Global Variome shared LOVD
IKBKG (inhibitor of kappa light polypeptide gene en...)
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Curator:
Francesca Fusco
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Unique variants in the IKBKG gene
The variants shown are described using the NM_003639.3 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.
165 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
+/., +?/.
37
3i_10_
c.(399+1_400-1)_*585{0}
r.?
p.?
ACMG
likely pathogenic, pathogenic, pathogenic (dominant)
g.(153784592_153786746)_(153793261_?)del
g.(154556377_154558531)_(154565046_?)del
del ex4-10
-
IKBKG_000004
94 kb deletion from IKBKG to IKBKGP, ACMG PM2, PVS1, mosaic deletion
PubMed: Aradhya 2001
,
PubMed: Kawai 2020
,
PubMed: Lee 2009
,
PubMed: Weisschuh 2024
-
-
De novo, Germline, Germline/De novo (untested), Somatic
-
-
-
-
(male), XCI pattern 0.500, XCI pattern 0.513, XCI pattern 0.584, XCI pattern 0.653, XCI pattern 0.671,
7 more items
Johan den Dunnen
./.
1
-
c.-210479_*929753dup
-
-
-
pathogenic
g.153565841_154722429dup
g.154337626_155494214dup
-
-
DKC1_000000
increased gene dosage
PubMed: DDDS 2015
,
Journal: DDDS 2015
-
-
Germline
-
-
-
-
-
Johan den Dunnen
./.
1
-
c.-205912_*68432dup
-
-
-
pathogenic
g.153570408_153861108dup
g.154342193_154632893dup
GDI1
-
FLNA_000307
-
PubMed: Hu 2016
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.-5839G>A
r.(?)
p.(=)
-
VUS
g.153770481G>A
-
IKBKG(NM_001099856.2):c.3G>A (p.(Met1?))
-
IKBKG_000159
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
-
c.-5831T>C
r.(?)
p.(=)
-
VUS
g.153770489T>C
g.154542274T>C
IKBKG(NM_001099856.4):c.11T>C (p.V4A)
-
IKBKG_000086
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/., ?/.
2
-
c.-5823G>A
r.(?)
p.(=)
-
likely benign, VUS
g.153770497G>A
g.154542282G>A
IKBKG(NM_001099856.4):c.19G>A (p.V7M), IKBKG(NM_001099856.6):c.19G>A (p.V7M)
-
G6PD_000198
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
?/.
1
-
c.-5730G>A
r.(?)
p.(=)
-
VUS
g.153770590G>A
g.154542375G>A
IKBKG(NM_001099856.4):c.112G>A (p.E38K)
-
IKBKG_000087
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/.
1
-
c.-5694C>T
r.(?)
p.(=)
-
benign
g.153770626C>T
g.154542411C>T
IKBKG(NM_001099856.4):c.148C>T (p.R50C)
-
G6PD_000199
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.-5691A>G
r.(?)
p.(=)
-
likely benign
g.153770629A>G
g.154542414A>G
IKBKG(NM_001099856.4):c.151A>G (p.T51A)
-
IKBKG_000088
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.-5669C>T
r.(?)
p.(=)
-
VUS
g.153770651C>T
-
IKBKG(NM_001099856.4):c.173C>T (p.A58V)
-
IKBKG_000160
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.-5657G>C
r.(?)
p.(=)
-
likely benign
g.153770663G>C
g.154542448G>C
IKBKG(NM_001099856.2):c.185G>C (p.(Gly62Ala))
-
G6PD_000200
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-/., ?/.
2
-
c.-2076T>G
r.(?)
p.(=)
-
benign, VUS
g.153774244T>G
g.154546029T>G
G6PD(NM_000402.4):c.210+7A>C (p.(=))
-
IKBKG_000071
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
+/.
1
-
c.-2048T>C
-
-
-
pathogenic
g.153774272T>C
g.154546057T>C
-
-
G6PD_000060
WHO classification-Class I
PubMed: Hirono 1995
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1449
-
c.-2044T>C
r.?
p.?
