Global Variome shared LOVD
SAG (S-antigen; retina and pineal gland (arrestin))
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Global Variome, with Curator vacancy
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Unique variants in the SAG gene
This database is one of the
"Eye disease"
gene variant databases.
The variants shown are described using the NM_000541.4 transcript reference sequence.
Legend
Please note that a short description of a certain column can be displayed when you move your mouse cursor over the column's header and hold it still. Below, a more detailed description is shown per column.
Effect
: The variant's effect on the function of the gene/protein, displayed in the format 'R/C'. R is the value reported by the source (publication, submitter) and this classification may vary between records. C is the value concluded by the curator. Note that in some database the curator uses Summary records to give details on the classification of the variant.Values used: '+' indicating the variant affects function, '+?' probably affects function, '-' does not affect function, '-?' probably does not affect function, '?' effect unknown, '.' effect was not classified.
Reported
: The number of times this variant has been reported in the database.
Exon
: number of exon/intron containing variant; 2 = exon 2, 12i = intron 12, 2i_7i = from intron 2 to intron 7, 8i_9 = intron 8/exon 9 boundary, _1 = 5' to exon 1, 18_ = 3' of exon 18, _1_18_ = encompassing the entire 18-exon gene
DNA change (cDNA)
: description of variant at DNA level, based on a coding DNA reference sequence (following HGVS recommendations); e.g. c.123C>T, c.123_145del, c.123_126dup. For deletions/duplications extending beyond the reference transcript resp. {0}/{2} is used to replace del/dup. Extent of the deletion/duplication should be specified using the genomic description (g.). "-" indicates the variant described on genomic level does not affect the coding DNA reference sequence.
RNA change
: description of variant at RNA level (following HGVS recommendations).
r.123c>u
r.? = unknown
r.(?) = RNA not analysed but probably transcribed copy of DNA variant
r.spl? = RNA not analysed but variant probably affects splicing
r.(spl?) = RNA not analysed but variant may affect splicing
r.0? = change expected to abolish transcription
Protein
: description of variant at protein level (following HGVS recommendations).
p.(Arg345Pro) = change predicted from DNA (RNA not analysed)
p.Arg345Pro = change derived from RNA analysis
p.? = unknown effect
p.0? = probably no protein produced
Classification method
: The method used for the clinical classification of this variant.
All options:
ACMG
ACGS
EAHAD-CFDB
ENIGMA
IARC
InSiGHT
kConFab
other
Clinical classification
: Clinical classification of variant, preferably based on standardised criteria (e.g. ACMG), directed on the clinical consequences as published/submitted, indicated using an enriched system including inheritance: e.g. pathogenic, pathogenic (dominant), pathogenic (recessive), pathogenic (!), pathogenic (maternal), pathogenic (paternal). Standard inheritance is covered by dominant/recessive, imprinting by maternal/paternal. A '!' warns for exceptional circumstances to be explained in the 'Remarks' field (low penetrance, variants pathogenic in heterozygous state only, hypomorphic/hypermorphic variants, protective variants, etc.). Non-disease consequences (e.g. drug metabolism (pharmacogenetics), risk factor, blood group, tasting bitter) are indicated using additions to the benign classification; benign (dominant), benign (recessive), benign (!), etc. The value 'association' is used for variants associated with a phenotype and 'NA' for variants from in vitro/in silico records. NOTE: classification may differ from the opinion of the curator as given in a variant SUMMARY-record or the 'Functional effect concluded'). NOTE: pathogenic/likely pathogenic should go together with "variant (probably) affects function" In ClassFunctional.
All options:
pathogenic
pathogenic (dominant)
pathogenic (recessive)
pathogenic (!)
pathogenic (maternal)
pathogenic (paternal)
likely pathogenic
likely pathogenic (dominant)
likely pathogenic (recessive)
likely pathogenic (!)
likely pathogenic (maternal)
likely pathogenic (paternal)
VUS
VUS (!)
likely benign
likely benign (dominant)
likely benign (recessive)
likely benign (!)
likely benign (maternal)
likely benign (paternal)
benign
benign (dominant)
benign (recessive)
benign (!)
