Global Variome shared LOVD
EMD (emerin)
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Curator:
Johan den Dunnen
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Unique variants in the EMD gene
This database is one of the gene variant databases from the
Leiden Muscular Dystrophy pages
The variants shown are described using the NM_000117.2 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%)
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Operator
Column type
Example
Matches
Text
Arg
all entries containing 'Arg'
space
Text
Arg Ser
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Arg|Ser
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!
Text
!fs
all entries not containing 'fs'
^
Text
^p.(Arg
all entries beginning with 'p.(Arg'
$
Text
Ser)$
all entries ending with 'Ser)'
=""
Text
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all entries with this field empty
=""
Text
="p.0"
all entries exactly matching 'p.0'
!=""
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!=""
all entries with this field not empty
!=""
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!="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
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>
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
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all entries containing 'Asian', 'asian', including 'Caucasian', 'caucasian', etc.
Asian !Caucasian
all entries containing 'Asian' but not containing 'Caucasian'
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all entries containing 'Asian' or 'African', but not containing 'Caucasian'
"South Asian"
all entries containing 'South Asian', but not containing 'South East Asian'
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197 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
-
c.-897039_*349dup
r.0?
p.0?
-
pathogenic
g.152710806_153609906dup
-
MECP2
-
MECP2_002820
-
PubMed: Hu 2016
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
./.
1
-
c.-42004_*1112872dup
-
-
-
pathogenic
g.153565841_154722429dup
g.154337626_155494214dup
-
-
DKC1_000000
increased gene dosage
PubMed: DDDS 2015
,
Journal: DDDS 2015
-
-
Germline
-
-
-
-
-
Johan den Dunnen
./.
1
-
c.-37437_*251551dup
r.0?
p.0?
-
pathogenic
g.153570408_153861108dup
g.154342193_154632893dup
GDI1
-
FLNA_000307
-
PubMed: Hu 2016
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
1
c.-4721_*13846del
r.0
p.0
-
pathogenic
g.153603124_153623403del
g.154374755_154395034del
-
-
EMD_000008
21kb deletion
PubMed: Small
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
1
-
c.-2784A>G
r.(?)
p.(=)
-
benign
g.153605061A>G
-
FLNA(NM_001110556.2):c.-2304T>C
-
FLNA_000573
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
1
-
c.(?_-252)_(*252_?)del
r.?
p.?
-
pathogenic (recessive)
g.(?_153607593)_(153609809_?)del
-
chrX:153607593-153609809
-
EMD_000187
2.2 kb deletion
PubMed: Topf 2020
-
-
Germline
-
1/1001 cases
-
-
-
Johan den Dunnen
+/.
6
1, _1_6_
c.(?_-248)_(*326_?)del
r.0, r.0?
p.0, p.0?
-
pathogenic
g.(?_153607597del)_(153609883_?)del, g.[153551430_(1535714)del;153603341_(153613227)del]
-
-
-
EMD_000007, EMD_000059
34kb deletion in inverted FLNA/EMD allele, breakpoints repeat unit estimated,
1 more item
EMD-Muller, 1996, EMD-Wehnert, 1998,
PubMed: Fujimoto
,
PubMed: Funakoshi 1999
,
PubMed: Small 1998
,
1 more item
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
1
1
c.(?_-248)_(*326_?)inv
r.(=)
p.(=)
-
benign
g.(153564000_153607597)_(153609883_153624000)inv
-
-
-
EMD_000089
48kb inversion FLNA/EMD
PubMed: Small 1997
-
-
Germline
-
19/106 controls
-
-
-
Johan den Dunnen
?/.
2
1
c.-139_-118del
r.(?)
p.(=)
-
VUS
g.153607706_153607727del
g.154379346_154379367del
-150_-129del
-
EMD_000001
-
PubMed: Manilal
,
PubMed: Wulff
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
1
c.?
r.55_83del
p.Tyr19Ilefs*4
-
pathogenic
g.?
-
-
-
EMD_000012
-
PubMed: Bione
,
PubMed: Romeo
,
PubMed: Sabatelli 1998
,
OMIM:var0003
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
4
1
c.1A>G
r.(?), r.1a>g
p.0?
-
pathogenic
g.153607845A>G
g.154379485A>G
-
-
EMD_000058
-
EMD-Toniolo, 1996, EMD-Toniolo, 1998,
PubMed: Bione
,
OMIM:var0002
,
PubMed: Markiewicz 2002
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
1
c.2T>C
r.(?)
p.0?
