Global Variome shared LOVD
DOK7 (docking protein 7)
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Curators:
Tom Winder
and
Johan den Dunnen
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Unique variants in the DOK7 gene
This database is one of the gene variant databases from the
Leiden Muscular Dystrophy pages
The variants shown are described using the NM_173660.4 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.
176 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
-/.
2
-
c.-30G>T
r.(=), r.(?)
p.(=)
-
benign
g.3465073G>T
g.3463346G>T
-
-
DOK7_000109
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Andreas Laner
,
VKGL-NL_Nijmegen
-/.
1
-
c.-6C>G
r.(?)
p.(=)
-
benign
g.3465097C>G
g.3463370C>G
-
-
DOK7_000157
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/., +?/., -?/.
13
2, 4, 6, 7
c.?
-, r.(?), r.[55_100del, 55_532del, 101_532del]
p.?, p.Arg158Ala, p.Arg159Ala, p.Arg174Ala, p.fs*, p.Leu241Ala, p.Leu245Ala, p.Leu248Ala, p.Pro421*,
2 more items
-
NA, pathogenic
g.?
-
414C>T, 55_100del
-
DOK7_000000
expression cloning HEK 293T cells significantly reduced Tyr phoshorylation,
7 more items
PubMed: Hamuro 2008
,
PubMed: Lashley 2010
,
PubMed: Selcen 2008
-
-
Germline, In vitro (cloned)
-
-
-
-
-
Johan den Dunnen
,
Tom Winder
+/.
3
1
c.7G>A
r.(?)
p.(Glu3Lys), p.Glu3Lys
-
NA, pathogenic, pathogenic (recessive)
g.3465109G>A
g.3463382G>A
-
-
DOK7_000052
1 more item
PubMed: Cossins 2012
-
-
Germline, In vitro (cloned)
-
-
-
-
-
Johan den Dunnen
,
Angela Abicht
?/.
2
1
c.32T>C
r.(?)
p.(Ala11Val), p.(Val11Ala)
-
VUS
g.3465134T>C
g.3463407T>C
-
-
DOK7_000032
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Tom Winder
,
VKGL-NL_Nijmegen
-?/.
1
1
c.42G>C
r.(?)
p.(=)
-
likely benign
g.3465144G>C
g.3463417G>C
R14R
-
DOK7_000075
-
PubMed: Cossins 2012
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
1
c.48C>T
r.0?
p.0?
-
pathogenic (recessive)
g.3465150C>T
g.3463423C>T
r.1-119del
-
DOK7_000058
exon trap cloning exon skipped
PubMed: Cossins 2012
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
3
1i
c.54+12_54+23del
r.spl
p.(=), p.?
-
likely pathogenic, likely pathogenic (recessive)
g.3465168_3465179del
g.3463441_3463452del
54+11_54+22del
-
DOK7_000140
-
PubMed: Gonzalez-Quereda 2020
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
,
Ana Topf
+/., +?/.
7
1i
c.54+25_55-38del
r.54_55ins54+1_55-1{54+14_55-38del}, r.spl?, r.[54_55ins54+1_55-1; 54+14_55-38del]
p.?, p.Trp19Valfs*22, p.Trp19Valfs*23
-
likely pathogenic, pathogenic
g.3465181_3465195del
g.3463454_3463468del
54+14_+28delGGGGGGGGGGGGCGC, 54+19_54+33delCGCGGGGGGGGGGGG, 54+22_36del, IVS1+14del15
-
DOK7_000014
intron 1 inclusion; not in 200 control chromosomes; intron 1 inclusion 100 control cDNAs
PubMed: BenAmmar 2010
,
PubMed: Lashley 2010
,
PubMed: Schara 2009
,
PubMed: Selcen 2008
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Tom Winder
+/.
4
1i
c.54+25_55+38del
r.spl?
p.?
