Global Variome shared LOVD
GJA8 (gap junction protein, alpha 8, 50kDa)
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Unique variants in the GJA8 gene
This database is one of the
"Eye disease"
gene variant databases.
The variants shown are described using the NM_005267.4 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.
97 entries on 1 page. Showing entries 1 - 97.
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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.-1622618_*1555328del
r.?
p.?
-
pathogenic
g.145752381_148936712del
-
-
-
ACP6_000003
decreased gene dosage
PubMed: DDDS 2015
,
Journal: DDDS 2015
-
-
Germline
-
-
-
-
-
Johan den Dunnen
./.
1
-
c.-1609566_*869589dup
r.?
p.?
-
pathogenic
g.145765433_148250973dup
-
-
-
ACP6_000002
increased gene dosage
PubMed: DDDS 2015
,
Journal: DDDS 2015
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
2
c.9C>G
r.(?)
p.(Asp3Glu)
-
likely pathogenic
g.147380091C>G
g.147907964C>G
-
-
GJA8_000030
-
-
-
-
Germline
yes
-
-
-
-
Zongfu Cao
+?/.
1
-
c.10T>C
r.(?)
p.(Trp4Arg)
-
likely pathogenic (dominant)
g.147380092T>C
g.147907965T>C
-
-
GJA8_000093
-
PubMed: Guo 2025
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.19_30del
r.(?)
p.(Gly8_Leu11del)
-
VUS
g.147380101_147380112del
-
GJA8(NM_005267.5):c.19_30delCTGGGGAACATC (p.G8_L11del)
-
GJA8_000035
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
+/., +?/.
4
2
c.20T>C
r.(?)
p.(Leu7Pro)
ACMG
likely pathogenic (dominant), pathogenic (dominant)
g.147380102T>C
g.147907975T>C
-
-
GJA8_000079
ACMG PS2_mod, PS4_sup, PM2_sup, PP1, PP3
PubMed: Liu 2023
,
PubMed: Mackay 2014
,
PubMed: Wang 2024
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
?/.
1
-
c.22G>A
r.(?)
p.(Gly8Arg)
-
VUS
g.147380104G>A
-
GJA8(NM_005267.5):c.22G>A (p.G8R)
-
GJA8_000036
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
+?/.
2
-
c.23_34del
r.(?)
p.(Gly8_Leu11del)
-
likely pathogenic (dominant)
g.147380105_147380116del
g.147907978_147907989del
21_32del
-
GJA8_000071
-
PubMed: Astiazaran 2018
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
?/.
1
-
c.50A>C
r.(?)
p.(His17Pro)
ACMG
VUS
g.147380132A>C
g.147908005A>C
-
-
GJA8_000094
ACMG PM2_sup, PP1_sup, PP3, PP4
PubMed: Wang 2024
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.51C>T
r.(?)
p.(His17=)
-
likely benign
g.147380133C>T
g.147908006C>T
GJA8(NM_005267.4):c.51C>T (p.H17=)
-
GJA8_000044
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/.
1
-
c.64G>A
r.(?)
p.(Gly22Ser)
-
likely pathogenic (dominant)
g.147380146G>A
g.147908019G>A
-
-
GJA8_000061
-
PubMed: Fernandez-Alcalde 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.64G>C
r.(?)
p.(Gly22Arg)
-
likely pathogenic (dominant)
g.147380146G>C
g.147908019G>C
-
-
GJA8_000067
-
PubMed: Amine 2023
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
+/.
