Global Variome shared LOVD
RS1 (retinoschisin 1)
LOVD v.3.0 Build 30b [
Current LOVD status
]
Register as submitter
|
Log in
Curators:
Johan den Dunnen
and
Markus Preising
View all genes
View RS1 gene homepage
View graphs about the RS1 gene database
Create a new gene entry
View all transcripts
View all transcripts of gene RS1
Create a new transcript information entry
View all variants
View all variants affecting transcripts
View unique variants in gene RS1
View all variants in gene RS1
Full data view for gene RS1
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 RS1
Create a new data submission
View active custom columns
Enable more custom columns
View all diseases
View all diseases associated with gene RS1
Create a new disease information entry
View available phenotype columns
View all screenings
View all screenings for gene RS1
Create a new data submission
View active custom columns
Enable more custom columns
Submit new data
Unique variants in the RS1 gene
This database is one of the
"Eye disease"
gene variant databases.
The variants shown are described using the NM_000330.3 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.
563 entries on 6 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
3
4
5
6
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
3i_6_
c.(184+1_185-1)_(*2316_?)del
r.?
p.?
-
pathogenic (recessive)
g.(?_18657808)_(18665453_18674772)del
g.(?_18639688)_(18647333_18656652)del
ex4-6del
-
RS1_000485
-
PubMed: Gao 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
2
5i_6_
c.(522+1_523-1)_(*2316_?)del
r.spl
p.(?)
-
likely pathogenic
g.(?_18657808)_(18660277_18662549)del
g.(?_18639688)_(18642157_18644429)del
RS1, variant 1 :Deletion exon 6
-
RS1_000011
solved, hemizygous
PubMed: Weisschuh 2020
-
-
Unknown
?
-
-
-
-
LOVD
+/.
1
_1_1i
c.-2577_52+260del
r.0?
p.0?
-
pathogenic (recessive)
g.18689879_18692767del
g.18671759_18674647del
[-5303_-3784del; -2579_52+258del]
-
RS1_000198
fragment cloned
PubMed: Huopaniemi 2000
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
+?/.
1
_1_1i
c.-1758_52+2664del
r.0?
p.0?
-
likely pathogenic
g.18687473_18691946del
g.18669353_18673826del
c.(-35)-1723_c.51+2664del4472
-
RS1_000324
error in annotation, exon 1 is ending on nucleotide 52
PubMed: D'Souza
-
-
Unknown
?
-
-
-
-
LOVD
+/.
1
_1_1i
c.-1492_53-421del
r.0?
p.0?
ACMG
pathogenic
g.18676206_18691680del
g.18658086_18673560del
del ex1
-
RS1_000422
-
PubMed: Chen 2020
-
-
Germline/De novo (untested)
?
1/90 cases RS
-
-
-
LOVD
?/.
1
1
c.(?_-473)_(17+1_?)del
r.(?)
p.?
-
VUS
g.18690171_18690661del
-
exon 1 deletion
-
RS1_000278
Check also: Bowles 2011
PubMed: Avela 2019
-
-
Germline
-
-
-
-
-
LOVD
+?/.
5
_1_1i
c.(?_-35)_(52+1_53-1)del
r.0?
p.0?
-
likely pathogenic
g.(18675786_18690136)_(18690223_?)del
g.(18657666_18672016)_(18672103_?)del
Exon 1 deletion
-
RS1_000010
-
PubMed: Sergeev 2013
-
-
Unknown
?
-
-
-
-
LOVD
+?/.
3
_1_1i
c.(?_-35)_(52+1_53-1)del
r.0?
p.0?
-
likely pathogenic
g.(18675786_18690136)_(18690223_?)del
g.(18657666_18672016)_(18672103_?)del
exon 1 deletion
-
RS1_000010
-
PubMed: Pennesi 2018
-
-
Unknown
?
-
-
-
-
LOVD
?/.
2
_1_2i
c.(?_-35)_(78+1_79-1)del
r.0?
p.0?
-
VUS
g.(18674879_18675759)_(18690223_?)del
g.(18656759_18657639)_(18672103_?)del
RS1 nucleotide 1, protein 1:exon1del, p.?
-
RS1_000307
hemizygous, ACMG unclassified - no access to supplementary table 2
PubMed: Hull 2020
-
-
Germline
?
-
-
-
-
LOVD
+?/.
1
_1_3i
c.(?_-35)_(184+1_185-1)del
r.0?
p.0?
-
likely pathogenic (maternal)
g.(18665453_18674772)_(18690223_?)del
g.(18647333_18656652)_(18672103_?)del
E1-3del
-
RS1_000123
-
PubMed: Liu-2020
-
-
Germline
-
-
-
-
-
LOVD
+/.
8
_1_1i
c.(?_-35)_(52+1_53-1)del
r.0?
p.0?, p.?
ACMG
pathogenic (recessive)
g.(18675786_18690136)_(18690223_?)del
g.(18657666_18672016)_(18672103_?)del
(?_1-1)_(52+1_53-1)del, del ex1, (1-?_52+?)del, del ex1-4, (1-?_326+?)del, del ex4-5, (185-?_522+?)del,
1 more item
-
RS1_000010
ACMG PVS1, PS4, PM2, PP4
PubMed: Bender 2022
,
PubMed: Georgiou 2022
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
_1_3i
c.(?_-35)_(184+1_185-1)del
r.0?
p.0?