-
pathogenic (recessive)
g.3465181_3465271del
-
54+25_39del15
-
DOK7_000059
1 more item
PubMed: Cossins 2012
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
1
-
c.54+32_54+33del
r.(=)
p.(=)
-
benign
g.3465188_3465189del
g.3463461_3463462del
-
-
DOK7_000132
-
-
-
rs71180187
Germline
-
-
-
-
-
Andreas Laner
+/.
1
1i
c.55-2A>C
r.55_65del
p.Trp19Valfs*70
-
pathogenic
g.3465231A>C
g.3463504A>C
IVS1-2A>C
-
DOK7_000016
-
PubMed: Selcen 2008
-
-
Germline
-
-
-
-
-
Tom Winder
+/.
2
2
c.91C>A
r.(?)
p.(Pro31Thr), p.Pro31Thr
-
NA, pathogenic (recessive)
g.3465269C>A
g.3463542C>A
-
-
DOK7_000060
1 more item
PubMed: Cossins 2012
-
-
Germline, In vitro (cloned)
-
-
-
-
-
Johan den Dunnen
+/.
3
1, 2
c.98C>T
r.(?)
p.(Ala33Val), p.Ala33Val
-
NA, pathogenic
g.3465276C>T
g.3463549C>T
98G>A
-
DOK7_000025
expression cloning, fails to induce MUSK (Tyr phosphorylation),
1 more item
PubMed: Hamuro 2008
,
PubMed: Muller 2007
-
-
Germline, In vitro (cloned)
-
-
-
-
-
Johan den Dunnen
,
Angela Abicht
+/.
2
2i_3
c.101-124_141del
r.spl?
p.?
-
pathogenic (recessive)
g.3475009_3475173del
g.3473282_3473446del
-
-
DOK7_000061
-
PubMed: Cossins 2012
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
1
-
c.101-26G>A
r.(=)
p.(=)
-
benign
g.3475107G>A
g.3473380G>A
-
-
DOK7_000133
-
-
-
-
Germline
-
-
-
-
-
Andreas Laner
+/.
1
2i_3
c.101-24_141del
r.spl?
p.?
-
pathogenic
g.3475109_3475173del
g.3473382_3473446del
-
-
DOK7_000050
-
PubMed: Jephson 2010
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
2i
c.101-19A>G
r.spl
p.(=)
-
VUS
g.3475114A>G
g.3473387A>G
-
-
DOK7_000057
-
-
-
-
Unknown
-
-
-
-
-
Damien Sternberg
-/.
1
-
c.101-11G>A
r.(=)
p.(=)
-
benign
g.3475122G>A
g.3473395G>A
-
-
DOK7_000158
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
2i
c.101-1G>C
r.spl
p.?
-
pathogenic
g.3475132G>C
g.3473405G>C
IVS2-1G>T
-
DOK7_000008
-
PubMed: Beeson 2006
,
OMIM:var0008
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
2i
c.101-1G>T
r.spl
p.?
-
pathogenic
g.3475132G>T
g.3473405G>T
IVS2-1G>T
-
DOK7_000029
exon trap cloning suggests ex3 skipping
PubMed: Muller 2007
-
-
Germline
-
-
-
-
-
Angela Abicht
+/.
1
2i_6i
c.(100+1_101-1)_(772+1_773-1)del
r.101_772del
p.fs*
-
pathogenic
g.(3465279_3475132)_(3491524_3494485)del
-
101_652del
-
DOK7_000012
1 more item
PubMed: Selcen 2008
-
-
Germline
-
-
-
-
-
Tom Winder
+/.
1
3
c.101_331del
r.(?)
p.Asp34_Glu110del
-
NA
g.3475133_3475363del
g.3473406_3473636del
-
-
DOK7_000000
1 more item
PubMed: Selcen 2008
-
-
In vitro (cloned)
-
-
-
-
-
Tom Winder
+/.
1
3
c.101_532del
r.(?)
p.Asp34_Trp178delinsGly
-
NA
g.3475133_3478269del
g.3473406_3476542del
-
-
DOK7_000000
pCMV-Tag2 (co-)expression cloning with MUSK in HEK293/C2C12 cells; failure to phosphorylate MUSK
PubMed: Selcen 2008
-
-
In vitro (cloned)
-
-
-
-
-
Tom Winder
+/.
