Global Variome shared LOVD
KBTBD13 (kelch repeat and BTB (POZ) domain containi...)
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Curator:
Johan den Dunnen
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Unique variants in the KBTBD13 gene
This database is one of the gene variant databases from the
Leiden Muscular Dystrophy pages
The variants shown are described using the NM_001101362.2 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%)
How to query this table
All list views have search fields which can be used to search data. You can search for a complete word or you can search for a part of a search term. If you enclose two or more words in double quotes, LOVD will search for the combination of those words only exactly in the order you specify. Note that search terms are case-insensitive and that wildcards such as * are treated as normal text! For all options, like "and", "or", and "not" searches, or searching for prefixes or suffixes, see the table below.
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.
58 entries on 1 page. Showing entries 1 - 58.
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Legend
How to query
Effect
Reported
Exon
DNA change (cDNA)
RNA change
Protein
Classification method
Clinical classification
DNA change (genomic) (hg19)
DNA change (hg38)
Published as
ISCN
DB-ID
Variant remarks
Reference
ClinVar ID
dbSNP ID
Origin
Segregation
Frequency
Re-site
VIP
Methylation
Owner
?/.
1
-
c.23T>C
r.(?)
p.Leu8Pro
ACMG
VUS
g.65369176T>C
g.65076838T>C
-
-
KBTBD13_000027
-
-
-
rs755992942
Germline
-
-
-
-
-
Andreas Laner
-?/.
1
-
c.43G>A
r.(?)
p.(Gly15Ser)
-
likely benign
g.65369196G>A
g.65076858G>A
KBTBD13(NM_001101362.2):c.43G>A (p.G15S)
-
KBTBD13_000016
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
1
c.58G>C
r.(?)
p.(Ala20Pro)
-
VUS
g.65369211G>C
g.65076873G>C
-
-
KBTBD13_000009
-
-
-
-
Germline
?
-
-
-
-
Tom Winder
-?/., ?/.
2
-
c.89G>A
r.(?)
p.(Gly30Asp)
-
likely benign
g.65369242G>A
g.65076904G>A
KBTBD13(NM_001101362.2):c.89G>A (p.(Gly30Asp))
-
KBTBD13_000029
VKGL data sharing initiative Nederland
PubMed: Sevy 2016
,
PubMed: Cerino 2020
-
-
CLASSIFICATION record, Germline/De novo (untested)
-
-
-
-
-
VKGL-NL_Leiden
,
Mathieu Cerino
-?/.
1
-
c.115G>C
r.(?)
p.(Gly39Arg)
-
likely benign
g.65369268G>C
-
KBTBD13(NM_001101362.2):c.115G>C (p.(Gly39Arg))
-
KBTBD13_000050
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
-
c.140A>G
r.(?)
p.(Glu47Gly)
-
VUS
g.65369293A>G
g.65076955A>G
-
-
KBTBD13_000028
-
PubMed: Saini 2018
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.154G>A
r.(?)
p.(Val52Ile)
-
likely benign
g.65369307G>A
g.65076969G>A
KBTBD13(NM_001101362.2):c.154G>A (p.V52I)
-
KBTBD13_000017
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.162C>G
r.(?)
p.(Ser54Arg)
-
likely benign
g.65369315C>G
-
KBTBD13(NM_001101362.2):c.162C>G (p.S54R)
-
KBTBD13_000043
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.163G>A
r.(?)
p.(Ala55Thr)
-
likely benign
g.65369316G>A
g.65076978G>A
KBTBD13(NM_001101362.2):c.163G>A (p.(Ala55Thr))
-
KBTBD13_000030
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
1
c.172_191del
r.(?)
p.(Phe58AlafsTer80)
-
VUS
g.65369325_65369344del
g.65076987_65077006del
-
-
KBTBD13_000046
-
PubMed: Ganapathy 2019
-
rs778782105
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.182C>T
r.(?)
p.(Thr61Met)
-
VUS
g.65369335C>T
-
KBTBD13(NM_001101362.2):c.182C>T (p.(Thr61Met))
-
KBTBD13_000058
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
1
-
c.188A>T
r.(?)
p.(Gln63Leu)
-
likely benign
g.65369341A>T
-
KBTBD13(NM_001101362.2):c.188A>T (p.Q63L)
-
KBTBD13_000048
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-?/.
1
-
c.228C>G
r.(?)
p.(Asp76Glu)
-
likely benign
g.65369381C>G
-
KBTBD13(NM_001101362.2):c.228C>G (p.(Asp76Glu))
-
KBTBD13_000051
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
1
-
c.229G>C
r.(?)
p.(Glu77Gln)
-
likely benign
g.65369382G>C
-
-
-
KBTBD13_000044
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/., ?/.