-
pathogenic (recessive)
g.(18665453_18674772)_(18779695_18797127)del
g.(18647333_18656652)_(18761577_18779009)del
ex1-3del
-
RS1_000123
-
PubMed: Gao 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
_1_4i
c.(?_-35)_(326+1_327-1)del
r.0?
p.?
ACMG
pathogenic (recessive)
g.(18665453_18674772)_(18690223_?)del
g.(18647333_18656652)_(18672103_?)del
-
-
RS1_000123
ACMG PVS1, PM2, PP4
PubMed: Bender 2022
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
2
_1_6_
c.(?_-35)_(*2316_?)del
r.0
p.0
-
likely pathogenic
g.(?_18657808)_(18690223_?)del
g.(?_18639688)_(18672103_?)del
170bp deletion Xp22.13
-
RS1_000000, RS1_000023
large deletion chrX (p22.2-p22.13) including OFD1 and RS1
PubMed: Bravo-Gil 2017
,
PubMed: Holtan 2020
-
-
Germline
-
1/899 cases
-
-
-
Global Variome, with Curator vacancy
,
Nereida Bravo Gil
+/.
8
_1_1i
c.(?_-35)_(52+1_53-1)del
r.0?
p.0?
-
pathogenic
g.(18675786_18690136)_(18690223_?)del
g.(18657666_18672016)_(18672103_?)del
del ex1
-
RS1_000010
deletion upto 1 kb 5' ATG
PubMed: Hiriyanna 1999
,
PubMed: Eksandh 2000
,
PubMed: Eksandh 2005
,
PubMed: Shinoda 2000
,
2 more items
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Dorothy Trump
+/.
2
_1_3i
c.(?_-35)_(184+1_185-1)del
r.0?
p.0?
-
pathogenic, pathogenic (recessive)
g.(18665453_18674772)_(18690223_?)del, g.(18665453_18674772)_(18779695_18797127)del
g.(18647333_18656652)_(18672103_?)del, g.(18647333_18656652)_(18761577_18779009)del
-
-
RS1_000123
136 kb deletion from PPEF intron 9 to RS1 intron 3 also involving CDKL5 (STK9)
PubMed: Huopaniemi
,
PubMed: Yu 2001
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
?/.
1
_1
-
r.?
p.?
-
VUS
g.18693972_18695491del
g.18675852_18677371del
[-5303_-3784del;-2579_52+258del]
-
RS1_000308
fragment cloned
PubMed: Huopaniemi 2000
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
3
_1_1i
c.(?_-35)_(52+1_53-1)del
r.0?
p.0?
-
likely pathogenic
g.(18675786_18690136)_(18690223_?)del
g.(18657666_18672016)_(18672103_?)del
del ex1, RS1 exon1 deletion
-
RS1_000010
-
PubMed: Avela 2019
,
PubMed: Chen 2014
,
PubMed: Fahim 2017
-
-
Germline, Germline/De novo (untested)
yes
-
-
-
-
LOVD
+/.
1
_1_6_
c.=
r.(=)
p.(=)
-
NA
g.=
-
-
-
RS1_000000
expression construct in pcDNA3.1, COS-7 cell expression
-
-
-
In vitro (cloned)
-
-
-
-
-
Johan den Dunnen
+/.
3
_1_1i
c.(?_-35)_(52+1_53-1)del
r.0?
p.0?
-
pathogenic
g.(18675786_18690136)_(18690223_?)del
g.(18657666_18672016)_(18672103_?)del
-
-
RS1_000010
deletion upto 1 kb 5' ATG
PubMed: Chan 2004
,
PubMed: Renner 2008
,
PubMed: Tsang 2007
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
1
c.1A>G
r.(?)
p.(Met1?)
-
pathogenic, pathogenic (recessive)
g.18690188T>C
g.18672068T>C
-
-
RS1_000093
-
PubMed: Mashima 1999
,
PubMed: Shinoda 2000
-
-
Germline, Germline/De novo (untested)
-
-
-MboII
-
-
Johan den Dunnen
+/., +?/.
8
1
c.1A>T
r.(?), r.0?
p.(Met1?)
ACMG
likely pathogenic, pathogenic, pathogenic (recessive)
g.18690188T>A
g.18672068T>A
c.1A>T, p.Met1Lle, RS1 c.1A>T, p.Met1Leu, [35T>A;52+5G>C]
-
RS1_000160
ACMG PVS1, PS4, PM2, PS3>M, PP3, PP4, not in 100 control chromosomes,
1 more item
PubMed: Bender 2022
,
PubMed: Kim 2006
,
PubMed: Pennesi 2018
,
PubMed: Stone 2017
,
1 more item
-
-
Germline, Unknown
?
-
-MboII
-
-
Johan den Dunnen
+?/.
1
-
c.2T>A
r.(?)
p.?
-
likely pathogenic
g.18690187A>T
-
-
-
RS1_000429
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/., +?/.
2
1
c.2T>C
r.(?)
p.(Met1?)
-
likely pathogenic, pathogenic
g.18690187A>G
g.18672067A>G
-
-
RS1_000419
VKGL data sharing initiative Nederland
PubMed: RS-consortium 1998, group 5
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Nijmegen
+/., +?/.
6
-
c.3G>A
r.(?)
p.(Met1?)
ACMG
likely pathogenic, pathogenic (recessive)
g.18690186C>T
g.18672066C>T
c.3G> A (p.M1) in the RS1 gene
-
RS1_000408
ACMG PS1, PP4, PP5, PM2, PM3
PubMed: D'Anna Mardero 2024
,
PubMed: Xiao 2016
-
-
Germline, Germline/De novo (untested)
yes
-
-
-
-
Johan den Dunnen
+/., +?/.