-
pathogenic
g.153774276T>C
g.154546061T>C
-
-
G6PD_000037
-
Zou 2019, submitted
-
-
Germline
-
-
-
-
-
Lin Zou
?/.
1
-
c.-1974G>A
-
-
-
VUS
g.153774346G>A
g.154546131G>A
-
-
IKBKG_000094
-
PubMed: Hamada 2010
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.-1326G>C
r.(?)
p.(=)
-
likely benign
g.153774994G>C
-
G6PD(NM_000402.4):c.82+10C>G
-
IKBKG_000143
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/., ?/.
3
-
c.-1323C>G
r.(?)
p.(=)
-
likely benign, VUS
g.153774997C>G
g.154546782C>G
G6PD(NM_000402.4):c.82+7G>C (p.(=))
-
IKBKG_000073
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
-?/.
1
-
c.-1321G>C
r.(?)
p.(=)
-
likely benign
g.153774999G>C
-
G6PD(NM_001042351.3):c.-8-621C>G
-
IKBKG_000144
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-/.
1
-
c.-1305C>A
r.(?)
p.(=)
-
benign
g.153775015C>A
g.154546800C>A
G6PD(NM_000402.4):c.71G>T (p.R24L)
-
IKBKG_000075
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.-1293C>A
r.(?)
p.(=)
-
likely benign
g.153775027C>A
g.154546812C>A
G6PD(NM_000402.4):c.59G>T (p.G20V)
-
IKBKG_000089
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.-1282G>A
r.(?)
p.(=)
-
likely benign
g.153775038G>A
g.154546823G>A
G6PD(NM_000402.4):c.48C>T (p.G16=)
-
IKBKG_000076
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.-1273T>G
r.(?)
p.(=)
-
likely benign
g.153775047T>G
-
G6PD(NM_000402.4):c.39A>C (p.T13=)
-
IKBKG_000145
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.-1254G>A
r.(?)
p.(=)
-
likely benign
g.153775066G>A
g.154546851G>A
G6PD(NM_000402.4):c.20C>T (p.A7V)
-
IKBKG_000077
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.-1220C>G
r.(=)
p.(=)
-
likely benign
g.153775100C>G
-
G6PD:c.-9+583G>C
-
IKBKG_000142
-
PubMed: Maranhao 2015
-
-
Germline
-
5/25 families
-
-
-
LOVD
-?/.
1
-
c.-14C>T
r.(?)
p.(=)
-
likely benign
g.153780204C>T
g.154551989C>T
IKBKG(NM_001099856.4):c.191C>T (p.P64L)
-
IKBKG_000090
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
_1
-
r.?
p.?
-
pathogenic (recessive)
g.153775961G>T
-
-
-
IKBKG_000133
affects splice site upstream exon, r.[-469_-360del,-360_-15ins[u,-358_-16]]
PubMed: Mooster 2010
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
+/.
4
3i_10_
c.(399+1_400-1)_*585{0}
r.?
p.?
-
pathogenic (dominant)
g.(153784592_153786746)_(153793261_?)del
g.(154556377_154558531)_(154565046_?)del
del ex4-10, del ex4-10, c.400_1260del861, NEMOdel4_10 400-?_*585+?del
-
IKBKG_000004
11.7 kb microdeletion involving ex4-10,
1 more item
PubMed: Aradhya 2001
,
PubMed: Fusco 2012
,
PubMed: Washington 2023
-
-
De novo, Germline
-
-
-
-
highly skewed Xi pattern of 99:1 (like affected) mother
Johan den Dunnen
,
Francesca Fusco
+/.
1
_1_2i
c.-258_(187+1_188-1){0}
r.0?
p.0
-
pathogenic (dominant)
g.(153770000_153774414)_(153776269_1537850000)del
-
-
-
IKBKG_000108
4.6kb deletion
PubMed: Fusco 2012
-
-
Germline/De novo (untested)
-
-
-
-
-
Francesca Fusco
+/.
