Global Variome shared LOVD
SCNN1A (sodium channel, non-voltage-gated 1 alpha s...)
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Curator:
Abul Kalam Azad
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Unique variants in the SCNN1A gene
The variants shown are described using the NM_001038.5 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.
75 entries on 1 page. Showing entries 1 - 75.
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Legend
How to query
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
-/.
1
-
c.-1909A>G
r.(?)
p.(=)
-
benign
g.6486525T>C
g.6377359T>C
-
-
LTBR_000001
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/?
1
1
c.-93A>G
r.(?)
p.(=)
-
VUS
g.6484709T>C
g.6375543T>C
-
-
SCNN1A_000001
-
PubMed: Mutesa 2008
;
PubMed: 19462466
-
rs10849447
Germline
-
0.49
-
-
-
Abul Kalam Azad
-?/., ?/.
2
-
c.-55+2T>C
r.spl?
p.?
-
likely benign, VUS
g.6484669A>G
g.6375503A>G
SCNN1A(NM_001038.5):c.-55+2T>C, SCNN1A(NM_001038.6):c.-55+2T>C
-
LTBR_000005
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
?/?
1
1i
c.-55+5G>C
r.(?)
p.(?)
-
VUS
g.6484666C>G
g.6375500C>G
-
-
SCNN1A_000002
-
PubMed: Mutesa 2008
;
PubMed: 19462466
-
rs13306617
Germline
-
0.003
-
-
-
Abul Kalam Azad
?/?
1
1i
c.-54-14C>T
r.(?)
p.(?)
-
VUS
g.6484017G>A
g.6374851G>A
-
-
SCNN1A_000003
-
PubMed: 19462466
-
rs61758858
Germline
-
-
-
-
-
Abul Kalam Azad
?/.
1
-
c.-39G>A
r.(?)
p.(=)
-
VUS
g.6483988C>T
g.6374822C>T
NM_001159576.1:c.139G>A (Glu47Lys)
-
SCNN1A_000062
-
PubMed: Rinne 2020
-
-
Germline
no
-
-
-
-
Johan den Dunnen
?/?
1
2
c.-28T>C
r.(?)
p.(=)
-
VUS
g.6483977A>G
g.6374811A>G
-
-
SCNN1A_000004
-
PubMed: Mutesa 2008
-
rs61759919
Germline
-
-
-
-
-
Abul Kalam Azad
-?/.
1
-
c.47C>A
r.(?)
p.(Ser16Tyr)
-
likely benign
g.6483903G>T
-
-
-
LTBR_000011
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/?
1
2
c.99C>T
r.(?)
p.(=)
-
VUS
g.6483851G>A
g.6374685G>A
-
-
SCNN1A_000005
-
PubMed: Mutesa 2008
;
PubMed: 19462466
-
rs13306619
Germline
-
-
-
-
-
Abul Kalam Azad
-?/.
1
-
c.107C>T
r.(?)
p.(Ala36Val)
-
likely benign
g.6483843G>A
g.6374677G>A
SCNN1A(NM_001159576.1):c.284C>T (p.A95V)
-
LTBR_000004
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
2
c.166C>T
r.(?)
p.(Arg56*)
-
pathogenic (recessive)
g.6483784G>A
g.6374618G>A
-
-
SCNN1A_000024
-
PubMed: Kerem 1999
-
rs778872550
Germline
yes
-
-
-
-
Susan Tzotzos
?/?
1
2
c.183C>A
r.(?)
p.(Phe61Leu)
-
VUS
g.6483767G>T
g.6374601G>T
-
-
SCNN1A_000006
-
PubMed: 19462466
-
rs61758859
Germline
-
-
-
-
-
Abul Kalam Azad
+/.
1
2
c.189C>A
r.(?)
p.(Cys63*)
-
pathogenic (recessive)
g.6483761G>T
g.6374595G>T
-
-
SCNN1A_000025
-
PubMed: Welzel 2013
-
-
Germline
yes
-
-
-
-
Susan Tzotzos
+/.
3
2
c.203_204del
r.(?)
p.(Ile68Thrfs*76)
-
pathogenic (recessive)
g.6483746_6483747del
g.6374580_6374581del
-
-
SCNN1A_000026
-
PubMed: Chang 1996
ClinVar-9263
rs765835593
Germline
yes
-
-
-
-
Susan Tzotzos
+/.