-
pathogenic
g.153607846T>C
g.154379486T>C
-
-
EMD_000002
-
PubMed: Yates
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
1
c.2T>G
r.(?)
p.0?
-
pathogenic
g.153607846T>G
g.154379486T>G
-
-
EMD_000003
-
PubMed: Manilal
,
PubMed: Yates
,
PubMed: Paquis
,
PubMed: Mora
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., ?/.
3
1
c.3G>A
r.(?), r.3g>a
p.(Met1?), p.0, p.0?
-
pathogenic, VUS
g.153607847G>A
g.154379487G>A
-
-
EMD_000004
-
from website {DBsub-Emory},
1 more item
-
-
Germline, Unknown
-
-
-
-
-
Ieke Ginjaar
,
Lab Müller-Reible
,
Madhuri Hegde
+/.
11
1
c.(3_31del?), c.3_31del
r.(?)
p.(fs*), p.0?
-
pathogenic
g.153607847_153607875del, g.?
g.154379487_154379515del
29bp del
-
EMD_000000, EMD_000087
-
PubMed: Boriani 2003
,
PubMed: Funakoshi 1999
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
1
c.7_188-23delinsN[130]
r.?
p.(Asn3*)
-
pathogenic
g.153607851_153608279delinsN[130]
-
7_188-23del429ins103
-
EMD_000015
insertion not specified
PubMed: Bione
,
PubMed: Yates
,
PubMed: Yates
,
PubMed: Ellis 1998
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
1
c.12C>T
r.(?)
p.(=)
-
VUS
g.153607856C>T
g.154379496C>T
-
-
EMD_000148
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
1
-
c.19C>G
r.(?)
p.(Leu7Val)
-
VUS
g.153607863C>G
-
EMD(NM_000117.3):c.19C>G (p.L7V)
-
FLNA_000556
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/.
1
-
c.30C>G
r.(?)
p.(Thr10=)
-
likely benign
g.153607874C>G
g.154379514C>G
-
-
FLNA_000382
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
1
c.31del
r.(?)
p.(Glu11Serfs*2)
-
pathogenic
g.153607875del
g.154379515del
31delG
-
EMD_000061
-
PubMed: Kubo
,
PubMed: Funakoshi 1999
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
1
c.57C>G
r.(?)
p.(Tyr19*)
-
pathogenic
g.153607901C>G
g.154379541C>G
-
-
EMD_000060
-
EMD-Toniolo, 1996
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
1
-
c.66G>T
r.(?)
p.(Pro22=)
-
benign
g.153607910G>T
g.154379550G>T
EMD(NM_000117.3):c.66G>T (p.P22=)
-
FLNA_000383
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
1
-
c.71G>C
r.(?)
p.(Gly24Ala)
-
VUS
g.153607915G>C
g.154379555G>C
-
-
FLNA_000384
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
2
1
c.82+1del
r.54_82del, r.spl
p.?, p.Tyr19Ilefs*4
-
pathogenic
g.153607927del
g.154379567del
82+1delG
-
EMD_000005
c.82+1delG
PubMed: Bione
,
PubMed: Manilal
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
1
c.82+1G>T
r.spl
p.?
-
pathogenic
g.153607927G>T
g.154379567G>T
-
-
EMD_000062
-
PubMed: Nigro
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
1
c.82+5G>C
r.54_82del
p.fs?
-
pathogenic
g.153607931G>C
g.154379571G>C
-
-
EMD_000006
-
PubMed: Hasegawa
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.82+5G>T
r.spl?
p.?
-
likely benign
g.153607931G>T
g.154379571G>T
-
-
FLNA_000385
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
1
c.82+6_82+7insCG
r.spl?
p.(?)
-
pathogenic
g.153607932_153607933insCG
g.154379572_154379573insCG
-
-
EMD_000063
-
PubMed: Bione
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.82+17G>A
r.(=)
p.(=)
-
likely benign
g.153607943G>A
g.154379583G>A
EMD(NM_000117.3):c.82+17G>A
-
EMD_000117
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-/.
2
-
c.83-13C>G
r.(=)
p.(=)
-
benign
g.153608037C>G
g.154379677C>G
EMD(NM_000117.2):c.83-13C>G, EMD(NM_000117.3):c.83-13C>G
-
EMD_000161
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
+/.
1
2
c.83-4_83del
r.spl
p.?
