Global Variome shared LOVD
EDA (ectodysplasin A)
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Curator:
Céline Cluzeau
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Unique variants in the EDA gene
The variants shown are described using the NM_001399.4 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.
196 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_1i
c.-242_(396+1_397-1){0}
r.0?
p.0?
-
pathogenic
g.(?_68835911)_(68836549_69176876)del
g.(?_69616067)_(69616705_69957026)del
c.1-?_396+?del
-
EDA_000154
-
PubMed: van der Hout 2008
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+?/.
1
8
c.1027 T>G
r.(?)
p.(Tyr343Asp)
-
likely pathogenic (recessive)
g.69255310T>G
g.70035460T>G
-
-
EDA_000177
-
PubMed: Martínez-Romero 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., +?/.
4
_1_1i
c.(?_-181)_(396+1_397-1)del
r.0?
p.0?
-
pathogenic (dominant), pathogenic (recessive)
g.(?_68835972)_(68836549_69176876)del
g.(?_69616128)_(69616705_69957026)del
deletion exon 1, exon 1 del
-
EDA_000015
gene exons described as 1a, 3a, 4-9
PubMed: Kere 1996
,
PubMed: Kere 1996
PubMed: Paakkonen 2001
,
PubMed: Paakkonen 2001
,
1 more item
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Céline Cluzeau
+/., -?/.
2
-
c.-56G>T
r.(?)
p.(=)
-
likely benign, pathogenic (recessive)
g.68836097G>T
g.69616253G>T
187G>T
-
EDA_000096
-
PubMed: Kere 1996
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.-16G>A
r.(?)
p.(=)
-
likely benign
g.68836137G>A
g.69616293G>A
227G>A
-
EDA_000097
-
PubMed: Kere 1996
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
+/.
2
_1_1i
c.-242_(396+1_397-1){0}
r.0?
p.0?
-
pathogenic (recessive)
g.(?_68835911)_(68836549_69176876)del
g.(?_69616067)_(69616705_69957026)del
del ex1
-
EDA_000154
-
PubMed: Martínez-Romero 2019
-
-
De novo, Germline
-
-
-
-
X-inactivation analysis 80:20 (in unaffected non-carrier mother 36:64)
Johan den Dunnen
+/.
1
_1_8_
c.-242_*3863{0}
r.0
p.0
-
pathogenic (recessive)
g.(?_68835911)_(69259322_?)del
g.(?_69616067)_(70039472_?)del
gene deletion
-
EDA_000153
-
PubMed: Martínez-Romero 2019
-
-
De novo
-
-
-
-
X-inactivation analysis not informative
Johan den Dunnen
+/.
1
1
c.2T>A
r.(?)
p.(Met1?)
-
pathogenic
g.68836154T>A
g.69616310T>A
Met1Lys
-
EDA_000156
-
PubMed: van der Hout 2008
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
1
c.2T>G
r.(?)
p.(M1R)
-
likely pathogenic
g.68836154T>G
g.69616310T>G
-
-
EDA_000016
-
PubMed: Cluzeau 2011
-
-
Unknown
-
-
-
-
-
Céline Cluzeau
+/.
1
-
c.4G>T
r.(?)
p.(Gly2Cys)
-
pathogenic (dominant)
g.68836156G>T
g.69616312G>T
-
-
EDA_000098
-
PubMed: Schneider 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.44dup
r.(?)
p.(Ala16Serfs*84)
-
pathogenic (recessive)
g.68836196dup
g.69616352dup
287insC
-
EDA_000093
-
PubMed: Kere 1996
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+/.
2
-
c.45_49del
r.(?)
p.(Pro17Glyfs*81)
-
pathogenic (dominant)
g.68836197_68836201del
g.69616353_69616357del
-
-
EDA_000099
-
PubMed: Schneider 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
1
c.58C>T
r.(?)
p.(Arg20Ter)
-
pathogenic (recessive)
g.68836210C>T
g.69616366C>T
-
-
EDA_000157
-
PubMed: Martínez-Romero 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
4
1
c.64_71dup
r.(?)
p.(Cys25Alafs*35)
-
pathogenic (dominant)
g.68836216_68836223dup
g.69616372_69616379dup
64_71dup8
-
EDA_000037
-
PubMed: Wohlfart 2016
-
-
Germline
-
4/124 cases
-
-
-
Sigrun Maier-Wohlfart
+/.