2
1
c.3G>T
r.(?)
p.(Met1?)
-
likely pathogenic, pathogenic (recessive)
g.18690186C>A
g.18672066C>A
(Met1Ile)
-
RS1_000289
VKGL data sharing initiative Nederland
PubMed: Hahn 2022
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Nijmegen
+/.
2
_1_1i
c.(-63_3)_(52+3913_52+4861)del
r.0?
p.0?
-
pathogenic (recessive)
g.(18685276_18686224)_(18690186_18690251)del
g.(18667156_18668104)_(18672066_18672131)del
-
arr[GRCh37] Xp22.13(18685276x1,18686224_18690186x0,18690251x1)
RS1_000480
-
PubMed: Fortunato 2023
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., -?/.
2
1
c.7C>G
r.(?)
p.(Arg3Gly)
-
likely benign, pathogenic (recessive)
g.18690182G>C
g.18672062G>C
RS1(NM_000330.3):c.7C>G (p.(Arg3Gly))
-
RS1_000219
VKGL data sharing initiative Nederland
PubMed: Chowdury 2024
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Leiden
+?/.
1
-
c.14del
r.(?)
p.(Ile5LysfsTer121)
-
likely pathogenic (recessive)
g.18690175del
g.18672055del
14delT
-
RS1_000511
-
PubMed: Gao 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
4
1
c.20del
r.(?)
p.(Gly7AlafsTer119)
-
pathogenic (recessive)
g.18690170del
g.18672050del
-
-
RS1_000482
ACMG PVS1, PM2, PP3, PP4
PubMed: Georgiou 2022
,
PubMed: Kousal 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
1
c.26del
r.(?)
p.(Leu9Cysfs*117)
-
pathogenic
g.18690167del
g.18672047del
-
-
RS1_000162
-
PubMed: Li 2007
,
PubMed: Ma 2008
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
1
c.26T>A
r.(?)
p.(Leu9*)
-
likely pathogenic
g.18690163A>T
g.18672043A>T
c.26A>T, p.(Leu9*)
-
RS1_000296
error in annotation: c.26A>T instead of T>A, hemizygous
PubMed: Wang 2019
-
-
Germline
?
-
-
-
-
LOVD
+/., +?/.
7
1
c.33_36del
r.(?)
p.(Leu11Phefs*114), p.(Leu11PhefsTer114)
-
likely pathogenic, pathogenic, pathogenic (recessive)
g.18690155_18690158del
g.18672035_18672038del
RS1, variant 1: c.33_36del/p.L11Ffs*114
-
RS1_000012
solved, hemizygous
PubMed: Kousal 2021
,
PubMed: RS-consortium 1998, group 3
,
PubMed: RS-consortium 1998, group 5
,
1 more item
ClinVar-000098944.7
-
Germline, Unknown
?
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.34_35insGATA
r.(?)
p.(Leu12ArgfsTer7)
ACMG
likely pathogenic (recessive)
g.18690154_18690155insTATC
g.18672034_18672035insTATC
-
-
RS1_000481
ACMG PVS1, PP4, PM2
PubMed: D'Anna Mardero 2024
-
-
Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
+/., +?/., ?/.
15
1
c.35T>A
-, r.(?)
p.(Leu12His), p.Leu12His
ACMG
likely pathogenic, likely pathogenic (recessive), NA, pathogenic, pathogenic (recessive), VUS
g.18690154A>T
g.18672034A>T
Leu12His, RS1 c.35T>A, p.(Leu12His), RS1 c.35T>A, p.Leu12His, [35T>A;52+5G>C]
-
RS1_000016
ACMG PM1, PM2, PP2, PP3, PP4, PP5, ACMG PS4, PM2, PS3>M, PP3, PP4,
2 more items
PubMed: Bender 2022
,
PubMed: Fahim 2017
,
PubMed: Georgiou 2022
,
PubMed: RS-consortium 1998, group 3
,
2 more items
-
rs62645879
Germline, In vitro (cloned), Unknown
?
-
-
-
-
Johan den Dunnen
,
Dorothy Trump
+?/.
1
1
c.35T>C
r.(?)
p.(Leu12Pro)
-
likely pathogenic
g.18690154A>G
g.18672034A>G
RS1 c.35T>C, p.Leu12Pro
-
RS1_000407
-
PubMed: Kondo 2019
-
-
Unknown
?
-
-
-
-
LOVD
+?/., -?/., ?/.
3
1
c.37C>T
-, r.(?)
p.(Leu13Phe), p.Leu13Phe
-
likely pathogenic, NA, VUS
g.18690152G>A
g.18672032G>A
Leu13Phe, RS1 c.37C>T, p.Leu13Phe, RS1(NM_000330.3):c.37C>T (p.L13F)
-
RS1_000258
in vitro analysis COS-7 cells shows readily secreted RS1 in cellular fraction,
1 more item
PubMed: Vijayasarathy 2010
-
-
CLASSIFICATION record, In vitro (cloned), Unknown
?
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Rotterdam
+/., +?/.
4
1
c.38T>C
-, r.(?)
p.(Leu13Pro), p.Leu13Pro
-
likely pathogenic, NA, pathogenic
g.18690151A>G
g.18672031A>G
Leu13Pro, RS1 c.38T>C, p.Leu13Pro
-
RS1_000109
1 more item
PubMed: Hiriyanna 1999
,
PubMed: Kondo 2019
,
PubMed: Vijayasarathy 2010
-
rs104894935
Germline, In vitro (cloned), Unknown
?
