Global Variome shared LOVD
GJB3 (gap junction protein, beta 3, 31kDa)
LOVD v.3.0 Build 30b [
Current LOVD status
]
Register as submitter
|
Log in
Curator:
Michel van Geel
View all genes
View GJB3 gene homepage
View graphs about the GJB3 gene database
Create a new gene entry
View all transcripts
View all transcripts of gene GJB3
Create a new transcript information entry
View all variants
View all variants affecting transcripts
View unique variants in gene GJB3
View all variants in gene GJB3
Full data view for gene GJB3
Create a new data submission
View active genomic custom columns
Enable more genomic custom columns
View all individuals
View all individuals with variants in gene GJB3
Create a new data submission
View active custom columns
Enable more custom columns
View all diseases
View all diseases associated with gene GJB3
Create a new disease information entry
View available phenotype columns
View all screenings
View all screenings for gene GJB3
Create a new data submission
View active custom columns
Enable more custom columns
Submit new data
Unique variants in the GJB3 gene
The variants shown are described using the NM_024009.2 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
All list views have search fields which can be used to search data. You can search for a complete word or you can search for a part of a search term. If you enclose two or more words in double quotes, LOVD will search for the combination of those words only exactly in the order you specify. Note that search terms are case-insensitive and that wildcards such as * are treated as normal text! For all options, like "and", "or", and "not" searches, or searching for prefixes or suffixes, see the table below.
Operator
Column type
Example
Matches
Text
Arg
all entries containing 'Arg'
space
Text
Arg Ser
all entries containing 'Arg' and 'Ser'
|
Text
Arg|Ser
all entries containing 'Arg' or 'Ser'
!
Text
!fs
all entries not containing 'fs'
^
Text
^p.(Arg
all entries beginning with 'p.(Arg'
$
Text
Ser)$
all entries ending with 'Ser)'
=""
Text
=""
all entries with this field empty
=""
Text
="p.0"
all entries exactly matching 'p.0'
!=""
Text
!=""
all entries with this field not empty
!=""
Text
!="p.0"
all entries not exactly matching 'p.0?'
combination
Text
*|Ter !fs
all entries containing '*' or 'Ter' but not containing 'fs'
Date
2020
all entries matching the year 2020
|
Date
2020-03|2020-04
all entries matching March or April, 2020
!
Date
!2020-03
all entries not matching March, 2020
<
Date
<2020
all entries before the year 2020
<=
Date
<=2020-06
all entries in or before June, 2020
>
Date
>2020-06
all entries after June, 2020
>=
Date
>=2020-06-15
all entries on or after June 15th, 2020
combination
Date
2019|2020 <2020-03
all entries in 2019 or 2020, and before March, 2020
Numeric
23
all entries exactly matching 23
|
Numeric
23|24
all entries exactly matching 23 or 24
!
Numeric
!23
all entries not exactly matching 23
<
Numeric
<23
all entries lower than 23
<=
Numeric
<=23
all entries lower than, or equal to, 23
>
Numeric
>23
all entries higher than 23
>=
Numeric
>=23
all entries higher than, or equal to, 23
combination
Numeric
>=20 <30 !23
all entries with values from 20 to 29, but not equal to 23
Some more advanced examples:
Example
Matches
Asian
all entries containing 'Asian', 'asian', including 'Caucasian', 'caucasian', etc.
Asian !Caucasian
all entries containing 'Asian' but not containing 'Caucasian'
Asian|African !Caucasian
all entries containing 'Asian' or 'African', but not containing 'Caucasian'
"South Asian"
all entries containing 'South Asian', but not containing 'South East Asian'
To sort on a certain column, click on the column header or on the arrows. If that column is already selected to sort on, the sort order will be swapped. The column currently sorted on has a darker blue background color than the other columns. The up and down arrows next to the column name indicate the current sorting direction. When sorting on any field other than the default, LOVD will sort secondarily on the default sort column.
66 entries on 1 page. Showing entries 1 - 66.