6
_1_10_
c.-258_*585{0}
r.0
p.0
-
pathogenic (dominant)
g.(153750000_153757815)_(153803560_153815000)del, g.(153750000_153757815)_(153821090_153830000)del,
4 more items
-
-
-
IKBKG_000103, IKBKG_000104, IKBKG_000105, IKBKG_000106, IKBKG_000107, IKBKG_000108
115kb deletion, 22.2kb deletion, 26,6kb deletion, 45.7kb deletion, 63.2kb deletion, 65.2kb deletion
PubMed: Fusco 2012
,
PubMed: Fusco et al. 2012
-
-
De novo, Germline/De novo (untested)
-
-
-
-
-
Francesca Fusco
+/.
1
-
c.-258_*585{0}del
r.0?
p.0?
-
pathogenic
g.(153744322_153759773)_(153793401_153798250)del
-
-
-
IKBKG_000102
-
-
-
-
Germline/De novo (untested)
-
-
-
-
-
Gemeinschaftspraxis für Humangenetik Dresden
+/.
1
2i_10_
c.188-1577_*585{0}
r.?
p.?
-
pathogenic (dominant)
g.153782803_153876971del
g.154554572_154648740del
g.153782803-153876971del
-
IKBKG_000150
deletion from IKBKG gene to IKBKGP; mosaic deletion in father’s peripheral blood leukocytes
PubMed: Kawai 2022
-
-
Germline
-
-
-
-
-
Miki Kawai, Hiroki Kurahashi
+/.
1
3i_10_
c.400-517_*585{0}
r.?
p.?
-
pathogenic (dominant)
g.153786230_153797929del
g.154558015_154569714del
del ex4-10
-
IKBKG_000111
-
PubMed: Smahi 2000
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.14T>G
r.(?)
p.(Leu5Arg)
-
VUS
g.153780231T>G
g.154552016T>G
IKBKG(NM_001099856.3):c.218T>G (p.(Leu73Arg))
-
IKBKG_000078
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
-
c.32G>A
r.(?)
p.(Cys11Tyr)
-
VUS
g.153780249G>A
-
IKBKG(NM_001099857.2):c.32G>A (p.C11Y)
-
IKBKG_000146
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.84C>A
r.(?)
p.(Gly28=)
-
likely benign
g.153780301C>A
g.154552086C>A
IKBKG(NM_001099857.2):c.84C>A (p.G28=)
-
IKBKG_000079
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.101G>A
r.(?)
p.(Gly34Glu)
-
VUS
g.153780318G>A
-
IKBKG(NM_001099857.5):c.101G>A (p.(Gly34Glu))
-
IKBKG_000161
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
1
-
c.111dup
r.111dup
p.Asn2_Met38del
-
pathogenic (recessive)
g.153780328dup
-
110_111insC
-
IKBKG_000109
-
PubMed: Niehaus 2004
,
PubMed: Puel 2006
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.117_127dup
r.(?)
p.(Ser43Cysfs*76)
-
pathogenic (dominant)
g.153780334_153780344dup
-
duo117-127
-
IKBKG_000005
-
PubMed: Aradhya 2001
-
-
Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
+/.
1
2
c.120_129dup
r.(?)
p.(Glu44Profs*9)
-
pathogenic (dominant)
g.153780337_153780346dup
-
-
-
IKBKG_000110
-
PubMed: Smahi 2000
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.121C>G
r.(?)
p.(Leu41Val)
-
likely benign
g.153780338C>G
-
IKBKG(NM_001099857.4):c.121C>G (p.L41V)
-
IKBKG_000154
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
?/?
1
2
c.133C>T
r.(?)
p.(Gln45*)
-
VUS
g.153780350C>T
g.154552135C>T
-
-
IKBKG_000006
2 familial cases.
PubMed: Fusco et al. 2008
-
-
Unknown
-
-
-
-
-
Francesca Fusco
-?/.
1
-
c.138C>T
r.(?)
p.(Gly46=)
-
likely benign
g.153780355C>T
g.154552140C>T
IKBKG(NM_001099857.2):c.138C>T (p.G46=)
-
IKBKG_000091
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.144T>A
r.(?)
p.(Pro48=)
-
likely benign
g.153780361T>A
-
IKBKG(NM_001099857.2):c.144T>A (p.P48=)
-
IKBKG_000147
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.151C>T
r.(?)
p.(Leu51Phe)
-
likely benign
g.153780368C>T
g.154552153C>T
IKBKG(NM_001099857.2):c.151C>T (p.L51F)
-
IKBKG_000080
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/?