-
-
-
-
Johan den Dunnen
-?/.
1
1
c.44G>A
r.(?)
p.(Gly15Asp)
-
likely benign
g.18690145C>T
g.18672025C>T
RS1(NM_000330.3):c.44G>A (p.(Gly15Asp))
-
RS1_000244
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/., +?/.
2
1_1i
c.48_52+2dup
r.spl, r.spl?
p.(?), p.0?
ACMG
likely pathogenic, pathogenic
g.18690135_18690141dup
g.18672015_18672021dup
c.52+2_3insTGAAGGT, RS1 c.52+2_3 ins tgaaggt
-
RS1_000404
error in annotation, variant automapped to c.48_52+2dup,
1 more item
PubMed: Chen 2014
,
PubMed: Chen 2020
-
-
Germline, Unknown
?, yes
1/90
-
-
-
LOVD
+?/.
1
1
c.49G>T
r.(?)
p.(Glu17Ter)
-
likely pathogenic
g.18690140C>A
g.18672020C>A
RS1 c.49G>T, p.Glu17*
-
RS1_000406
-
PubMed: Kondo 2019
-
-
Unknown
?
-
-
-
-
LOVD
+/., +?/.
4
1
c.52G>A
r.(?), r.spl
p.(Ala18Thr), p.0?, p.?
-
likely pathogenic (recessive), pathogenic (recessive)
g.18690137C>T
g.18672017C>T
RS1 c.673C>T, p.Arg213Trp
-
RS1_000322
in vitro analysis mini-gene splicing assay shows splicing is affected
PubMed: Hahn 2022
,
PubMed: Vijayasarathy 2010
,
PubMed: Wei 2024
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
+/.
8
1i
c.52+1G>A
r.(?), r.spl
p.?
ACMG
pathogenic, pathogenic (recessive)
g.18690136C>T
g.18672016C>T
-
-
RS1_000013
ACMG PVS1, PS4, PM2, PP4
PubMed: Bender 2022
,
PubMed: Mashima 1999
,
PubMed: RS-consortium 1998, group 2
,
PubMed: Shinoda 2000
,
1 more item
-
-
Germline, Germline/De novo (untested)
-
-
+MboII
-
-
Johan den Dunnen
+/.
2
1
c.52+1G>C
r.(?), r.spl
p.?
ACMG
pathogenic, pathogenic (recessive)
g.18690136C>G
g.18672016C>G
RS1 c.52+1g>c
-
RS1_000405
number of alleles does not match number of patients (91 vs 90)
PubMed: Chen 2020
,
PubMed: Jimenez 2022
-
-
Germline, Unknown
?
1/90
-
-
-
Johan den Dunnen
+/., ?/.
6
1, 1i
c.52+1G>T
r.(?), r.spl
p.?
ACMG
pathogenic, pathogenic (recessive), VUS
g.18690136C>A
g.18672016C>A
RS1 c.52+1g>t
-
RS1_000014
ACMG PVS1, PM2, PP3, PP4, ACMG PVS1, PM2, PP4,
1 more item
PubMed: Bender 2022
,
PubMed: Chen 2020
,
PubMed: Georgiou 2022
,
PubMed: Lamey 2010
,
1 more item
-
rs281865336
Germline, Unknown
?
1/90
-
-
-
Johan den Dunnen
+/.
1
1i
c.52+2dup
r.spl?
p.?
-
pathogenic
g.18690135dup
g.18672015dup
-
-
RS1_000015
-
PubMed: RS-consortium 1998, group 3
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.52+2T>A
r.spl
p.?
-
pathogenic (recessive)
g.18690135A>T
g.18672015A>T
-
-
RS1_000510
-
PubMed: Gao 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., +?/.
10
1, 1i
c.52+2T>C
r.spl
p.(?), p.?
-
likely pathogenic, pathogenic, pathogenic (recessive)
g.18690135A>G
g.18672015A>G
c.52+2T>C, IVS1+2 T to C, RS1 c.52+2T>C, RS1 c.52+2T>C, p.?, Splice site IVS1+2T>C
-
RS1_000128
ACMG PVS1, PM2, PP3, PP4
PubMed: Fahim 2017
,
PubMed: Georgiou 2022
,
PubMed: Khan 2001
,
PubMed: Li 2007
,
PubMed: Ma 2008
,
5 more items
-
rs281865334
Germline, Unknown
?
-
-
-
-
Johan den Dunnen
,
Dorothy Trump
+?/., ?/.
4
1i
c.52+3A>G
r.spl?
p.(?), p.?
-
likely pathogenic, VUS
g.18690134T>C
g.18672014T>C
Allele 1 c.52+3A>G, Allele 2 not applicable
-
RS1_000243
hemizygous, VKGL data sharing initiative Nederland
PubMed: Khan 2019
-
-
CLASSIFICATION record, Germline/De novo (untested)
?
-
-
-
-
VKGL-NL_Nijmegen
?/.
6
1i
c.52+5G>C
r.(?), r.spl?
p.(=), p.?
ACMG
VUS
g.18690132C>G, g.52515354A>G
g.18672012C>G, g.51941218A>G
-
-
ATP7B_000001, RS1_000017
ACMG PS4, PM2, PP4, PP3, BP2
PubMed: Bender 2022
,
PubMed: RS-consortium 1998, group 3
-
-
Germline
-
-
-
-
-
Gerard C.P. Schaafsma
,
Johan den Dunnen
+/.