1
3
c.101_772del
r.(?)
p.Asp34_Gly257del
-
NA
g.3475133_3491523del
g.3473406_3489796del
-
-
DOK7_000000
1 more item
PubMed: Selcen 2008
-
-
In vitro (cloned)
-
-
-
-
-
Tom Winder
+/., -/., -?/., ?/.
8
3
c.134C>T
r.(?)
p.(Ser45Leu)
-
benign, likely benign, pathogenic, VUS
g.3475166C>T
g.3473439C>T
134C>T, DOK7(NM_001164673.1):c.134C>T (p.(Ser45Leu)), DOK7(NM_173660.4):c.134C>T (p.S45L)
-
DOK7_000026
VKGL data sharing initiative Nederland
PubMed: BenAmmar 2010
,
PubMed: Cossins 2012
,
PubMed: Mihaylova 2010
,
PubMed: Muller 2007
-
rs62272670
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
Angela Abicht
,
Andreas Laner
,
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
-?/.
1
3
c.160C>T
r.(?)
p.(Arg54Cys)
-
likely benign
g.3475192C>T
g.3473465C>T
Arg54Tyr)
-
DOK7_000076
-
PubMed: Cossins 2012
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
3
3
c.176_206delinsAG
r.(?), r.?)
p.(Leu59GlnfsTer24)
-
pathogenic, pathogenic (recessive)
g.3475208_3475238delinsAG
g.3473481_3473511delinsAG
-
-
DOK7_000117
-
PubMed: Cossins 2012
,
PubMed: Jephson 2010
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., ?/.
2
3
c.190G>A
r.(?)
p.(Gly64Arg)
-
pathogenic, VUS
g.3475222G>A
g.3473495G>A
-
-
DOK7_000053
PolyPhen-2: prob. dam. (PSIC:0,995), Mut.Taster: disease causing
-
-
-
Germline
-
-
-
-
-
Angela Abicht
,
Andreas Laner
+/.
2
3
c.190G>M
r.(?)
p.(Gly64Arg)
-
pathogenic
g.3475222G>M
g.3473495G>M
190G>A?
-
DOK7_000039
-
PubMed: Mihaylova 2010
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
1
-
c.220=
r.(=)
p.(Leu74=)
-
benign
g.3475252T>C
g.3473525=
DOK7(NM_173660.5):c.220T>C (p.L74=)
-
DOK7_000177
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-/., -?/.
2
3
c.220C>T
r.(?)
p.(=)
-
benign, likely benign
g.3475252=
g.3473525C>T
L74L
-
DOK7_000078
-
PubMed: Cossins 2012
-
rs4325970
Germline, Unknown
?
0.99 controls
-
-
-
Johan den Dunnen
,
Florian Erger
+/.
2
3
c.230C>T
r.(?)
p.(Thr77Met), p.Thr77Met
-
NA, pathogenic (recessive)
g.3475262C>T
g.3473535C>T
-
-
DOK7_000062
1 more item
PubMed: Cossins 2012
-
-
Germline, In vitro (cloned)
-
-
-
-
-
Johan den Dunnen
?/.
1
3
c.245G>T
r.(?)
p.(Cys82Phe)
-
VUS
g.3475277G>T
g.3473550G>T
-
-
DOK7_000036
-
-
-
-
Germline
-
-
-
-
-
Tom Winder
-?/.
2
3
c.282C>T
r.(?)
p.(=), p.(His94=)
-
likely benign
g.3475314C>T
g.3473587C>T
DOK7(NM_001164673.1):c.282C>T (p.(His94=)), H49H
-
DOK7_000077
VKGL data sharing initiative Nederland
PubMed: Cossins 2012
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Leiden
-?/., ?/.