1
-
c.67C>T
r.(?)
p.(Gln23*)
-
pathogenic (recessive)
g.68836219C>T
g.69616375C>T
366C>T
-
EDA_000100
-
PubMed: Kere 1996
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
+/.
2
1
c.78del
r.(?)
p.(Cys27Valfs*30), p.(Cys27ValfsTer30)
-
pathogenic
g.68836230del
g.69616386del
c.77delG, EDA(NM_001399.5):c.78delG (p.C27Vfs*30)
-
EDA_000147
VKGL data sharing initiative Nederland
PubMed: van der Hout 2008
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Groningen
+/.
1
-
c.88del
r.(?)
p.(Ala30Profs*27)
-
pathogenic
g.68836240del
-
EDA(NM_001399.5):c.88delG (p.A30Pfs*27)
-
EDA_000192
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-?/.
1
-
c.93T>C
r.(?)
p.(Pro31=)
-
likely benign
g.68836245T>C
g.69616401T>C
EDA(NM_001399.5):c.93T>C (p.P31=)
-
EDA_000065
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+?/.
1
1
c.119_120insTGTG
r.(?)
p.(Leu41Valfs*60)
-
pathogenic (dominant)
g.68836271_68836272insTGTG
g.69616427_69616428insTGTG
-
-
EDA_000013
-
PubMed: Gunadi 2009
-
-
Unknown
-
-
-
-
-
Céline Cluzeau
+/.
1
-
c.121dup
r.(?)
p.(Leu41Profs*59)
-
pathogenic (recessive)
g.68836273dup
g.69616429dup
363insC
-
EDA_000101
-
PubMed: Kere 1996
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
+?/.
1
1
c.164T>C
r.(?)
p.(Leu55Pro)
-
likely pathogenic (recessive)
g.68836316T>C
g.69616472T>C
-
-
EDA_000158
-
PubMed: Martínez-Romero 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.170C>G
r.(?)
p.(Thr57Arg)
-
likely pathogenic
g.68836322C>G
g.69616478C>G
EDA(NM_001399.5):c.170C>G (p.T57R)
-
EDA_000066
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+/.
1
-
c.181T>C
r.(?)
p.(Tyr61His)
-
pathogenic (recessive)
g.68836333T>C
g.69616489T>C
423T>C
-
EDA_000102
-
PubMed: Kere 1996
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
+/.
1
-
c.183C>G
r.(?)
p.(Tyr61*)
-
pathogenic (recessive)
g.68836335C>G
g.69616491C>G
-
425C>G
EDA_000094
-
PubMed: Yotsumoto 1998
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.185del
r.(?)
p.(Leu62Glnfs*29)
-
pathogenic (recessive)
g.68836337del
g.69616493del
427delT
-
EDA_000103
-
PubMed: Kere 1996
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
+/.
1
-
c.187G>A
r.(?)
p.(Glu63Lys)
-
pathogenic (recessive)
g.68836339G>A
g.69616495G>A
429G>A
-
EDA_000104
-
PubMed: Kere 1996
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
+/., -/., -?/., ?/.
8
-
c.206G>T
r.(?)
p.(Arg69Leu)
-
benign, likely benign, pathogenic (dominant), pathogenic (recessive), VUS
g.68836358G>T
g.69616514G>T
206G>T/991C>T, 448G>T,
1 more item
-
EDA_000067
VKGL data sharing initiative Nederland
PubMed: Burger 2014
,
PubMed: Kere 1996
,
PubMed: Schneider 2011
-
-
CLASSIFICATION record, Germline
yes
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
,
VKGL-NL_Utrecht
+/.
1
-
c.242C>A
r.(?)
p.(Ser81Ter)
-
pathogenic
g.68836394C>A
g.69616550C>A
EDA(NM_001399.5):c.242C>A (p.S81*)
-
EDA_000068
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+/.