1
1i_2i
c.53-859_78+276del
r.(53_78del)
p.(Ala18Glyfs*2)
-
pathogenic (recessive)
g.18675485_18676645del
g.18657365_18658525del
(A18Pfs*108)
-
RS1_000484
-
PubMed: Jimenez 2022
,
PubMed: Wen 2023
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
1i_2i
c.53-713_78+266del
r.(?)
p.(Ala18Glyfs*2)
-
likely pathogenic
g.18675494_18676498del
g.18657374_18658378del
c.53-713_78+266del
-
RS1_000301
-
PubMed: Maggi_2021
-
-
Germline
yes
-
-
-
-
LOVD
+/., +?/.
4
1i
c.53-34A>G
r.(53_78del), r.(?), r.spl
p.(=), p.(Ala18fs), p.?
ACMG
likely pathogenic (recessive), pathogenic, pathogenic (recessive)
g.18675819T>C
g.18657699T>C
IVS1-34A>G
-
RS1_000179
ACMG PM1, PM2, PP4, ACMG PP1>S, PS4, PS3-PM, PM2, PP3, PP4
PubMed: Bender 2022
,
PubMed: Georgiou 2022
,
PubMed: Gerth 2008
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
-
c.53-1G>A
r.spl
p.?
-
pathogenic (recessive)
g.18675786C>T
g.18657666C>T
-
-
RS1_000479
-
PubMed: Wang 2022
,
PubMed: Wei 2024
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
+/., +?/., ?/.
33
1i_2i
c.(52+1_53-1)_(78+1_79-1)del
r.(53_78del), r.(?), r.?
p.(?), p.(Ala18Glyfs*2), p.(Ala18GlyfsTer2), p.?
ACMG
likely pathogenic, pathogenic, pathogenic (recessive), VUS
g.(18674879_18675759)_(18675786_18690136)del
g.(18656759_18657639)_(18657666_18672016)del
arr[hg19] Xp22.13 (18674960 x1, 18675273-18681809 x0, 18681810 x1) mat; c.53-?_78+?del, del ex2,
5 more items
-
RS1_000018
ACMG PVS1, PS4, PM2, PP4, no amplification exon 2 ,
1 more item
PubMed: Abalem 2018
,
PubMed: Bender 2022
,
PubMed: Georgiou 2022
,
PubMed: Hewitt 2005
,
PubMed: Wei 2024
,
6 more items
-
-
Germline, Unknown
?, yes
-
-
-
-
Johan den Dunnen
+/., +?/.
6
1i_3i
c.(52+1_53-1)_(184+1_185-1)del
r.(53_184del), r.(?), r.?
p.(?), p.(Ala18Pro61del), p.?
ACMG
likely pathogenic, pathogenic (recessive), VUS
g.(18665453_18674772)_(18675786_18690136)del
c.(52+1_53-1)_(184+1_185-1)del, g.(18647333_18656652)_(18657666_18672016)del
del ex2-3, RS1 Deletion of exons 2 and 3, RS1 deletion of exons 2, 3, RS1 exon2-3 del
-
RS1_000424
deletion of exons 2, 3
PubMed: Fahim 2017
,
PubMed: Georgiou 2022
,
PubMed: Kondo 2019
,
PubMed: Ores 2018
,
PubMed: Wei 2024
,
1 more item
-
-
Germline, Unknown
?
-
-
-
-
Johan den Dunnen
+?/.
2
_1_4i
c.(?_-35)_(326+1_327-1)del
r.0?
p.0?
-
likely pathogenic
g.(18662746_18665310)_(18690223_?)del
g.(18644626_18647190)_(18672103_?)del
RS1 deletion of exons 1, 2, 3, 4
-
RS1_000427
deletion of exons 1, 2, 3, 4
PubMed: Ores 2018
-
-
Unknown
?
-
-
-
-
LOVD
+?/.
1
1i_5i
c.(52+1_53-1)_(522+1_523-1)del
r.?
p.?
-
likely pathogenic
g.(18660277_18662549)_(18675786_18690136)del
g.(18642157_18644429)_(18657666_18672016)del
RS1 deletion of exons 2, 3, 4, 5
-
RS1_000426
deletion of exons 2, 3, 4, 5
PubMed: Ores 2018
-
-
Unknown
?
-
-
-
-
LOVD
+?/.
1
2_3
c.(53_184)delN[?]
r.?
p.?
-
likely pathogenic
g.(18674775_18675787)delN[?]
g.(18656655_18657667)delN[?]
deletion in ex2 and/or ex3
-
RS1_000000
-
PubMed: Khan 2001
-
-
Germline/De novo (untested)
?
-
-
-
-
LOVD
+/.
2
2
c.61G>T
r.(?)
p.(Gly21*)
-
pathogenic
g.18675777C>A
g.18657657C>A
-
-
RS1_000137
-
PubMed: Hewitt 2005
-
-
Germline
-
-
+DraIII
-
-
Johan den Dunnen
+/.
2
2
c.68C>A
r.(?)
p.(Ser23*), p.(Ser23Ter)
ACMG
pathogenic, pathogenic (recessive)
g.18675770G>T
g.18657650G>T
-
-
RS1_000019
ACMG PVS1, PM2, PP4
PubMed: Bender 2022
,
PubMed: RS-consortium 1998, group 4, 5
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
2
c.76G>T
r.(?)
p.(Glu26*)
-
pathogenic
g.18675762C>A
g.18657642C>A
-
-
RS1_000020
-
PubMed: RS-consortium 1998, group 4
-
-
Germline
-
-
+MaeI
-
-
Johan den Dunnen
+/., +?/.