10 per page
25 per page
50 per page
100 per page
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
-/-
2
1i
c.-25-477_-25-474del
r.(?)
p.(=)
-
benign
g.35249862_35249865del
g.34784261_34784264del
632del4
-
GJB3_000015
-
PubMed: Lopez-Bigas 2000
-
-
Unknown
?
14/103 controls, 15/110 cases
-
-
-
Johan den Dunnen
-/-
2
1i
c.-25-427_-25-426del
r.(=)
p.(=)
-
benign
g.35249912_35249913del
g.34784311_34784312del
581del2
-
GJB3_000014
-
PubMed: Lopez-Bigas 2000
-
-
Unknown
?
30/103 controls, 33/110 cases
-
-
-
Johan den Dunnen
?/.
1
-
c.-25G>C
r.(?)
p.(=)
-
VUS
g.35250339G>C
-
GJB3(NM_024009.2):c.-25G>C (p.?)
-
GJB3_000063
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
1
-
c.33C>T
r.(?)
p.(=)
-
likely benign
g.35250396C>T
-
GJB3(NM_024009.3):c.33C>T (p.S11=)
-
GJB3_000057
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
2
2
c.34G>A
r.(?)
p.(Gly12Ser)
-
pathogenic
g.35250397G>A
g.34784796G>A
G12S
-
GJB3_000022
VKGL data sharing initiative Nederland
PubMed: Richard 2001
-
-
CLASSIFICATION record, Unknown
?
-
-
-
-
Michel van Geel
,
VKGL-NL_Nijmegen
+/.
1
2
c.34G>C
r.(?)
p.(Gly12Arg)
-
pathogenic
g.35250397G>C
g.34784796G>C
-
-
GJB3_000003
-
PubMed: Richard 1998
,
OMIM:var0001
-
-
Unknown
?
-
-
-
-
Michel van Geel
+/.
1
2
c.35G>A
r.(?)
p.(Gly12Asp)
-
pathogenic
g.35250398G>A
g.34784797G>A
-
-
GJB3_000004
-
PubMed: Richard 1998
,
OMIM:var0002
-
-
Unknown
?
-
-
-
-
Michel van Geel
?/.
1
-
c.48C>A
r.(?)
p.(Tyr16Ter)
-
VUS
g.35250411C>A
g.34784810C>A
-
-
GJB3_000045
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
-
c.64C>T
r.(?)
p.(Arg22Cys)
-
VUS
g.35250427C>T
-
GJB3(NM_024009.3):c.64C>T (p.(Arg22Cys))
-
GJB3_000065
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
-
c.69C>G
r.(?)
p.(Ile23Met)
-
VUS
g.35250432C>G
-
GJB3(NM_024009.3):c.69C>G (p.(Ile23Met))
-
GJB3_000066
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
-
c.70T>C
r.(?)
p.(Trp24Arg)
-
VUS
g.35250433T>C
-
-
-
GJB3_000056
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
2
c.76T>A
r.(?)
p.(ser26Thr)
-
pathogenic
g.35250439T>A
g.34784838T>A
S26T
-
GJB3_000024
-
PubMed: Richard 2001
-
-
Unknown
?
-
-
-
-
Michel van Geel
?/.
1
-
c.79G>A
r.(?)
p.(Val27Met)
-
VUS
g.35250442G>A
-
GJB3(NM_024009.3):c.79G>A (p.V27M)
-
GJB3_000064
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-/-, -/., -?/.
7
2
c.94C>T
r.(?)
p.(Arg32Trp)
-
benign, likely benign
g.35250457C>T
g.34784856C>T
GJB3(NM_024009.3):c.94C>T (p.R32W)
-
GJB3_000011
VKGL data sharing initiative Nederland
PubMed: Eisenberger 2018
,
PubMed: Kelsell 2000
,
PubMed: Lopez-Bigas 2000
-
rs1805063
CLASSIFICATION record, Germline, Unknown
?
2/110 controls, 7/153 cases, 8/46 controls
-
-
-
Johan den Dunnen
,
VKGL-NL_Groningen
,
VKGL-NL_AMC
+/.