4
1
c.252del
r.(?)
p.(Gly85Alafs*6)
-
pathogenic (dominant), pathogenic (recessive)
g.68836404del
g.69616560del
494delT
-
EDA_000043
-
PubMed: Kere 1996
,
PubMed: Wohlfart 2016
-
-
Germline
yes
1/124 cases
-
-
-
Johan den Dunnen
,
Sigrun Maier-Wohlfart
+/.
1
-
c.351_353delinsG
r.(?)
p.(Pro118Glyfs*8)
-
pathogenic (recessive)
g.68836503_68836505delinsG
g.69616659_69616661delinsG
593A>G;594-595delCC
-
EDA_000105
-
PubMed: Kere 1996
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
+?/.
1
1
c.358G>T
r.(?)
p.(Glu120X)
-
likely pathogenic
g.68836510G>T
g.69616666G>T
-
-
EDA_000017
-
PubMed: Cluzeau 2011
-
-
Unknown
-
-
-
-
-
Céline Cluzeau
+?/.
1
1
c.361del
r.(?)
p.(Ala121Profs*16)
-
likely pathogenic
g.68836513del
g.69616669del
-
-
EDA_000032
-
PubMed: Cluzeau 2011
-
-
Unknown
-
-
-
-
-
Céline Cluzeau
+?/.
1
-
c.374C>G
r.(?)
p.(Ser125Cys)
-
likely pathogenic
g.68836526C>G
g.69616682C>G
g.616C>G
-
EDA_000149
-
-
-
-
Germline
-
-
-
-
-
Sigrun Maier-Wohlfart
+/.
1
1
c.376_379del
r.(?)
p.(Asp126Profs*10)
-
pathogenic (dominant)
g.68836528_68836531del
g.69616684_69616687del
376_379del4
-
EDA_000040
-
PubMed: Wohlfart 2016
-
-
Germline
-
1/124 cases
-
-
-
Sigrun Maier-Wohlfart
+/.
1
-
c.390del
r.(?)
p.(His131Thrfs*6)
-
pathogenic
g.68836542del
-
EDA(NM_001399.5):c.390delG (p.H131Tfs*6)
-
EDA_000189
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+/.
1
-
c.394C>T
r.(?)
p.(Gln132*)
-
pathogenic (recessive)
g.68836546C>T
g.69616702C>T
636C>T
-
EDA_000106
-
PubMed: Kere 1996
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
+/., +?/.
2
1i
c.396+5G>A
r.spl?
p.0?, p.?
-
likely pathogenic (dominant), pathogenic (dominant)
g.68836553G>A
g.69616709G>A
-
-
EDA_000050
-
PubMed: Prasad 2016
,
PubMed: Wohlfart 2016
-
-
Germline
-
1/124 cases
-
-
-
Johan den Dunnen
,
Sigrun Maier-Wohlfart
-?/.
1
1i
c.396+32900dup
r.(=)
p.(=)
-
likely benign
g.68869448dup
g.69649604dup
68869433insT
-
EDA_000059
-
PubMed: Kumar 2016
,
Journal: Kumar 2016
-
rs367558953
Germline
-
-
-
-
-
Thuong Ha
-?/.
1
1i
c.396+36982del
r.(?)
p.(=)
-
likely benign
g.68873530del
g.69653686del
68873526_68873527delA
-
EDA_000060
-
PubMed: Kumar 2016
,
Journal: Kumar 2016
-
-
Germline
-
-
-
-
-
Thuong Ha
-?/.
1
1i
c.396+44701A>T
r.(?)
p.(=)
-
likely benign
g.68881249A>T
g.69661405A>T
-
-
EDA_000061
-
PubMed: Kumar 2016
,
Journal: Kumar 2016
-
rs7471344
Germline
-
-
-
-
-
Thuong Ha
-?/.
1
-
c.396+53503C>A
r.(=)
p.(=)
-
likely benign
g.68890051C>A
-
EDA(NM_001005613.4):c.397-3C>A
-
EDA_000190
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-?/.
1
-
c.396+53503_396+53508del
r.(=)
p.(=)
-
likely benign
g.68890051_68890056del
g.69670207_69670212del
EDA(NM_001005613.2):c.397-3_399del (p.?)
-
EDA_000085
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
-
c.396+53504_396+53508del
r.(=)
p.(=)
-
VUS
g.68890052_68890056del
g.69670208_69670212del
EDA(NM_001005609.1):c.396+53503_396+53507del (p.?)