3
2
c.68G>C
r.(?)
p.(Arg23Thr)
ACMG
pathogenic, pathogenic (dominant)
g.147380150G>C
g.147908023G>C
68G>T
-
GJA8_000022
ACMG PM1, PP1, PP3, PS3, LOVD2: GJ8_00005
PubMed: Khidiyatova 2023
,
PubMed: Willoughby 2003
,
OMIM:var0004
-
rs80358203
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.73T>C
r.(?)
p.(Trp25Arg)
ACMG
likely pathogenic (dominant)
g.147380155T>C
g.147908028T>C
-
-
GJA8_000068
incomplete penetrance
PubMed: Javadiyan 2017
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.77T>C
r.(?)
p.(Leu26Pro)
-
likely pathogenic
g.147380159T>C
g.147908032T>C
c.77T-->C; p.Leu26Pro
-
GJA8_000053
no Sanger sequencing; heterozygous
PubMed: Patel 2019
-
-
Germline
?
-
-
-
-
LOVD
+/.
1
-
c.89dupT
r.(?)
p.(Ile31HisfsTer18)
-
pathogenic (recessive)
g.147380171dup
g.147908044dup
89dupT
-
GJA8_000072
father unavailable
PubMed: Ma 2016
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
2
-
c.101T>C
r.(?)
p.(Ile34Thr)
ACMG
likely pathogenic (dominant)
g.147380183T>C
g.147908056T>C
-
-
GJA8_000105
ACMG PM2_P, PP2_P, PP3_S
PubMed: Lecca 2024
-
-
Germline, Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.102C>T
r.(?)
p.(Ile34=)
-
likely benign
g.147380184C>T
-
GJA8(NM_005267.4):c.102C>T (p.I34=)
-
GJA8_000047
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/.
1
-
c.116C>G
r.(?)
p.(Thr39Arg)
-
likely pathogenic (dominant)
g.147380198C>G
g.147908071C>G
-
-
GJA8_000095
-
PubMed: Guo 2025
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.117G>A
r.(?)
p.(Thr39=)
-
likely benign
g.147380199G>A
g.147908072G>A
GJA8(NM_005267.4):c.117G>A (p.T39=)
-
GJA8_000037
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/.
1
-
c.119C>T
r.(?)
p.(Ala40Val)
-
likely pathogenic (dominant)
g.147380201C>T
g.147908074C>T
-
-
GJA8_000073
-
PubMed: Ma 2016
-
-
De novo
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.126G>C
r.(?)
p.(Glu42Asp)
-
VUS
g.147380208G>C
-
GJA8(NM_005267.5):c.126G>C (p.(Glu42Asp))
-
GJA8_000090
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/., +?/.
4
-
c.130G>A
r.(?)
p.(Val44Met)
-
likely pathogenic (dominant), pathogenic (dominant)
g.147380212G>A
g.147908085G>A
-
-
GJA8_000062
-
PubMed: Guo 2025
,
PubMed: Zhang 2018
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
2
c.131T>A
r.(?)
p.(Val44Glu)
-
pathogenic
g.147380213T>A
g.147908086T>A
-
-
GJA8_000023
LOVD2: GJ8_00006
PubMed: Devi 2006
,
OMIM:var0005
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.133_142del
r.(?)
p.(Trp45SerfsTer43)
ACMG
pathogenic (dominant)
g.147380215_147380224del
g.147908088_147908097del
-
-
GJA8_000080
ACMG PVS1, PM1, PM2
PubMed: Khidiyatova 2023
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.134G>A
r.(?)
p.(Trp45Ter)
-
pathogenic (recessive)
g.147380216G>A
g.147908089G>A
-
-
GJA8_000081
-
PubMed: Liu 2023
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., +?/.
4
2
c.134G>C
r.(?)
p.(Trp45Ser)
ACMG
likely pathogenic, likely pathogenic (dominant), pathogenic (dominant)
g.147380216G>C
g.147908089G>C
GJA8(NM_005267.4):c.134G>C(p.W45S)
-
GJA8_000009
-
PubMed: Javadiyan 2017
,
PubMed: Liu 2023
,
PubMed: Ma 2016
,
PubMed: Sun 2018
rs864309688
rs864309688
De novo, Germline, Germline/De novo (untested)
?, yes
152
-
-
-
Johan den Dunnen
+/., +?/.
