Global Variome shared LOVD
STXBP1 (syntaxin binding protein 1)
LOVD v.3.0 Build 30b [
Current LOVD status
]
Register as submitter
|
Log in
Curator:
Global Variome, with Curator vacancy
View all genes
View STXBP1 gene homepage
View graphs about the STXBP1 gene database
Create a new gene entry
View all transcripts
View all transcripts of gene STXBP1
Create a new transcript information entry
View all variants
View all variants affecting transcripts
View unique variants in gene STXBP1
View all variants in gene STXBP1
Full data view for gene STXBP1
Create a new data submission
View active genomic custom columns
Enable more genomic custom columns
View all individuals
View all individuals with variants in gene STXBP1
Create a new data submission
View active custom columns
Enable more custom columns
View all diseases
View all diseases associated with gene STXBP1
Create a new disease information entry
View available phenotype columns
View all screenings
View all screenings for gene STXBP1
Create a new data submission
View active custom columns
Enable more custom columns
Submit new data
Unique variants in the STXBP1 gene
The variants shown are described using the
NM_003165.3
NM_001032221.3
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
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.
155 entries on 2 pages. Showing entries 1 - 100.
10 per page
25 per page
50 per page
100 per page
Legend
How to query
« First
Prev
1
2
Next
Last »
Effect
Reported
Exon
DNA change (cDNA)
RNA change
Protein
Classification method
Clinical classification
DNA change (genomic) (hg19)
DNA change (hg38)
Published as
ISCN
DB-ID
Variant remarks
Reference
ClinVar ID
dbSNP ID
Origin
Segregation
Frequency
Re-site
VIP
Methylation
Owner
+/+
1
1_4i
c.(?_-197)_(246+1_247-1)del
r.0?
p.0?
-
pathogenic
g.130374486_130420730del
-
-
-
STXBP1_000035
1 more item
PubMed: Boone 2010
-
-
De novo
-
-
-
-
-
LOVD
+/?
1
1_1i
c.(?_-120)_(37+1_38-1)del
r.0?
p.0?
-
pathogenic
g.?
-
-
-
STXBP1_000011
deletion 42.3-116 Kb, might affect FAM129B gene
PubMed: Deprez 2010
-
-
De novo
-
1/106
-
-
-
LOVD
+/+
1
1_20
c.(?_-181)_(*1958+?)del
r.0?
p.0?
-
pathogenic
g.?
-
-
-
STXBP1_000029
deletion 3.14-3.3 Mb
PubMed: Mignot 2011
-
-
De novo
-
-
-
-
-
LOVD
+/?
1
1_20
c.0
r.0
p.0
-
pathogenic
g.((?_130374486)_(130454995_?)del
-
-
-
STXBP1_000014
deletion 2 Mb (FAM125B to CEECAM1) incl. entire gene
PubMed: Saitsu 2008
-
-
De novo
-
-
-
-
-
LOVD
-/.
1
-
c.38-16_38-15del
r.(=)
p.(=)
-
benign
g.130413866_130413867del
g.127651587_127651588del
STXBP1(NM_003165.6):c.38-16_38-15delCT
-
STXBP1_000061
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-?/., ?/.
2
-
c.38-3T>C
r.spl?
p.?
-
likely benign, VUS
g.130413879T>C
g.127651600T>C
STXBP1(NM_003165.6):c.38-3T>C
-
STXBP1_000041
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Andreas Laner
,
VKGL-NL_Utrecht
-/.
1
-
c.88-53C>T
r.(=)
p.(=)
-
benign
g.130415941C>T
-
STXBP1(NM_001374308.2):c.46-53C>T
-
STXBP1_000151
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/.
1
-
c.88-51_246+205del
r.(88_246del)
p.(Val30_Lys82del)
-
pathogenic (dominant)
g.130415943_130420935del
g.127653664_127658656del
-
-
STXBP1_000111
-
PubMed: Lionel 2018
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.125C>T
r.(?)
p.(Ser42Phe)
-
pathogenic (dominant)
g.130416031C>T
g.127653752C>T
-
-
STXBP1_000124
-
PubMed: Carvill 2013
-
-
De novo
-
-
-
-
-
Johan den Dunnen
./.