4
2
c.78G>C
r.(?)
p.(Glu26Asp)
-
likely pathogenic (recessive), pathogenic
g.18675760C>G
g.18657640C>G
-
-
RS1_000142
ACMG PVS1, PM2, PP2, PP3, PP4
PubMed: Georgiou 2022
,
PubMed: Pimenides 2005
-
-
Germline
-
-
+MaeI
-
-
Johan den Dunnen
+/., +?/.
2
2i
c.78+1G>A
r.spl
p.(?), p.?
ACMG
likely pathogenic, pathogenic
g.18675759C>T
g.18657639C>T
RS1 c.78+1G>A, p.(?)
-
RS1_000152
hemizygous
PubMed: Inoue 2002
,
PubMed: Kim 2021
-
-
Germline, Unknown
?
-
-
-
-
Johan den Dunnen
+/.
1
2i
c.78+1G>C
r.spl
p.?
-
pathogenic
g.18675759C>G
g.18657639C>G
-
-
RS1_000155
-
PubMed: Lesch 2008
,
PubMed: Lesch 2008
-
-
Germline
-
-
+AluI
-
-
Viktoria Szabo
+/., +?/.
6
2i
c.78+1G>T
r.(?), r.spl
p.(?), p.?
-
likely pathogenic, pathogenic, pathogenic (recessive)
g.18675759C>A
g.18657639C>A
c.78+1G>T, RS1 c.78+1G>T , p.?
-
RS1_000169
not in 108 control chromosomes
PubMed: Kim 2009
;Kim, ASHG2007 P1124,
PubMed: Lee 2025
,
PubMed: Sudha 2017
,
PubMed: Sudha 2018
-
-
Germline, Unknown
?, yes
-
-
-
-
Johan den Dunnen
+?/.
1
2i
c.78+2T>C
r.spl
p.(?)
-
likely pathogenic
g.18675758A>G
g.18657638A>G
RS1 c.78+2T>C, undetermined splicing defect
-
RS1_000403
-
PubMed: Kondo 2019
-
-
Unknown
?
-
-
-
-
LOVD
+/., ?/.
2
2i
c.78+5G>A
r.spl?
p.?
ACMG
pathogenic, VUS
g.18675755C>T
g.18657635C>T
-
-
RS1_000170
ACMG PM2, PP4, not in 108 control chromosomes
PubMed: Bender 2022
,
PubMed: Kim 2009
;Kim, ASHG2007 P1124
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Viktoria Szabo
+/., +?/.
3
2
c.78+5G>T
r.(?), r.spl, r.spl?
p.(?), p.?
ACMG
likely pathogenic, pathogenic (recessive)
g.18675755C>A
g.18657635C>A
c.78+5G>T, RS1 c.78+5G>T
-
RS1_000402
number of alleles does not match number of patients (91 vs 90)
PubMed: Chen 2014
,
PubMed: Chen 2020
,
PubMed: Gao 2021
-
-
Germline, Unknown
?, yes
1/90
-
-
-
Johan den Dunnen
+/.
1
2i
c.78+5_78+7delinsTTT
r.spl
p.?
-
pathogenic (recessive)
g.18675753_18675755delinsAAA
g.18657633_18657635delinsAAA
-
-
RS1_000478
-
PubMed: Hahn 2022
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
2i
c.78+380T>C
r.(=)
p.(=)
-
VUS
g.18675380A>G
g.18657260A>G
-
-
RS1_000209
-
-
-
-
Germline
-
-
-
-
-
Yu Sun
+/.
3
2i
c.79-2A>G
r.spl
p.?
-
pathogenic
g.18674880T>C
g.18656760T>C
-
-
RS1_000001
-
PubMed: RS-consortium 1998, group W
,
PubMed: RS-consortium 1998; group 6
,
PubMed: Pimenides 2005
,
1 more item
-
-
Germline
-
-
-PstI
-
-
Johan den Dunnen
,
Dorothy Trump
+/., +?/.
12
2i_3i
c.(78+1_79-1)_(184+1_185-1)del
r.(79_184del), r.(?), r.spl
p.(?), p.(Asp27Asnfs*64), p.(Asp27fs)
-
likely pathogenic, pathogenic, pathogenic (recessive)
g.(18665453_18674772)_(18674879_18675759)del
g.(18647333_18656652)_(18656759_18657639)del
del ex3, RS1 exon3 del, RS1, variant 1 :Deletion exon 3
-
RS1_000021
no amplification exon 3 , solved, hemizygous
PubMed: Hahn 2022
,
PubMed: Hewitt 2005
,
PubMed: Kondo 2019
,
PubMed: RS-consortium 1998, group 1
,
3 more items
-
-
Germline, Unknown
?, yes
-
-
-
-
Johan den Dunnen
-?/., ?/.
2
3
c.87C>T
r.(?)
p.(Gly29=)
-
likely benign, VUS
g.18674870G>A
-
RS1(NM_000330.3):c.87C>T (p.G29=)
-
RS1_000270
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Nijmegen
+/.
1
-
c.96del
r.(?)
p.(Trp33GlyfsTer93)
-
pathogenic (recessive)
g.18674864del
g.18656744del
96delC
-
RS1_000477
-
PubMed: Wei 2024
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., +?/.