-
EDA_000076
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
-
c.396+53508C>T
r.(=)
p.(=)
-
VUS
g.68890056C>T
g.69670212C>T
EDA(NM_001005609.1):c.396+53508C>T (p.?)
-
EDA_000077
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
1
1i
c.397-78326_397-78325del
r.(?)
p.(=)
-
likely benign
g.69098551_69098552del
g.69878701_69878702del
-
-
EDA_000056
-
-
-
-
Germline
-
-
-
-
-
Yu Sun
-?/.
2
1i
c.397-78293_397-78292del
r.(?)
p.(=)
-
likely benign
g.69098584_69098585del
g.69878734_69878735del
-
-
EDA_000057
-
-
-
-
Germline
-
-
-
-
-
Yu Sun
+/.
1
1i_2i
c.397-6065_502+3117dup
r.?
p.?
-
pathogenic (dominant)
g.69170812_69180099dup
g.69950962_69960249dup
duplication exon 2
-
EDA_000054
-
PubMed: Wohlfart 2016
-
-
Unknown
-
1/124 cases
-
-
-
Sigrun Maier-Wohlfart
+/.
3
1i_2i
c.397-5853_502+3446dup
r.?
p.?
-
pathogenic (dominant)
g.69171024_69180428dup
g.69951174_69960578dup
duplication exon 2
-
EDA_000036
-
PubMed: Wohlfart 2016
-
-
Germline
-
3/124 cases
-
-
-
Sigrun Maier-Wohlfart
+?/.
1
2
c.397-1G>A
r.(?)
p.(?)
-
likely pathogenic
g.69176876G>A
g.69957026G>A
-
-
EDA_000030
-
PubMed: Cluzeau 2011
-
-
Unknown
-
-
-
-
-
Céline Cluzeau
+/.
2
1i_2i
c.(396+1_397-1)_(502+1_503-1)del
r.?
p.?
-
pathogenic (dominant)
g.(68836549_69176876)_(69176983_69243067)del
g.(69616705_69957026)_(69957133_70023217)del
deletion exon 2, 397-?_502+?del, exon 3 del
-
EDA_000055
gene exons described as 1a, 3a, 4-9
PubMed: Schneider 2011
,
PubMed: Wohlfart 2016
-
-
Germline, Unknown
-
1/124 cases
-
-
-
Johan den Dunnen
,
Sigrun Maier-Wohlfart
+/.
1
1i_2i
c.(396+1_397-1)_(502+1_503-1)dup
r.?
p.?
-
pathogenic (dominant)
g.(68836549_69176876)_(69176983_69243067)dup
g.(69616705_69957026)_(69957133_70023217)dup
-
-
EDA_000064
Testing revealed a partial duplication including exon 2.
-
-
-
Unknown
-
-
-
-
-
Alizabeth Woodruff
+/.
1
-
c.449_456del
r.(?)
p.(Glu150Alafs*6)
-
pathogenic (dominant)
g.69176929_69176936del
g.69957079_69957086del
-
-
EDA_000107
-
PubMed: Schneider 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
7
2
c.457C>T
r.(?), r.spl
p.(Arg153Cys)
-
pathogenic (dominant), pathogenic (recessive)
g.69176937C>T
g.69957087C>T
-
-
EDA_000108
-
PubMed: Burger 2014
,
PubMed: Martínez-Romero 2019
,
PubMed: Schneider 2011
,
PubMed: Wohlfart 2016
-
-
De novo, Germline
-
2/124 cases
-
-
-
Johan den Dunnen
+/., -?/.
2
-
c.458G>A
r.(?)
p.(Arg153His)
-
likely benign, pathogenic
g.69176938G>A
g.69957088G>A
EDA(NM_001005609.1):c.458G>A (p.(Arg153His))
-
EDA_000078
VKGL data sharing initiative Nederland
PubMed: Gostain 2020
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Leiden
+/.