1
-
c.147_148delinsCA
r.(=)
p.(=)
-
pathogenic
g.130416053_130416054delCinsCA
g.127653774_127653775delinsCA
-
-
STXBP1_000056
1 more item
PubMed: DDDS 2015
,
Journal: DDDS 2015
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+/+
1
3
c.157G>T
r.(?)
p.(Glu53*)
-
pathogenic
g.130416063G>T
g.127653784G>T
-
-
STXBP1_000018
-
PubMed: Saitsu 2010
-
-
De novo
-
-
-
-
-
LOVD
+/?
1
3i
c.169+1G>A
r.[169_170ins169+1_169+570{169+1g>a}, 169_170ins169+1_169+1168{169+1g>a}, ...]
p.Ile57Asnfs*8
-
pathogenic
g.130416076G>A
g.127653797G>A
-
-
STXBP1_000008
1 more item
PubMed: Hamdan 2009
,
PubMed: Hamdan 2011
-
-
De novo
-
-
-
-
-
LOVD
+?/.
1
-
c.169+2T>C
r.([169_170ins[gc;169+3_169+1168];169_170ins[gc;169+3_169+1334]])
p.(Ile57Serfs7*)
-
pathogenic
g.130416077T>C
g.127653798T>C
-
-
STXBP1_000112
-
-
-
-
De novo
-
-
-
-
-
Silvia Russo
-?/.
1
-
c.170-460G>A
r.(=)
p.(=)
-
likely benign
g.130420194G>A
-
STXBP1(NM_001032221.6):c.170-460G>A
-
STXBP1_000168
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
1
-
c.170-2A>G
r.spl
p.?
-
pathogenic (dominant)
g.130420652A>G
g.127658373A>G
ref?:c.170-2A>G
-
STXBP1_000172
-
PubMed: Soden 2014
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.187A>T
r.(?)
p.(Lys63Ter)
ACMG
pathogenic
g.130420671A>T
g.127658392A>T
K63X
-
STXBP1_000155
-
PubMed: Zhou 2018
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+/+
1
4
c.232_235del
r.(?)
p.(Thr78Hisfs*41)
-
pathogenic
g.130420716_130420719del
g.127658437_127658440del
-
-
STXBP1_000026
-
PubMed: Otsuka 2010
-
-
De novo
-
-
-
-
-
LOVD
+/.
1
-
c.238T>C
r.(?)
p.(Ser80Pro)
-
pathogenic (dominant)
g.130420722T>C
g.127658443T>C
-
-
STXBP1_000125
-
PubMed: Carvill 2013
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.246_246+2del
r.spl?
p.?
-
pathogenic
g.130420730_130420732del
g.127658451_127658453del
STXBP1(NM_003165.6):c.246_246+2delGGT
-
STXBP1_000113
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-/.
1
-
c.246+12A>G
r.(=)
p.(=)
-
benign
g.130420742A>G
g.127658463A>G
STXBP1(NM_003165.6):c.246+12A>G
-
STXBP1_000062
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-?/.
3
-
c.247-8A>G
r.(=)
p.(=)
-
likely benign
g.130422301A>G
g.127660022A>G
STXBP1(NM_003165.3):c.247-8A>G, STXBP1(NM_003165.6):c.247-8A>G
-
STXBP1_000063
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
,
VKGL-NL_Utrecht
?/.
1
-
c.247-1del
r.spl?
p.?
-
VUS
g.130422308del
g.127660029del
-
-
STXBP1_000042
-
-
-
-
Germline
-
-
-
-
-
Andreas Laner
+/?
1
5
c.251T>A
r.(?)
p.(Val84Asp)
-
pathogenic
g.130422313T>A
g.127660034T>A
-
-
STXBP1_000001
not in 500 control chromosomes
PubMed: Saitsu 2008
-
-
De novo
-
-
-
-
-
LOVD
-?/.
1
-
c.281C>T
r.(?)
p.(Pro94Leu)
-
likely benign
g.130422343C>T
-
STXBP1(NM_003165.6):c.281C>T (p.P94L)
-
STXBP1_000140
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
?/.
1
-
c.284C>T
r.(?)
p.(Pro95Leu)
-
VUS
g.130422346C>T
g.127660067C>T
-
-
STXBP1_000043
PolyPhen-2: PSIC: 0.025
-
-
-
Germline
-
-
-
-
-
Andreas Laner
+?/.