5
3
c.96dup
r.(?)
p.(Trp33Leufs*53), p.(Trp33LeufsTer53)
ACMG
likely pathogenic, pathogenic, pathogenic (recessive)
g.18674864dup
g.18656744dup
92_96insC, RS1 c.(92_97)insC, (p.W33fs), RS1 c.96dup, p.(Trp33Leufs*53)
-
RS1_000211
ACMG PVS1, PS4, PM2, PP4, error in annotation, variant automapped to c.96dup
Kucinskas, ASHG2017, P1110,
PubMed: Bender 2022
,
PubMed: Ores 2018
,
PubMed: Strupaite 2018
-
-
Germline, Unknown
?, yes
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.96dupC
r.(?)
p.(Trp33Leufs*53)
-
likely pathogenic
g.18674864dup
g.18656744dup
c.96dupC, p.Trp33Leu*53
-
RS1_000211
-
PubMed: Pennesi 2018
-
-
Unknown
?
-
-
-
-
LOVD
+/., +?/.
11
3
c.97del
r.(?)
p.(Trp33Glyfs*93), p.(Trp33GlyfsTer93)
ACMG
likely pathogenic, pathogenic (recessive)
g.18674860del
g.18656740del
97delT, c.97delT, RS1 c.97del, p.(Trp33Glyfs*93)
-
RS1_000261
ACMG PVS1, PM2, PP4, no protein change given
PubMed: Bender 2022
,
PubMed: Gao 2021
,
PubMed: Holtan 2020
,
PubMed: Lai 2015
,
PubMed: Lee 2025
,
1 more item
-
-
Germline, Unknown
?, yes
1/899 cases
-
-
-
Global Variome, with Curator vacancy
,
Johan den Dunnen
+/., +?/.
3
3
c.98G>A
r.(?)
p.(Trp33*), p.(Trp33Ter)
ACMG
likely pathogenic, pathogenic
g.18674859C>T
g.18656739C>T
c.98G>A, p.W33X, RS1 c.98G>A, p.Trp33*, RS1 c.98G>A, p.W33X
-
RS1_000401
number of alleles does not match number of patients (91 vs 90)
PubMed: Chen 2014
,
PubMed: Chen 2020
,
PubMed: Kondo 2019
-
-
Germline, Unknown
?, yes
1/90
-
-
-
LOVD
+/., +?/.
3
3
c.99G>A
r.(?)
p.(Trp33*), p.(Trp33Ter)
ACMG
likely pathogenic, pathogenic (recessive)
g.18674858C>T
g.18656738C>T
c.99G>A, p.Trp33*
-
RS1_000400
ACMG PVS1, PS4, PM2, PP4
PubMed: Bender 2022
,
PubMed: Pennesi 2018
-
-
Germline, Unknown
?
-
-
-
-
Johan den Dunnen
+/., +?/.
4
3
c.103C>T
r.(?)
p.(Gln35*), p.(Gln35Ter)
ACMG
likely pathogenic, pathogenic, pathogenic (recessive)
g.18674854G>A
g.18656734G>A
Substitution 103C>T, p. Gln35X
-
RS1_000138
ACMG PVS1, PM2, PP3, ACMG PVS1, PM2, PP4
PubMed: Bender 2022
,
PubMed: Georgiou 2022
,
PubMed: Hewitt 2005
,
PubMed: Sergeev 2013
-
-
Germline, Unknown
?
-
-KpnI
-
-
Johan den Dunnen
+/.
1
3
c.108del
r.(?)
p.(Ala37Hisfs*89)
-
pathogenic
g.18674853del
g.18656733del
c.108del, p.(Ala37Hisfs*89)
-
RS1_000295
error in annotation: c.108del instead of, hemizygous
PubMed: Wang 2019
-
-
Germline
?
-
-
-
-
LOVD
+/., +?/.
15
3
c.120C>A
r.(?)
p.(Cys40*), p.(Cys40Ter)
ACMG
likely pathogenic, pathogenic, pathogenic (recessive)
g.18674837G>T
g.18656717G>T
RS1 c.120C>A, p.(Cys40X)
-
RS1_000002
ACMG PVS1, PM2, PP4, BP4, ACMG PVS1, PS4, PM2, PP4
PubMed: Bender 2022
,
PubMed: Eksandh 2000
,
PubMed: Fahim 2017
,
PubMed: Georgiou 2022
,
8 more items
-
rs62645885
Germline, Unknown
?
-
-
-
-
Johan den Dunnen
,
Dorothy Trump
+?/.
1
-
c.127C>T
r.(?)
p.(Gln43Ter)
-
likely pathogenic
g.18674830G>A
-
-
-
RS1_000432
-
-
-
-
CLASSIFICATION record
-
-
-
-
-
MobiDetails
+/.
1
3
c.130_140del
r.(?)
p.(Gly44CysfsTer38)
-
pathogenic (recessive)
g.18674821_18674831del
g.18656701_18656711del
-
-
RS1_000476
-
PubMed: Lee 2025
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
3
c.140dup
r.(?)
p.(Asn47Lysfs*39)
-
pathogenic
g.18674818dup
g.18656698dup
c.140dup, p.(Asn47Lysfs*39)
-
RS1_000294
error in annotation: c.140dup instead of, hemizygous
PubMed: Wang 2019
-
-
Germline
yes
-
-
-
-
LOVD
+/.
2
3
c.149G>A
r.(?)
p.(Trp50*), p.(Trp50Ter)
-
pathogenic, pathogenic (recessive)
g.18674808C>T
g.18656688C>T
-
-
RS1_000135
-
PubMed: Eksandh 2005
,
PubMed: Fortunato 2023
-
-
Germline
-
-
+AciI
-
-
Johan den Dunnen
+/., +?/.