2
2
c.101T>C
r.(?)
p.(Leu34Pro)
-
pathogenic
g.35250464T>C
g.34784863T>C
-
-
GJB3_000026
-
PubMed: Gottfried 2002
-
rs28937583
Unknown
?
-
-
-
-
Michel van Geel
?/.
1
2
c.124C>T
r.(?)
p.(Arg42Cys)
ACMG
VUS
g.35250487C>T
-
-
-
GJB3_000052
inherited from unaffected mother
PubMed: Sampaio-Silva 2018
-
rs976148533
Germline
no
-
-
-
-
Karina Lezirovitz Mandelbaum
+/.
3
2
c.125G>C
r.(?)
p.(Arg42Pro)
-
pathogenic
g.35250488G>C
g.34784887G>C
-
-
GJB3_000006
-
PubMed: Richard 1998
,
OMIM:var0008
,
PubMed: Richard 2000
,
PubMed: Wilgoss 1999
,
OMIM:var0008
-
-
Unknown
?
-
-
-
-
Michel van Geel
+/.
1
2
c.134G>A
r.(?)
p.(Gly45Glu)
-
pathogenic
g.35250497G>A
g.34784896G>A
G45E
-
GJB3_000023
-
PubMed: Richard 2001
-
-
Unknown
?
-
-
-
-
Michel van Geel
-?/.
1
-
c.186C>T
r.(?)
p.(=)
-
likely benign
g.35250549C>T
-
GJB3(NM_024009.3):c.186C>T (p.N62=)
-
GJB3_000059
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/.
1
-
c.187G>A
r.(?)
p.(Val63Ile)
-
likely benign
g.35250550G>A
g.34784949G>A
GJB3(NM_024009.2):c.187G>A (p.V63I)
-
GJB3_000047
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/., ?/.
2
2
c.196_198del
r.(?)
p.(Asp66del)
-
pathogenic, VUS
g.35250559_35250561del
g.34784958_34784960del
GJB3(NM_024009.3):c.196_198del (p.(Asp66del))
-
GJB3_000021
VKGL data sharing initiative Nederland
PubMed: Lopez-Bigas 2001
,
OMIM:var0009
-
-
CLASSIFICATION record, Germline
yes
-
-
-
-
Michel van Geel
,
VKGL-NL_Leiden
?/.
1
-
c.197A>G
r.(?)
p.(Asp66Gly)
-
VUS
g.35250560A>G
-
GJB3(NM_024009.3):c.197A>G (p.D66G)
-
GJB3_000053
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+?/., ?/.
2
-
c.250G>A
r.(?)
p.(Val84Ile)
-
likely pathogenic (dominant), VUS
g.35250613G>A
g.34785012G>A
c.G250A, GJB3(NM_024009.3):c.250G>A (p.(Val84Ile))
-
GJB3_000036
rs145751680, VKGL data sharing initiative Nederland
PubMed: Choi 2013
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Leiden
+/.
1
2
c.256T>A
r.(?)
p.(Cys86Ser)
-
pathogenic
g.35250619T>A
g.34785018T>A
-
-
GJB3_000005
-
PubMed: Richard 1998
,
OMIM:var0003
-
-
Unknown
?
-
-
-
-
Michel van Geel
-?/.
2
-
c.264G>A
r.(?)
p.(Ser88=)
-
likely benign
g.35250627G>A
g.34785026G>A
GJB3(NM_024009.3):c.264G>A (p.S88=)
-
GJB3_000048
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
,
VKGL-NL_AMC
?/.
1
-
c.268C>A
r.(?)
p.(Leu90Met)
-
VUS
g.35250631C>A
-
GJB3(NM_024009.3):c.268C>A (p.(Leu90Met))
-
GJB3_000067
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
1
-
c.290A>C
r.(?)
p.(Tyr97Ser)
-
likely benign
g.35250653A>C
g.34785052A>C
-
-
GJB3_000046
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
-
c.311G>A
r.(?)
p.(Arg104Gln)
-
VUS
g.35250674G>A
-
GJB3(NM_024009.3):c.311G>A (p.R104Q)
-
GJB3_000055
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/.