9
-
c.134G>T
r.(?)
p.(Trp45Leu)
-
likely pathogenic (dominant), pathogenic, pathogenic (dominant)
g.147380216G>T
g.147908089G>T
GJA8 c.134G>T, p.(Gln155Ter), GJA8(NM_005267.4):c.134G>T (p.(Trp45Leu))
-
GJA8_000056
heterozygous, VKGL data sharing initiative Nederland
PubMed: Bell 2021
,
PubMed: Guo 2025
,
PubMed: Jackson 2020
,
PubMed: Liu 2023
-
-
CLASSIFICATION record, De novo, Germline
yes
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Leiden
+?/.
4
2
c.136G>A
r.(?)
p.(Gly46Arg)
-
likely pathogenic (dominant)
g.147380218G>A
g.147908091G>A
-
-
GJA8_000074
-
PubMed: Fan 2020
,
PubMed: Guo 2025
,
PubMed: Liu 2023
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
+/.
1
2
c.139G>A
r.(?)
p.(Asp47Asn)
-
pathogenic
g.147380221G>A
g.147908094G>A
-
-
GJA8_000025
LOVD2: GJ8_00008
PubMed: He 2011
,
OMIM:var0007
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
2
c.139G>C
r.(?)
p.(Asp47His)
-
pathogenic (dominant)
g.147380221G>C
g.147908094G>C
-
-
GJA8_000082
-
PubMed: Liu 2023
rs121434643
rs121434643
Germline
yes
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.140A>G
r.(?)
p.(Asp47Gly)
-
likely pathogenic
g.147380222A>G
g.147908095A>G
-
-
GJA8_000038
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
2
c.142G>A
r.(?)
p.(Glu48Lys)
-
pathogenic
g.147380224G>A
g.147908097G>A
-
-
GJA8_000020
LOVD2: GJ8_00003
PubMed: Berry 1999
,
OMIM:var0002
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., +?/.
2
-
c.143A>G
r.(?)
p.(Glu48Gly)
ACMG
likely pathogenic (dominant), pathogenic (dominant)
g.147380225A>G
g.147908098A>G
-
-
GJA8_000083
ACMG PM1, PM2, PM5, PP3, ACMG PS2, PM2_sup, PM5, PP3, PP4
PubMed: Khidiyatova 2023
,
PubMed: Wang 2024
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., ?/.
2
2
c.145C>G
r.(?)
p.(Gln49Glu)
-
pathogenic, VUS
g.147380227C>G
g.147908100C>G
-
-
GJA8_000001
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Juhua Yang
,
VKGL-NL_Nijmegen
+?/.
3
-
c.151G>A
r.(?)
p.(Asp51Asn)
-
likely pathogenic (dominant)
g.147380233G>A
g.147908106G>A
-
-
GJA8_000075
-
PubMed: Guo 2025
,
PubMed: Ma 2016
-
-
De novo, Germline
-
-
-
-
-
Johan den Dunnen
+?/.
2
-
c.154T>C
r.(?)
p.(Phe52Leu)
ACMG
likely pathogenic, likely pathogenic (dominant)
g.147380236T>C
g.147908109T>C
GJA8(NM_005267.4):c.154T>C(p.F52L)
-
GJA8_000058
ACMG PM1 PM2 PP3 PP4
PubMed: Li 2019
,
PubMed: Sun 2018
-
-
Germline, Germline/De novo (untested)
?
137
-
-
-
Johan den Dunnen
?/.
1
-
c.157G>T
r.(?)
p.(Val53Leu)
-
VUS
g.147380239G>T
-
GJA8(NM_005267.4):c.157G>T (p.(Val53Leu))
-
GJA8_000088
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+?/., ?/.
3
-
c.163A>G
r.(?)
p.(Asn55Asp)
ACMG
likely pathogenic, likely pathogenic (dominant), VUS
g.147380245A>G
g.147908118A>G
GJA8(NM_005267.4):c.163A>G(p.N55D)
-
GJA8_000059
ACMG PS2_mod, PS4_sup, PM2_sup, PP3
PubMed: Guo 2025
,
PubMed: Sun 2018
,
PubMed: Wang 2024
-
-
Germline, Germline/De novo (untested)
?