benign (maternal)
benign (paternal)
conflicting
association
NA
DNA change (genomic) (hg19)
: HGVS description of variant at DNA level, based on the genomic (chromosomal) DNA reference sequence; e.g. g.12345678C>T, g.12345679del, g.12345678_12345890dup
DNA change (hg38)
: HGVS description of variant at DNA level, based on the hg38 genomic (chromosomal) eference sequence; e.g. g.12345678C>T, g.12345679del, g.12345678_12345890dup
Published as
: listed only when different from "DNA change"; variant as reported originally (e.g. 521delT). Variants seen in animal models, tested in vitro, predicted from RNA analysis, etc. are described between brackets like c.(456C>G)
ISCN
: description of the variant according to ISCN nomenclature
DB-ID
: database ID of variant, grouping multiple observations of the same variant together, starting with the HGNC gene symbol, followed by an underscore (_) and a six digit number (e.g. DMD_012345). _000000 is used for variants where DNA was not analysed (change predicted from RNA analysis), variants seen in animal models or variants not seen in humans but functionally tested in vitro
Variant remarks
: remarks regarding variant described, e.g. germline mosaicism in mother, 345 kb deletion, muscle RNA analysed, not in 200 control chromosomes tested, on founder haplotype, etc.
Reference
: publication describing the variant submitted, incl. links to OMIM, PubMed or other source, e.g. "den Dunnen ASHG2003 P2346"
ClinVar ID
: ID of variant in ClinVar database
dbSNP ID
: the dbSNP ID
Origin
: Origin of variant/record: Germline = in all cells, De novo = in all cells, but not in either parent, Germline/De novo (untested) = in all cells, parents not tested (use only when De novo is likely, e.g. isolated/sporadic cases with dominant disease), Somatic = present in a subset of cells, but not in either parent, Uniparental disomy = from parental disomy (maternal or paternal), CLASSIFICATION record = submitter only sharing variant classification (note another report may share Individual data), SUMMARY record = master summary record from curator (may link to another database), In vitro (cloned) = data resulting from in vitro functional assays, animal model = data from animal model, Artefact = false positive variant call, DUPLICATE record = variant already described on another chromosome (e.g. unbalanced translocation, duplicating transposition, 2nd fusion transcript, etc.)
All options:
Germline
De novo
Germline/De novo (untested)
Somatic
Uniparental disomy
Uniparental disomy, maternal allele
Uniparental disomy, paternal allele
CLASSIFICATION record
SUMMARY record
In vitro (cloned)
In silico
animal model
Artefact
DUPLICATE record
Unknown
Not applicable
Segregation
: Indicates whether the variant segregates with the phenotype (yes), does not segregate with the phenotype (no) or segregation is unknown (?)
All options:
? = unknown
yes = segregates with phenotype
no = does not segregate with phenotype
- = not applicable
Frequency
: frequency in which the variant was found; e.g 5/760 chromosomes (in 5 of 760 chromosomes tested), 1/33 patients (in 1 of 33 patients analysed in study), 0.05 controls (in 5% of control cases tested)
Re-site
: restriction enzyme recognition site created (+) or destroyed (-); e.g. BglII+;BamHI-
VIP
: variant VIP-status was requested for matchmaking - need collaboration(s) to crack the case - please contact the submitter/curator. NOTE: to get VIP status ask the curator.
Methylation
: result of methylation test; GOM (gain of methylation), LOM (loss of methylation), 30% (30% methylated). NOTE: when several tests were done mention the method as well (e.g. MS-PCR 75%)
How to query this table
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.
77 entries on 1 page. Showing entries 1 - 77.
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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.-28-4_-25del
r.spl?
p.?
-
VUS
g.234217804_234217811del
g.233309158_233309165del
SAG(NM_000541.4):c.-28-4_-25delCAAGGTGG
-
SAG_000041
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
1i_2i
c.-25_75+3124del
r.0?
p.0?
-
pathogenic (recessive)
g.234217811_234221034del
g.233309165_233312388del
-
-
SAG_000040
-
PubMed: Huang 2012
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
3
2
c.31G>A
r.(?)
p.(Glu11Lys)
-
VUS
g.234217866G>A
g.233309220G>A
p.SAG-E11K, SAG(NM_000541.5):c.31G>A (p.E11K)
-
SAG_000002
VKGL data sharing initiative Nederland
PubMed: Schorderet-2013
-
-
CLASSIFICATION record, Unknown
no
-
-
-
-
VKGL-NL_Nijmegen
,
VKGL-NL_AMC
-?/.