1
-
c.313C>A
r.(?)
p.(His105Asn)
-
benign
g.35250676C>A
-
GJB3(NM_024009.3):c.313C>A (p.H105N)
-
GJB3_000058
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/-, -/.
3
2
c.357C>T
r.(?)
p.(=), p.(Asn119=)
-
benign
g.35250720C>T
g.34785119C>T
GJB3(NM_024009.3):c.357C>T (p.N119=), N119N
-
GJB3_000012
VKGL data sharing initiative Nederland
PubMed: Richard 2000
-
-
CLASSIFICATION record, Unknown
?
0.3
-
-
-
Johan den Dunnen
,
VKGL-NL_Nijmegen
,
VKGL-NL_AMC
+/.
1
2
c.409T>C
r.(?)
p.(Phe137Leu)
-
pathogenic
g.35250772T>C
g.34785171T>C
-
-
GJB3_000019
de novo, in patient
PubMed: Richard 2000
-
-
De novo
-
-
-
-
-
Michel van Geel
+/.
2
2
c.421A>G
r.(?)
p.(Ile141Val)
-
pathogenic
g.35250784A>G
g.34785183A>G
-
-
GJB3_000010
-
PubMed: Liu 2000
,
OMIM:var0007
-
-
Unknown
yes
-
-
-
-
Michel van Geel
+/.
2
2
c.421_423del
r.(?)
p.(Ile141del)
-
pathogenic
g.35250784_35250786del
g.34785183_34785185del
-
-
GJB3_000009
-
PubMed: Liu 2000
,
OMIM:var0006
-
-
Unknown
yes
-
-
-
-
Michel van Geel
?/.
1
-
c.422_423insCA
r.(?)
p.(Glu142Metfs*127)
-
VUS
g.35250785_35250786insCA
-
GJB3(NM_024009.3):c.422_423insCA (p.(Glu142MetfsTer127))
-
GJB3_000060
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
-
c.424dup
r.(?)
p.(Glu142Glyfs*64)
-
VUS
g.35250787dup
-
GJB3(NM_024009.3):c.424dup (p.(Glu142GlyfsTer64))
-
GJB3_000061
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
-
c.476C>T
r.(?)
p.(Pro159Leu)
-
VUS
g.35250839C>T
-
GJB3(NM_024009.3):c.476C>T (p.(Pro159Leu))
-
GJB3_000062
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-/-, -/.
3
2
c.477G>A
r.(?)
p.(=), p.(Pro159=)
-
benign
g.35250840G>A
g.34785239G>A
GJB3(NM_024009.3):c.477G>A (p.P159=), P159P
-
GJB3_000016
not in 92 control chromosomes, VKGL data sharing initiative Nederland
PubMed: Lopez-Bigas 2000
-
-
CLASSIFICATION record, Unknown
?
24/153 cases, 6/46 controls
-
-
-
Johan den Dunnen
,
VKGL-NL_AMC
?/.
1
-
c.478C>T
r.(?)
p.(Arg160Cys)
-
VUS
g.35250841C>T
-
GJB3(NM_024009.3):c.478C>T (p.(Arg160Cys))
-
GJB3_000068
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
2
-
c.479G>A
r.(?)
p.(Arg160His)
-
likely benign
g.35250842G>A
g.34785241G>A
GJB3(NM_024009.3):c.479G>A (p.R160H)
-
GJB3_000037
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
,
VKGL-NL_AMC
+/.
1
2
c.497A>G
r.(?)
p.(Asn166Ser)
-
pathogenic
g.35250860A>G
g.34785259A>G
-
-
GJB3_000033
GJB2 1 bp deletion; possible di-genic inheritance
PubMed: Liu 2009
-
-
Unknown
?
-
-
-
-
Michel van Geel
-/.