1
2
c.151del
r.(?)
p.(Leu51Serfs*64)
-
VUS
g.153780368del
g.154552153del
-
-
IKBKG_000007
-
-
-
-
Unknown
?
-
-
-
-
Francesca Fusco
?/.
1
-
c.157C>A
r.(?)
p.(Arg53Ser)
-
VUS
g.153780374C>A
g.154552159C>A
IKBKG(NM_001099856.3):c.361C>A (p.(Arg121Ser))
-
IKBKG_000092
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
-
c.157C>T
r.(?)
p.(Arg53Cys)
-
VUS
g.153780374C>T
g.154552159C>T
IKBKG(NM_001099857.2):c.157C>T (p.R53C)
-
IKBKG_000095
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/., -/., -?/., ?/.
5
2
c.169G>A
r.(?)
p.(Glu57Lys)
-
benign, likely benign, pathogenic (dominant), VUS
g.153780386G>A
g.154552171G>A
G169A,
1 more item
-
IKBKG_000008
VKGL data sharing initiative Nederland
PubMed: Aradhya 2001
,
PubMed: Fusco 2004
-
-
CLASSIFICATION record, Germline/De novo (untested)
-
-
-
-
-
Francesca Fusco
,
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
,
VKGL-NL_Utrecht
+/.
3
2
c.184C>T
r.(?)
p.(Arg62*), p.(Arg62Ter)
-
pathogenic, pathogenic (dominant)
g.153780401C>T
g.154552186C>T
C184T
-
IKBKG_000009
-
PubMed: Aradhya 2001
,
PubMed: Kawai 2020
,
PubMed: Smahi 2000
-
-
Germline, Germline/De novo (untested)
yes
-
-
-
XCI pattern 0.974
Johan den Dunnen
,
Francesca Fusco
-?/.
1
-
c.188-20A>G
r.(=)
p.(=)
-
likely benign
g.153784360A>G
-
IKBKG(NM_001099857.5):c.188-20A>G
-
IKBKG_000149
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+/.
1
3
c.214del
r.(?)
p.(Leu72Cysfs*43)
-
pathogenic (dominant)
g.153784406del
g.154556191del
delC214
-
IKBKG_000010
-
PubMed: Aradhya 2001
-
-
Germline/De novo (untested)
-
-
-
-
-
Francesca Fusco
+/.
1
3
c.220del
r.(?)
p.(Glu74Serfs*41)
-
pathogenic (dominant)
g.153784412del
g.154556197del
delG220
-
IKBKG_000011
-
PubMed: Aradhya 2001
-
-
Germline/De novo (untested)
-
-
-
-
-
Francesca Fusco
?/?
1
3
c.229del
r.(?)
p.(Glu77Argfs*38)
-
VUS
g.153784421del
g.154556206del
-
-
IKBKG_000012
-
PubMed: Fusco et al.2008
-
-
Unknown
?
-
-
-
-
Francesca Fusco
+/.
1
-
c.239T>C
r.(?)
p.(Leu80Pro)
-
pathogenic (recessive)
g.153784431T>C
-
-
-
IKBKG_000132
-
PubMed: Ku 2005
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/?
1
3
c.256C>T
r.(?)
p.(Gln86*)
-
VUS
g.153784448C>T
g.154556233C>T
-
-
IKBKG_000013
-
PubMed: Fusco et al.2008
-
-
Unknown
?
-
-
-
-
Francesca Fusco
?/?
1
3
c.262del
r.(?)
p.(Glu88Argfs*27)
-
VUS
g.153784454del
g.154556239del
-
-
IKBKG_000014
-
-
-
-
Unknown
?
-
-
-
-
Francesca Fusco
+/.
2
3
c.268A>T
r.(268a>u), r.(?)
p.(Lys90*), p.(Lys90Ter)
-
pathogenic
g.153784460A>T
g.154556245A>T
K90X
-
IKBKG_000097
-
PubMed: Kawai 2020
-
-
Germline
-
-
-
-
XCI pattern 0.984
Johan den Dunnen
,
Miki Kawai, Hiroki Kurahashi
?/?
