p.(Leu177=)
-
likely benign
g.143018555C>T
g.143321462C>T
CLCN1(NM_000083.2):c.531C>T (p.L177=)
-
CLCN1_000318
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/., ?/?
2
4
c.537C>G
r.(?)
p.(Cys179Trp)
-
pathogenic, VUS
g.143018561C>G
g.143321468C>G
-
-
CLCN1_000023
-
Ginjaar, unpublished
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Ieke Ginjaar
?/.
1
-
c.556G>A
r.(?)
p.(Ala186Thr)
-
VUS
g.143018580G>A
-
-
-
CLCN1_000393
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/?
1
4
c.557C>T
r.(?)
p.(Ala186Val)
-
VUS
g.143018581C>T
g.143321488C>T
-
-
CLCN1_000024
-
-
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.562+1G>C
r.spl?
p.?
-
pathogenic
g.143018587G>C
g.143321494G>C
-
-
CLCN1_000312
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/?
1
4i
c.562+29A>G
r.(?)
p.(=)
-
VUS
g.143018615A>G
g.143321522A>G
-
-
CLCN1_000025
-
-
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/-, -/?
17
4i
c.563-23A>T
r.(?)
p.(=), p.(?)
-
benign
g.143018785A>T
g.143321692A>T
-
-
CLCN1_000027
-
-
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Birgit Neitzel
?/?
2
4i
c.563-17T>G
r.(?)
p.(=)
-
VUS
g.143018791T>G
g.143321698T>G
-
-
CLCN1_000026
-
-
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
2
-
c.563-9C>T
r.(=)
p.(=)
-
likely benign
g.143018799C>T
-
CLCN1(NM_000083.2):c.563-9C>T, CLCN1(NM_000083.3):c.563-9C>T
-
CLCN1_000329
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
+?/.
1
5
c.563G>C
r.(?)
p.(Gly188Ala)
-
likely pathogenic
g.143018808G>C
g.143321715G>C
-
-
CLCN1_000284
-
-
-
-
Germline
-
-
-
-
-
Raffaella Brugnoni
+?/.
1
5
c.563G>T
r.(spl?)
p.(Gly188Val)
-
likely pathogenic
g.143018808G>T
g.143321715G>T
-
-
CLCN1_000277
-
-
-
-
Germline
-
-
-
-
-
Carmen Palma
+/., +/?, +?/., ?/.
9
5
c.568G>A
r.(?)
p.(Gly190Arg), p.(Gly190His)
-
likely pathogenic, pathogenic, VUS
g.143018813G>A
g.143321720G>A
-
-
CLCN1_000185
PolyPhen-2: prob.dam. (PSIC:1,0), MutTaster: dis.causing (0,99); index mother normal,
1 more item
Ginjaar, unpublished
-
-
CLASSIFICATION record, Germline
yes
-
-
-
CarmenPalma
Ieke Ginjaar
,
Birgit Neitzel
,
Andreas Laner
,
Carmen Palma
,
VKGL-NL_Nijmegen
+/?
1
5
c.568G>C
r.(?)
p.(Gly190Ala)
-
pathogenic
g.143018813G>C
g.143321720G>C
-
-
CLCN1_000193
-
-
-
-
Germline
-
-
-
-
-
Birgit Neitzel
+/+
2
5
c.568G>M
r.(?)
p.(Gly190Arg)
-
pathogenic
g.g143018813G>M
-
G190R
-
CLCN1_000028
-
Laboratory for Diagnostic Genome Analyses
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/+, +/?, +?/.
3
5
c.568G>T
r.(?)
p.(Gly190*)
-
likely pathogenic, pathogenic
g.143018813G>T
g.143321720G>T
CLCN1(NM_000083.2):c.568G>T (p.(Gly190Ter))
-
CLCN1_000029
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
Birgit Neitzel
,
VKGL-NL_Leiden
+/.
12
5
c.568_569delinsTC
r.(?)
p.(Gly190Ser)
-
pathogenic, pathogenic (dominant), pathogenic (recessive)
g.143018813_143018814delinsTC
g.143321720_143321721delinsTC
568-569GG>TC, c.568-569GG>TC, c.568GG>TC, G190S, G190S/L861P*
-
CLCN1_000282
-
Ginjaar, unpublished,
PubMed: Brugnoni 2022
,
Journal: Brugnoni 2022
,
1 more item
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Ieke Ginjaar
,
Raffaella Brugnoni
,
Evgeniya Ivanova
+/+
1
5
c.569C>G
r.(?)
p.(Gly190Ala)
-
pathogenic
g.143018814C>G
g.143321721C>G
-
-
CLCN1_000030
1 more item
-
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.569G>C
r.(?)
p.(Gly190Ala)
-
likely pathogenic
g.143018814G>C
-
CLCN1(NM_000083.2):c.569G>C (p.(Gly190Ala))
-
CLCN1_000394
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/+, +/.
2
5
c.577G>A
r.(?)
p.(Glu193Lys)
-
pathogenic
g.143018822G>A
g.143321729G>A
-
-
CLCN1_000031
-
Colding-Jorgensen 2003;Grunnet 2003
-
rs80356686
Germline
-
-
-
-
-
Johan den Dunnen
,
Andreas Laner
+/+
3
5
c.577G>T
r.(?)
p.(Glu193*)
-
pathogenic
g.143018822G>T
g.143321729G>T
-
-
CLCN1_000032
-
Wu 2002
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/+, +/.