-
pathogenic
g.153608046_153608050del
g.154379686_154379690del
-
-
EMD_000021
-
PubMed: Yates
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
2
c.83-2A>C
r.spl
p.?
-
pathogenic
g.153608048A>C
g.154379688A>C
-
-
EMD_000025
-
PubMed: Bione
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.83-2A>G
r.spl?
p.?
-
pathogenic
g.153608048A>G
-
-
-
EMD_000200
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
2
c.96_155del
r.(?)
p.(Arg32_Pro51del)
-
pathogenic
g.153608063_153608122del
g.154379703_154379762del
-
-
EMD_000020
-
PubMed: Talkop
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
2
c.99_157del
r.(?)
p.(Tyr34Valfs*7)
-
pathogenic
g.153608066_153608124del
g.154379706_154379764del
59nt del g.329_388del
-
EMD_000088
-
Wehnert (1999) Hum.Genet. 104:195
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
2
c.100del
r.(?)
p.(Tyr34Thrfs*31)
-
pathogenic
g.153608067del
g.154379707del
100delT
-
EMD_000009
-
PubMed: Nevo 2001
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
2
c.102C>G
r.(?), r.102c>g
p.(Tyr34*), p.Tyr34*
-
pathogenic
g.153608069C>G
g.154379709C>G
-
-
EMD_000010
-
Tverskaya ESHG2006 P0740,
PubMed: Yates
,
PubMed: Yates
,
PubMed: de Koning Gans
-
-
Germline
-
-
-
-
-
Ieke Ginjaar
,
Svetlana Tverskaya
-?/.
1
-
c.105G>A
r.(?)
p.(Glu35=)
-
likely benign
g.153608072G>A
-
EMD(NM_000117.2):c.105G>A (p.E35=)
-
EMD_000184
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
2
c.106A>T
r.(?)
p.(Lys36*)
-
pathogenic
g.153608073A>T
g.154379713A>T
-
-
EMD_000107
-
PubMed: Nigro 2010
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/., -?/.
3
-
c.108G>A
r.(?)
p.(Lys36=)
-
benign, likely benign
g.153608075G>A
g.154379715G>A
EMD(NM_000117.2):c.108G>A (p.K36=), EMD(NM_000117.3):c.108G>A (p.K36=)
-
EMD_000118
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Nijmegen
,
VKGL-NL_AMC
+?/.
1
-
c.110_112del
r.(?)
p.(Lys37del)
-
likely pathogenic
g.153608077_153608079del
g.154379717_154379719del
-
-
EMD_000162
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
2
c.123C>G
r.(?)
p.(Tyr41*)
-
pathogenic
g.153608090C>G
g.154379730C>G
-
-
EMD_000064
-
PubMed: Bione
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
2
c.124G>T
r.(?)
p.(Glu42*)
-
pathogenic
g.153608091G>T
g.154379731G>T
-
-
EMD_000115
-
-
-
-
Germline
-
-
-
-
-
Tom Winder
+/.
4
2
c.130C>T
r.(?), r.130c>u
p.(Gln44*), p.Gln44*
-
pathogenic
g.153608097C>T
g.154379737C>T
-
-
EMD_000011
RNA reduced
EMD-Kaplan, 1998,
PubMed: Buckley 1999
,
PubMed: Manilal
,
PubMed: Manilal
,
1 more item
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
1
-
c.132G>A
r.(?)
p.(Gln44=)
-
benign
g.153608099G>A
g.154379739G>A
EMD(NM_000117.3):c.132G>A (p.Q44=)
-
EMD_000182
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
1
2
c.138_163del
r.138_163del
p.Ser49Leufs*3
-
pathogenic
g.153608105_153608130del
g.154379745_154379770del
-
-
EMD_000013
-
PubMed: Nevo 1999
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
2
c.139_140dup
r.139_140dup
p.Leu48Glyfs*18
-
pathogenic
g.153608106_153608107dup
g.154379746_154379747dup
-
-
EMD_000024
-
PubMed: Bione
,
PubMed: Romeo
,
OMIM:var0004
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
2
-
c.144C>T
r.(?)
p.(Leu48=)
-
benign
g.153608111C>T
g.154379751C>T
EMD(NM_000117.2):c.144C>T (p.L48=), EMD(NM_000117.3):c.144C>T (p.L48=)
-
EMD_000119
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
?/.
2
-
c.148C>T
r.(?)
p.(Pro50Ser)
-
VUS
g.153608115C>T
g.154379755C>T
EMD(NM_000117.3):c.148C>T (p.P50S)
-
EMD_000163
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
,
VKGL-NL_AMC
?/.