1
2
c.206A>G
r.(?)
p.(His69Arg)
-
pathogenic (recessive)
g.6483744T>C
g.6374578T>C
-
-
SCNN1A_000053
-
PubMed: Cayir (2019)
-
-
Germline
yes
-
-
-
-
Susan Tzotzos
+/.
1
2
c.217C>T
r.(?)
p.(Arg73Cys)
-
pathogenic (recessive)
g.6483733G>A
g.6374567G>A
-
-
SCNN1A_000054
-
PubMed: Gopal-Kothandapani (2019)
-
rs763345732
Germline
yes
-
-
-
-
Susan Tzotzos
?/?
1
2
c.241C>T
r.(?)
p.(Arg81Cys)
-
VUS
g.6483709G>A
g.6374543G>A
-
-
SCNN1A_000007
-
PubMed: 19462466
-
rs61759860
Germline
-
-
-
-
-
Abul Kalam Azad
+/.
1
2
c.301C>A
r.(?)
p.(Gln101Lys)
-
pathogenic (recessive)
g.6483649G>T
g.6374483G>T
-
-
SCNN1A_000027
-
PubMed: Mora-Lopez 2012
-
-
Germline
yes
-
-
-
-
Susan Tzotzos
?/?
1
2
c.340G>A
r.(?)
p.(Val114Ile)
-
VUS
g.6483610C>T
g.6374444C>T
-
-
SCNN1A_000008
-
PubMed: 19462466
-
rs61759861
Germline
-
-
-
-
-
Abul Kalam Azad
+/.
1
2
c.416G>A
r.(?)
p.(Arg139Lys)
-
pathogenic (recessive)
g.6483534C>T
g.6374368C>T
-
-
SCNN1A_000028
-
PubMed: Kala Ahluwalia 2014
-
rs370256768
Germline
yes
-
-
-
-
Susan Tzotzos
+/.
1
3
c.505_506del
r.(?)
p.(Thr169Serfs*36)
-
pathogenic (recessive)
g.6472788_6472789del
g.6363622_6363623del
-
-
SCNN1A_000029
-
PubMed: Kerem 1999
-
rs1014590535
Germline
yes
-
-
-
-
Susan Tzotzos
-/.
1
-
c.540G>T
r.(?)
p.(Leu180=)
-
benign
g.6472753C>A
g.6363587C>A
SCNN1A():g.6472753C>A
-
SCNN1A_000023
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/?
1
3
c.[540G>T;541C>T]
r.(?)
p.(Arg181Trp)
-
VUS
g.[6472752G>A;6472753C>A]
-
c.540_541delinsTT
-
SCNN1A_000009
variants 540G>T and 541C>T always occur in cis, in dbSNP rs61759862 and rs55797039
PubMed: 19462466
-
-
Germline
-
0.024
-
-
-
Abul Kalam Azad
?/?
1
3
c.541C>T
r.(?)
p.(Arg181Trp)
-
VUS
g.6472752G>A
g.6363586G>A
-
-
SCNN1A_000010
NOTE: variant reported to occur always in cis with c.540G>T
PubMed: 19462466
-
rs55797039
Germline
-
-
-
-
-
Abul Kalam Azad
+/.
1
-
c.574del
r.(?)
p.(Arg192Glyfs*57)
-
pathogenic
g.6472719del
-
SCNN1A(NM_001038.6):c.574delA (p.R192Gfs*57)
-
LTBR_000014
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
+/.
2
3
c.588dup
r.(?)
p.(Pro197Alafs*9), p.(ProAla197fs*9)
-
pathogenic (recessive)
g.6472705dup
g.6363539dup
-
-
SCNN1A_000030
-
PubMed: Welzel 2013
-
-
Germline
yes
-
-
-
-
Susan Tzotzos
+/.
1
3
c.598dup
r.(?)
p.(Ala200Glyfs*6)
-
pathogenic (recessive)
g.6472698dup
g.6363532dup
-
-
SCNN1A_000052
-
PubMed: Cayir (2019)
-
rs759611286
Germline
yes
-
-
-
-
Susan Tzotzos
+/.
