Global Variome shared LOVD
GJB2 (gap junction protein, beta 2, 26kDa)
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Unique variants in the GJB2 gene
The variants shown are described using the NM_004004.5 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.
132 entries on 2 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
-/.
1
1
c.-216T>G
r.(?)
p.(=)
-
benign
g.20767115A>C
g.20192976A>C
-
-
GJB2_000064
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/.
1
1
c.-205T>G
r.(?)
p.(=)
-
likely benign
g.20767104A>C
g.20192965A>C
-
-
GJB2_000063
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/.
1
1
c.-127G>C
r.(?)
p.(=)
-
benign
g.20767026C>G
g.20192887C>G
-
-
GJB2_000062
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/., -?/.
2
1
c.-45C>A
r.(?)
p.(=)
-
benign, likely benign
g.20766944G>T
g.20192805G>T
GJB2(NM_004004.6):c.-45C>A
-
GJB2_000048
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Nijmegen
-?/.
1
1
c.-35C>T
r.(?)
p.(=)
-
likely benign
g.20766934G>A
g.20192795G>A
-
-
GJB2_000061
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/., +?/.
9
1i
c.-23+1G>A
r.spl, r.spl?
p.?
-
likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive)
g.20766921C>T
g.20192782C>T
GJB2(NM_004004.6):c.-23+1G>A
-
GJB2_000011
12 heterozygous, no homozygous;
Clinindb (India)
, ACMG PVS1, PP5, PM2, BP4,
1 more item
PubMed: Baux 2017
,
Journal: Baux 2017
,
PubMed: Dalamon 2013
,
PubMed: Narang 2020
,
Journal: Narang 2020
,
3 more items
-
rs80338940
CLASSIFICATION record, Germline, Germline/De novo (untested)
-
1/476 cases, 12/2787 individuals
-
-
-
Johan den Dunnen
,
David Baux
,
Mieke Wesdorp
,
VKGL-NL_Rotterdam
,
VKGL-NL_Nijmegen
,
Viviana Karina Dalamón
,
Mohammed Faruq
-?/.
1
1i
c.-23+12G>A
r.(=)
p.(=)
-
likely benign
g.20766910C>T
g.20192771C>T
-
-
GJB2_000060
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/.
1
1
c.-22-12C>T
r.(=)
p.(=)
-
benign
g.20763754G>A
g.20189615G>A
-
-
GJB2_000059
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/.
2
1i
c.-22-6T>C
r.(=)
p.(=)
-
likely benign
g.20763748A>G
g.20189609A>G
GJB2(NM_004004.6):c.-22-6T>C
-
GJB2_000047
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Nijmegen
+?/.
3
1, 2
c.-22-2A>C
r.spl, r.spl?
p.0?, p.?
-
likely pathogenic, pathogenic (recessive)
g.20763744T>G
g.20189605T>G
GJB2(NM_004004.6):c.-22-2A>C
-
GJB2_000058
VKGL data sharing initiative Nederland
-
551912
rs201895089
CLASSIFICATION record, Germline
?
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Nijmegen
,
Viviana Karina Dalamón
-/.
1
-
c.-15C>T
r.(?)
p.(=)
-
benign
g.20763735G>A
g.20189596G>A
-
-
GJB2_000096
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/.
1
2
c.-6T>A
r.(?)
p.(=)
-
benign
g.20763726A>T
g.20189587A>T
-
-
GJB2_000057
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/.
1
-
c.-1G>A
r.(?)
p.(=)
-
likely benign
g.20763721C>T
-
GJB2(NM_004004.5):c.-1G>A (p.?)
-
GJB2_000131
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/., +?/.
3
2
c.1A>G
r.(?)
p.(Met1?), p.?
-
likely pathogenic, pathogenic
g.20763720T>C
g.20189581T>C
GJB2(NM_004004.6):c.1A>G (p.(Met1?))
-
GJB2_000031
5 heterozygous, no homozygous;
Clinindb (India)
, VKGL data sharing initiative Nederland
PubMed: Baux 2017
,
Journal: Baux 2017
,
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs111033293
CLASSIFICATION record, Germline, Germline/De novo (untested)
-
5/2793 individuals
-
-
-
David Baux
,
VKGL-NL_Leiden
,
Mohammed Faruq
-?/.
3
-
c.11G>A
r.(?)
p.(Gly4Asp)
-
likely benign
g.20763710C>T
g.20189571C>T
GJB2(NM_004004.6):c.11G>A (p.G4D)
-
GJB2_000046
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
+?/.
1
-
c.16C>G
r.(?)
p.(Leu6Val)
-
likely pathogenic
g.20763705G>C
g.20189566G>C
-
-
GJB2_000106
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/., ?/.