2
3
c.296C>T
r.(?)
p.(Ala99Val)
-
likely benign, VUS
g.3475328C>T
g.3473601C>T
-
-
DOK7_000079
conflicting interpretations of pathogenicity; 1 heterozygous, no homozygous;
Clinindb (India)
PubMed: Cossins 2012
,
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs138010842
Germline
-
1/2793 individuals
-
-
-
Johan den Dunnen
,
Mohammed Faruq
?/.
1
-
c.308G>A
r.(?)
p.(Arg103Gln)
-
VUS
g.3475340G>A
g.3473613G>A
-
-
DOK7_000160
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
2
3
c.313C>T
r.(?)
p.(Arg105Cys)
-
VUS
g.3475345C>T
g.3473618C>T
-
-
DOK7_000033
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Tom Winder
,
VKGL-NL_Nijmegen
+/.
3
3
c.325G>T
r.(?)
p.(Gly109Cys), p.Gly109Cys
-
NA, pathogenic, pathogenic (recessive)
g.3475357G>T
g.3473630G>T
-
-
DOK7_000051
1 more item
PubMed: Cossins 2012
,
PubMed: Jephson 2010
-
-
Germline, In vitro (cloned)
-
-
-
-
-
Johan den Dunnen
+/.
7
3i
c.331+1G>T
r.spl, r.[101_331del, 101_532del, 55_532del]
p.?, p.[Asp34_Glu110del, fs*]
-
pathogenic
g.3475364G>T
g.3473637G>T
IVS3+1G>T
-
DOK7_000018
not in 378 control chromosomes, unique exon 3 skip, not observed in 100 control cDNAs
PubMed: Selcen 2008
,
PubMed: Vogt 2009
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Tom Winder
-/.
1
-
c.331+27G>A
r.(=)
p.(=)
-
benign
g.3475390G>A
g.3473663G>A
-
-
DOK7_000136
-
-
-
-
Germline
-
-
-
-
-
Andreas Laner
-/.
1
-
c.332-112G>A
r.(=)
p.(=)
-
benign
g.3477957G>A
g.3476230G>A
-
-
DOK7_000137
-
-
-
rs34427903
Germline
-
-
-
-
-
Andreas Laner
-/.
1
-
c.332-82G>A
r.(=)
p.(=)
-
benign
g.3477987G>A
g.3476260G>A
-
-
DOK7_000138
-
-
-
-
Germline
-
-
-
-
-
Andreas Laner
-/.
1
-
c.332-69A>G
r.(=)
p.(=)
-
benign
g.3478000A>G
g.3476273A>G
-
-
DOK7_000139
-
-
-
rs2857985
Germline
-
-
-
-
-
Andreas Laner
-/.
1
-
c.332-56delinsAT
r.(=)
p.(=)
-
benign
g.3478013delinsAT
g.3476286delinsAT
-
-
DOK7_000105
-
-
-
-
Germline
-
-
-
-
-
Andreas Laner
-/.
1
-
c.332-50T>C
r.(=)
p.(=)
-
benign
g.3478019T>C
g.3476292T>C
-
-
DOK7_000106
-
-
-
rs114112298
Germline
-
-
-
-
-
Andreas Laner
-/.
1
-
c.332-49C>T
r.(=)
p.(=)
-
benign
g.3478020C>T
g.3476293C>T
-
-
DOK7_000107
c.332-121_65del
-
-
-
Germline
-
-
-
-
-
Andreas Laner
-?/.
1
-
c.332-43_332-18del
r.(=)
p.(=)
-
likely benign
g.3478026_3478051del
g.3476299_3476324del
DOK7(NM_001164673.1):c.332-43_332-18del (p.(=))
-
DOK7_000143
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-/.
2
-
c.332-39T>C
r.(=)
p.(=)
-
benign
g.3478030T>C
g.3476303T>C
-
-
DOK7_000108
-
-
-
rs114948213
Germline
-
-
-
-
-
Andreas Laner
-/.
2
-
c.332-38G>A
r.(=)
p.(=)
-
benign
g.3478031G>A
g.3476304G>A
-
-
DOK7_000111
-
-
-
rs115931214
Germline
-
-
-
-
-
Andreas Laner
-/.