1
3
c.268_270del
r.(?)
p.(Lys90del)
-
VUS
g.153784460_153784462del
g.154556245_154556247del
-
-
IKBKG_000015
-
PubMed: Fusco et al. 2004
-
-
Unknown
?
-
-
-
-
Francesca Fusco
+/.
1
3
c.292C>T
r.(?)
p.(Gln98*)
-
pathogenic (dominant)
g.153784484C>T
g.154556269C>T
C292T
-
IKBKG_000016
-
PubMed: Aradhya 2001
-
-
Germline/De novo (untested)
-
-
-
-
-
Francesca Fusco
+/.
1
-
c.297_304del
r.(?)
p.(Ala100Thrfs*35)
-
pathogenic
g.153784489_153784496del
-
IKBKG(NM_001099857.5):c.297_304delGGCCAGGA (p.A100Tfs*35)
-
IKBKG_000151
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-?/., ?/?
4
3
c.337G>A
r.(?)
p.(Asp113Asn)
-
likely benign, VUS
g.153784529G>A
g.154556314G>A
IKBKG(NM_001099857.4):c.337G>A (p.D113N), IKBKG(NM_001099857.5):c.337G>A (p.D113N)
-
IKBKG_000001
VKGL data sharing initiative Nederland,
1 more item
PubMed: Tarpey 2009
-
-
CLASSIFICATION record, Germline
-
2/208 cases
-
-
-
Johan den Dunnen
,
VKGL-NL_Groningen
,
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
+/.
1
3
c.343A>T
r.(343a>u)
p.(Lys115*)
-
pathogenic
g.153784535A>T
g.154556320A>T
K115X
-
IKBKG_000098
-
PubMed: Kawai 2020
-
-
Germline
-
-
-
-
XCI pattern 0.824
Miki Kawai, Hiroki Kurahashi
?/?
1
3
c.358C>T
r.(?)
p.(Gln120*)
-
VUS
g.153784550C>T
g.154556335C>T
-
-
IKBKG_000017
-
-
-
-
Unknown
?
-
-
-
-
Francesca Fusco
?/?
1
3
c.367C>T
r.(?)
p.(Arg123Trp)
-
VUS
g.153784559C>T
g.154556344C>T
-
-
IKBKG_000018
-
PubMed: Fusco et al. 2004
-
-
Unknown
?
-
-
-
-
Francesca Fusco
+/.
1
-
c.373del
r.(?)
p.(Val125Trpfs*4)
-
pathogenic
g.153784565del
g.154556350del
-
-
IKBKG_000158
-
-
-
-
De novo
-
-
-
-
-
Gemeinschaftspraxis für Humangenetik Dresden
?/?
1
3
c.382dup
r.(?)
p.(Leu128Profs*10)
-
VUS
g.153784574dup
g.154556359dup
-
-
IKBKG_000019
-
-
-
-
Unknown
?
-
-
-
-
Francesca Fusco
+/.
1
3i
c.399+1G>C
r.spl
p.?
-
pathogenic (dominant)
g.153784592G>C
g.154556377G>C
IVS3GT>CT
-
IKBKG_000020
-
PubMed: Aradhya 2001
-
-
Germline/De novo (untested)
-
-
-
-
-
Francesca Fusco
-?/.
1
-
c.399+19G>C
r.(=)
p.(=)
-
likely benign
g.153784610G>C
g.154556395G>C
IKBKG(NM_001099857.5):c.399+19G>C
-
IKBKG_000081
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
?/?
1
4
c.433C>T
r.(?)
p.(Gln145*)
-
VUS
g.153786780C>T
g.154558565C>T
-
-
IKBKG_000021
-
-
-
-
Unknown
?
-
-
-
-
Francesca Fusco
+/.
1
4
c.436_471delinsT
r.(?)
p.(Val146*)
-
pathogenic (dominant)
g.153786783_153786818delinsT
g.154558568_154558603delinsT
-
-
IKBKG_000003
-
-
-
-
Germline/De novo (untested)
-
-
-
-
-
Francesca Fusco
+/.