4
1
c.242C>T
r.(?)
p.(Ala81Val), p.Ala81Val
-
benign, VUS
g.65369395C>T
g.65077057C>T
KBTBD13(NM_001101362.2):c.242C>T (p.A81V)
-
KBTBD13_000006
VKGL data sharing initiative Nederland
-
-
rs2919358
CLASSIFICATION record, Germline
-
0.31-0.50
-
-
-
Johan den Dunnen
,
Andreas Laner
,
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
-?/., ?/.
2
-
c.247G>C
r.(?)
p.(Glu83Gln)
-
likely benign, VUS
g.65369400G>C
g.65077062G>C
-
-
KBTBD13_000036
present in affected daughter, other affected family members not tested,
1 more item
PubMed: Westra 2019
-
-
CLASSIFICATION record, Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Nijmegen
?/.
2
-
c.251G>C
r.(?)
p.(Cys84Ser)
-
VUS
g.65369404G>C
g.65077066G>C
KBTBD13(NM_001101362.2):c.251G>C (p.C84S)
-
KBTBD13_000018
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
,
VKGL-NL_VUmc
?/.
1
-
c.252C>A
r.(?)
p.(Cys84*)
-
VUS
g.65369405C>A
g.65077067C>A
-
-
KBTBD13_000042
no variant 2nd chromosome
PubMed: Yu 2017
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.272_277dup
r.(?)
p.(Pro91_Ala92dup)
-
VUS
g.65369425_65369430dup
-
KBTBD13(NM_001101362.3):c.272_277dup (p.(Pro91_Ala92dup))
-
KBTBD13_000053
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
-
c.341C>T
r.(?)
p.(Ala114Val)
-
VUS
g.65369494C>T
-
KBTBD13(NM_001101362.3):c.341C>T (p.(Ala114Val))
-
KBTBD13_000054
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
-
c.358G>A
r.(?)
p.(Asp120Asn)
-
VUS
g.65369511G>A
-
KBTBD13(NM_001101362.3):c.358G>A (p.(Asp120Asn))
-
KBTBD13_000055
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-/.
1
-
c.361G>A
r.(?)
p.(Val121Met)
-
benign
g.65369514G>A
g.65077176G>A
KBTBD13(NM_001101362.2):c.361G>A (p.V121M)
-
KBTBD13_000019
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-/., ?/.
4
1
c.378G>T
r.(=), r.(?)
p.(=), p.(Ala126=)
-
benign, VUS
g.65369531G>T
g.65077193G>T
KBTBD13(NM_001101362.2):c.378G>T (p.A126=)
-
KBTBD13_000007
VKGL data sharing initiative Nederland
-
-
rs2946642
CLASSIFICATION record, Germline
-
0.50
-
-
-
Johan den Dunnen
,
Andreas Laner
,
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
?/.
1
-
c.383T>G
r.(?)
p.(Phe128Cys)
-
VUS
g.65369536T>G
-
KBTBD13(NM_001101362.2):c.383T>G (p.(Phe128Cys))
-
KBTBD13_000059
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
-
c.434C>G
r.(?)
p.(Ala145Gly)
-
VUS
g.65369587C>G
-
KBTBD13(NM_001101362.2):c.434C>G (p.(Ala145Gly))
-
KBTBD13_000060
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+?/.
1
-
c.444C>G
r.(?)
p.(Tyr148Ter)
-
likely pathogenic
g.65369597C>G
g.65077259C>G
-
-
KBTBD13_000024
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
-
c.445G>A
r.(?)
p.(Val149Met)
-
VUS
g.65369598G>A
-
KBTBD13(NM_001101362.3):c.445G>A (p.V149M)
-
KBTBD13_000065
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
?/.
1
-
c.469T>G
r.(?)
p.(Tyr157Asp)
-
VUS
g.65369622T>G
-
KBTBD13(NM_001101362.3):c.469T>G (p.(Tyr157Asp))
-
KBTBD13_000056
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
-
c.471C>G
r.(?)
p.(Tyr157Ter)
-
VUS
g.65369624C>G
g.65077286C>G
-
-
KBTBD13_000031
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
-
c.499C>G
r.(?)
p.Pro167Ala
ACMG
VUS
g.65369652C>G
g.65077314C>G
-
-
KBTBD13_000026
-
-
-
rs749520412
Germline
-
-
-
-
-
Andreas Laner
-?/.