1
-
c.332-24C>A
r.(=)
p.(=)
-
benign
g.3478045C>A
g.3476318C>A
-
-
DOK7_000112
-
-
-
rs112637134
Germline
-
-
-
-
-
Andreas Laner
-/.
1
-
c.332-18T>C
r.(=)
p.(=)
-
benign
g.3478051T>C
g.3476324T>C
-
-
DOK7_000161
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/.
1
4i
c.332-1G>T
r.spl
p.?
-
likely pathogenic
g.3478068G>T
g.3476341G>T
-
-
DOK7_000103
-
-
-
-
Germline
-
-
-
-
-
Tom Winder
+/.
1
4
c.346G>A
r.(?)
p.(Val116Met)
-
pathogenic
g.3478083G>A
g.3476356G>A
-
-
DOK7_000042
-
PubMed: BenAmmar 2010
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
4
c.379G>A
r.(?)
p.(Gly127Ser)
-
likely pathogenic
g.3478116G>A
g.3476389G>A
-
-
DOK7_000102
-
-
-
-
Germline
-
-
-
-
-
Tom Winder
+/.
3
4
c.396C>G
r.(?)
p.(His132Gln)
-
pathogenic
g.3478133C>G
g.3476406C>G
396C>G
-
DOK7_000022
-
PubMed: Muller 2007
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Angela Abicht
-?/.
1
-
c.405T>C
r.(?)
p.(=)
-
likely benign
g.3478142T>C
-
DOK7(NM_001164673.1):c.405T>C (p.(Asn135=))
-
DOK7_000201
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
3
4
c.414C>T
r.[=, 332_425del]
p.[(=, Val111Glyfs*104)]
-
pathogenic (recessive)
g.3478151C>T
g.3476424C>T
r.[=, 397_490del]
-
DOK7_000063
exon trap cloning
PubMed: Cossins 2012
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
5
4
c.415G>C
r.(?)
p.(Val139Leu), p.Val139Leu
-
NA, pathogenic, pathogenic (recessive)
g.3478152G>C
g.3476425G>C
-
-
DOK7_000049
1 more item
PubMed: Cossins 2012
,
PubMed: Jephson 2010
-
-
Germline, In vitro (cloned)
-
-
-
-
-
Johan den Dunnen
+/., ?/.
4
4
c.437C>T
r.(?)
p.(Pro146Leu)
-
pathogenic, VUS
g.3478174C>T
g.3476447C>T
-
-
DOK7_000034
functional studies needed, VKGL data sharing initiative Nederland
PubMed: BenAmmar 2010
,
PubMed: Westra 2019
-
-
CLASSIFICATION record, Germline, Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
,
Tom Winder
,
VKGL-NL_Nijmegen
+/.
3
4
c.437del
r.(?)
p.(Pro146Argfs*100)
-
pathogenic (recessive)
g.3478174del
g.3476447del
437delC
-
DOK7_000064
-
PubMed: Cossins 2012
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.439dup
r.(?)
p.(Ala147Glyfs*10)
ACMG
pathogenic
g.3478176dup
g.3476449dup
-
-
DOK7_000196
-
-
-
-
Germline
-
-
-
-
-
Martin Krenn
+/.
1
4
c.470T>G
r.(?)
p.(Leu157Arg)
-
pathogenic
g.3478207T>G
g.3476480T>G
-
-
DOK7_000043
-
PubMed: BenAmmar 2010
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.472C>T
r.(?)
p.(Arg158Trp)
ACMG
likely pathogenic
g.3478209C>T
-
-
-
DOK7_000197
-
-
-
-
Germline
-
-
-
-
-
Martin Krenn
+/.