1
-
c.437T>G
r.(?)
p.(Val146Gly)
-
pathogenic
g.153786784T>G
-
-
-
IKBKG_000115
-
PubMed: Devora 2010
-
-
Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
?/?
1
4
c.454del
r.(?)
p.(Glu152Serfs*129)
-
VUS
g.153786801del
g.154558586del
-
-
IKBKG_000022
-
PubMed: Fusco et al.2008
-
-
Unknown
?
-
-
-
-
Francesca Fusco
+/.
1
-
c.458T>G
r.458u>g
p.Leu153Arg
-
pathogenic (recessive)
g.153786805T>G
-
-
-
IKBKG_000138
-
PubMed: Orange 2002
,
PubMed: Orange 2004
-
-
Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
?/?
1
4
c.484del
r.(?)
p.(Ala162Leufs*119)
-
VUS
g.153786831del
g.154558616del
-
-
IKBKG_000023
-
-
-
-
Unknown
?
-
-
-
-
Francesca Fusco
?/?
1
4
c.502C>T
r.(?)
p.(Gln168*)
-
VUS
g.153786849C>T
g.154558634C>T
-
-
IKBKG_000024
-
-
-
-
Unknown
?
-
-
-
-
Francesca Fusco
?/?
1
4
c.509T>C
r.(?)
p.(Leu170Pro)
-
VUS
g.153786856T>C
g.154558641T>C
-
-
IKBKG_000025
-
-
-
-
Unknown
?
-
-
-
-
Francesca Fusco
?/?
1
4
c.511G>T
r.(?)
p.(Glu171*)
-
VUS
g.153786858G>T
g.154558643G>T
-
-
IKBKG_000026
-
-
-
-
Unknown
?
-
-
-
-
Francesca Fusco
?/?
1
4
c.515_516insC
r.(?)
p.(Arg173Serfs*15)
-
VUS
g.153786862_153786863insC
g.154558647_154558648insC
-
-
IKBKG_000027
-
PubMed: Fusco et al.2008
-
-
Unknown
?
-
-
-
-
Francesca Fusco
+/.
1
4
c.516del
r.(?)
p.(Arg173Glyfs*108)
-
pathogenic (dominant)
g.153786863del
g.154558648del
delT516
-
IKBKG_000028
-
PubMed: Aradhya 2001
-
-
Germline/De novo (untested)
-
-
-
-
-
Francesca Fusco
+/.
1
-
c.517C>G
r.517c>g
p.Arg173Gly
-
pathogenic (recessive)
g.153786864C>G
-
C518G
-
IKBKG_000137
-
PubMed: Ku 2007
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/?
1
4
c.518G>A
r.(?)
p.(Arg173Gln)
-
VUS
g.153786865G>A
g.154558650G>A
-
-
IKBKG_000029
-
-
-
-
Unknown
?
-
-
-
-
Francesca Fusco
+/.
1
4i
c.518+2T>A
r.spl
p.?
-
pathogenic (dominant)
g.153786867T>A
g.154558652T>A
IVS4 GT>GA
-
IKBKG_000030
-
PubMed: Aradhya 2001
-
-
Germline/De novo (untested)
-
-
-
-
-
Francesca Fusco
?/?
1
4i
c.518+2T>G
r.(?)
p.(?)
-
VUS
g.153786867T>G
g.154558652T>G
-
-
IKBKG_000031
-
-
-
-
Unknown
?
-
-
-
-
Francesca Fusco
+/., ?/?
4
4i
c.519-3_519dup
r.(?), r.spl?
p.(?), p.(Ala174Glnfs*15)
-
pathogenic (dominant), VUS
g.153788619_153788622dup
g.154560404_154560407dup
520-523dupCAGG
-
IKBKG_000032
-
PubMed: Lee 2009
-
-
Germline, Unknown
?
-
-
-
-
Johan den Dunnen
,
Francesca Fusco
+/.