2
2
c.23C>T
r.(?)
p.(Thr8Met), p.(Trp8Met)
-
pathogenic (recessive), VUS
g.20763698G>A
g.20189559G>A
GJB2(NM_004004.5):c.23C>T (p.(Thr8Met))
-
GJB2_000084
VKGL data sharing initiative Nederland
PubMed: Dalamon 2013
-
-
CLASSIFICATION record, Germline
-
1/476 cases
-
-
-
VKGL-NL_Leiden
,
Viviana Karina Dalamón
-?/.
2
2
c.24G>A
r.(=), r.(?)
p.(=), p.(Thr8=)
-
likely benign
g.20763697C>T
g.20189558C>T
-
-
GJB2_000075
VKGL data sharing initiative Nederland
PubMed: Dalamon 2013
-
-
CLASSIFICATION record, Germline
-
1/74 cases
-
-
-
VKGL-NL_Nijmegen
,
Viviana Karina Dalamón
+/.
1
-
c.29T>C
r.(?)
p.(Leu10Pro)
-
pathogenic (dominant)
g.20763692A>G
g.20189553A>G
-
-
GJB2_000077
-
PubMed: Dalamon 2013
-
-
Germline
-
-
-
-
-
Viviana Karina Dalamón
+/.
1
-
c.31_68del
r.(?)
p.(Gly11Leufs*24)
ACMG
pathogenic
g.20763653_20763690del
g.20189514_20189551del
-
-
GJB2_000055
1 more item
-
-
rs397516873
Germline
-
-
-
-
-
Andreas Laner
+?/.
1
-
c.32G>A
r.(?)
p.(Gly11Glu)
ACMG
likely pathogenic
g.20763689C>T
g.20189550C>T
-
-
GJB2_000016
-
PubMed: Trujillano 2017
-
-
De novo
-
-
-
-
-
Daniel Trujillano
+/+, +/., +?/+, +?/., ./.
130
2
c.35del
r.(35del), r.(?), r.35del
p.(Gly12Valfs*2), p.(Gly12ValfsTer2), p.Gly12Valfs*2
ACMG
likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive)
g.20763686del, g.20763691del
g.20189552del
35delG, c.35delG, g.8429delG,
1 more item
-
GJB2_000001, GJB2_000007
ACMG PM2, PVS1, PP5, ACMG PVS1, PS3, PP3, PP5, ACMG PVS1, PS3, PP5, PP3, no variant 2nd chromosome,
2 more items
{CV:94392},
PubMed: Batissoco 2021
,
PubMed: Baux 2017
,
Journal: Baux 2017
,
PubMed: Boucher 2020
,
14 more items
ClinVar-32043
rs1801002
,
rs80338939
CLASSIFICATION record, Germline, Germline/De novo (untested), Unknown
?, yes
1/476 cases, 2/476 cases, 26/476 cases, 3/476 cases, 4/476 cases
-
-
-
Johan den Dunnen
,
David Baux
,
Atteeq Rehman
,
Mieke Wesdorp
,
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
Jan Traeger-Synodinos
,
VKGL-NL_Groningen
,
Viviana Karina Dalamón
,
Anaïs Begemann
,
MobiDetails
,
Karina Lezirovitz Mandelbaum
+/.
1
-
c.35delG
r.(?)
p.(Gly12Valfs*2)
-
pathogenic
g.20763691del
g.20189552del
GJB2 c.35delG, p.(Gly12Valfs*2)
-
GJB2_000001
-
PubMed: Niepokoj 2018
-
-
Germline
yes
-
-
-
-
LOVD
+/+
1
2
c.35dup
r.(?)
p.(Val13Cysfs*35)
-
pathogenic
g.20763691dup
g.20189552dup
35insG
-
GJB2_000005
-
{PMID:Estivill 1998:09482292:},
Journal: Estivill 1998
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
+?/.
3
2
c.35G>T
r.(?)
p.(Gly12Val)
-
likely pathogenic, pathogenic (recessive)
g.20763686C>A
g.20189547C>A
-
-
GJB2_000083
VKGL data sharing initiative Nederland
PubMed: Dalamon 2013
-
-
CLASSIFICATION record, Germline
-
1/476 cases
-
-
-
VKGL-NL_Nijmegen
,
Viviana Karina Dalamón
./.
1
-
c.38_39del
r.(?)
p.(Val13Glufs*34)
-
likely pathogenic
g.20763685_20763686del
g.20189546_20189547del
NM_004004.5(GJB2):c.38_39del p.(Val13Glufs*34)
-
GJB2_000017
variant could not be associated with disease phenotype
PubMed: Vogelaar 2017
,
Journal: Vogelaar 2017
-
-
Germline
-
-
-
-
-
Marjolijn JL Ligtenberg
?/.
1
-
c.44A>C
r.(?)
p.(Lys15Thr)
-
VUS
g.20763677T>G
g.20189538T>G
-
-
GJB2_000056
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/.