1
-
c.529T>G
r.(?)
p.(Tyr177Asp)
-
benign
g.35250892T>G
-
GJB3(NM_024009.3):c.529T>G (p.Y177D)
-
GJB3_000031
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
1
2
c.538C>T
r.(?)
p.(Arg180*)
-
pathogenic
g.35250901C>T
g.34785300C>T
-
-
GJB3_000008
-
PubMed: Xia 1998
,
OMIM:var0005
-
-
Germline
yes
-
-
-
-
Michel van Geel
+/.
1
2
c.547G>A
r.(?)
p.(Glu183Lys)
-
pathogenic
g.35250910G>A
g.34785309G>A
-
-
GJB3_000007
-
PubMed: Xia 1998
,
OMIM:var0004
-
-
Germline
yes
-
-
-
-
Michel van Geel
-/-, -/.
3
2
c.567C>T
r.(?)
p.(=), p.(Tyr189=)
-
benign
g.35250930C>T
g.34785329C>T
GJB3(NM_024009.3):c.567C>T (p.Y189=), Y189Y
-
GJB3_000017
not in 92 control chromosomes, VKGL data sharing initiative Nederland
PubMed: Lopez-Bigas 2000
-
-
CLASSIFICATION record, Unknown
?
1/110 cases, 3/153 cases
-
-
-
Johan den Dunnen
,
VKGL-NL_AMC
-/.
2
-
c.579C>T
r.(?)
p.(Gly193=)
-
benign
g.35250942C>T
g.34785341C>T
GJB3(NM_024009.2):c.579C>T (p.G193=), GJB3(NM_024009.3):c.579C>T (p.G193=)
-
GJB3_000049
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
+/., -/., ?/.
3
2
c.580G>A
r.(?)
p.(Ala194Thr)
-
benign, pathogenic, VUS
g.35250943G>A
g.34785342G>A
GJB3(NM_024009.2):c.580G>A (p.(Ala194Thr)), GJB3(NM_024009.3):c.580G>A (p.A194T)
-
GJB3_000034
GJB2 1 bp deletion; possible di-genic inheritance, VKGL data sharing initiative Nederland
PubMed: Liu 2009
-
-
CLASSIFICATION record, Unknown
?
-
-
-
-
Michel van Geel
,
VKGL-NL_Leiden
,
VKGL-NL_AMC
?/.
1
-
c.586G>A
r.(?)
p.(Ala196Thr)
-
VUS
g.35250949G>A
g.34785348G>A
GJB3(NM_024009.3):c.586G>A (p.A196T)
-
GJB3_000038
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/-
2
2
c.597G>A
r.(?)
p.(Val200Ile)
-
benign
g.35250960G>A
g.34785359G>A
-
-
GJB3_000018
1 more item
PubMed: Lopez-Bigas 2000
-
-
Unknown
?
1/110 cases, 3/153 cases
-
-
-
Johan den Dunnen
-?/.
1
-
c.598G>A
r.(?)
p.(Val200Ile)
-
likely benign
g.35250961G>A
g.34785360G>A
GJB3(NM_024009.3):c.598G>A (p.V200I)
-
GJB3_000039
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
1
2
c.625C>T
r.(?)
p.(Leu209Phe)
-
pathogenic
g.35250988C>T
g.34785387C>T
L209F
-
GJB3_000025
-
PubMed: Richard 2001
-
-
Unknown
?
-
-
-
-
Michel van Geel
+/.
1
2
c.652_663del
r.(?)
p.(Leu218_Asp221del)
-
pathogenic
g.35251015_35251026del
g.34785414_34785425del
-
-
GJB3_000020
-
PubMed: Richard 2000
-
-
Unknown
?
-
-
-
-
Michel van Geel
+/.
2
2
c.667C>A
r.(?)
p.(Pro223Thr)
-
pathogenic
g.35251030C>A
g.34785429C>A
GJB3(NM_024009.2):c.667C>A (p.(Pro223Thr))
-
GJB3_000027
VKGL data sharing initiative Nederland
PubMed: Uyguner 2003
-
-
CLASSIFICATION record, Unknown
yes
-
-
-
-
Michel van Geel
,
VKGL-NL_Leiden
?/.