173
-
-
-
Johan den Dunnen
?/.
1
-
c.167C>T
r.(?)
p.(Thr56Ile)
-
VUS
g.147380249C>T
g.147908122C>T
-
-
GJA8_000033
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/.
1
-
c.175C>T
r.(?)
p.(Pro59Ser)
-
likely pathogenic (dominant)
g.147380257C>T
g.147908130C>T
-
-
GJA8_000096
-
PubMed: Guo 2025
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.179G>A
r.(?)
p.(Gly60Asp)
ACMG
likely pathogenic (dominant)
g.147380261G>A
g.147908134G>A
-
-
GJA8_000084
ACMG PM1, PM2, PP3
PubMed: Khidiyatova 2023
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.184G>A
r.(?)
p.(Glu62Lys)
-
likely pathogenic (dominant)
g.147380266G>A
g.147908139G>A
-
-
GJA8_000097
-
PubMed: Guo 2025
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
2
-
c.197A>G
r.(?)
p.(Tyr66Cys)
-
likely pathogenic (dominant)
g.147380279A>G
g.147908152A>G
-
-
GJA8_000098
-
PubMed: Guo 2025
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.200A>G
r.(?)
p.(Asp67Gly)
-
pathogenic (dominant)
g.147380282A>G
g.147908155A>G
-
-
GJA8_000076
-
PubMed: Reis 2013
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
?/.
1
-
c.206C>T
r.(?)
p.(Ala69Val)
ACMG
VUS
g.147380288C>T
g.147908161C>T
-
-
GJA8_000099
ACMG PM2_sup, PM5, PP3, PP4
PubMed: Wang 2024
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.208T>C
r.(?)
p.(Phe70Leu)
-
likely pathogenic (dominant)
g.147380290T>C
g.147908163T>C
-
-
GJA8_000100
-
PubMed: Guo 2025
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
2
c.218C>T
r.(?)
p.(Ser73Phe)
-
pathogenic
g.147380300C>T
g.147908173C>T
-
-
GJA8_000007
-
PubMed: Hansen 2009
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.226C>G
r.(?)
p.(Arg76Gly)
-
likely pathogenic (dominant)
g.147380308C>G
g.147908181C>G
-
-
GJA8_000063
-
PubMed: Fernandez-Alcalde 2021
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+/., +?/., ?/.
4
-
c.226C>T
r.(?)
p.(Arg76Cys)
-
likely pathogenic (dominant), pathogenic (dominant), VUS
g.147380308C>T
g.147908181C>T
-
-
GJA8_000064
father not available, VUS PS4sup, PM2, PP3
PubMed: Fernandez-Alcalde 2021
,
PubMed: Kessel 2021
,
PubMed: Reichsteiner 2021
,
PubMed: Reis 2013
-
-
De novo, Germline, Germline/De novo (untested)
yes
-
-
-
-
Johan den Dunnen
+/.
2
-
c.227G>A
r.(?)
p.(Arg76His)
-
pathogenic
g.147380309G>A
-
GJA8(NM_005267.4):c.227G>A (p.(Arg76His))
-
GJA8_000048
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Nijmegen
-?/.
1
-
c.250T>A
r.(?)
p.(Phe84Ile)
-
likely benign
g.147380332T>A
g.147908205T>A
-
-
GJA8_000034
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
2
c.262C>T
r.(?)
p.(Pro88Ser)
-
pathogenic
g.147380344C>T
g.147908217C>T
-
-
GJA8_000019
LOVD2: GJ8_00002
PubMed: Shiels 1998
,
OMIM:var0001
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.263C>T
r.(?)
p.(Pro88Leu)
ACMG
VUS
g.147380345C>T
g.147908218C>T
GJA8 c.263C>T p.(Pro88Leu) het
-
GJA8_000055
heterozygous
PubMed: Lenassi 2020
-
-
Germline
?