3
2
c.149C>A
r.(?)
p.(Pro50His)
-
VUS
g.153608116C>A
g.154379756C>A
EMD(NM_000117.3):c.149C>A (p.P50H)
-
EMD_000120
VKGL data sharing initiative Nederland
PubMed: Nallamilli 2018
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Madhuri Hegde
,
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
+/.
5
2
c.153del
r.(?)
p.(Ser52Alafs*13)
-
pathogenic
g.153608120del
g.154379760del
153delC
-
EMD_000014
-
EMD-D.Toniolo, 1998, EMD-Toniolo, 1996,
PubMed: Manilal
,
2 more items
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.157T>G
r.(?)
p.(Ser53Ala)
-
VUS
g.153608124T>G
g.154379764T>G
-
-
EMD_000178
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
2
c.161C>T
r.(?)
p.(Ser54Phe)
-
pathogenic
g.153608128C>T
g.154379768C>T
-
-
EMD_000065
-
PubMed: Ellis
, EMD-Muller, 1996
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.165C>A
r.(?)
p.(Ala55=)
-
likely benign
g.153608132C>A
g.154379772C>A
-
-
EMD_000164
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
2
c.165del
r.(?)
p.(Ala56Profs*9)
-
pathogenic
g.153608132del
g.154379772del
164delC
-
EMD_000095
-
UMD: Wehnert unpublished
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
2
c.174_175del
r.(?)
p.(Tyr59*)
-
pathogenic
g.153608141_153608142del
g.154379781_154379782del
-
-
EMD_000066
-
EMD-Kaplan, 1998
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
2
c.177T>A
r.(?)
p.(Tyr59*)
-
pathogenic
g.153608144T>A
g.154379784T>A
-
-
EMD_000016
-
EMD-Toniolo, 1996,
PubMed: Yates
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
2
c.178dup
r.(?)
p.(Ser60Lysfs*4)
-
pathogenic
g.153608145dup
g.154379785dup
178dupA
-
EMD_000017
-
PubMed: Nevo 2001
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
2
c.184dup
r.(?)
p.(Ser62Phefs*2)
-
pathogenic
g.153608151dup
g.154379791dup
184dupT
-
EMD_000018
-
PubMed: Canki-Klain 2000
-
-
Germline
-
-
MboII+
-
-
Johan den Dunnen
+/.
1
2
c.186dup
r.(?)
p.(Asp63*)
-
pathogenic
g.153608153dup
g.154379793dup
186dupT
-
EMD_000104
-
PubMed: Yates
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
2
c.187+1G>A
r.spl
p.?
-
pathogenic
g.153608155G>A
g.154379795G>A
-
-
EMD_000019
-
PubMed: Yates
,
PubMed: Yates
,
PubMed: Deymeer
,
PubMed: Yates
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
3
c.187+1G>C
r.spl
p.?
ACMG
pathogenic (maternal)
g.153608155G>C
g.154379795G>C
-
-
EMD_000195
-
-
-
-
Germline/De novo (untested)
yes
-
-
-
-
Emanuele Micaglio
-/.
1
-
c.188-13C>T
r.(=)
p.(=)
-
benign
g.153608289C>T
-
EMD(NM_000117.3):c.188-13C>T
-
EMD_000121
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/.
1
-
c.188-13_188-11del
r.(=)
p.(=)
-
benign
g.153608289_153608291del
g.154379929_154379931del
EMD(NM_000117.3):c.188-13_188-11delCCT
-
EMD_000122
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/.
2
-
c.188-12C>T
r.(=)
p.(=)
-
likely benign
g.153608290C>T
g.154379930C>T
EMD(NM_000117.2):c.188-12C>T
-
EMD_000123
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
-?/.
1
-
c.206G>A
r.(?)
p.(Gly69Glu)
-
likely benign
g.153608320G>A
-
EMD(NM_000117.2):c.206G>A (p.G69E)
-
EMD_000185
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
2
-
c.212C>T
r.(?)
p.(Ala71Val)
-
likely benign
g.153608326C>T
g.154379966C>T
EMD(NM_000117.2):c.212C>T (p.A71V)
-
EMD_000124
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
+/., ?/.
2
3
c.215A>T
r.(?)
p.(Asp72Val)
-
pathogenic, VUS
g.153608329A>T
g.154379969A>T
-
-
EMD_000100
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
,
Svetlana Tverskaya
+/.