6
4
c.473G>A
r.(?)
p.(Arg158Gln), p.Arg158Gln
-
NA, pathogenic, pathogenic (recessive)
g.3478210G>A
g.3476483G>A
-
-
DOK7_000038
expression cloning HEK 293T cells, fails to bind MUSK,
2 more items
PubMed: Cossins 2012
,
PubMed: Hamuro 2008
,
PubMed: Lashley 2010
,
PubMed: Palace 2007
-
-
Germline, In vitro (cloned)
-
-
BpmI+
-
-
Johan den Dunnen
+/.
1
4
c.480C>A
r.(?)
p.(Tyr160*)
-
pathogenic
g.3478217C>A
g.3476490C>A
-
-
DOK7_000040
-
PubMed: Anderson 2007
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., +?/.
5
4
c.481G>A
r.(?)
p.(Gly161Arg), p.Gly161Arg
-
likely pathogenic, NA, pathogenic (recessive)
g.3478218G>A
g.3476491G>A
-
-
DOK7_000066
VKGL data sharing initiative Nederland,
1 more item
PubMed: Cossins 2012
-
-
CLASSIFICATION record, Germline, In vitro (cloned)
-
-
-
-
-
Johan den Dunnen
,
Tom Winder
,
VKGL-NL_Nijmegen
+/.
4
4
c.496G>A
r.(?)
p.(Gly166Arg), p.Gly166Arg
-
NA, pathogenic, pathogenic (recessive)
g.3478233G>A
g.3476506G>A
-
-
DOK7_000047
1 more item
PubMed: Cossins 2012
,
PubMed: Jephson 2010
,
PubMed: Lashley 2010
-
-
Germline, In vitro (cloned)
-
-
-
-
-
Johan den Dunnen
+/.
1
4
c.511G>C
r.(?)
p.(Gly171Arg)
-
pathogenic
g.3478248G>C
g.3476521G>C
-
-
DOK7_000044
-
PubMed: BenAmmar 2010
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
4
c.512G>A
r.(?)
p.(Gly171Asp), p.Gly171Asp
-
NA, pathogenic (recessive)
g.3478249G>A
g.3476522G>A
-
-
DOK7_000068
1 more item
PubMed: Cossins 2012
-
-
Germline, In vitro (cloned)
-
-
-
-
-
Johan den Dunnen
+/.
2
4
c.513C>T
r.512_532del
p.(Gly172_Trp178del)
-
pathogenic (recessive)
g.3478250C>T
g.3476523C>T
r.577_597del
-
DOK7_000069
exon trap cloning
PubMed: Cossins 2012
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., ?/.
2
4
c.514G>A
r.(?)
p.(Gly172Arg)
-
pathogenic, VUS
g.3478251G>A
g.3476524G>A
-
-
DOK7_000045
VKGL data sharing initiative Nederland
PubMed: BenAmmar 2010
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Nijmegen
-/.
1
-
c.532+122C>A
r.(=)
p.(=)
-
benign
g.3478391C>A
g.3476664C>A
-
-
DOK7_000113
-
-
-
rs2857987
Germline
-
frequency up to 0,01%
-
-
-
Andreas Laner
+/.
2
4i, 5i
c.533-37_533-11del
r.spl?
p.(=)
-
pathogenic (recessive)
g.3487229_3487255del
g.3485502_3485528del
533-37_533-11del27
-
DOK7_000070
-
PubMed: Cossins 2012
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
1
-
c.533-31A>G
r.(=)
p.(=)
-
benign
g.3487235A>G
g.3485508A>G
-
-
DOK7_000114
-
-
-
rs181786949
Germline
-
-
-
-
-
Andreas Laner
+/.
6
5
c.539G>C
r.(?)
p.(Gly180Ala), p.Gly180Ala
-
NA, pathogenic, pathogenic (recessive)
g.3487272G>C
g.3485545G>C
539G>C (593G>C), 593G>C
-
DOK7_000009
change in phosphotyrosine binding domain; no change in CHRNA, CHRNB, CHRND, CHRNE, RAPSN, COLQ, CHAT,
2 more items
PubMed: Beeson 2006
,
OMIM:var0006
,
PubMed: Beeson 2006
,
PubMed: Palace 2007
,
OMIM:var0006
,
2 more items
-
-
Germline, In vitro (cloned)
-
-
BsaHI-
-
-
Johan den Dunnen
+/.