1
-
c.35C>T
r.(?)
p.(Pro12Leu)
-
likely benign
g.234217870C>T
g.233309224C>T
SAG(NM_000541.5):c.35C>T (p.P12L)
-
SAG_000003
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+?/.
1
-
c.72_75+15delATC GGTGAGTGGT GCACAA
r.spl
p.(?)
-
likely pathogenic
g.55973730_55973731del
g.54213970_54213971del
different transcript: NM_001142763.1(PCDH15):c.1081_1082delGA; p.Asp361Leufs
-
SAG_000001
Ocular disease gene; heterozygous variant
PubMed: Wan 2018
-
-
Unknown
?
-
-
-
-
LOVD
?/.
1
-
c.74C>T
r.(?)
p.(Ser25Leu)
ACMG
VUS
g.234217909C>T
g.233309263C>T
SAG:NM_000541 c.C74T, p.S25L
-
SAG_000059
heterozygous, individual solved, variant non-causal
PubMed: Rodriguez-Munoz 2020
-
-
Germline
?
-
-
-
-
LOVD
+?/.
1
-
c.75G>A
r.(?)
p.(Ser25=)
-
likely pathogenic
g.234217910G>A
g.233309264G>A
SAG, variant 1: c.75G>A/p.?, variant 2: c.75G>A/p.?
-
SAG_000062
possibly solved, homozygous
PubMed: Weisschuh 2020
-
-
Unknown
?
-
-
-
-
LOVD
-?/.
1
-
c.93G>A
r.(?)
p.(Gly31=)
-
likely benign
g.234224738G>A
g.233316092G>A
SAG(NM_000541.4):c.93G>A (p.G31=)
-
SAG_000004
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/.
1
-
c.107T>C
r.(?)
p.(Ile36Thr)
-
likely pathogenic (recessive)
g.234224752T>C
g.233316106T>C
-
-
SAG_000054
-
PubMed: Xu 2014
-
rs188789430
Germline
-
1/314 case chromosomes
-
-
-
LOVD
+?/.
1
-
c.136+2T>G
r.spl
p.(?)
-
likely pathogenic
g.234224783T>G
g.233316137T>G
SAG, variant 1: c.136+2T>G/p.?, variant 2: c.806+4A>G/p.?
-
SAG_000063
possibly solved, compound heterozygous
PubMed: Weisschuh 2020
-
-
Unknown
?
-
-
-
-
LOVD
?/.
1
-
c.180A>G
r.(?)
p.(=)
-
VUS
g.234227440A>G
-
SAG(NM_000541.5):c.180A>G (p.(Lys60=))
-
SAG_000074
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-/.
1
-
c.181+82A>G
r.(=)
p.(=)
-
benign
g.234227523A>G
g.233318877A>G
SAG(NM_000541.5):c.181+82A>G
-
SAG_000005
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
?/.
1
-
c.182-2A>G
r.spl?
p.?
-
VUS
g.234229274A>G
g.233320628A>G
SAG(NM_000541.5):c.182-2A>G
-
SAG_000006
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/.
2
-
c.201C>T
r.(=)
p.(=)
-
likely benign
g.234229295C>T
g.233320649C>T
-
-
SAG_000046
3 homozygous;
Clinindb (India)
, 69 heterozygous;
Clinindb (India)
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs72976383
Germline
-
3/2794 individuals, 69/2794 individuals
-
-
-
Mohammed Faruq
?/.
1
-
c.212A>C
r.(?)
p.(Tyr71Ser)
-
VUS
g.234229306A>C
-
SAG(NM_000541.4):c.212A>C (p.Y71S)
-
SAG_000048
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/.
3
-
c.226A>G
r.(?)
p.(Ile76Val)
-
benign
g.234229320A>G
g.233320674A>G
-
-
SAG_000025
VKGL data sharing initiative Nederland
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
rs7565275
CLASSIFICATION record, Germline
-
14/1204 cases with retinitis pigmentosa, 156/1204 cases with retinitis pigmentosa
-
-
-
VKGL-NL_Nijmegen
,
Yoshito Koyanagi
-/.