1
-
c.684+2T>A
r.spl
p.?
-
pathogenic (recessive)
g.6472607A>T
g.6363441A>T
-
-
SCNN1A_000031
-
PubMed: Ekinci 2013
-
-
Germline
yes
-
-
-
-
Susan Tzotzos
+/.
1
-
c.685-1G>A
r.spl
p.?
-
pathogenic
g.6471408C>T
g.6362242C>T
-
-
SCNN1A_000061
-
PubMed: Serra G, 2021
-
-
Germline
yes
-
-
-
-
Susan Tzotzos
+/.
1
4
c.727T>C
r.(?)
p.(Ser243Pro)
-
pathogenic (recessive)
g.6471365A>G
g.6362199A>G
-
-
SCNN1A_000032
-
PubMed: Dirlewanger 2011
-
rs776069930
Germline
yes
-
-
-
-
Susan Tzotzos
+/.
2
4
c.729del
r.(?)
p.(Val245Trpfs*4)
-
pathogenic (recessive)
g.6471363del
g.6362197del
-
-
SCNN1A_000033
-
PubMed: Schaedel 1999
ClinVar-9266
,
ClinVar-RCV000009848.2
rs1592074026
Germline
yes
-
-
-
-
Susan Tzotzos
+/.
1
4
c.729_730del
r.(?)
p.(Val245Glyfs*65)
-
pathogenic (recessive)
g.6471362_6471363del
g.6362196_6362197del
-
-
SCNN1A_000060
-
PubMed: Huneif, 2021
-
-
Germline
yes
-
-
-
-
Susan Tzotzos
+/.
1
4
c.742del
r.(?)
p.(Val248*)
-
pathogenic (recessive)
g.6471351del
g.6362185del
-
-
SCNN1A_000051
-
PubMed: Welzel 2013
-
-
Germline
yes
-
-
-
-
Susan Tzotzos
+/.
1
4
c.814dup
r.(?)
p.(Glu272Glyfs*39)
-
pathogenic (recessive)
g.6471279dup
g.6362113dup
-
-
SCNN1A_000037
-
PubMed: Wang (2013)
-
rs747904876
Germline
yes
-
-
-
-
Susan Tzotzos
+?/.
1
-
c.875+1G>A
r.spl?
p.?
-
likely pathogenic
g.6471216C>T
-
-
-
SCNN1A_000058
-
-
-
-
Germline
-
-
-
-
-
Sha Hong
+/.
1
-
c.875+2dup
r.(spl?)
p.?
-
pathogenic (recessive)
g.6471215dup
g.6362049dup
-
-
SCNN1A_000038
-
PubMed: Silva (2013)
ClinVar-871275
-
Germline
yes
-
-
-
-
Susan Tzotzos
-?/.
1
-
c.876-13C>T
r.(=)
p.(=)
-
likely benign
g.6465059G>A
-
SCNN1A(NM_001038.6):c.876-13C>T
-
LTBR_000012
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/.
1
5
c.979G>T
r.(?)
p.(Gly327Cys)
-
pathogenic (recessive)
g.6464943C>A
g.6355777C>A
-
-
SCNN1A_000039
-
PubMed: Edelheit (2005)
-
rs974854786
Germline
yes
-
-
-
-
Susan Tzotzos
?/.
2
-
c.997C>T
r.(?)
p.(Arg333Cys)
-
VUS
g.6464584G>A
g.6355418G>A
-
-
SCNN1A_000057
1 homozygous;
Clinindb (India)
, 3 heterozygous;
Clinindb (India)
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs146177203
Germline
-
1/2795 individuals, 3/2795 individuals
-
-
-
Mohammed Faruq
-/., ?/?
2
6
c.1000G>A
r.(?)
p.(Ala334Thr)
-
benign, VUS
g.6464581C>T
g.6355415C>T
-
-
SCNN1A_000011
VKGL data sharing initiative Nederland
PubMed: Mutesa 2008
;
PubMed: 19462466
-
rs11542844
CLASSIFICATION record, Germline
-
0.039
-
-
-
Abul Kalam Azad
,
VKGL-NL_Nijmegen
?/.