-
-
-
-
LOVD
+/., ./.
2
-
c.290T>G
r.(?)
p.(Val97Gly)
-
pathogenic, pathogenic (dominant)
g.147380372T>G
g.147908245T>G
-
-
GJA8_000026
-
PubMed: DDDS 2015
,
Journal: DDDS 2015
,
PubMed: Jackson 2020
-
-
De novo, Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.293A>C
r.(?)
p.(His98Pro)
-
pathogenic (dominant)
g.147380375A>C
g.147908248A>C
-
-
GJA8_000085
-
PubMed: Mackay 2014
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
+?/., ?/.
2
2
c.293A>G
r.(?)
p.(His98Arg)
ACMG
likely pathogenic, VUS
g.147380375A>G
g.147908248A>G
GJA8 c.293A>G p.(His98Arg) het
-
GJA8_000028
1 more item
PubMed: Gillespie 2014
,
Journal: Gillespie 2014
,
PubMed: Lenassi 2020
-
-
Germline
?
-
-
-
-
Johan den Dunnen
+?/.
2
-
c.302G>T
r.(?)
p.(Arg101Leu)
-
likely pathogenic (dominant)
g.147380384G>T
g.147908257G>T
-
-
GJA8_000101
-
PubMed: Guo 2025
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.333G>A
r.(?)
p.(Ala111=)
-
likely benign
g.147380415G>A
g.147908288G>A
GJA8(NM_005267.4):c.333G>A (p.A111=)
-
GJA8_000039
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.339G>C
r.(?)
p.(Glu113Asp)
-
likely benign
g.147380421G>C
-
GJA8(NM_005267.4):c.339G>C (p.(Glu113Asp))
-
GJA8_000089
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+?/.
1
-
c.367G>A
r.(?)
p.(Gly123Ser)
-
likely pathogenic (dominant)
g.147380449G>A
g.147908322G>A
-
-
GJA8_000102
-
PubMed: Guo 2025
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.385G>A
r.(?)
p.(Val129Ile)
-
likely benign
g.147380467G>A
g.147908340G>A
GJA8(NM_005267.4):c.385G>A (p.V129I)
-
GJA8_000031
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.406A>G
r.(?)
p.(Lys136Glu)
-
VUS
g.147380488A>G
-
GJA8(NM_005267.5):c.406A>G (p.(Lys136Glu))
-
GJA8_000092
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
1
-
c.460C>G
r.(?)
p.(His154Asp)
-
pathogenic (dominant)
g.147380542C>G
g.147908415C>G
-
-
GJA8_000077
-
PubMed: Patel 2017
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.477C>T
r.(?)
p.(Thr159=)
-
likely benign
g.147380559C>T
g.147908432C>T
GJA8(NM_005267.4):c.477C>T (p.T159=)
-
GJA8_000040
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.484G>A
r.(?)
p.(Glu162Lys)
ACMG
VUS
g.147380566G>A
g.147908439G>A
-
-
GJA8_000069
-
PubMed: Javadiyan 2017
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
?/.
2
-
c.553C>T
r.(?)
p.(Arg185Trp)
-
VUS
g.147380635C>T
g.147908508C>T
GJA8(NM_005267.4):c.553C>T (p.R185W), GJA8(NM_005267.5):c.553C>T (p.R185W)
-
GJA8_000041
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_VUmc
+?/.
1
-
c.565C>G
r.(?)
p.(Pro189Ala)
-
likely pathogenic (dominant)
g.147380647C>G
g.147908520C>G
-
-
GJA8_000065
-
PubMed: Fernandez-Alcalde 2021
-
-
Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.566C>T
r.(?)
p.(Pro189Leu)
-
pathogenic (dominant)
g.147380648C>T
-
565C>T (Pro189Leu)
-
GJA8_000011
-
PubMed: Hansen 2009
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., +?/.