1
-
c.231C>T
r.(?)
p.(Asp77=)
-
benign
g.234229325C>T
g.233320679C>T
SAG(NM_000541.5):c.231C>T (p.D77=)
-
SAG_000007
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
1
-
c.238G>T
r.(?)
p.(Gly80Cys)
-
VUS
g.234229332G>T
g.233320686G>T
-
-
SAG_000029
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
-
Germline
-
1/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
-/.
1
-
c.250C>T
r.(?)
p.(Arg84Cys)
-
benign
g.234229344C>T
g.233320698C>T
SAG(NM_000541.5):c.250C>T (p.R84C)
-
SAG_000008
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
1
-
c.254G>C
r.(?)
p.(Arg85Thr)
-
VUS
g.234229348G>C
g.233320702G>C
-
-
SAG_000030
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
-
Germline
-
1/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
+/.
1
-
c.271C>T
r.(?)
p.(Arg91Trp)
-
pathogenic
g.234229365C>T
g.233320719C>T
-
-
SAG_000053
-
PubMed: Costa 2017
-
-
Germline
-
-
-
-
-
LOVD
-?/.
1
-
c.300C>T
r.(?)
p.(Ala100=)
-
likely benign
g.234229394C>T
g.233320748C>T
SAG(NM_000541.4):c.300C>T (p.A100=)
-
SAG_000009
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/., ?/.
3
-
c.301G>A
r.(?)
p.(Ala101Thr)
-
benign, VUS
g.234229395G>A
g.233320749G>A
SAG(NM_000541.4):c.301G>A (p.A101T), SAG(NM_000541.5):c.301G>A (p.A101T)
-
SAG_000010
VKGL data sharing initiative Nederland
PubMed: Wang 2014
-
rs141521563
CLASSIFICATION record, Germline
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
-?/.
1
-
c.357G>A
r.(?)
p.(=)
-
likely benign
g.234229451G>A
-
SAG(NM_000541.5):c.357G>A (p.T119=)
-
SAG_000072
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+?/., -?/., ?/.
6
5
c.374C>T
r.(?)
p.(Thr125Met)
-
likely benign, likely pathogenic, VUS
g.234229468C>T
g.233320822C>T
c.374C>T, SAG(NM_000541.4):c.374C>T (p.T125M), SAG(NM_000541.5):c.374C>T (p.T125M), Thr125Met
-
SAG_000028
VKGL data sharing initiative Nederland
PubMed: Eisenberger-2013
,
PubMed: Olivé 2015
,
PubMed: Wang 2014
-
rs137886124
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
+/.
1
-
c.374del
r.(?)
p.(Thr125SerfsTer10)
-
pathogenic
g.234229468del
g.233320822del
SAG(NM_000541.5):c.374delC (p.T125Sfs*10)
-
SAG_000011
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/.
1
-
c.375+11C>T
r.(=)
p.(=)
-
benign
g.234229480C>T
g.233320834C>T
-
-
SAG_000026
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
-
c.379C>T
r.(?)
p.(Pro127Ser)
-
VUS
g.234231595C>T
g.233322949C>T
-
-
SAG_000042
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
6
c.398C>T
r.(?)
p.(Ser133Leu)
-
pathogenic
g.234231614C>T
-
c.398C>T: p. (Ser133Leu)
-
SAG_000067
-
PubMed: Colombo 2019
-
-
Germline
yes
-
-
-
-
LOVD
-?/.
1
-
c.420A>G
r.(?)
p.(Pro140=)
-
likely benign
g.234231636A>G
-
SAG(NM_000541.4):c.420A>G (p.P140=)
-
SAG_000047
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/.
2
6i
c.436-18G>C
r.(=), r.(?)
p.(=)
-
benign
g.234235749G>C
g.233327103G>C
IVS6-18G>C, SAG(NM_000541.5):c.436-18G>C
-
SAG_000012
VKGL data sharing initiative Nederland
PubMed: Huang 2012
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Groningen
?/.