1
-
c.1183A>C
r.(?)
p.(Thr395Pro)
-
VUS
g.6463975T>G
-
SCNN1A(NM_001159576.1):c.1360A>C (p.T454P)
-
LTBR_000009
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
-
c.1242+1G>A
r.spl?
p.?
-
pathogenic
g.6463915C>T
-
-
-
LTBR_000007
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
2
8
c.1305del
r.(?)
p.(Tyr436Ilefs*46)
-
pathogenic (recessive)
g.6463659del
g.6354493del
-
-
SCNN1A_000034
-
PubMed: Kerem (1999)
,
PubMed: Kerem 1999
-
rs758014063
Germline
yes
-
-
-
-
Susan Tzotzos
+/.
1
8
c.1311del
r.(?)
p.(Arg438Glyfs*44)
-
pathogenic (recessive)
g.6463653del
g.6354487del
-
-
SCNN1A_000050
-
PubMed: Wang (2013)
-
-
Germline
yes
-
-
-
-
Susan Tzotzos
+/.
1
8
c.1339dup
r.(?)
p.(Tyr447Leufs*13)
-
pathogenic (recessive)
g.6463625dup
g.6354459dup
-
-
SCNN1A_000049
-
PubMed: Saxena (2002)
-
rs754323537
Germline
yes
-
-
-
-
Susan Tzotzos
+/.
1
8
c.1344_1347dup
r.(?)
p.(His450Lysfs*11)
-
pathogenic (recessive)
g.6463619_6463622dup
g.6354453_6354456dup
1342_1343insTACA
-
SCNN1A_000048
-
PubMed: Welzel (2013)
-
-
Germline
yes
-
-
-
-
Susan Tzotzos
+/.
1
8
c.1356del
r.(?)
p.(Trp453Glyfs*29)
-
pathogenic (recessive)
g.6463609del
g.6354443del
-
-
SCNN1A_000047
-
PubMed: Edelheit (2005)
-
-
Germline
yes
-
-
-
-
Susan Tzotzos
?/.
1
-
c.1358G>T
r.(?)
p.(Trp453Leu)
-
VUS
g.6463606C>A
g.6354440C>A
SCNN1A(NM_001159576.1):c.1535G>T (p.W512L)
-
LTBR_000003
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
-
c.1360+1G>C
r.spl?
p.?
-
pathogenic
g.6463603C>G
g.6354437C>G
-
-
SCNN1A_000022
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
2
-
c.1360+1G>T
r.spl, r.spl?
p.?
-
pathogenic, pathogenic (recessive)
g.6463603C>A
g.6354437C>A
-
-
SCNN1A_000021
VKGL data sharing initiative Nederland
PubMed: Kala Ahluwalia 2014
-
rs573376286
CLASSIFICATION record, Germline
yes
-
-
-
-
VKGL-NL_Nijmegen
,
Susan Tzotzos
+/.
1
-
c.1361-2A>G
r.(spl?)
p.?
-
pathogenic (recessive)
g.6458573T>C
g.6349407T>C
-
-
SCNN1A_000046
-
PubMed: Welzel (2013)
ClinVar-992425
-
Germline
yes
-
-
-
-
Susan Tzotzos
?/.
1
-
c.1367G>A
r.(?)
p.(Cys456Tyr)
-
VUS
g.6458565C>T
-
SCNN1A(NM_001038.6):c.1367G>A (p.C456Y)
-
LTBR_000013
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/., ?/.
2
-
c.1435T>C
r.(?)
p.(Cys479Arg)
-
pathogenic, VUS
g.6458497A>G
g.6349331A>G
SCNN1A(NM_001038.6):c.1435T>C (p.C479R)
-
SCNN1A_000020
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
+/., +?/.
3
-
c.1439+1G>C
r.0?, r.spl?
p.?
-
likely pathogenic, pathogenic (recessive)
g.6458492C>G
g.6349326C>G
-
-
SCNN1A_000045
-
PubMed: Wang (2013)
-
rs1369791519
Germline
yes
-
-
-
-
Susan Tzotzos
,
Sha Hong
?/?
1
9i
c.1439+26G>A
r.(?)
p.(?)