1
-
c.511G>C
r.(?)
p.(Glu171Gln)
-
likely benign
g.65369664G>C
g.65077326G>C
KBTBD13(NM_001101362.2):c.511G>C (p.E171Q)
-
KBTBD13_000020
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
2
-
c.561G>A
r.(?)
p.(Trp187Ter)
-
VUS
g.65369714G>A
g.65077376G>A
KBTBD13(NM_001101362.2):c.561G>A (p.W187*, p.(Trp187*))
-
KBTBD13_000032
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
?/.
1
-
c.605C>A
r.(?)
p.(Ala202Asp)
-
VUS
g.65369758C>A
-
KBTBD13(NM_001101362.3):c.605C>A (p.(Ala202Asp))
-
KBTBD13_000066
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
1
-
c.617C>T
r.(?)
p.(Thr206Met)
-
likely benign
g.65369770C>T
g.65077432C>T
KBTBD13(NM_001101362.2):c.617C>T (p.T206M)
-
KBTBD13_000037
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
?/.
1
-
c.685T>C
r.(?)
p.(Phe229Leu)
-
VUS
g.65369838T>C
-
KBTBD13(NM_001101362.2):c.685T>C (p.(Phe229Leu))
-
KBTBD13_000061
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
2
1
c.742C>A
r.(?)
p.(Arg248Ser)
-
pathogenic
g.65369895C>A
g.65077557C>A
-
-
KBTBD13_000003
1 heterozygous, no homozygous;
Clinindb (India)
, not in 400 control chromosomes
PubMed: Narang 2020
,
Journal: Narang 2020
,
PubMed: Sambuughin 2010
,
OMIM:var0003
-
rs200549195
Germline
-
1/2767 individuals
-
-
-
Nyamkhishig Sambuughin
,
Mohammed Faruq
+/., -?/., ?/.
6
-
c.742C>T
r.(?)
p.(Arg248Cys)
-
likely benign, pathogenic, VUS
g.65369895C>T
g.65077557C>T
1 more item
-
KBTBD13_000025
3 heterozygous, no homozygous;
Clinindb (India)
, no segregation analysis,
1 more item
PubMed: Narang 2020
,
Journal: Narang 2020
,
PubMed: Westra 2019
-
rs200549195
CLASSIFICATION record, Germline, Germline/De novo (untested)
-
3/2762 individuals
-
-
-
Johan den Dunnen
,
VKGL-NL_Leiden
,
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
,
VKGL-NL_VUmc
,
Mohammed Faruq
-/.
1
-
c.769G>C
r.(?)
p.(Asp257His)
-
benign
g.65369922G>C
-
KBTBD13(NM_001101362.3):c.769G>C (p.(Asp257His))
-
KBTBD13_000067
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
2
-
c.783C>A
r.(?)
p.(Tyr261Ter)
-
VUS
g.65369936C>A
g.65077598C>A
KBTBD13(NM_001101362.2):c.783C>A (p.(Tyr261*))
-
KBTBD13_000033
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Nijmegen
-?/.
1
-
c.794G>A
r.(?)
p.(Gly265Asp)
-
likely benign
g.65369947G>A
g.65077609G>A
KBTBD13(NM_001101362.2):c.794G>A (p.(Gly265Asp))
-
KBTBD13_000034
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-/., ?/.
4
1
c.798A>G
r.(=), r.(?)
p.(=), p.(Glu266=)
-
benign, VUS
g.65369951A>G
g.65077613A>G
KBTBD13(NM_001101362.3):c.798A>G (p.E266=)
-
KBTBD13_000004
VKGL data sharing initiative Nederland
-
-
rs2919359
,
rs2919360
CLASSIFICATION record, Germline
-
0.50-1.00
-
-
-
Johan den Dunnen
,
Andreas Laner
,
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
?/.
1
1
c.880C>G
r.(?)
p.(Pro294Ala)
-
VUS
g.65370033C>G
g.65077695C>G
-
-
KBTBD13_000047
-
PubMed: Karthikeyan 2024
-
-
Germline/De novo (untested)
-
-
-
-
-
Lakshmi Bremadesam
-/.
1
-
c.954C>G
r.(?)
p.(Thr318=)
-
benign
g.65370107C>G
g.65077769C>G
KBTBD13(NM_001101362.2):c.954C>G (p.T318=)
-
KBTBD13_000021
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-?/.
1
-
c.958G>T
r.(?)
p.(Val320Leu)
-
likely benign
g.65370111G>T
-
KBTBD13(NM_001101362.2):c.958G>T (p.V320L)
-
KBTBD13_000049
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/., ?/.