1
-
c.325+6T>C
r.spl
p.(=)
-
likely pathogenic (dominant)
g.130422393T>C
g.127660114T>C
-
-
STXBP1_000108
-
PubMed: Papuc 2019
-
-
De novo
-
-
-
-
-
Anaïs Begemann
-/., -?/.
3
-
c.325+8C>T
r.(=)
p.(=)
-
benign, likely benign
g.130422395C>T
g.127660116C>T
STXBP1(NM_003165.3):c.325+8C>T, STXBP1(NM_003165.6):c.325+8C>T
-
STXBP1_000064
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
,
VKGL-NL_Utrecht
-?/.
2
-
c.326-8A>G
r.(=)
p.(=)
-
likely benign
g.130423373A>G
g.127661094A>G
STXBP1(NM_003165.3):c.326-8A>G, STXBP1(NM_003165.6):c.326-8A>G
-
STXBP1_000065
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
?/.
1
-
c.335A>G
r.(?)
p.(Asp112Gly)
-
VUS
g.130423390A>G
-
STXBP1(NM_001032221.6):c.335A>G (p.(Asp112Gly))
-
STXBP1_000169
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
1
-
c.364C>T
r.(?)
p.(Arg122Ter)
-
pathogenic
g.130423419C>T
g.127661140C>T
-
-
STXBP1_000091
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
-
c.365dup
r.(?)
p.(Ala123SerfsTer19)
-
pathogenic
g.130423420dup
-
STXBP1(NM_003165.6):c.365dupG (p.A123Sfs*19)
-
STXBP1_000114
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
+/.
1
-
c.385del
r.(?)
p.(Thr129Leufs*2)
-
pathogenic
g.130423440del
-
STXBP1(NM_003165.3):c.385delA (p.T129Lfs*2)
-
STXBP1_000152
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/+
1
6
c.388_389del
r.(?)
p.(Leu130Aspfs*11)
-
pathogenic
g.130423443_130423444del
g.127661164_127661165del
388_389delCT
-
STXBP1_000019
-
PubMed: Saitsu 2010
-
-
De novo
-
-
-
-
-
LOVD
?/.
1
-
c.394G>A
r.(?)
p.(Glu132Lys)
-
VUS
g.130423449G>A
-
STXBP1(NM_003165.6):c.394G>A (p.E132K)
-
STXBP1_000141
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+/.
1
-
c.416C>T
r.(?)
p.(Pro139Leu)
-
pathogenic
g.130423471C>T
g.127661192C>T
-
-
STXBP1_000122
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/?
1
6i
c.429+1G>A
r.spl?
p.(fs*)
-
pathogenic
g.130423485G>A
g.127661206G>A
-
-
STXBP1_000010
not in 500 control chromosomes
PubMed: Deprez 2010
-
-
De novo
-
1/106
-
-
-
LOVD
+/.
1
-
c.429+5G>C
r.spl?
p.?
-
pathogenic
g.130423489G>C
g.127661210G>C
-
-
STXBP1_000092
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/.
1
-
c.430G>T
r.(?)
p.(Val144Phe)
-
likely pathogenic
g.130425484G>T
-
-
-
STXBP1_000158
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
./.
1
-
c.436_438delinsT
r.(?)
p.(Ser146Phefs*5)
-
pathogenic
g.130425490_130425492delTCinsT
g.127663211_127663213delinsT
-
-
STXBP1_000055
1 more item
PubMed: DDDS 2015
,
Journal: DDDS 2015
-
-
De novo
-
-
-
-
-
Johan den Dunnen
-?/?
1
7
c.455C>G
r.(?)
p.(Ser152Cys)
-
likely benign
g.130425509C>G
g.127663230C>G
-
-
STXBP1_000013
-
PubMed: Hamdan 2009
-
-
Unknown
-
-
-
-
-
LOVD
+?/.
1
-
c.469T>C
r.(?)
p.(Tyr157His)
-
likely pathogenic
g.130425523T>C
-
-
-
STXBP1_000154
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
-
c.524A>C
r.(?)
p.(Gln175Pro)
-
VUS
g.130425578A>C
g.127663299A>C
-
-
STXBP1_000115
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/?