-
VUS
g.6458467C>T
g.6349301C>T
-
-
SCNN1A_000012
-
PubMed: 19462466
-
rs61759912
Germline
-
-
-
-
-
Abul Kalam Azad
+/.
6
10
c.1449del
r.(?)
p.(Tyr484Thrfs*13), p.(Tyr484ThrfsTer13)
-
pathogenic, pathogenic (recessive)
g.6458378del
g.6349212del
SCNN1A(NM_001159576.2):c.1626delC (p.Y543Tfs*13)
-
SCNN1A_000035
VKGL data sharing initiative Nederland
PubMed: Schaedel (1999)
,
PubMed: Schaedel 1999
RSV000009847.2
ClinVar-9265
,
ClinVar-9265
,
ClinVar-RCV000009847.2
rs756434927
,
rs765434927
CLASSIFICATION record, Germline
yes
-
-
-
-
VKGL-NL_Nijmegen
,
VKGL-NL_VUmc
,
Susan Tzotzos
+/.
3
10
c.1474C>T
r.(?)
p.(Arg492*)
-
pathogenic (recessive)
g.6458353G>A
g.6349187G>A
-
-
SCNN1A_000044
-
PubMed: Bonny (2002)
-
rs775543049
Germline
yes
-
-
-
-
Susan Tzotzos
-/., -?/., ?/?
5
10
c.1477T>C
r.(?)
p.(Trp493Arg)
-
benign, likely benign, VUS
g.6458350A>G
g.6349184A>G
SCNN1A(NM_001159576.2):c.1654T>C (p.W552R)
-
SCNN1A_000013
2 homozygous;
Clinindb (India)
, VKGL data sharing initiative Nederland
PubMed: 19462466
,
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs5742912
CLASSIFICATION record, Germline
-
0.018, 2/2795 individuals
-
-
-
Abul Kalam Azad
,
VKGL-NL_Groningen
,
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
,
Mohammed Faruq
-?/.
3
-
c.1484C>T
r.(?)
p.(Ser495Leu)
-
likely benign
g.6458343G>A
g.6349177G>A
SCNN1A(NM_001038.6):c.1484C>T (p.S495L), SCNN1A(NM_001159576.1):c.1661C>T (p.S554L)
-
SCNN1A_000056
16 heterozygous, no homozygous;
Clinindb (India)
, VKGL data sharing initiative Nederland
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs148749888
CLASSIFICATION record, Germline
-
16/2794 individuals
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
,
Mohammed Faruq
+/.
7
11
c.1522C>T
r.(?)
p.(Arg508*)
-
pathogenic (recessive)
g.6458147G>A
g.6348981G>A
-
-
SCNN1A_000043
-
PubMed: Belot (2008)
,
PubMed: Chang (1996)
,
PubMed: Gopal-Kothandapani, 2019
,
PubMed: Kerem (1999)
,
1 more item
9264,
ClinVar-RCV000009846.2
,
ClinVar-9264
rs137852634
Germline
yes
-
-
-
-
Susan Tzotzos
-?/.
1
-
c.1540G>A
r.(?)
p.(Val514Ile)
-
likely benign
g.6458129C>T
-
SCNN1A(NM_001038.6):c.1540G>A (p.V514I)
-
LTBR_000015
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/.
1
12
c.1571C>T
r.(?)
p.(Val524Ala)
-
pathogenic (recessive)
g.6457951A>G
g.6348785A>G
-
-
SCNN1A_000059
-
PubMed: Gopal-Kothandapani 2019
-
-
Germline
yes
-
-
-
-
Susan Tzotzos
+/.
1
12
c.1571T>C
r.(?)
p.(Val524Ala)
-
pathogenic (recessive)
g.6457951A>G
g.6348785A>G
-
-
SCNN1A_000059
-
PubMed: Gopal-Kothandapani 2019
-
-
Germline
yes
-
-
-
-
Susan Tzotzos
+/., +?/., ?/?
5
12
c.1582_1584del
r.(?)
p.(Phe528del)
-
likely pathogenic, pathogenic (recessive), VUS
g.6457943_6457945del
g.6348777_6348779del
-
-
SCNN1A_000014, SCNN1A_000042
VKGL data sharing initiative Nederland
PubMed: 19462466
,
PubMed: Gopal-Kothandapani, 2019
,
PubMed: Turan (2018)
-
rs61759913
CLASSIFICATION record, Germline
yes
-
-
-
-
Abul Kalam Azad
,
VKGL-NL_Nijmegen
,
Susan Tzotzos
+/.