18
2, 3
c.463C>T
r.(?)
p.(Arg155Cys)
-
pathogenic, pathogenic (dominant), pathogenic (recessive)
g.69176943C>T
g.69957093C>T
C704T, EDA(NM_001399.4):c.463C>T (p.(Arg155Cys)), EDA(NM_001399.5):c.463C>T (p.R155C)
-
EDA_000069
VKGL data sharing initiative Nederland
PubMed: Bodemer 2019
,
PubMed: Martínez-Romero 2019
,
PubMed: Monreal 1998
,
PubMed: Schneider 2011
,
2 more items
-
-
CLASSIFICATION record, De novo, Germline
-
8/124 cases
-
-
-
Johan den Dunnen
,
VKGL-NL_Leiden
,
VKGL-NL_Groningen
,
VKGL-NL_Utrecht
+?/.
1
2
c.466C>G
r.(?)
p.(Arg156Gly)
-
likely pathogenic
g.69176946C>G
g.69957096C>G
-
-
EDA_000018
-
PubMed: Cluzeau 2011
-
-
Unknown
-
-
-
-
-
Céline Cluzeau
+/.
9
2, 3
c.466C>T
r.(?)
p.(Arg156Cys)
ACMG
pathogenic, pathogenic (dominant), pathogenic (recessive)
g.69176946C>T
g.69957096C>T
707C>T (R156C), C707T, R156C
-
EDA_000002
ACMG grading: PS3,PM1,PM2,PM5,PP3, frequent variant in HED, located in the furin cleavage site
PubMed: Bayes 1998
,
PubMed: Bodemer 2019
,
PubMed: Burger 2014
,
PubMed: Cluzeau 2011
,
4 more items
-
rs132630313
De novo, Germline, Unknown
-
1/124 cases
-
-
-
Johan den Dunnen
,
Andreas Laner
,
Céline Cluzeau
+/.
24
2, 3
c.467G>A
r.(?)
p.(Arg156His)
-
pathogenic, pathogenic (dominant), pathogenic (recessive)
g.69176947G>A
g.69957097G>A
708G>A (R156H), EDA(NM_001399.5):c.467G>A (p.R156H), G708A
-
EDA_000003
located in the furin cleavage site, VKGL data sharing initiative Nederland
PubMed: Bodemer 2019
,
PubMed: Burger 2014
,
PubMed: Cluzeau 2011
,
PubMed: Martínez-Romero 2019
,
6 more items
-
-
CLASSIFICATION record, De novo, Germline, Unknown
-
8/124 cases
-
-
-
Johan den Dunnen
,
Céline Cluzeau
,
VKGL-NL_Groningen
+/.
1
-
c.467G>T
r.(?)
p.(Arg156Leu)
-
pathogenic (dominant)
g.69176947G>T
g.69957097G>T
-
-
EDA_000109
-
PubMed: Wohlfart 2016
-
-
Germline
-
1/124 cases
-
-
-
Johan den Dunnen
+/.
3
2
c.467_468del
r.(?)
p.(Arg156Glnfs*2)
-
pathogenic (dominant)
g.69176947_69176948del
g.69957097_69957098del
467_468del2
-
EDA_000041
-
PubMed: Wohlfart 2016
-
-
Germline
-
3/124 cases
-
-
-
Sigrun Maier-Wohlfart
-?/.
2
-
c.491A>C
r.(?)
p.(Glu164Ala)
-
likely benign
g.69176971A>C
g.69957121A>C
EDA(NM_001399.4):c.491A>C (p.E164A), EDA(NM_001399.5):c.491A>C (p.E164A)
-
EDA_000090
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
+/.
6
-
c.502+1G>A
r.spl, r.spl?
p.?
-
pathogenic, pathogenic (dominant)
g.69176983G>A
g.69957133G>A
EDA(NM_001399.5):c.502+1G>A
-
EDA_000079
1 heterozygous, no homozygous;
Clinindb (India)
, VKGL data sharing initiative Nederland
PubMed: Burger 2014
,
PubMed: Narang 2020
,
Journal: Narang 2020
,
PubMed: Wohlfart 2016
-
rs727505013
CLASSIFICATION record, Germline
-
1/2793 individuals, 3/124 cases
-
-
-
Johan den Dunnen
,
VKGL-NL_Groningen
,
Mohammed Faruq
-?/.
1
-
c.502+4A>G
r.spl?
p.?
-
likely benign
g.69176986A>G
-
EDA(NM_001399.5):c.502+4A>G
-
EDA_000191
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-?/.