1
3
c.221_222del
r.(?)
p.(Tyr74*)
-
pathogenic
g.153608335_153608336del
g.154379975_154379976del
221_222delAT
-
EMD_000068
-
PubMed: Bione
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/., -?/.
3
-
c.234G>A
r.(?)
p.(Lys78=)
-
benign, likely benign
g.153608348G>A
g.154379988G>A
EMD(NM_000117.2):c.234G>A (p.K78=), EMD(NM_000117.3):c.234G>A (p.K78=)
-
EMD_000125
VKGL data sharing initiative Nederland
-
-
rs781889152
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
,
MobiDetails
+/.
1
3
c.239_240del
r.(?)
p.(Glu80Glyfs*12)
-
pathogenic
g.153608353_153608354del
g.154379993_154379994del
-
-
EMD_000022
-
PubMed: Wulff
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.241G>A
r.(?)
p.(Asp81Asn)
-
likely benign
g.153608355G>A
-
EMD(NM_000117.2):c.241G>A (p.D81N)
-
EMD_000189
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
3
c.243C>T
r.(?)
p.(=)
-
VUS
g.153608357C>T
g.154379997C>T
-
-
EMD_000149
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/.
5
3
c.251_255del
r.(?)
p.(Leu84Profs*7)
-
pathogenic
g.153608365_153608369del
g.154380005_154380009del
251_255delTCTAC
-
EMD_000023
de novo, germline mosaicism in mother, no second variant
EMD-Yates, 1998,
PubMed: Hoeltzenbein
,
PubMed: Manilal
,
PubMed: Hoeltzenbein
,
OMIM:var0010
,
2 more items
-
-
De novo, Germline
-
-
-
-
-
Johan den Dunnen
,
Madhuri Hegde
+/.
1
3
c.256C>T
r.(?)
p.(Gln86*)
-
pathogenic
g.153608370C>T
g.154380010C>T
-
-
EMD_000069
-
EMD-Toniolo, 1998
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
1
-
c.265+12G>C
r.(=)
p.(=)
-
benign
g.153608391G>C
g.154380031G>C
EMD(NM_000117.3):c.265+12G>C
-
EMD_000126
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+?/.
1
-
c.266-35_266-18del
r.(=)
p.(=)
-
likely pathogenic
g.153608559_153608576del
-
EMD(NM_000117.3):c.266-35_266-18delATGCCCCCTCTGCTACCG
-
EMD_000165
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
-?/.
1
-
c.266-35_266-18dup
r.(=)
p.(=)
-
likely benign
g.153608559_153608576dup
g.154380199_154380216dup
EMD(NM_000117.3):c.266-17delCinsATGCCCCCTCTGCTACCGC
-
EMD_000127
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+/., +?/.
2
3i, 4
c.266-27_266-10del
r.(spl?), r.spl?
p.(=), p.(?)
-
likely pathogenic, pathogenic
g.153608567_153608584del
g.154380207_154380224del
-
-
EMD_000071
variant found in affected maternal grandfather
Wehnert (2000) Hum.Genet. 105:151
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Tom Winder
+/.
2
4
c.266-3A>G
r.[266_308del, 265_266ins266-86_266-1;266-3a>g, 26, r.[266_308del,265_266ins[265+1_266-4;gag]]
p.fs?, p.[Tyr90Leufs*18,Tyr90Lysfs*25]
-
pathogenic, pathogenic (recessive)
g.153608591A>G
g.154380231A>G
-
-
EMD_000028
cryptic splice acceptor, intron retention
PubMed: Bione
,
OMIM:var0005
,
PubMed: Bournazos 2022
-
-
Germline, Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
+/.
3
3i, 4
c.266-2A>G
r.265_266insg, r.spl
p.?, p.Tyr90Leufs*3
-
pathogenic
g.153608592A>G
g.154380232A>G
-
-
EMD_000027
-
from website {DBsub-Emory},
PubMed: Nevo 1999
,
1 more item
-
-
Germline, Unknown
-
-
-
-
-
Johan den Dunnen
,
Madhuri Hegde
+/.
1
4
c.266-1G>C
r.spl
p.?
-
pathogenic
g.153608593G>C
g.154380233G>C
-
-
EMD_000070
-
EMD-Kress, 1998
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
4
c.266-?_*325+?del
r.(266_1090del?)
p.(?)