2
2
c.569A>G
r.(?)
p.(Asn190Ser)
-
likely pathogenic, pathogenic
g.147380651A>G
g.147908524A>G
-
-
GJA8_000018
-
PubMed: Huang 2015
-
-
Germline
-
-
-
-
-
Juhua Yang
+?/.
1
-
c.590C>T
r.(?)
p.(Ser197Phe)
-
likely pathogenic (dominant)
g.147380672C>T
g.147908545C>T
-
-
GJA8_000103
-
PubMed: Guo 2025
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
3
2
c.592C>T
r.(?)
p.(Arg198Trp)
-
pathogenic (dominant)
g.147380674C>T
g.147908547C>T
-
-
GJA8_000012
-
PubMed: Fernandez-Alcalde 2021
,
PubMed: Liu 2023
-
-
De novo, Germline
yes
-
-
-
-
Johan den Dunnen
+/.
2
2
c.593G>A
r.(?)
p.(Arg198Gln)
-
pathogenic, pathogenic (dominant)
g.147380675G>A
g.147908548G>A
-
-
GJA8_000024
de novo in mother, LOVD2: GJ8_00007
PubMed: Devi 2006
,
OMIM:var0006
,
PubMed: Prokudin 2014
-
-
De novo, Germline
yes
-
-
-
-
Johan den Dunnen
,
Ivan Prokudin
?/.
1
-
c.596C>G
r.(?)
p.(Pro199Arg)
-
VUS
g.147380678C>G
-
-
-
GJA8_000060
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
-
c.601G>A
r.(?)
p.(Glu201Lys)
ACMG
pathogenic (dominant)
g.147380683G>A
g.147908556G>A
-
-
GJA8_000104
ACMG PS2_mod, PS3_sup, PM2_sup, PP1_strong, PP3, PP4
PubMed: Wang 2024
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.602A>C
r.(?)
p.(Glu201Ala)
-
VUS
g.147380684A>C
-
GJA8(NM_005267.5):c.602A>C (p.E201A)
-
GJA8_000070
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
?/.
3
-
c.634G>T
r.(?)
p.(Val212Leu)
ACMG
VUS
g.147380716G>T
g.147908589G>T
-
-
GJA8_000106
ACMG PM2_P, PP2_P, PP3_M
PubMed: Lecca 2024
-
-
Germline, Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
-/., -?/.
5
2
c.658A>G
r.(?)
p.(Asn220Asp)
-
benign, likely benign
g.147380740A>G
g.147908613A>G
GJA8(NM_005267.4):c.658A>G (p.N220D, p.(Asn220Asp))
-
GJA8_000032
in unaffected father, VKGL data sharing initiative Nederland
PubMed: Cao 2018
,
PubMed: Ma 2016
-
rs138140155
CLASSIFICATION record, Germline
-
1/340 control chromosomes, 1/38 chromosomes cases CTRCT
-
-
-
Johan den Dunnen
,
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
+?/.
2
-
c.667G>A
r.(?)
p.(Glu223Lys)
-
likely pathogenic, likely pathogenic (dominant)
g.147380749G>A
g.147908622G>A
c.667G>A, p.(Glu223Lys)
-
GJA8_000054
heterozygous
PubMed: Guo 2025
,
PubMed: Wang 2019
-
-
Germline
?
-
-
-
-
Johan den Dunnen
?/.
1
-
c.727C>T
r.(?)
p.(Pro243Ser)
-
VUS
g.147380809C>T
-
-
-
GJA8_000045
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/., -/., -?/., ?/.
6
2
c.741T>G
r.(?)
p.(Ile247Met)
ACMG
benign, likely benign, pathogenic, VUS
g.147380823T>G
g.147908696T>G
GJA8(NM_005267.4):c.741T>G (p.I247M, p.(Ile247Met))
-
GJA8_000021
ACMG PS4, PP4, BS3, LOVD2: GJ8_00004, VKGL data sharing initiative Nederland
PubMed: Khidiyatova 2023
,
PubMed: Polyakov 2001
,
OMIM:var0003
,
PubMed: Reis 2013
-
rs80358202
CLASSIFICATION record, Germline
no
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
VKGL-NL_Nijmegen
?/.