1
-
c.670C>T
r.(?)
p.(Arg224*)
-
VUS
g.35251033C>T
-
-
-
GJB3_000054
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/.
1
-
c.697G>A
r.(?)
p.(Ala233Thr)
-
likely benign
g.35251060G>A
g.34785459G>A
GJB3(NM_024009.3):c.697G>A (p.A233T)
-
GJB3_000040
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
1
-
c.764C>G
r.(?)
p.(Pro255Arg)
-
VUS
g.35251127C>G
g.34785526C>G
GJB3(NM_024009.3):c.764C>G (p.P255R)
-
GJB3_000050
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/.
1
-
c.778C>T
r.(?)
p.(Leu260=)
-
likely benign
g.35251141C>T
g.34785540C>T
GJB3(NM_024009.2):c.778C>T (p.L260=)
-
GJB3_000051
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/., -?/?
2
2
c.798C>T
r.(?)
p.(=), p.(Asn266=)
-
benign, likely benign
g.35251161C>T
g.34785560C>T
801C>T (N267N), GJB3(NM_024009.3):c.798C>T (p.N266=)
-
GJB3_000028
VKGL data sharing initiative Nederland
PubMed: Uyguner 2003
-
-
CLASSIFICATION record, Unknown
yes
-
-
-
-
Michel van Geel
,
VKGL-NL_AMC
-/-
4
2
c.798G>T
r.(?)
p.(=)
-
benign
g.35251161G>T
g.34785560G>T
1931C>T (N266N), N266N
-
GJB3_000013
1 more item
PubMed: Lopez-Bigas 2000
,
PubMed: Richard 2000
-
-
Unknown
?
0.1, 1/110 cases, 3/153 cases, 6/110 cases
-
-
-
Johan den Dunnen
-?/.
1
-
c.*13G>A
r.(=)
p.(=)
-
likely benign
g.35251189G>A
g.34785588G>A
GJB3(NM_024009.3):c.*13G>A
-
GJB3_000041
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/.
1
-
c.*43C>A
r.(=)
p.(=)
-
benign
g.35251219C>A
g.34785618C>A
GJB3(NM_024009.3):c.*43C>A
-
GJB3_000001
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/-
1
2
c.856C>A
r.(?)
p.(=)
-
benign
g.35251219C>A
g.34785618C>A
-
-
GJB3_000001
1 more item
PubMed: Richard 1998
-
-
Unknown
?
0.24
-
-
-
Johan den Dunnen
-/.
1
-
c.*53G>A
r.(=)
p.(=)
-
benign
g.35251229G>A
g.34785628G>A
GJB3(NM_024009.3):c.*53G>A
-
GJB3_000002
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/-
1
2
c.866G>A
r.(?)
p.(=)
-
benign
g.35251229G>A
g.34785628G>A
-
-
GJB3_000002
1 more item
PubMed: Richard 1998
-
-
Unknown
?
0.34
-
-
-
Johan den Dunnen
-/.
1
-
c.*369G>C
r.(=)
p.(=)
-
benign
g.35251545G>C
g.34785944G>C
GJB3(NM_024009.3):c.*369G>C
-
GJB3_000042
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/.
1
-
c.*493C>T
r.(=)
p.(=)
-
benign
g.35251669C>T
g.34786068C>T
GJB3(NM_024009.3):c.*493C>T
-
GJB3_000043
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/.
1
-
c.*559G>C
r.(=)
p.(=)
-
benign
g.35251735G>C
g.34786134G>C
GJB3(NM_024009.3):c.*559G>C
-
GJB3_000044
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
10 per page
25 per page
50 per page
100 per page
Legend
How to query
Screenscraping/webscraping (downloading large amounts of data using scripts) is strictly prohibited.
Use our
APIs
to retrieve data.
Powered by
LOVD v.3.0
Build 30b
LOVD software ©2004-2024
Leiden University Medical Center
Database contents © by their respective submitters and curators