2
13
c.1678G>A
r.(?)
p.(Gly560Ser)
-
pathogenic (recessive)
g.6457371C>T
g.6348205C>T
-
-
SCNN1A_000041
-
PubMed: Huppmann (2011)
,
PubMed: Turan (2018)
-
rs772866436
Germline
yes
-
-
-
-
Susan Tzotzos
+/.
1
13
c.1684T>C
r.(?)
p.(Ser562Pro)
-
pathogenic (recessive)
g.6457365A>G
g.6348199A>G
-
-
SCNN1A_000040
-
PubMed: Riepe (2009)
-
-
Germline
yes
-
-
-
-
Susan Tzotzos
+/.
3
13
c.1685C>T
r.(?)
p.(Ser562Leu)
-
pathogenic (recessive)
g.6457364G>A
g.6348198G>A
-
-
SCNN1A_000036
-
PubMed: Nur (2017)
,
PubMed: Schaedel (1999)
ClinVar-RCV000009849
,
ClinVar-9267
,
ClinVar-RCV0009849
,
ClinVar-9267
rs137852635
Germline
yes
-
-
-
-
Susan Tzotzos
-?/., ?/?
2
13
c.1717G>A
r.(?)
p.(Val573Ile)
-
likely benign, VUS
g.6457332C>T
g.6348166C>T
SCNN1A(NM_001038.6):c.1717G>A (p.V573I)
-
SCNN1A_000015
VKGL data sharing initiative Nederland
PubMed: Mutesa 2008
-
rs59142484
CLASSIFICATION record, Germline
-
-
-
-
-
Abul Kalam Azad
,
VKGL-NL_Utrecht
?/.
1
-
c.1772G>A
r.(?)
p.(Arg591Gln)
-
VUS
g.6457277C>T
g.6348111C>T
SCNN1A(NM_001159576.1):c.1949G>A (p.R650Q)
-
LTBR_000002
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/.
1
-
c.1853G>T
r.(?)
p.(Cys618Phe)
-
benign
g.6457196C>A
g.6348030C>A
-
-
SCNN1A_000055
10 heterozygous, no homozygous;
Clinindb (India)
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs3741913
Germline
-
10/2770 individuals
-
-
-
Mohammed Faruq
-?/.
1
-
c.1935C>T
r.(?)
p.(Ala645=)
-
likely benign
g.6457114G>A
-
SCNN1A(NM_001038.6):c.1935C>T (p.A645=)
-
LTBR_000008
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-?/.
1
-
c.1978A>G
r.(?)
p.(Ser660Gly)
-
likely benign
g.6457071T>C
g.6347905T>C
-
-
SCNN1A_000019
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/., ?/?
3
13
c.1987A>G
r.(?)
p.(Thr663Ala)
-
benign, VUS
g.6457062T>C
g.6347896T>C
SCNN1A(NM_001159576.2):c.2164A>G (p.T722A)
-
SCNN1A_000016
VKGL data sharing initiative Nederland
PubMed: Mutesa 2008
;
PubMed: 19462466
-
rs2228576
CLASSIFICATION record, Germline
-
0.648
-
-
-
Abul Kalam Azad
,
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
?/., ?/?
2
13
c.2004dup
r.(?)
p.(Pro669Alafs*62), p.(Pro669AlafsTer62)
-
VUS
g.6457051dup
g.6347885dup
SCNN1A(NM_001159576.1):c.2181dupG (p.P728Afs*62)
-
LTBR_000006, SCNN1A_000017
VKGL data sharing initiative Nederland
PubMed: 19462466
-
rs61759914
CLASSIFICATION record, Germline
-
-
-
-
-
VKGL-NL_Rotterdam
,
Abul Kalam Azad
-?/.
1
-
c.2004G>T
r.(?)
p.(Gly668=)
-
likely benign
g.6457045C>A
-
SCNN1A(NM_001038.6):c.2004G>T (p.G668=)
-
LTBR_000010
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
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