1
8
c.539G>A
r.(?)
p.(Cys180Tyr)
-
pathogenic
g.130425593G>A
g.127663314G>A
-
-
STXBP1_000002
not in 500 control chromosomes
PubMed: Saitsu 2008
-
-
De novo
-
-
-
-
-
LOVD
+/+
1
7
c.548T>G
r.(?)
p.(Leu183Arg)
-
pathogenic
g.130425602T>G
g.127663323T>G
-
-
STXBP1_000034
-
PubMed: Milh 2011
-
-
De novo
-
-
-
-
-
LOVD
+?/.
1
7
c.550_552del
r.(?)
p.(Lys184del)
ACMG
likely pathogenic
g.130425604_130425606del
g.127663325_127663327del
-
-
STXBP1_000165
-
-
ClinVar-3387775
-
De novo
-
-
-
-
-
Marketa Wayhelova
+?/.
1
-
c.558C>A
r.(?)
p.(Tyr186Ter)
-
likely pathogenic
g.130425612C>A
g.127663333C>A
STXBP1(NM_003165.6):c.558C>A (p.Y186*)
-
STXBP1_000067
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/.
1
-
c.568C>T
r.(?)
p.(Arg190Trp)
-
pathogenic (dominant)
g.130425622C>T
g.127663343C>T
-
-
STXBP1_000126
-
PubMed: Carvill 2013
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+/., +?/., ?/.
5
-
c.569G>A
r.(?)
p.(Arg190Gln)
-
likely pathogenic, pathogenic, VUS
g.130425623G>A
g.127663344G>A
STXBP1(NM_003165.3):c.569G>A (p.(Arg190Gln)), STXBP1(NM_003165.6):c.569G>A (p.R190Q)
-
STXBP1_000068
conflicting interpretations of pathogenicity; 2 heterozygous, no homozygous;
Clinindb (India)
,
1 more item
PubMed: Minardi 2020
,
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs796053356
CLASSIFICATION record, Germline, Unknown
?
2/2795 individuals
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Utrecht
,
VKGL-NL_VUmc
,
Francesca Bisulli
,
Mohammed Faruq
+/.
1
-
c.578+1G>A
r.spl?
p.?
-
pathogenic
g.130425633G>A
g.127663354G>A
STXBP1(NM_003165.6):c.578+1G>A
-
STXBP1_000069
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
?/.
1
-
c.578+11G>T
r.(=)
p.(=)
-
VUS
g.130425643G>T
g.127663364G>T
-
-
STXBP1_000044
-
-
-
-
Germline
-
-
-
-
-
Andreas Laner
+/.
1
-
c.579-3C>G
r.spl?
p.?
-
pathogenic
g.130427523C>G
g.127665244C>G
-
-
STXBP1_000093
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
2
-
c.579-2A>G
r.spl?
p.?
-
pathogenic
g.130427524A>G
g.127665245A>G
STXBP1(NM_003165.6):c.579-2A>G
-
STXBP1_000070
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
+/+
1
7i_14
c.(578+1_579-1)_(1249+1_1250-1)del
r.(580_1249+1del)
p.(Glu194Glyfs*20)
-
pathogenic
g.130427526_130438221del
-
-
-
STXBP1_000033
1 more item
PubMed: Milh 2011
-
-
De novo
-
-
-
-
-
LOVD
-?/.
1
-
c.627C>T
r.(?)
p.(Leu209=)
-
likely benign
g.130427574C>T
g.127665295C>T
STXBP1(NM_003165.3):c.627C>T (p.L209=)
-
STXBP1_000116
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
-
c.663+1G>A
r.(?)
p.(?)
ACMG
pathogenic
g.130427611G>A
g.127665332G>A
-
-
STXBP1_000123
ACMG grading: PVS1,PM2,PM6,PP4,PP5; Yamamoto et al. 2016. Brain 38: 280
-
-
-
Germline
-
-
-
-
-
Andreas Laner
+/+
1
8i
c.663+5G>A
r.579_663del
p.Glu194Alafs*10
-
pathogenic
g.130427615G>A
g.127665336G>A
-
-
STXBP1_000020
exon 8 skipped
PubMed: Saitsu 2010
-
-
De novo
-
-
-
-
-
LOVD
-/., -?/.