1
2
c.56G>C
r.(?)
p.(Ser19Thr)
-
pathogenic (recessive)
g.20763665C>G
g.20189526C>G
-
-
GJB2_000082
-
PubMed: Dalamon 2013
-
-
Germline
-
1/476 cases
-
-
-
Viviana Karina Dalamón
+/+, +/., +?/+
44
2
c.71G>A
r.(?)
p.(Trp24*), p.(Trp24Ter)
ACMG
likely pathogenic, pathogenic, pathogenic (recessive)
g.20763650C>T
g.20189511C>T
c.71G>A (p.Trp24*), GJB2(NM_004004.6):c.71G>A (p.W24*, p.(Trp24Ter))
-
GJB2_000003
32 heterozygous, no homozygous;
Clinindb (India)
, ACMG PVS1, PM1, PP3, PP5,
1 more item
PubMed: Baux 2017
,
Journal: Baux 2017
,
PubMed: Falk 2012
,
Journal: Falk 2012
,
PubMed: Hull-2016
,
6 more items
-
rs104894396
CLASSIFICATION record, Germline, Germline/De novo (untested)
yes
32/2795 individuals
-
-
-
Johan den Dunnen
,
David Baux
,
Atteeq Rehman
,
Mieke Wesdorp
,
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
Jan Traeger-Synodinos
,
Anju Shukla
,
Mohammed Faruq
-/., -?/.
13
2
c.79G>A
r.(?)
p.(Val27Ile)
-
benign, likely benign
g.20763642C>T
g.20189503C>T
g.8473G>A, GJB2(NM_004004.6):c.79G>A (p.V27I)
-
GJB2_000045
10 homozygous;
Clinindb (India)
, 121 heterozygous;
Clinindb (India)
,
2 more items
PubMed: Dalamon 2013
,
PubMed: Narang 2020
,
Journal: Narang 2020
36279
rs2274084
CLASSIFICATION record, Germline, Germline/De novo (untested)
?, no
0.04538, 10/2795 individuals, 121/2795 individuals, 29/125 cases non-syndromic deafness,
2 more items
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
,
Viviana Karina Dalamón
,
Mohammed Faruq
,
Guillermina García Sánchez
+?/.
1
2
c.79_82delinsAGA
r.(?)
p.(Val27Argfs*8)
ACMG
pathogenic
g.20763639_20763642delinsTCT
g.20189500_20189503delinsTCT
-
-
GJB2_000116
-
PubMed: Batissoco 2021
ClinVar-SCV001792212
-
Germline
?
-
-
-
-
Karina Lezirovitz Mandelbaum
-?/.
1
-
c.84C>T
r.(?)
p.(Leu28=)
-
likely benign
g.20763637G>A
g.20189498G>A
-
-
GJB2_000101
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/.
1
-
c.94C>G
r.(?)
p.(Arg32Gly)
-
likely pathogenic
g.20763627G>C
-
-
-
GJB2_000117
-
-
-
-
CLASSIFICATION record
-
-
-
-
-
MobiDetails
+/.
13
-
c.94C>T
r.(?)
p.(Arg32Cys)
ACMG
pathogenic (recessive)
g.20763627G>A
g.20189488G>A
-
-
GJB2_000126
-
PubMed: Dia 2022
-
-
Germline
yes
2/148 controls
-
-
-
Yacouba Dia
+/., +?/.
3
2
c.95G>A
r,(?), r.(?)
p.(Arg32His)
-
likely pathogenic, pathogenic, pathogenic (recessive)
g.20763626C>T
g.20189487C>T
-
-
GJB2_000032
3 heterozygous, no homozygous;
Clinindb (India)
, combination of alleles not reported
PubMed: Baux 2017
,
Journal: Baux 2017
,
PubMed: Narang 2020
,
Journal: Narang 2020
,
PubMed: Wu 2019
-
rs111033190
Germline
yes
1/1291 cases hearing loss, 3/2795 individuals
-
-
-
Johan den Dunnen
,
David Baux
,
Mohammed Faruq
+/., +?/.
21
2
c.101T>C
r.(?)
p.(Met34Thr)
-
likely pathogenic, pathogenic, pathogenic (recessive)
g.20763620A>G
g.20189481A>G
GJB2(NM_004004.6):c.101T>C (p.M34T, p.(Met34Thr))
-
GJB2_000012
VKGL data sharing initiative Nederland
PubMed: Baux 2017
,
Journal: Baux 2017
,
PubMed: Boucher 2020
,
PubMed: Dalamon 2013
,
PubMed: Papuc 2019
,
2 more items
-
rs35887622
CLASSIFICATION record, Germline, Germline/De novo (untested)
yes
1/476 cases, 2/476 cases
-
-
-
Johan den Dunnen
,
David Baux
,
Mieke Wesdorp
,
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
,
Viviana Karina Dalamón
,
Anaïs Begemann
,
MobiDetails
+?/.
1
-
c.102G>A
r.(?)
p.(Met34Ile)
-
likely pathogenic
g.20763619C>T
g.20189480C>T
-
-
GJB2_000095
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/., ?/.