4
5
c.585_589del
r.(?)
p.(Lys195Asnfs*62)
-
pathogenic
g.143018830_143018834del
g.143321737_143321741del
-
-
CLCN1_000033
-
Ginjaar, unpublished, Laboratory for Diagnostic Genome Analyses
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Ieke Ginjaar
+/+, +/?
4
5
c.592C>G
r.(?)
p.(Leu198Val)
-
pathogenic
g.143018837C>G
g.143321744C>G
-
-
CLCN1_000034
-
Simpson 2004
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Birgit Neitzel
+?/.
1
5
c.593T>A
r.(?)
p.(Leu198His)
-
likely pathogenic
g.143018838T>A
g.143321745T>A
-
-
CLCN1_000212
-
-
-
-
Germline
-
-
-
-
-
Raffaella Brugnoni
+/.
1
5
c.593T>C
r.(?)
p.(Leu198Pro)
-
pathogenic
g.143018838T>C
g.143321745T>C
-
-
CLCN1_000285
-
-
-
-
Germline
-
-
-
-
-
Raffaella Brugnoni
?/.
1
-
c.596G>A
r.(?)
p.(Arg199His)
-
VUS
g.143018841G>A
-
CLCN1(NM_000083.2):c.596G>A (p.R199H)
-
CLCN1_000313
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
+/+
1
5
c.598G>A
r.(?)
p.(Gly200Arg)
-
pathogenic
g.143018843G>A
g.143321750G>A
-
-
CLCN1_000035
-
Mailander 1996 ref func:Wollnik 1997
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/?
1
5
c.599G>A
r.(?)
p.(Gly200Glu)
-
VUS
g.143018844G>A
g.143321751G>A
-
-
CLCN1_000036
-
-
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/+
1
5
c.601dup
r.(?)
p.(Val201Glyfs*58)
-
pathogenic
g.143018846dup
g.143321753dup
-
-
CLCN1_000037
-
Esteban 1998
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.601G>A
r.(?)
p.(Val201Ile)
-
VUS
g.143018846G>A
g.143321753G>A
-
-
CLCN1_000246
Polyphen-2: probably damaging (PSIC: 0,966)
-
-
-
Germline
-
-
-
-
-
Andreas Laner
?/.
1
-
c.613G>A
r.(?)
p.(Glu205Lys)
-
VUS
g.143018858G>A
g.143321765G>A
-
-
CLCN1_000247
PolyPhen-2: prob.damaging (PSIC 0.99)
-
-
-
Germline
-
-
-
-
-
Andreas Laner
-?/.
1
-
c.633C>T
r.(?)
p.(Ala211=)
-
likely benign
g.143018878C>T
-
CLCN1(NM_000083.2):c.633C>T (p.(=))
-
CLCN1_000354
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+?/?
1
5
c.650T>A
r.(?)
p.Val217Asp
-
likely pathogenic
g.143018895T>A
g.143321802T>A
-
-
CLCN1_000215
-
-
-
-
Unknown
-
-
-
-
-
Raffaella Brugnoni
+/+, ?/.
2
5
c.652G>A
r.(?)
p.(Ala218Thr)
-
pathogenic, VUS
g.143018897G>A
g.143321804G>A
-
-
CLCN1_000038
1 heterozygous, no homozygous;
Clinindb (India)
de Diego 1999,
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs189963844
Germline
-
1/2795 individuals
-
-
-
Johan den Dunnen
,
Mohammed Faruq
-/., ?/.
2
-
c.663G>A
r.(=), r.(?)
p.(=), p.(Ala221=)
-
benign, VUS
g.143018908G>A
g.143321815G>A
CLCN1(NM_000083.2):c.663G>A (p.(Ala221=))
-
CLCN1_000248
LOVD, VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Andreas Laner
,
VKGL-NL_Leiden
-/-
1
5
c.663G>T
r.(?)
p.(Ala221Ala)
-
benign
g.143018908G>T
g.143321815G>T
-
-
CLCN1_000041
-
-
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
5
c.677G>A
r.(?)
p.(Gly226Asp)
-
VUS
g.143018922G>A
g.143321829G>A
-
-
CLCN1_000332
-
-
-
rs1304529237
Germline
-
-
-
-
-
LOVD
?/.
1
-
c.685G>A
r.(?)
p.(Val229Met)
-
VUS
g.143018930G>A
g.143321837G>A
-
-
CLCN1_000303
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
-
c.688G>A
r.(?)
p.(Gly230Arg)
-
VUS
g.143018933G>A
-
-
-
CLCN1_000355
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/+, +/., +?/.
7
5
c.689G>A
r.(?)
p.(Gly230Glu)
-
likely pathogenic, pathogenic
g.143018934G>A
g.143321841G>A
-
-
CLCN1_000039
VKGL data sharing initiative Nederland
George 1994; Meyer-Kleine 1995; Koty,
PubMed: Granger 2022
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Nijmegen
+?/.
1
-
c.696G>A
r.(?)
p.(Glu232Glu)
ACMG
likely pathogenic
g.143018941G>A
g.143321848G>A
-
-
CLCN1_000323
ACMG: PM2,PM3,PP3,PP4
-
-
rs923380712
Germline
-
-
-
-
-
Andreas Laner
+/+
1
5i
c.696+2T>A
r.spl
p.?
-
pathogenic
g.143018943T>A
g.143321850T>A
-
-
CLCN1_000040
-
Brugnoni 1999
-
-
Germline
-
-
-
-
-
Johan den Dunnen
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