2
-
c.663+17C>T
r.(=)
p.(=)
-
benign, likely benign
g.130427627C>T
g.127665348C>T
STXBP1(NM_003165.6):c.663+17C>T
-
STXBP1_000045
VKGL data sharing initiative Nederland
-
-
rs140247913
CLASSIFICATION record, Germline
-
-
-
-
-
Andreas Laner
,
VKGL-NL_Utrecht
+/.
1
-
c.664-2A>C
r.spl?
p.?
-
pathogenic
g.130428443A>C
g.127666164A>C
-
-
STXBP1_000094
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/+, +/.
3
9
c.703C>T
r.(?)
p.(Arg235*), p.(Arg235Ter)
ACMG
pathogenic
g.130428484C>T
g.127666205C>T
STXBP1(NM_003165.6):c.703C>T (p.R235*)
-
STXBP1_000021
VKGL data sharing initiative Nederland
PubMed: Saitsu 2010
,
PubMed: Trujillano 2017
-
-
CLASSIFICATION record, De novo
-
-
-
-
-
Daniel Trujillano
,
VKGL-NL_Utrecht
./.
1
-
c.704G>A
r.(?)
p.(Arg235Gln)
-
pathogenic
g.130428485G>A
g.127666206G>A
-
-
STXBP1_000051
-
PubMed: DDDS 2015
,
Journal: DDDS 2015
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.721del
r.(?)
p.(Ser241ProfsTer7)
-
pathogenic
g.130428502del
g.127666223del
STXBP1(NM_003165.3):c.721delT (p.S241Pfs*7)
-
STXBP1_000073
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/.
1
-
c.721T>C
r.(?)
p.(Ser241Pro)
-
likely pathogenic
g.130428502T>C
g.127666223T>C
STXBP1(NM_003165.6):c.721T>C (p.S241P)
-
STXBP1_000072
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
+?/.
1
-
c.734A>G
r.(?)
p.(His245Arg)
-
likely pathogenic
g.130428515A>G
-
STXBP1(NM_003165.6):c.734A>G (p.H245R)
-
STXBP1_000142
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
+/+
1
9
c.747dup
r.(?)
p.(Gln250Serfs*6)
-
pathogenic
g.130428528dup
g.127666249dup
-
-
STXBP1_000022
-
PubMed: Saitsu 2010
-
-
De novo
-
-
-
-
-
LOVD
+/.
1
-
c.755T>C
r.(?)
p.(Met252Thr)
-
pathogenic (dominant)
g.130428536T>C
g.127666257T>C
-
-
STXBP1_000110
-
PubMed: Lionel 2018
-
-
De novo
-
-
-
-
-
Johan den Dunnen
./.
1
-
c.778G>T
r.(?)
p.(Glu260*)
-
pathogenic
g.130428559G>T
g.127666280G>T
-
-
STXBP1_000052
-
PubMed: DDDS 2015
,
Journal: DDDS 2015
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.785A>T
r.(?)
p.(Asp262Val)
-
likely pathogenic
g.130428566A>T
g.127666287A>T
STXBP1(NM_003165.6):c.785A>T (p.D262V)
-
STXBP1_000074
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/.
1
-
c.785del
r.(?)
p.(Asp262Valfs*15)
-
pathogenic
g.130428566del
g.127666287del
785delA
-
STXBP1_000106
-
-
-
-
Unknown
-
-
-
-
-
IMGAG
+/.
1
9
c.785_787delinsCC
r.(?)
p.(Asp262Alafs*15)
-
pathogenic
g.130428566_130428568delinsCC
g.127666287_127666289delinsCC
-
-
STXBP1_000060
-
-
-
-
Germline/De novo (untested)
-
-
-
-
-
IMGAG
+?/.
1
-
c.794+5G>A
r.spl
p.?
ACMG
likely pathogenic (dominant)
g.130428580G>A
g.127666301G>A
-
-
STXBP1_000133
ACMG PS1, PM2, PM6
PubMed: Johannesen 2020
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.795-1G>A
r.spl
p.?
-
likely pathogenic
g.130430358G>A
g.127668079G>A
IVS9-1G>A
-
STXBP1_000146
-
PubMed: Srivastava 2014
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+?/.