2
-
c.107T>C
r,(?), r.(?)
p.(Leu36Pro)
-
pathogenic (recessive), VUS
g.20763614A>G
g.20189475A>G
-
-
GJB2_000104
4 heterozygous, no homozygous;
Clinindb (India)
, combination of alleles not reported
PubMed: Narang 2020
,
Journal: Narang 2020
,
PubMed: Wu 2019
-
rs587783644
Germline
-
1/1291 cases hearing loss, 4/2795 individuals
-
-
-
Johan den Dunnen
,
Mohammed Faruq
+/., +?/., -?/.
29
2
c.109G>A
r,(?), r.(?)
p.(Val37Ile)
-
likely benign, likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive)
g.20763612C>T
g.20189473C>T
GJB2(NM_004004.6):c.109G>A (p.V37I, p.(Val37Ile)), V37I
-
GJB2_000013
1 heterozygous, no homozygous;
Clinindb (India)
, combination of alleles not reported,
2 more items
PubMed: Baux 2017
,
Journal: Baux 2017
,
PubMed: Dalamon 2013
,
PubMed: del Castillo 2005
,
9 more items
-
rs72474224
CLASSIFICATION record, Germline, Germline/De novo (untested)
yes
1/2792 individuals, 1/476 cases, 1608/1291 cases hearing loss, frequency 0.096
-
-
-
Global Variome, with Curator vacancy
,
Johan den Dunnen
,
David Baux
,
Mieke Wesdorp
,
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
,
VKGL-NL_VUmc
,
Viviana Karina Dalamón
,
Mohammed Faruq
+?/.
1
2
c.119C>A
r.(?)
p.(Ala40Glu)
-
likely pathogenic
g.20763602G>T
g.20189463G>T
-
-
GJB2_000027
-
PubMed: Baux 2017
,
Journal: Baux 2017
-
rs111033296
Germline/De novo (untested)
-
-
-
-
-
David Baux
+?/.
1
-
c.119C>G
r.(?)
p.(Ala40Gly)
-
likely pathogenic
g.20763602G>C
g.20189463G>C
-
-
GJB2_000105
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
-
c.126G>T
r.(?)
p.(Glu42Asp)
-
VUS
g.20763595C>A
g.20189456C>A
-
-
GJB2_000094
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
2
c.128T>G
r.(?)
p.(Val43Gly)
-
VUS
g.20763593A>C
g.20189454A>C
-
-
GJB2_000009
-
PubMed: Sommen 2016
,
Journal: Sommen 2016
-
-
Germline
-
-
-
-
-
Manou Sommen
+/.
3
2
c.132G>A
r.(?)
p.(p.(Trp44Ter), p.(Trp44Ter)
ACMG
pathogenic, pathogenic (recessive)
g.20763589C>T
g.20189450C>T
-
-
GJB2_000093
VKGL data sharing initiative Nederland
PubMed: Dia 2022
-
-
CLASSIFICATION record, Germline
yes
-
-
-
-
VKGL-NL_Nijmegen
,
Yacouba Dia
?/.
1
-
c.136G>T
r.(?)
p.(Asp46Tyr)
-
VUS
g.20763585C>A
-
GJB2(NM_004004.6):c.136G>T (p.D46Y)
-
GJB2_000115
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/., +?/.
9
2
c.139G>T
r.(?)
p.(Glu47*), p.(Glu47Ter)
-
pathogenic, pathogenic (recessive)
g.20763582C>A
g.20189443C>A
GJB2(NM_004004.6):c.139G>T (p.E47*)
-
GJB2_000028
VKGL data sharing initiative Nederland
PubMed: Baux 2017
,
Journal: Baux 2017
,
PubMed: Dalamon 2013
-
rs104894398
CLASSIFICATION record, Germline, Germline/De novo (untested)
-
1/476 cases, 2/476 cases
-
-
-
David Baux
,
VKGL-NL_Rotterdam
,
VKGL-NL_VUmc
,
Viviana Karina Dalamón
+/.
3
-
c.148G>A
r.(?)
p.(Asp50Asn)
ACMG
pathogenic, pathogenic (dominant)
g.20763573C>T
g.20189434C>T
-
-
GJB2_000054
0/60 healthy controls from Cameroon, VKGL data sharing initiative Nederland
PubMed: Wonkam 2013
-
-
CLASSIFICATION record, Germline/De novo (untested)
?
-
-
-
-
VKGL-NL_Nijmegen
,
Yacouba Dia
-?/.
1
-
c.156C>T
r.(?)
p.(Val52=)
-
likely benign
g.20763565G>A
g.20189426G>A
-
-
GJB2_000100
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/+, +?/.
13
2
c.167del
r.(?)
p.(Leu56Argfs*26)
-
pathogenic, pathogenic (recessive)
g.20763554del
g.20189415del
167delT
-
GJB2_000006
-
PubMed: Dalamon 2013
,
PubMed: Lerer 2001
,
Journal: Lerer 2001
-
-
Germline
yes
1/476 cases, 2/476 cases, 4/476 cases
-
-
-
Johan den Dunnen
,
Viviana Karina Dalamón
+/.