2
-
c.1013G>A
r.(?)
p.(Arg338His)
-
likely benign, VUS
g.65370166G>A
g.65077828G>A
KBTBD13(NM_001101362.2):c.1013G>A (p.R338H)
-
KBTBD13_000039
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Nijmegen
-?/.
1
-
c.1098C>G
r.(?)
p.(His366Gln)
-
likely benign
g.65370251C>G
-
KBTBD13(NM_001101362.2):c.1098C>G (p.H366Q)
-
KBTBD13_000045
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
3
-
c.1107C>G
r.(?)
p.(Ile369Met)
-
VUS
g.65370260C>G
g.65077922C>G
KBTBD13(NM_001101362.2):c.1107C>G (p.I369M), KBTBD13(NM_001101362.3):c.1107C>G (p.I369M)
-
KBTBD13_000022
no segregation analysis, VKGL data sharing initiative Nederland
PubMed: Westra 2019
-
-
CLASSIFICATION record, Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Groningen
,
VKGL-NL_Utrecht
+/., ?/.
3
1
c.1170G>C
r.(?)
p.(Lys390Asn)
-
pathogenic (dominant), VUS
g.65370323G>C
g.65077985G>C
-
-
KBTBD13_000002
mapped by linkage; not 532 in control chromosomes, VKGL data sharing initiative Nederland
PubMed: Sambuughin 2010
,
OMIM:var0001
,
PubMed: Westra 2019
-
-
CLASSIFICATION record, Germline
yes
-
-
-
-
Johan den Dunnen
,
Nyamkhishig Sambuughin
,
VKGL-NL_Nijmegen
-/.
1
-
c.1197C>T
r.(?)
p.(=)
-
benign
g.65370350C>T
-
KBTBD13(NM_001101362.3):c.1197C>T (p.(Arg399=))
-
KBTBD13_000057
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-/., ?/.
4
1
c.1200T>C
r.(=), r.(?)
p.(=), p.(Gly400=)
-
benign, VUS
g.65370353T>C
g.65078015T>C
KBTBD13(NM_001101362.3):c.1200T>C (p.G400=)
-
KBTBD13_000005
VKGL data sharing initiative Nederland
-
-
rs2919359
,
rs2919360
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
Andreas Laner
,
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
?/.
1
-
c.1222C>A
r.(?)
p.(Arg408Ser)
-
VUS
g.65370375C>A
g.65078037C>A
-
-
KBTBD13_000035
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/., -?/.
5
1
c.1222C>T
r.(?)
p.(Arg408Cys)
-
NA, pathogenic, pathogenic (dominant)
g.65370375C>T
g.65078037C>T
-
-
KBTBD13_000001
expression cloining inC2C12, mouse cardiomyocytes, no abnormalities obeserved,
2 more items
PubMed: Sambuughin 2010
,
OMIM:var0002
,
PubMed: Sambuughin 2010
,
PubMed: Gommans 2003
,
1 more item
-
-
CLASSIFICATION record, Germline, In vitro (cloned)
yes
-
-
-
-
Nyamkhishig Sambuughin
,
VKGL-NL_Nijmegen
-?/.
1
-
c.1235T>A
r.(?)
p.(Leu412Gln)
-
likely benign
g.65370388T>A
-
KBTBD13(NM_001101362.2):c.1235T>A (p.(Leu412Gln))
-
KBTBD13_000062
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
1
-
c.1255G>A
r.(?)
p.(Gly419Ser)
-
likely benign
g.65370408G>A
-
KBTBD13(NM_001101362.2):c.1255G>A (p.(Gly419Ser))
-
KBTBD13_000063
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
1
-
c.1264C>T
r.(?)
p.(Arg422Trp)
-
likely benign
g.65370417C>T
-
KBTBD13(NM_001101362.3):c.1264C>T (p.R422W)
-
KBTBD13_000064
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-/.
1
-
c.1363A>G
r.(?)
p.(Thr455Ala)
-
benign
g.65370516A>G
g.65078178A>G
KBTBD13(NM_001101362.2):c.1363A>G (p.T455A)
-
KBTBD13_000023
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-/.
1
-
c.*122T>C
r.(=)
p.(=)
-
benign
g.65370652T>C
g.65078314T>C
-
-
KBTBD13_000014
-
-
-
rs12901617
Germline
-
-
-
-
-
Andreas Laner
?/.
1
1
c.*305C>T
r.(?)
p.(=)
-
VUS
g.65370835C>T
g.65078497C>T
-
-
KBTBD13_000008
-
-
-
rs2946643
Germline
-
0.50-1.00
-
-
-
Johan den Dunnen
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