1
5
c.523dup
r.(?)
p.(Arg175Profs*13)
-
pathogenic (dominant)
g.153788626dup
g.154560411dup
insC523
-
IKBKG_000033
-
PubMed: Aradhya 2001
-
-
Germline/De novo (untested)
-
-
-
-
-
Francesca Fusco
+/.
1
-
c.524G>C
r.(?)
p.(Arg175Pro)
-
pathogenic
g.153788627G>C
-
R175P
-
IKBKG_000118
-
PubMed: Doffinger 2001
-
-
Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.549G>C
r.(?)
p.(Gln183His)
-
pathogenic (dominant)
g.153788652G>C
-
-
-
IKBKG_000134
-
PubMed: Lee 2009
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
5
c.551_588del
r.(?)
p.(Leu184Argfs*57)
-
pathogenic (dominant)
g.153788654_153788691del
g.154560439_154560476del
del551-588
-
IKBKG_000034
-
PubMed: Aradhya 2001
-
-
Germline/De novo (untested)
-
-
-
-
-
Francesca Fusco
+/.
1
5
c.570del
r.(?)
p.(Leu191Cysfs*90)
-
pathogenic (dominant)
g.153788673del
g.154560458del
del%570
-
IKBKG_000035
-
PubMed: Aradhya 2001
-
-
Germline/De novo (untested)
-
-
-
-
-
Francesca Fusco
?/?
1
5
c.570_574del
r.(?)
p.(Leu191Alafs*61)
-
VUS
g.153788673_153788677del
g.154560458_154560462del
-
-
IKBKG_000036
-
-
-
-
Unknown
?
-
-
-
-
Francesca Fusco
?/.
1
-
c.578A>C
r.(?)
p.(Gln193Pro)
-
VUS
g.153788681A>C
-
IKBKG(NM_001099857.5):c.578A>C (p.Q193P)
-
IKBKG_000155
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+/.
1
5
c.638_642dup
r.(?)
p.(Met215Serfs*68)
-
pathogenic (dominant)
g.153788741_153788745dup
g.154560526_154560530dup
dup638-642
-
IKBKG_000037
-
PubMed: Aradhya 2001
-
-
Germline/De novo (untested)
-
-
-
-
-
Francesca Fusco
+/.
1
5
c.639_645del
r.(?)
p.(Arg214Serfs*65)
-
pathogenic (dominant)
g.153788742_153788748del
g.154560527_154560533del
del639-645
-
IKBKG_000038
-
PubMed: Aradhya 2001
-
-
Germline/De novo (untested)
-
-
-
-
-
Francesca Fusco
?/.
1
-
c.643A>C
r.(?)
p.(Met215Leu)
-
VUS
g.153788746A>C
-
IKBKG(NM_001099857.5):c.643A>C (p.M215L)
-
IKBKG_000156
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
?/?
1
5
c.653_659dup
r.(?)
p.(Ser221Glyfs*35)
-
VUS
g.153788756_153788762dup
g.154560541_154560547dup
-
-
IKBKG_000069
-
-
-
-
Unknown
?
-
-
-
-
Francesca Fusco
?/?
1
5
c.662C>A
r.(?)
p.(Ser221*)
-
VUS
g.153788765C>A
g.154560550C>A
-
-
IKBKG_000039
-
PubMed: Fusco et al.2008
-
-
Unknown
?
-
-
-
-
Francesca Fusco
+/.
1
-
c.667G>A
r.(?)
p.(Glu223Lys)
-
pathogenic (recessive)
g.153788770G>A
-
-
-
IKBKG_000130
-
PubMed: Karamchandani-Patel 2011
-
-
Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.667_669del
r.(?)
p.(Glu223del)
-
pathogenic (recessive)
g.153788770_153788772del
-
667_669delGAG
-
IKBKG_000129
-
PubMed: Karamchandani-Patel 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.671+1G>A
r.spl?
p.?
-
pathogenic
g.153788775G>A
-
IKBKG(NM_001099857.5):c.671+1G>A
-
IKBKG_000153
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+/.
1
-
c.671+2T>G
r.spl?
p.?
-
pathogenic
g.153788776T>G
-
IKBKG(NM_001099857.5):c.671+2T>G
-
IKBKG_000152
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
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