1
-
c.437C>A
r.(?)
p.(Ser146Tyr)
-
VUS
g.234235768C>A
g.233327122C>A
-
-
SAG_000031
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
-
Germline
-
1/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
+/.
16
7
c.440G>T
r.(?)
p.(Cys147Phe)
ACMG
pathogenic
g.234235771G>T
-
c.440G>T; p.Cys147Phe
-
SAG_000068
-
Villafuerte-de la Cruz RA, et al., 2023. Submitted,
PubMed: Sullivan 2017
ClinVar-1391715
,
ClinVar-3353
,
ClinVar-862338
rs753107507
Germline
yes
-
-
-
-
Rocio Villafuerte-de la Cruz
+?/.
1
-
c.442G>A
r.(?)
p.(Gly148Arg)
-
likely pathogenic (recessive)
g.234235773G>A
g.233327127G>A
-
-
SAG_000055
-
PubMed: Xu 2014
-
-
Germline
-
1/314 case chromosomes
-
-
-
LOVD
-?/.
1
-
c.468C>T
r.(?)
p.(=)
-
likely benign
g.234235799C>T
-
SAG(NM_000541.5):c.468C>T (p.F156=)
-
SAG_000071
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/., +?/., ?/.
4
7
c.473C>A
r.(?)
p.(Thr158Lys)
-
likely pathogenic, pathogenic, VUS
g.234235804C>A
g.233327158C>A
c.473C>A, SAG(NM_000541.4):c.473C>A (p.T158K), SAG(NM_000541.5):c.473C>A (p.T158K)
-
SAG_000013
VKGL data sharing initiative Nederland
PubMed: Costa 2017
,
PubMed: Eisenberger-2013
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
-/.
1
-
c.489C>T
r.(?)
p.(Ala163=)
-
benign
g.234235820C>T
g.233327174C>T
SAG(NM_000541.5):c.489C>T (p.A163=)
-
SAG_000014
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-?/., ?/.
2
-
c.511A>G
r.(?)
p.(Lys171Glu)
-
likely benign, VUS
g.234235842A>G
g.233327196A>G
SAG(NM_000541.4):c.511A>G (p.K171E, p.(Lys171Glu))
-
SAG_000015
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
+?/.
1
-
c.(512+1_513-1)_(806+1_807-1)del
r.(?)
p.(?)
ACMG
likely pathogenic
g.?
g.?
SAG c.(512+1_513-1) _(806+1_807-1)del, BBS9 c.1277_1280del, p.(Gln426Argfs*16)
-
SNRNP200_000007
single heterozygous variant (recessive)
PubMed: Jespersgaar 2019
-
-
Germline
?
-
-
-
-
LOVD
+/., ?/.
2
8
c.523C>T
r.(?)
p.(Arg175*)
-
pathogenic (recessive), VUS
g.234237134C>T
g.233328488C>T
744C>T (Arg175*)
-
SAG_000037
-
PubMed: Nakamura 2004
,
PubMed: Zeitz-2009
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
1
-
c.525A>G
r.(?)
p.(Arg175=)
-
benign
g.234237136A>G
g.233328490A>G
SAG(NM_000541.5):c.525A>G (p.R175=)
-
SAG_000016
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
1
-
c.548A>G
r.(?)
p.(His183Arg)
ACMG
VUS
g.234237159A>G
g.233328513A>G
SAG:NM_000541 c.A548G, p.H183R
-
SAG_000060
heterozygous, individual unsolved, causality of variants unknown
PubMed: Rodriguez-Munoz 2020
-
-
Germline
?
-
-
-
-
LOVD
+/., +?/., ?/.
16
8
c.577C>T
r.(?)
p.(Arg193*), p.(Arg193Ter)
ACMG
likely pathogenic, pathogenic, pathogenic (recessive), VUS
g.234237188C>T
g.233328542C>T
c.577C>T, c.577C>T, p.Arg193Ter, SAG Ex.8 c.577C>T p.(Arg193*), Ex.8 c.577C>T p.(Arg193*),
3 more items
-
SAG_000036
ACMG PM2, PVS1, PP5, heterozygous, homozygous, solved, homozygous, Unknown 2nd allele,
1 more item
PubMed: Avila Fernandez 2010
,
PubMed: Eisenberger-2013
,
PubMed: Gao 2019
,
PubMed: Huang 2012
,
5 more items
-
rs201153410
CLASSIFICATION record, Germline, Germline/De novo (untested), Unknown
?, yes
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Rotterdam
-/., -?/.