2
2
c.169C>T
r.(?)
p.(Gln57*)
-
pathogenic
g.20763552G>A
g.20189413G>A
-
-
GJB2_000029
-
PubMed: Baux 2017
,
Journal: Baux 2017
-
rs111033297
Germline, Germline/De novo (untested)
yes
-
-
-
-
David Baux
+/.
2
-
c.176_191del
r,(?), r.(?)
p.(Gly59AlafsTer18)
-
pathogenic, pathogenic (recessive)
g.20763531_20763546del
g.20189392_20189407del
-
-
GJB2_000108
combination of alleles not reported, VKGL data sharing initiative Nederland
PubMed: Wu 2019
-
-
CLASSIFICATION record, Germline
-
2/1291 cases hearing loss
-
-
-
Johan den Dunnen
,
VKGL-NL_Nijmegen
?/.
1
-
c.179G>T
r.(?)
p.(Cys60Phe)
-
VUS
g.20763542C>A
-
-
-
GJB2_000132
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
-
c.200A>G
r.(?)
p.(His67Arg)
-
VUS
g.20763521T>C
-
GJB2(NM_004004.6):c.200A>G (p.H67R)
-
GJB2_000125
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-?/.
1
-
c.213C>T
r.(?)
p.(Ile71=)
-
likely benign
g.20763508G>A
g.20189369G>A
-
-
GJB2_000099
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
-
c.217C>A
r.(?)
p.(His73Asn)
-
VUS
g.20763504G>T
-
GJB2(NM_004004.6):c.217C>A (p.H73N)
-
GJB2_000119
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
3
2
c.223C>T
r.(?)
p.(Arg75Trp)
-
pathogenic, pathogenic (dominant), pathogenic (recessive)
g.20763498G>A
g.20189359G>A
-
-
GJB2_000053
VKGL data sharing initiative Nederland
PubMed: Dalamon 2013
,
PubMed: Richard 2019
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Nijmegen
,
Viviana Karina Dalamón
+/.
6
2
c.224G>A
r.(?)
p.(Arg75Gln)
-
pathogenic, pathogenic (dominant), pathogenic (recessive)
g.20763497C>T
g.20189358C>T
-
-
GJB2_000078
2 heterozygous, no homozygous;
Clinindb (India)
, VKGL data sharing initiative Nederland
PubMed: Dalamon 2013
,
PubMed: Narang 2020
,
Journal: Narang 2020
,
PubMed: Richard 2019
,
PubMed: Wu 2013
-
rs28931593
CLASSIFICATION record, Germline
yes
2/2795 individuals
-
-
-
Johan den Dunnen
,
VKGL-NL_Nijmegen
,
Viviana Karina Dalamón
,
Mohammed Faruq
+/., +?/.
3
2
c.229T>C
r.(?)
p.(Trp77Arg)
-
pathogenic, pathogenic (recessive)
g.20763492A>G
g.20189353A>G
GJB2(NM_004004.6):c.229T>C (p.W77R)
-
GJB2_000044
VKGL data sharing initiative Nederland
PubMed: Dalamon 2013
-
-
CLASSIFICATION record, Germline
-
1/476 cases
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Nijmegen
,
Viviana Karina Dalamón
+/., ?/+
27
2
c.231G>A
r.(?)
p.(Trp77*), p.(Trp77Ter)
-
pathogenic, pathogenic (recessive), VUS
g.20763490C>T
g.20189351C>T
GJB2(NM_004004.6):c.231G>A (p.W77*)
-
GJB2_000004
7 heterozygous, no homozygous;
Clinindb (India)
, VKGL data sharing initiative Nederland
PubMed: Kelsell 1997
,
PubMed: Naz 2017
,
PubMed: Narang 2020
,
Journal: Narang 2020
,
PubMed: Shaikh 2017
,
1 more item
-
rs80338944
CLASSIFICATION record, Germline
yes
7/2795 individuals
-
-
-
Johan den Dunnen
,
Atteeq Rehman
,
VKGL-NL_Rotterdam
,
VKGL-NL_Nijmegen
,
Mohammed Faruq
+/.
1
2
c.232dup
r.(?)
p.(Ala78Glyfs*24)
-
pathogenic (recessive)
g.20763491dup
g.20189352dup
-
-
GJB2_000074
-
PubMed: Dalamon 2013
553568
-
Germline
yes
-
-
-
-
Viviana Karina Dalamón
+/.
1
2
c.232G>T
r.(?)
p.(Ala78Ser)
-
pathogenic (recessive)
g.20763489C>A
g.20189350C>A
-
-
GJB2_000071
-
PubMed: Dalamon 2013
-
-
Germline
yes
singleton
-
-
-
Viviana Karina Dalamón
+/., +?/.