-
pathogenic
g.153608594_153609882del
-
-
-
EMD_000026
1 more item
PubMed: Manilal
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/., -?/.
2
-
c.272A>G
r.(?)
p.(Asn91Ser)
-
benign, likely benign
g.153608600A>G
g.154380240A>G
EMD(NM_000117.3):c.272A>G (p.N91S)
-
EMD_000166
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
,
VKGL-NL_AMC
-/., -?/.
2
-
c.276C>T
r.(?)
p.(Asp92=)
-
benign, likely benign
g.153608604C>T
g.154380244C>T
EMD(NM_000117.2):c.276C>T (p.D92=), EMD(NM_000117.3):c.276C>T (p.D92=)
-
EMD_000167
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
,
VKGL-NL_AMC
+/.
1
-
c.282C>A
r.(?)
p.(Tyr94Ter)
-
pathogenic
g.153608610C>A
-
-
-
EMD_000192
-
-
-
-
Unknown
-
-
-
-
-
IMGAG
+/.
1
4
c.282C>G
r.(?)
p.(Tyr94*)
-
pathogenic
g.153608610C>G
g.154380250C>G
-
-
EMD_000029
-
PubMed: Yates
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., ?/.
2
4
c.284_298del
r.(?)
p.(Tyr95_Tyr99del)
-
pathogenic, VUS
g.153608612_153608626del
g.154380252_154380266del
284_298delATGAAGAGAGCTACT
-
EMD_000030
-
from website {DBsub-Emory},
1 more item
-
-
Germline, Unknown
-
-
-
-
-
Johan den Dunnen
,
Madhuri Hegde
?/.
1
4
c.295T>C
r.(?)
p.(Tyr99His)
-
VUS
g.153608623T>C
g.154380263T>C
-
-
EMD_000150
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/.
1
4
c.301dup
r.(?)
p.(Thr101Asnfs*28)
-
pathogenic
g.153608629dup
g.154380269dup
301dupA
-
EMD_000031
-
PubMed: Wulff
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
4
c.314_315del
r.(?)
p.(Tyr105Trpfs*23)
-
pathogenic
g.153608642_153608643del
g.154380282_154380283del
-
-
EMD_000032
-
PubMed: Manilal
,
PubMed: Wulff
,
PubMed: Wulff 1997
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
3
4
c.315T>G
r.(?)
p.(Tyr105*)
-
pathogenic
g.153608643T>G
g.154380283T>G
-
-
EMD_000034
-
PubMed: Manilal
,
PubMed: Muntoni
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.320A>T
r.(?)
p.(Glu107Val)
-
VUS
g.153608648A>T
g.154380288A>T
EMD(NM_000117.2):c.320A>T (p.E107V)
-
EMD_000168
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/.
2
4
c.325G>T
r.(?)
p.(Glu109*)
-
pathogenic
g.153608653G>T
g.154380293G>T
-
-
EMD_000035
-
PubMed: Bione
,
PubMed: Kress
,
PubMed: Manilal
,
PubMed: Yates
,
PubMed: Hodgson
, {PMID1525559 :Cole}
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.353G>A
r.(?)
p.(Arg118His)
-
VUS
g.153608681G>A
g.154380321G>A
-
-
EMD_000169
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
4
c.355C>A
r.(?)
p.(Gln119Lys)
-
VUS
g.153608683C>A
g.154380323C>A
-
-
EMD_000151
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/.
1
4
c.356dup
r.(?)
p.(Ser120Valfs*9)
-
pathogenic
g.153608684dup
g.154380324dup
356dupA
-
EMD_000091
-
-
-
-
Germline
-
-
-
-
-
Rosário dos Santos
+/.
1
4
c.358del
r.(?)
p.(Ser120Glnfs*2)
-
pathogenic
g.153608686del
g.154380326del
358delT
-
EMD_000074
-
EMD-Wehnert, 1998
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., -?/.
2
4
c.359_362del
r.(?)
p.(Ser120*), p.(Ser120Ter)
-
likely benign, pathogenic
g.153608687_153608690del
g.154380327_154380330del
EMD(NM_000117.3):c.359_362delCAGT (p.S120*)
-
EMD_000036, EMD_000128
VKGL data sharing initiative Nederland
PubMed: Manilal
,
PubMed: Yates
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_AMC
?/.
1
4
c.385G>A
r.(?)
p.(Ala129Thr)
-
VUS
g.153608713G>A
g.154380353G>A
-
-
EMD_000152
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
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