2
10
c.842_846del
r.(?)
p.(Leu281ArgfsTer31)
-
likely pathogenic
g.130430406_130430410del
g.127668127_127668131del
c.841_845delCTGGA
-
STXBP1_000145
-
PubMed: Ganapathy 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.847G>A
r.(?)
p.(Glu283Lys)
-
pathogenic
g.130430411G>A
g.127668132G>A
-
-
STXBP1_000095
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
./.
1
-
c.853G>A
r.(?)
p.(Asp285Asn)
-
VUS
g.130430417G>A
g.127668138G>A
-
-
STXBP1_000059
-
PubMed: Bobbili 2018
,
Journal: Bobbili 2018
-
-
Germline
-
1/567 controls
-
-
-
Dheeraj Bobbili
+?/.
1
-
c.860T>C
r.(?)
p.(Leu287Pro)
-
likely pathogenic
g.130430424T>C
g.127668145T>C
STXBP1(NM_003165.6):c.860T>C (p.L287P)
-
STXBP1_000075
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+?/.
1
-
c.864G>A
r.(?)
p.Trp288*
ACMG
likely pathogenic
g.130430428G>A
g.127668149G>A
-
-
STXBP1_000109
1 more item
-
-
-
Germline
-
-
-
-
-
Andreas Laner
+/.
2
-
c.874C>T
r.(?)
p.(Arg292Cys)
ACMG
pathogenic
g.130430438C>T
g.127668159C>T
-
-
STXBP1_000096
ACMG PS1, PM2, PM5, PP2, PP3; mother not available, VKGL data sharing initiative Nederland
PubMed: Johannesen 2020
-
-
CLASSIFICATION record, Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Nijmegen
+/.
2
-
c.875G>A
r.(?)
p.(Arg292His)
ACMG
pathogenic, pathogenic (dominant)
g.130430439G>A
g.127668160G>A
R292H
-
STXBP1_000128
-
PubMed: Helbig 2016
,
PubMed: Zhou 2018
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.875G>T
r.(?)
p.(Arg292Leu)
ACMG
pathogenic (dominant)
g.130430439G>T
g.127668160G>T
-
-
STXBP1_000127
-
PubMed: Helbig 2016
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.889G>A
r.(?)
p.(Ala297Thr)
-
pathogenic (dominant)
g.130430453G>A
g.127668174G>A
-
-
STXBP1_000159
-
PubMed: Chuan 2022
-
-
Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
+/?
1
10
c.893_894del
r.(?)
p.(Glu298Glyfs*15)
-
pathogenic
g.130430457_130430458del
g.127668178_127668179del
-
-
STXBP1_000007
not in 500 control chromosomes; possibly de novo, parents not available
PubMed: Deprez 2010
-
-
Unknown
-
1/106
-
-
-
LOVD
+/+
1
10i
c.902+1G>A
r.spl?
p.?
-
pathogenic
g.130430467G>A
g.127668188G>A
-
-
STXBP1_000030
-
PubMed: Milh 2011
-
-
De novo
-
-
-
-
-
LOVD
+/+
1
10i
c.902+5G>A
r.902_903ins[guaaa;902+6_902+138]
p.Glu301*
-
pathogenic
g.130430471G>A
g.127668192G>A
-
-
STXBP1_000024
variant affects splicing, resulting in 138-bp intron 10 insert
PubMed: Saitsu 2010
-
-
Unknown
-
-
-
-
-
LOVD
?/.
2
-
c.902+7G>C
r.(=)
p.(=)
-
VUS
g.130430473G>C
g.127668194G>C
STXBP1(NM_001032221.3):c.902+7G>C (p.(=))
-
STXBP1_000046
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Andreas Laner
,
VKGL-NL_Leiden
-?/.
2
-
c.902+10C>T
r.(=)
p.(=)
-
likely benign
g.130430476C>T
g.127668197C>T
STXBP1(NM_003165.6):c.902+10C>T
-
STXBP1_000076
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
,
VKGL-NL_Utrecht
?/.
1
-
c.902+32G>C
r.(=)
p.(=)
-
VUS
g.130430498G>C
g.127668219G>C
-
-
STXBP1_000047
-
-
-
-
Germline
-
-
-
-
-
Andreas Laner
+?/.