15
11, 9
c.235del
r,(?), r.(?)
p.(Leu79Cysfs*3), p.(Leu79CysfsTer3)
ACMG
likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive)
g.20763486del, g.20763488del
g.20189349del
233delC, 235delC, c.235delC,
1 more item
-
GJB2_000052
combination of alleles not reported, rs80338943, VKGL data sharing initiative Nederland
PubMed: Choi 2013
,
PubMed: Sang 2019
,
PubMed: Shaikh 2017
,
PubMed: Wu 2019
,
PubMed: Xie 2022
,
1 more item
-
-
CLASSIFICATION record, Germline
-
215/1291 cases hearing loss
-
-
-
Johan den Dunnen
,
VKGL-NL_Leiden
,
VKGL-NL_Groningen
+/.
1
-
c.238C>A
r.(?)
p.(Gln80Lys)
-
pathogenic
g.20763483G>T
g.20189344G>T
-
-
GJB2_000051
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/.
1
-
c.239A>C
r.(?)
p.(Gln80Pro)
-
likely pathogenic
g.20763482T>G
-
-
-
GJB2_000110
-
-
-
rs727504302
CLASSIFICATION record
-
-
-
-
-
MobiDetails
+?/.
1
-
c.241C>G
r.(?)
p.(Leu81Val)
-
likely pathogenic
g.20763480G>C
-
-
-
GJB2_000109
-
-
-
rs145216882
CLASSIFICATION record
-
-
-
-
-
MobiDetails
+?/.
2
2
c.246C>G
r.(?)
p.(Ile82Met)
-
likely pathogenic, pathogenic (recessive)
g.20763475G>C
g.20189336G>C
-
-
GJB2_000081
VKGL data sharing initiative Nederland
PubMed: Dalamon 2013
-
-
CLASSIFICATION record, Germline
-
1/476 cases
-
-
-
VKGL-NL_Nijmegen
,
Viviana Karina Dalamón
-?/.
3
2
c.249C>G
r.(?)
p.(Phe83Leu)
-
likely benign
g.20763472G>C
g.20189333G>C
GJB2(NM_004004.6):c.249C>G (p.F83L)
-
GJB2_000043
VKGL data sharing initiative Nederland
PubMed: Dalamon 2013
-
-
CLASSIFICATION record, Germline
-
1/74 cases
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Nijmegen
,
Viviana Karina Dalamón
+/., +?/.
3
2
c.250G>C
r.(?)
p.(Val84Leu)
-
likely pathogenic, pathogenic, pathogenic (recessive)
g.20763471C>G
g.20189332C>G
-
-
GJB2_000014
VKGL data sharing initiative Nederland
PubMed: Dalamon 2013
,
PubMed: Zazo Seco 2017
,
Journal: Zazo Seco 2017
-
-
CLASSIFICATION record, Germline
-
1/476 cases
-
-
-
Mieke Wesdorp
,
VKGL-NL_Nijmegen
,
Viviana Karina Dalamón
?/.
1
-
c.250G>T
r.(?)
p.(Val84Leu)
-
VUS
g.20763471C>A
-
-
-
GJB2_000135
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
-
c.257C>T
r.(?)
p.(Thr86Met)
-
VUS
g.20763464G>A
-
-
-
GJB2_000127
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
-
c.263C>T
r,(?)
p.(Ala88Val)
-
pathogenic (dominant)
g.20763458G>A
g.20189319G>A
-
-
GJB2_000134
combination of alleles not reported
PubMed: Wu 2019
-
-
Germline
-
1/1291 cases hearing loss
-
-
-
Johan den Dunnen
+?/.
1
2
c.269dup
r.(?)
p.(Val91Serfs*11)
-
pathogenic (recessive)
g.20763452dup
g.20189313dup
269dupT
-
GJB2_000080
-
PubMed: Dalamon 2013
-
-
Germline
-
1/476 cases
-
-
-
Viviana Karina Dalamón
+/., +?/.
9
2
c.269T>C
r.(?)
p.(Leu90Pro)
-
likely pathogenic, pathogenic, pathogenic (recessive)
g.20763452A>G
g.20189313A>G
GJB2(NM_004004.6):c.269T>C (p.L90P, p.(Leu90Pro))
-
GJB2_000015
VKGL data sharing initiative Nederland
PubMed: Baux 2017
,
Journal: Baux 2017
,
PubMed: Dalamon 2013
,
1 more item
-
rs80338945
CLASSIFICATION record, Germline, Germline/De novo (untested)
yes
1/476 cases
-
-
-
David Baux
,
Mieke Wesdorp
,
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
,
Viviana Karina Dalamón
?/.
1
-
c.280C>G
r.(?)
p.(His94Asp)
-
VUS
g.20763441G>C
-
-
-
GJB2_000124
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/.