1
-
c.761C>T
r.(?)
p.(Ser254Phe)
-
VUS
g.147380843C>T
-
-
-
GJA8_000087
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/., ?/.
3
-
c.773C>T
r.(?)
p.(Ser258Phe)
-
likely pathogenic, VUS
g.147380855C>T
g.147908728C>T
GJA8(NM_005267.4):c.773C>T(p.S258F)
-
GJA8_000013
-
PubMed: Reichsteiner 2021
,
PubMed: Sun 2018
-
-
Germline, Germline/De novo (untested)
?
189
-
-
-
Johan den Dunnen
+/.
1
2
c.776C>A
r.(?)
p.(Ser259Tyr)
-
pathogenic (dominant)
g.147380858C>A
g.147908731C>A
836C>A
-
GJA8_000014
-
PubMed: Hansen 2009
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.804C>T
r.(?)
p.(Leu268=)
-
likely benign
g.147380886C>T
g.147908759C>T
-
-
GJA8_000066
-
PubMed: Li 2019
-
rs3766503
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
2
c.827C>T
r.(?)
p.(Ser276Phe)
-
VUS
g.147380909C>T
g.147908782C>T
-
-
GJA8_000015
-
-
-
rs45619342
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
2
c.914G>A
r.(?)
p.(Ser305Asn)
-
likely pathogenic
g.147380996G>A
g.147908869G>A
-
-
GJA8_000029
-
-
-
-
Germline
yes
-
-
-
-
Zongfu Cao
-?/.
1
-
c.1095C>T
r.(?)
p.(Ala365=)
-
likely benign
g.147381177C>T
-
GJA8(NM_005267.4):c.1095C>T (p.A365=)
-
GJA8_000049
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.1096G>A
r.(?)
p.(Val366Met)
-
likely benign
g.147381178G>A
-
GJA8(NM_005267.5):c.1096G>A (p.(Val366Met))
-
GJA8_000091
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
1
-
c.1102G>C
r.(?)
p.(Glu368Gln)
-
pathogenic (dominant)
g.147381184G>C
g.147909057G>C
-
-
GJA8_000078
-
PubMed: Senthil Kumar 2016
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
?/.
1
-
c.1105G>T
r.(?)
p.(Gly369Trp)
-
VUS
g.147381187G>T
-
GJA8(NM_005267.4):c.1105G>T (p.G369W)
-
GJA8_000046
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.1129G>A
r.(?)
p.(Val377Met)
-
likely benign
g.147381211G>A
-
GJA8(NM_005267.4):c.1129G>A (p.V377M)
-
GJA8_000050
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.1161G>T
r.(?)
p.(Gln387His)
-
likely benign
g.147381243G>T
g.147909116G>T
GJA8(NM_005267.4):c.1161G>T (p.(Gln387His))
-
GJA8_000042
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
1
-
c.1162T>G
r.(?)
p.(Ser388Ala)
-
likely benign
g.147381244T>G
g.147909117T>G
GJA8(NM_005267.4):c.1162T>G (p.(Ser388Ala))
-
GJA8_000043
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+?/.
1
-
c.1210_1211insACAC
r.(?)
p.(Leu404HisfsTer10)
-
likely pathogenic
g.147381292_147381293insACAC
g.147909165_147909166insACAC
1207_1208insCACA
-
GJA8_000086
-
PubMed: Liu 2023
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
2
c.1273C>T
r.(?)
p.(Arg425*)
-
pathogenic
g.147381355C>T
g.147909228C>T
-
-
GJA8_000027
-
PubMed: Gillespie 2014
,
Journal: Gillespie 2014
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.1300T>C
r.(?)
p.(*434Argext*?)
-
VUS
g.147381382T>C
-
-
-
GJA8_000057
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
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