1
-
c.915del
r.(?)
p.(Ser306LeufsTer2)
-
likely pathogenic
g.130432189del
g.127669910del
-
-
STXBP1_000148
-
-
-
-
Unknown
-
-
-
-
-
IMGAG
+/+
1
11
c.961A>T
r.(?)
p.(Lys321*)
-
pathogenic
g.130432235A>T
g.127669956A>T
-
-
STXBP1_000023
-
PubMed: Saitsu 2010
-
-
De novo
-
-
-
-
-
LOVD
+/?
1
11i_20
c.(962+1_963-1)_(*1958_?)del
r.(del)
p.(Thr322_Glu603del)
-
pathogenic
g.?
-
-
-
STXBP1_000012
deletion 23-35.4 Kb
PubMed: Deprez 2010
-
-
De novo
-
1/106
-
-
-
LOVD
+?/.
1
-
c.993del
r.(?)
p.(Lys332Argfs*24)
ACMG
likely pathogenic (dominant)
g.130434359del
g.127672080del
c.993delG
-
STXBP1_000153
ACMG PVS1, PM2, PP3
PubMed: Marinakis 2021
-
-
De novo
-
-
-
-
-
Jan Traeger-Synodinos
?/.
1
-
c.1000A>T
r.(?)
p.(Met334Leu)
-
VUS
g.130434366A>T
g.127672087A>T
-
-
STXBP1_000048
-
-
-
-
Germline
-
-
-
-
-
Andreas Laner
+/?
1
12i
c.1029+1G>T
r.[1029del,1029_1030ins[u;1029+2_1029+30],1029_1030ins[u;1029+2_1030-1]]
p.[Lys343Asnfs*13,Tyr344Leufs*12]
-
pathogenic
g.130434396G>T
g.127672117G>T
-
-
STXBP1_000009
not in 500 control chromosomes
PubMed: Deprez 2010
-
-
De novo
-
1/106
-
-
-
LOVD
-/.
1
-
c.1030-237T>A
r.(=)
p.(=)
-
benign
g.130435223T>A
g.127672944T>A
STXBP1(NM_003165.6):c.1030-237T>A
-
STXBP1_000077
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/.
1
-
c.1030-1G>T
r.spl?
p.?
-
pathogenic
g.130435459G>T
g.127673180G>T
STXBP1(NM_003165.6):c.1030-1G>T
-
STXBP1_000117
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+/.
1
-
c.1032C>A
r.(?)
p.(Tyr344Ter)
-
pathogenic
g.130435462C>A
-
-
-
STXBP1_000135
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/+
1
13
c.1039_1048dup
r.(?)
p.(Leu350Profs*6)
-
pathogenic
g.130435469_130435478dup
g.127673190_127673199dup
c.1039_1048dupCACCTGCACC
-
STXBP1_000028
-
PubMed: Mignot 2011
-
-
De novo
-
-
-
-
-
LOVD
+/.
3
-
c.1060T>C
r.(?)
p.(Cys354Arg)
ACMG
pathogenic, pathogenic (dominant)
g.130435490T>C
g.127673211T>C
NM_001032221:c.1060T>C
-
STXBP1_000057
-
PubMed: Carvill 2013
,
PubMed: Salinas 2020
,
PubMed: Trujillano 2017
VCV000280041.1
-
De novo, Unknown
-
-
-
-
-
Johan den Dunnen
,
Daniel Trujillano
./.
1
-
c.1099C>T
r.(?)
p.(Arg367*)
-
pathogenic
g.130435529C>T
g.127673250C>T
-
-
STXBP1_000053
-
PubMed: DDDS 2015
,
Journal: DDDS 2015
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.1108C>T
r.(?)
p.(Gln370Ter)
-
pathogenic
g.130435538C>T
g.127673259C>T
-
-
STXBP1_000097
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/.
1
-
c.1109A>C
r.(?)
p.(Gln370Pro)
-
likely pathogenic
g.130435539A>C
g.127673260A>C
STXBP1(NM_003165.6):c.1109A>C (p.Q370P)
-
STXBP1_000078
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
10 per page
25 per page
50 per page
100 per page
Legend
How to query
« First
Prev
1
2
Next
Last »
Screenscraping/webscraping (downloading large amounts of data using scripts) is strictly prohibited.
Use our
APIs
to retrieve data.
Powered by
LOVD v.3.0
Build 30b
LOVD software ©2004-2024
Leiden University Medical Center
Database contents © by their respective submitters and curators