2
3
c.150G>A
r.(?)
p.(Trp50*), p.(Trp50Ter)
-
likely pathogenic, pathogenic (recessive)
g.18674807C>T
g.18656687C>T
c.150G>A
-
RS1_000300
-
PubMed: Lee 2025
,
PubMed: Maggi_2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
3
c.160_163dup
r.(?)
p.(Thr55Serfs*32)
-
pathogenic
g.18674794_18674797dup
g.18656674_18656677dup
-
-
RS1_000122
-
PubMed: Hiraoka 2000
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
3
c.163del
r.(?)
p.(Thr55Profs*71), p.(Thr55ProfsTer71)
ACMG
pathogenic, pathogenic (recessive)
g.18674794del
g.18656674del
-
-
RS1_000101
ACMG PVS1, PM2, PP4
PubMed: Bender 2022
,
PubMed: Gehrig 1999
-
-
Germline
-
-
-BstXI +SduI
-
-
Johan den Dunnen
+/.
1
3
c.173_184+21del
r.spl
p.?
-
pathogenic
g.18674759_18674791del
g.18656639_18656671del
-
-
RS1_000107
-
PubMed: Shinoda 2000
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
5
3
c.175T>A
r.(?)
p.(Cys59Ser)
-
NA, pathogenic
g.18674782A>T
g.18656662A>T
-
-
RS1_000022
expression construct in pcDNA3.1, COS-7 cell expression
PubMed: Gehrig 1999
,
PubMed: Renner 2008
,
PubMed: RS-consortium 1998, group 2
,
PubMed: Wang 2002
,
1 more item
-
-
De novo, Germline, In vitro (cloned)
-
-
-
-
-
Johan den Dunnen
+?/.
6
3
c.175T>G
r.(?)
p.(Cys59Gly)
-
likely pathogenic
g.18674782A>C
g.18656662A>C
c.175A>C, p.(Cys59Gly), RS1 c.175T>G, p.Cys59Gly
-
RS1_000293
hemizygous
PubMed: Kondo 2019
,
PubMed: Wang 2019
-
-
Germline
yes
-
-
-
-
LOVD
+/., +?/.
5
3
c.176G>A
r.(?)
p.(Cys59Tyr)
ACMG
likely pathogenic (recessive), pathogenic
g.18674781C>T
g.18656661C>T
RS1 176G>A, Cys59Tyr, RS1 c.176G>A , p. C59Y
-
RS1_000320
ACMG PM1, PM2, PM5, PP1, PP2, PP3, PP4, PP5,
1 more item
PubMed: Bender 2022
,
PubMed: Chen 2020
,
PubMed: Yi 2012
-
-
Germline, Unknown
?
1/90
-
-
-
Johan den Dunnen
+/., +?/.
3
3
c.176G>C
r.(?)
p.(Cys59Ser)
ACMG
likely pathogenic, likely pathogenic (recessive)
g.18674781C>G
g.18656661C>G
RS1 c.176G>C (p.Cys59Ser)
-
RS1_000321
ACMG PS3, PM2, PP3, PP4, PP
PubMed: Bender 2022
,
PubMed: Skorczyk 2012
-
-
Germline, Unknown
?
-
-
-
-
Johan den Dunnen
+/.
1
3_3i
c.183_184+12del
r.spl
p.?
-
pathogenic
g.18674763_18674776del
g.18656643_18656656del
181-184+10del
-
RS1_000423
-
PubMed: RS-consortium 1998, group 5
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., +?/.
3
3i, 3_3i
c.184+1G>C
r.spl
p.(?), p.?
ACMG
likely pathogenic, pathogenic, pathogenic (recessive)
g.18674772C>G
g.18656652C>G
IVS3+1G to C
-
RS1_000399
ACMG PVS1, PM2, PP4
PubMed: Bender 2022
,
PubMed: Khan 2001
,
PubMed: RS-consortium 1998, group 3
-
-
Germline, Unknown
?
-
-
-
-
Johan den Dunnen
+/., +?/.
3
3i
c.184+2T>G
r.spl
p.(?), p.?
-
likely pathogenic, pathogenic (recessive)
g.18674771A>C
g.18656651A>C
RS1 c.184+2T>G, p.?, Splice site 184+2T>G
-
RS1_000398
ACMG PVS1, PM2, PP3, PP4, PP5
PubMed: Fahim 2017
,
PubMed: Georgiou 2022
,
PubMed: Sergeev 2013
-
rs1555959367
Germline, Unknown
?
-
-
-
-
Johan den Dunnen
-/., ?/.
4
3i
c.184+35T>C
r.(=)
p.(=)
-
benign, VUS
g.18674738A>G
g.18656618A>G
184+35C>T
-
RS1_000157
-
PubMed: Kim 2009
;Kim, ASHG2007 P1124,
PubMed: Kjellstrom 2010
-
rs6633109
Germline
-
0.07
-
-
-
Johan den Dunnen
-/.
2
3i
c.184+129T>G
r.(=)
p.(=)
-
benign
g.18674644A>C
g.18656524A>C
184+129G>T
-
RS1_000171
-
PubMed: Kim 2009
;Kim, ASHG2007 P1124
-
-
Germline
-
-
-
-
-
Johan den Dunnen
10 per page
25 per page
50 per page
100 per page
Legend
How to query
« First
Prev
1
2
3
4
5
6
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