1
2i
c.502+27544G>A
r.(?)
p.(=)
-
likely benign
g.69204526G>A
g.69984676G>A
-
-
EDA_000062
-
PubMed: Kumar 2016
,
Journal: Kumar 2016
-
-
Germline
-
-
-
-
-
Thuong Ha
+?/.
1
4
c.503-?_1176+?del
r.(?)
p.(?)
-
likely pathogenic
g.69243068_69255459del
g.70023218_70035609del
-
-
EDA_000035
1 more item
PubMed: Cluzeau 2011
-
-
Unknown
-
-
-
-
-
Céline Cluzeau
+/.
1
2i_8_
c.(502+1_503-1)_(*1_?)del
r.?
p.?
-
pathogenic (dominant)
g.(69176983_69243067)_(69255460_?)del
g.(69957133_70023217)_(70035610_?)del
exon 4-9 del
-
EDA_000095
gene exons described as 1a, 3a, 4-9
PubMed: Schneider 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
2i_8_
c.(502+1_503-1)_(*1_?)dup
r.(dup)
p.?
-
pathogenic (dominant)
g.(69176983_69243067)_(69255460_?)dup
-
dup ex3-8
-
EDA_000047
-
PubMed: Wohlfart 2016
-
-
Germline
-
1/124 cases
-
-
-
Sigrun Maier-Wohlfart
+?/.
1
3
c.516del
r.(?)
p.(Asn172Lysfs*108)
-
likely pathogenic (recessive)
g.69243081del
g.70023231del
c.516delC
-
EDA_000155
-
PubMed: Martínez-Romero 2019
-
-
De novo
-
-
-
-
X-inactivation analysis 40:60 (in unaffected non-carrier mother not informative)
Johan den Dunnen
+/.
1
3i_6i
c.527-3066_793+1017delinsGAGTACAG
r.(del)
p.?
-
pathogenic (dominant)
g.69244641_69251387delinsGAGTACAG
g.70024791_70031537delinsGAGTACAG
527-3066_793+1017del-ins8 (del ex4-6)
-
EDA_000045
1 more item
PubMed: Wohlfart 2016
-
-
Germline
-
1/124 cases
-
-
-
Sigrun Maier-Wohlfart
-/.
1
-
c.527-25del
r.(=)
p.(=)
-
benign
g.69247682del
g.70027832del
EDA(NM_001399.5):c.527-34delA
-
EDA_000070
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/., +?/.
3
-
c.527G>T
r.(?), r.spl
p.(Arg153Cys), p.(Gly176Val)
-
likely pathogenic, pathogenic (dominant)
g.69247707G>T
g.70027857G>T
g.411797G>T
-
EDA_000110
-
PubMed: Burger 2014
,
PubMed: Schneider 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Sigrun Maier-Wohlfart
+/.
5
-
c.533_552delinsCTGAA
r.(?)
p.(Lys178_Pro184delinsThrGlu)
-
pathogenic (dominant)
g.69247713_69247732delinsCTGAA
g.70027863_70027882delinsCTGAA
533_552del-ins5
-
EDA_000111
-
PubMed: Burger 2014
,
PubMed: Wohlfart 2016
-
-
Germline
-
3/124 cases
-
-
-
Johan den Dunnen
+/.
1
-
c.543_569del
r.(?)
p.(Asn185_Pro193del)
-
pathogenic
g.69247723_69247749del
g.70027873_70027899del
EDA(NM_001399.5):c.543_569delTCCTGGACCCAATGGCCCTCCAGGACC (p.N185_P193del)
-
EDA_000086
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+/.
7
4, 5
c.546_581del
r.(?)
p.(Asn185_Pro196del)
-
pathogenic, pathogenic (dominant), pathogenic (recessive)
g.69247726_69247761del
g.70027876_70027911del
536_571del36 (G180_P191del), 542_577del36, c.546_581del36, c.553_588del36,
1 more item
-
EDA_000049, EDA_000112, EDA_000113
VKGL data sharing initiative Nederland
PubMed: Burger 2014
,
PubMed: Martínez-Romero 2019
,
PubMed: van der Hout 2008
,
PubMed: Wohlfart 2016
-
-
CLASSIFICATION record, Germline
-
1/124 cases
-
-
-
Johan den Dunnen
,
Sigrun Maier-Wohlfart
,
VKGL-NL_Groningen
+/.