1
5
c.539G>T
r.(?)
p.(Gly180Val)
-
pathogenic
g.3487272G>T
g.3485545G>T
-
-
DOK7_000035
-
PubMed: BenAmmar 2010
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
3
5
c.548_551del
r.(?)
p.(Phe183Cysfs*62)
-
pathogenic, pathogenic (recessive)
g.3487281_3487284del
g.3485554_3485557del
548_551delTCCT, 549_551delTCCT
-
DOK7_000010
not in 200 control chromosomes
PubMed: Beeson 2006
,
PubMed: Palace 2007
,
OMIM:var0003
,
PubMed: Cossins 2012
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
5
c.555del
r.(?), r.555del
p.(Ser186Argfs*60), p.Ser186Argfs*60
-
pathogenic
g.3487288del
g.3485561del
555delC
-
DOK7_000023
-
PubMed: Muller 2007
,
PubMed: Schara 2009
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Angela Abicht
+/.
1
5
c.561del
r.(?)
p.(Glu188Argfs*58)
-
pathogenic
g.3487294del
g.3485567del
-
-
DOK7_000054
-
-
-
-
Germline
-
-
-
-
-
Angela Abicht
-/., -?/.
3
5
c.589G>A
r.(?)
p.(Asp197Asn)
-
benign, likely benign
g.3487322G>A
g.3485595G>A
DOK7(NM_001164673.1):c.578G>A (p.(Arg193Gln))
-
DOK7_000080
VKGL data sharing initiative Nederland
PubMed: Cossins 2012
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Leiden
,
VKGL-NL_Nijmegen
+/., +?/.
5
5
c.596del
r.(?)
p.(Ile199Thrfs*47), p.Ile199Thrfs*47
-
NA, pathogenic, pathogenic (recessive)
g.3487329del
g.3485602del
596delT
-
DOK7_000013
c.1124_1127dupTGCC,
1 more item
PubMed: Cossins 2012
,
PubMed: Jephson 2010
,
PubMed: Selcen 2008
-
-
Germline, In vitro (cloned)
-
-
-
-
-
Johan den Dunnen
,
Tom Winder
,
Andreas Laner
+/., +?/.
9
5
c.601C>T
r.(?), r.601c>u
p.(Arg201*), p.(Arg201Ter), p.Arg201*
-
NA, pathogenic, pathogenic (recessive)
g.3487334C>T
g.3485607C>T
601C>T, DOK7(NM_173660.5):c.601C>T (p.R201*)
-
DOK7_000011
expression cloning, fails to induce MUSK; accumulates in nucleus,
3 more items
PubMed: Beeson 2006
,
PubMed: Schara 2009
,
OMIM:var0007
,
PubMed: Chakravorty 2020
,
PubMed: Cossins 2012
,
3 more items
-
-
CLASSIFICATION record, Germline, In vitro (cloned)
-
-
-
-
-
Johan den Dunnen
,
Tom Winder
,
Angela Abicht
,
VKGL-NL_Utrecht
?/.
1
-
c.641C>T
r.(?)
p.(Pro214Leu)
-
VUS
g.3487374C>T
g.3485647C>T
DOK7(NM_001164673.1):c.630C>T (p.(=))
-
DOK7_000178
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
-
c.653-77C>G
r.(=)
p.(=)
-
VUS
g.3491327C>G
g.3489600C>G
-
-
DOK7_000110
-
-
-
-
Germline
-
-
-
-
-
Andreas Laner
-?/.