3
2
c.283G>A
r.(?)
p.(Val95Met)
-
likely pathogenic, pathogenic (recessive)
g.20763438C>T
g.20189299C>T
-
-
GJB2_000042
VKGL data sharing initiative Nederland
PubMed: Dalamon 2013
-
-
CLASSIFICATION record, Germline
-
1/476 cases
-
-
-
VKGL-NL_Nijmegen
,
Viviana Karina Dalamón
+/.
1
2
c.290dup
r.(?)
p.(Tyr97*)
-
pathogenic
g.20763431dup
g.20189292dup
290dupA
-
GJB2_000030
-
PubMed: Baux 2017
,
Journal: Baux 2017
-
rs786204491
Germline
yes
-
-
-
-
David Baux
+?/.
1
-
c.299A>C
r.(?)
p.(His100Pro)
-
likely pathogenic
g.20763422T>G
g.20189283T>G
-
-
GJB2_000050
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/., +?/.
5
-
c.299_300del
r,(?), r.(?)
p.(His100Argfs*14), p.(His100ArgfsTer14)
-
likely pathogenic (recessive), pathogenic, pathogenic (recessive)
g.20763421_20763422del
g.20189282_20189283del
299_300delAT, c.299_300del
-
GJB2_000107
combination of alleles not reported, rs111033204, VKGL data sharing initiative Nederland
PubMed: Choi 2013
,
PubMed: Wu 2019
-
-
CLASSIFICATION record, Germline
?
31/1291 cases hearing loss
-
-
-
Johan den Dunnen
,
VKGL-NL_Nijmegen
,
Seungmin Lee
+/.
1
-
c.310_323del
r.(?)
p.(Arg104Glyfs*6)
-
pathogenic (recessive)
g.20763398_20763411del
g.20189259_20189272del
310del14 (Lys105Argfs*2)
-
GJB2_000113
-
PubMed: Shaikh 2017
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/.
6
2
c.313_326del
r.(?)
p.(Lys105Glyfs*5), p.(Lys105GlyfsTer5)
-
pathogenic, pathogenic (recessive)
g.20763397_20763410del
g.20189258_20189271del
313_326del14, GJB2(NM_004004.6):c.313_326delAAGTTCATCAAGGG (p.K105Gfs*5)
-
GJB2_000026
2 heterozygous, no homozygous;
Clinindb (India)
, VKGL data sharing initiative Nederland
PubMed: Baux 2017
,
Journal: Baux 2017
,
PubMed: Dalamon 2013
,
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs111033253
CLASSIFICATION record, Germline
yes
1/476 cases, 2/2794 individuals
-
-
-
David Baux
,
VKGL-NL_Rotterdam
,
VKGL-NL_Nijmegen
,
Viviana Karina Dalamón
,
Mohammed Faruq
+/.
2
2
c.334_335del
r.(?)
p.(Lys112Glufs*2)
-
pathogenic
g.20763387_20763388del
g.20189248_20189249del
334_335delAA
-
GJB2_000019
-
PubMed: Baux 2017
,
Journal: Baux 2017
-
rs756484720
Germline, Germline/De novo (untested)
yes
-
-
-
-
David Baux
-/.
3
-
c.341A>G
r.(?)
p.(Glu114Gly)
-
benign
g.20763380T>C
g.20189241T>C
GJB2(NM_004004.6):c.341A>G (p.E114G)
-
GJB2_000041
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
+/.
1
2
c.353_356dup
r.(?)
p.(Glu119Aspfs*92)
-
pathogenic
g.20763366_20763369dup
g.20189227_20189230dup
-
-
GJB2_000018
-
PubMed: Baux 2017
,
Journal: Baux 2017
-
-
Germline
yes
-
-
-
-
David Baux
?/.
2
-
c.355G>A
r.(?)
p.(Glu119Lys)
-
VUS
g.20763366C>T
-
GJB2(NM_004004.6):c.355G>A (p.E119K)
-
GJB2_000040
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
+/.
1
-
c.355G>T
r.(?)
p.(Glu119Ter)
ACMG
pathogenic (recessive)
g.20763366C>A
g.20189227C>A
-
-
GJB2_000128
-
PubMed: Richard 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., +?/.
8
2
c.358_360del
r.(?)
p.(Glu120del)
-
likely pathogenic, pathogenic, pathogenic (recessive)
g.20763364_20763366del
g.20189225_20189227del
358_360delGAG,
1 more item
-
GJB2_000020
VKGL data sharing initiative Nederland
PubMed: Baux 2017
,
Journal: Baux 2017
,
PubMed: Dalamon 2013
,
PubMed: Marlin 2005
,
PubMed: Naz 2017
-
rs80338947
CLASSIFICATION record, Germline, Germline/De novo (untested)
yes
1/476 cases
-
-
-
Johan den Dunnen
,
David Baux
,
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
VKGL-NL_Nijmegen
,
Viviana Karina Dalamón
+/.