5
5
c.553_588del
r.(?)
p.(Asn185_Pro196del)
-
pathogenic, pathogenic (dominant), pathogenic (recessive)
g.69247733_69247768del
g.70027883_70027918del
c.553_588del36, del794_829, EDA(NM_001399.5):c.553_588del (p.N185_P196del)
-
EDA_000080
VKGL data sharing initiative Nederland
PubMed: Monreal 1998
,
PubMed: Schneider 2011
,
PubMed: van der Hout 2008
-
-
CLASSIFICATION record, De novo, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Groningen
+/.
1
-
c.557G>A
r.(?)
p.(Gly186Asp)
ACMG
pathogenic (recessive)
g.69247737G>A
-
-
-
EDA_000185
-
-
-
-
Germline
yes
-
-
-
-
Marketa Wayhelova
+/.
1
-
c.562_589del
r.(?)
p.(Pro188Argfs*83)
-
pathogenic (recessive)
g.69247742_69247769del
g.70027892_70027919del
del803-830
-
EDA_000114
-
PubMed: Monreal 1998
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+/.
5
4, 5
c.572_589del
r.(?)
p.(Pro191_Pro196del)
-
pathogenic, pathogenic (dominant), pathogenic (recessive)
g.69247752_69247769del
g.70027902_70027919del
c.559_576del18, c.572_589del18
-
EDA_000115
-
PubMed: Martínez-Romero 2019
,
PubMed: van der Hout 2008
,
PubMed: Wohlfart 2016
-
-
Germline
-
1/124 cases
-
-
-
Johan den Dunnen
+?/.
1
4
c.573_590del
r.(?)
p.(Gly192_Gln197del)
-
likely pathogenic
g.69247753_69247770del
g.70027903_70027920del
-
-
EDA_000034
-
PubMed: Cluzeau 2011
-
-
Unknown
-
-
-
-
-
Céline Cluzeau
+/.
1
-
c.574G>A
r.(?)
p.(Gly192Arg)
-
pathogenic (dominant)
g.69247754G>A
g.70027904G>A
-
-
EDA_000116
-
PubMed: Wohlfart 2016
-
-
Germline
-
1/124 cases
-
-
-
Johan den Dunnen
+?/.
1
4
c.599C>G
r.(?)
p.(Pro200Arg)
-
likely pathogenic (recessive)
g.69247779C>G
g.70027929C>G
-
-
EDA_000159
-
PubMed: Martínez-Romero 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.599dup
r.(?)
p.(Gly201ArgfsTer39)
-
pathogenic
g.69247779dup
g.70027929dup
EDA(NM_001399.5):c.599dupC (p.G201Rfs*39)
-
EDA_000081
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+/.
3
4
c.601G>T
r.(?)
p.(Gly201*)
-
pathogenic (dominant)
g.69247781G>T
g.70027931G>T
-
-
EDA_000039
-
PubMed: Wohlfart 2016
-
-
Germline
-
3/124 cases
-
-
-
Sigrun Maier-Wohlfart
+/.
2
4
c.608C>T
r.(?)
p.(Pro203Leu)
-
pathogenic (dominant)
g.69247788C>T
g.70027938C>T
-
-
EDA_000048
-
PubMed: Wohlfart 2016
-
-
Germline
-
2/124 cases
-
-
-
Sigrun Maier-Wohlfart
+?/.
1
4
c.612delG
r.(?)
p.(Ile205PhefsTer75)
-
likely pathogenic (recessive)
g.69247792del
g.70027942del
c.612delG
-
EDA_000160
-
PubMed: Martínez-Romero 2019
-
-
De novo
-
-
-
-
X-inactivation analysis 77:23 (in unaffected non-carrier mother 56:44)
Johan den Dunnen
+/.