1
-
c.653-20G>A
r.(=)
p.(=)
-
likely benign
g.3491384G>A
-
DOK7(NM_001164673.1):c.642-20G>A (p.(=))
-
DOK7_000200
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
-
c.653-20G>T
r.(=)
p.(=)
-
VUS
g.3491384G>T
g.3489657G>T
DOK7(NM_001164673.1):c.642-20G>T (p.(=))
-
DOK7_000179
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
1
-
c.653-19A>C
r.(=)
p.(=)
-
likely benign
g.3491385A>C
g.3489658A>C
DOK7(NM_001164673.1):c.642-19A>C (p.(=))
-
DOK7_000147
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
1
-
c.675G>C
r.(?)
p.(Ser225=)
-
likely benign
g.3491426G>C
g.3489699G>C
DOK7(NM_001164673.1):c.664G>C (p.(Asp222His))
-
DOK7_000148
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
2
6i
c.737-2A>G
r.spl
p.?
-
pathogenic
g.3491486A>G
-
-
-
DOK7_000041
1 more item
PubMed: BenAmmar 2010
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/., -?/.
4
6
c.753G>A
r.(=), r.(?)
p.(=), p.(Ala251=)
-
benign, likely benign
g.3491504G>A
g.3489777G>A
A251A, DOK7(NM_001164673.1):c.742G>A (p.(Gly248Arg))
-
DOK7_000081
VKGL data sharing initiative Nederland
PubMed: Cossins 2012
-
rs59932476
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
Andreas Laner
,
VKGL-NL_Leiden
,
VKGL-NL_Nijmegen
?/.
1
-
c.766A>C
r.(?)
p.(Ser256Arg)
-
VUS
g.3491517A>C
-
DOK7(NM_173660.5):c.766A>C (p.(Ser256Arg))
-
DOK7_000205
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+?/.
1
6i
c.772+2_772+4delinsCCGGGCAGGCGGGCA
r.spl
p.?
-
likely pathogenic
g.3491525_3491527delinsCCGGGCAGGCGGGCA
g.3489798_3489800delinsCCGGGCAGGCGGGCA
772+2_772+4delinsCCGGGCAGGCGGGCA
-
DOK7_000104
-
-
-
-
Germline
-
-
tlwinder
-
-
Tom Winder
-/.
1
-
c.773-67G>A
r.(=)
p.(=)
-
benign
g.3494419G>A
g.3492692G>A
-
-
DOK7_000118
-
-
-
rs62272676
Germline
-
-
-
-
-
Andreas Laner
-/.
1
-
c.773-56G>A
r.(=)
p.(=)
-
benign
g.3494430G>A
g.3492703G>A
-
-
DOK7_000119
(c.359-56G>A)
-
-
rs6830812
Germline
-
-
-
-
-
Andreas Laner
-/.
1
-
c.773-32del
r.(=)
p.(=)
-
benign
g.3494454del
g.3492727del
-
-
DOK7_000120
-
-
-
rs67004955
Germline
-
-
-
-
-
Andreas Laner
?/.
1
-
c.781C>T
r.(?)
p.(Arg261Cys)
-
VUS
g.3494494C>T
g.3492767C>T
-
-
DOK7_000188
conflicting interpretations of pathogenicity; 4 heterozygous, no homozygous;
Clinindb (India)
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs142821143
Germline
-
4/2795 individuals
-
-
-
Mohammed Faruq
-?/.
2
7
c.782G>A
r.(?)
p.(Arg261His)
-
likely benign
g.3494495G>A
g.3492768G>A
DOK7(NM_001164673.1):c.*3G>A (p.(=))
-
DOK7_000082
VKGL data sharing initiative Nederland
PubMed: Cossins 2012
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Leiden
-?/.
1
7
c.816C>G
r.(?)
p.(His272Gln)
-
likely benign
g.3494529C>G
g.3492802C>G
-
-
DOK7_000083
-
PubMed: Cossins 2012
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
3
7
c.831C>T
r.(?)
p.(=), p.(Ala277=)
-
likely benign
g.3494544C>T
g.3492817C>T
A277A, DOK7(NM_001164673.1):c.*52C>T (p.(=)), DOK7(NM_173660.5):c.831C>T (p.A277=)
-
DOK7_000084
VKGL data sharing initiative Nederland
PubMed: Cossins 2012
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Leiden
,
VKGL-NL_Utrecht
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