1
-
c.370C>T
r.(?)
p.(Gln124*)
-
pathogenic
g.20763351G>A
g.20189212G>A
-
-
GJB2_000103
3 heterozygous, no homozygous;
Clinindb (India)
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs397516874
Germline
-
3/2795 individuals
-
-
-
Mohammed Faruq
-/., -?/., ?/.
4
2
c.380G>A
r.(?)
p.(Arg127His)
-
benign, likely benign, VUS
g.20763341C>T
g.20189202C>T
GJB2(NM_004004.6):c.380G>A (p.R127H)
-
GJB2_000002
VKGL data sharing initiative Nederland
PubMed: Dalamon 2013
-
-
CLASSIFICATION record, Germline
-
1/74 cases
-
-
-
Yu Sun
,
VKGL-NL_Rotterdam
,
VKGL-NL_Nijmegen
,
Viviana Karina Dalamón
?/.
1
-
c.382A>G
r.(?)
p.(Ile128Val)
-
VUS
g.20763339T>C
-
GJB2(NM_004004.5):c.382A>G (p.(Ile128Val))
-
GJB2_000130
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
1
2
c.384C>T
r.(=)
p.(=)
-
likely benign
g.20763337G>A
g.20189198G>A
-
-
GJB2_000076
-
PubMed: Dalamon 2013
-
-
Germline
-
1/74 cases
-
-
-
Viviana Karina Dalamón
?/.
1
-
c.389G>C
r.(?)
p.(Gly130Ala)
-
VUS
g.20763332C>G
-
-
-
GJB2_000123
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
-
c.389G>T
r.(?)
p.(Gly130Val)
-
VUS
g.20763332C>A
g.20189193C>A
-
-
GJB2_000070
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/., ?/.
3
2
c.400T>C
r.(?)
p.(Trp134Arg)
-
pathogenic, VUS
g.20763321A>G
g.20189182A>G
-
-
GJB2_000008
VKGL data sharing initiative Nederland
PubMed: Behar 2014
,
Journal: Behar 2014
-
-
CLASSIFICATION record, Germline
yes
-
-
-
-
Johan den Dunnen
,
Zippi Brownstein
,
VKGL-NL_Nijmegen
+?/., ?/.
4
-
c.416G>A
r.(?)
p.(Ser139Asn)
-
likely pathogenic, VUS
g.20763305C>T
g.20189166C>T
GJB2(NM_004004.5):c.416G>A (p.(Ser139Asn)), GJB2(NM_004004.6):c.416G>A (p.S139N)
-
GJB2_000039
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
?/.
1
-
c.425T>C
r.(?)
p.(Phe142Ser)
-
VUS
g.20763296A>G
g.20189157A>G
-
-
GJB2_000092
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/., +?/.
15
2
c.427C>T
r,(?), r.(?)
p.(Arg143Trp)
ACMG
pathogenic, pathogenic (recessive)
g.20189155G>A, g.20763294G>A
g.20189155G>A
GJB2(NM_004004.6):c.427C>T (p.R143W)
-
GJB2_000038
combination of alleles not reported, VKGL data sharing initiative Nederland
PubMed: Dalamon 2013
,
PubMed: Dia 2022
,
PubMed: Wu 2019
17009
-
CLASSIFICATION record, Germline
yes
1/476 cases, 2/476 cases, 4/148 controls, 6/1291 cases hearing loss
-
-
-
Johan den Dunnen
,
VKGL-NL_Rotterdam
,
VKGL-NL_Nijmegen
,
Viviana Karina Dalamón
,
Yacouba Dia
+/.
2
-
c.428G>A
r,(?), r.(?)
p.(Arg143Gln)
ACMG
pathogenic (dominant), pathogenic (recessive)
g.20763293C>T
g.20189154C>T
-
-
GJB2_000091
combination of alleles not reported
PubMed: Sang 2019
,
PubMed: Wu 2019
-
-
Germline
-
1/1291 cases hearing loss
-
-
-
Johan den Dunnen
+?/.
1
2
c.428G>T
r.(?)
p.(Arg143Leu)
-
likely pathogenic
g.20763293C>A
g.20189154C>A
-
-
GJB2_000025
-
PubMed: Baux 2017
,
Journal: Baux 2017
-
rs104894401
Germline
yes
-
-
-
-
David Baux
+/., +?/.
3
2
c.439G>A
r.(?)
p.(Glu147Lys)
ACMG
likely pathogenic, pathogenic (recessive)
g.20763282C>T
g.20189143C>T
-
-
GJB2_000024
VKGL data sharing initiative Nederland
PubMed: Baux 2017
,
Journal: Baux 2017
,
PubMed: Sang 2019
-
rs1801002
CLASSIFICATION record, Germline
yes
-
-
-
-
Johan den Dunnen
,
David Baux
,
VKGL-NL_Nijmegen
+/.
1
-
c.449del
r.(?)
p.(Phe150Serfs*18)
-
pathogenic
g.20763273del
-
-
-
GJB2_000122
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
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