1
4
c.612_629del
r.(?)
p.(Ile205_Gly210del)
-
pathogenic (recessive)
g.69247792_69247809del
g.70027942_70027959del
c.612_629del18
-
EDA_000161
-
PubMed: Martínez-Romero 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
4
c.620G>T
r.(?)
p.(Gly207Val)
-
likely pathogenic
g.69247800G>T
g.70027950G>T
-
-
EDA_000019
-
PubMed: Cluzeau 2011
-
-
Unknown
-
-
-
-
-
Céline Cluzeau
+/., ?/.
2
-
c.626C>T
r.(?)
p.(Pro209Leu)
-
pathogenic (recessive), VUS
g.69247806C>T
g.70027956C>T
C867T
-
EDA_000088
1 heterozygous, no homozygous;
Clinindb (India)
PubMed: Monreal 1998
,
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs132630315
Germline
-
1/2782 individuals
-
-
-
Johan den Dunnen
,
Mohammed Faruq
+?/.
1
-
c.628G>A
r.(?)
p.(Gly210Arg)
-
likely pathogenic
g.69247808G>A
g.70027958G>A
EDA(NM_001399.5):c.628G>A (p.G210R)
-
EDA_000082
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+?/.
1
4
c.632C>G
r.(?)
p.(Thr211Arg)
-
likely pathogenic
g.69247812C>G
g.70027962C>G
-
-
EDA_000020
-
PubMed: Cluzeau 2011
-
-
Unknown
-
-
-
-
-
Céline Cluzeau
+?/.
1
4
c.640dup
r.(?)
p.(Met214Asnfs*26)
-
likely pathogenic
g.69247820dup
g.70027970dup
-
-
EDA_000033
-
PubMed: Cluzeau 2011
-
-
Unknown
-
-
-
-
-
Céline Cluzeau
+/.
1
-
c.643G>A
r.(?)
p.(Gly215Arg)
-
pathogenic (dominant)
g.69247823G>A
g.70027973G>A
-
-
EDA_000117
-
PubMed: Schneider 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.644G>A
r.(?)
p.(Gly215Glu)
-
pathogenic
g.69247824G>A
g.70027974G>A
EDA(NM_001399.5):c.644G>A (p.G215E)
-
EDA_000072
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+/.
2
4
c.648_665del
r.(?)
p.(Pro220_Pro225del)
-
pathogenic (dominant)
g.69247828_69247845del
g.70027978_70027995del
648_665del18 (Pro216_Gly221del
-
EDA_000052
-
PubMed: Wohlfart 2016
-
-
Germline
-
1/124 cases
-
-
-
Johan den Dunnen
,
Sigrun Maier-Wohlfart
+/.
2
-
c.653G>A
r.(?)
p.(Gly218Asp)
-
pathogenic (dominant)
g.69247833G>A
g.70027983G>A
-
-
EDA_000118
-
PubMed: Wohlfart 2016
-
-
Germline
-
2/124 cases
-
-
-
Johan den Dunnen
+/.
1
4
c.653G>T
r.(?)
p.(Gly218Val)
-
pathogenic (recessive)
g.69247833G>T
g.70027983G>T
-
-
EDA_000162
-
PubMed: Martínez-Romero 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
8
-
c.659_676del
r.(?)
p.(Pro220_Pro225del)
-
pathogenic (dominant)
g.69247839_69247856del
g.70027989_70028006del
-
-
EDA_000119
-
PubMed: Burger 2014
,
PubMed: Schneider 2011
,
PubMed: Wohlfart 2016
-
-
Germline
-
4/124 cases
-
-
-
Johan den Dunnen
+/.
2
4
c.663_697del
r.(?)
p.(Pro222Thrfs*6), p.(Pro222ThrfsTer6)
-
pathogenic (recessive)
g.69247843_69247877del
g.70027993_70028027del
c.663_697del35, del904-938
-
EDA_000120
-
PubMed: Martínez-Romero 2019
,
PubMed: Monreal 1998
-
-
De novo, Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.671G>C
r.(?)
p.(Gly224Ala)
-
pathogenic (recessive)
g.69247851G>C
g.70028001G>C
G912C
-
EDA_000121
-
PubMed: Monreal 1998
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
-
c.671G>T
r.(?)
p.(Gly224Val)
-
pathogenic (dominant)
g.69247851G>T
g.70028001G>T
-
-
EDA_000122
-
PubMed: Burger 2014
,
PubMed: Schneider 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
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