2
-
c.588G>A
r.(?)
p.(Ala196=)
-
benign, likely benign
g.234237199G>A
g.233328553G>A
SAG(NM_000541.4):c.588G>A (p.A196=), SAG(NM_000541.5):c.588G>A (p.A196=)
-
SAG_000017
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
?/.
1
-
c.624C>A
r.(?)
p.(His208Gln)
-
VUS
g.234237235C>A
g.233328589C>A
-
-
SAG_000056
-
PubMed: Xu 2014
-
rs183412440
Germline
-
1/314 case chromosomes
-
-
-
LOVD
-?/.
1
-
c.681C>T
r.(?)
p.(Thr227=)
-
likely benign
g.234238171C>T
-
SAG(NM_000541.5):c.681C>T (p.T227=)
-
SAG_000058
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+?/.
1
-
c.682G>A
r.(?)
p.(Val228Met)
-
likely pathogenic
g.234238172G>A
g.233329526G>A
SAG, variant 1: c.682G>A/p.V228M, variant 2 :Duplication exon 15-16
-
SAG_000064
possibly solved, compound heterozygous
PubMed: Weisschuh 2020
-
-
Unknown
?
-
-
-
-
LOVD
-/.
1
9i
c.733+31T>G
r.(?)
p.(=)
-
benign
g.234238254T>G
g.233329608T>G
IVS9+31T>G
-
SAG_000039
-
PubMed: Huang 2012
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.734-18C>A
r.(=)
p.(=)
-
likely benign
g.234240268C>A
g.233331622C>A
SAG(NM_000541.5):c.734-18C>A
-
SAG_000045
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/.
1
-
c.778G>A
r.(?)
p.(Val260Ile)
-
likely benign
g.234240330G>A
-
SAG(NM_000541.4):c.778G>A (p.V260I)
-
SAG_000050
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.787G>A
r.(?)
p.(Val263Met)
-
VUS
g.234240339G>A
-
-
-
SAG_000051
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/., -?/.
2
-
c.804G>T
r.(?)
p.(Ala268=)
-
benign, likely benign
g.234240356G>T
g.233331710G>T
SAG(NM_000541.4):c.804G>T (p.A268=), SAG(NM_000541.5):c.804G>T (p.A268=)
-
SAG_000018
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
+?/.
2
-
c.806+4A>G
r.spl?
p.(?), p.?
ACMG
likely pathogenic, likely pathogenic (recessive)
g.234240362A>G
g.233331716A>G
SAG, variant 1: c.136+2T>G/p.?, variant 2: c.806+4A>G/p.?
-
SAG_000065
ACMG PP3, PM2, PP5_STRONG, possibly solved, compound heterozygous
PubMed: Weisschuh 2020
,
PubMed: Weisschuh 2024
-
-
Germline, Unknown
?
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.862C>T
r.(?)
p.(Leu288=)
-
likely benign
g.234243663C>T
-
SAG(NM_000541.4):c.862C>T (p.L288=)
-
SAG_000052
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.866C>G
r.(?)
p.(Ala289Gly)
-
VUS
g.234243667C>G
-
SAG(NM_000541.4):c.866C>G (p.A289G)
-
SAG_000049
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.874C>G
r.(?)
p.(Arg292Gly)
ACMG
VUS
g.234243675C>G
g.233335029C>G
SAG:NM_000541 c.C874G, p.R292G
-
SAG_000061
heterozygous, individual solved, variant non-causal
PubMed: Rodriguez-Munoz 2020
-
-
Germline
?
-
-
-
-
LOVD
+/., +?/., ?/.
6
11
c.874C>T
r.(?)
p.(Arg292*)
-
likely pathogenic, pathogenic, pathogenic (recessive), VUS
g.234243675C>T
g.233335029C>T
1195C>T, c.874C>T, c.[874C>T]+[874C>T]
-
SAG_000001
-
PubMed: Li 2017
,
PubMed: Nakamura 2004
,
PubMed: O'Sullivan-2012
,
PubMed: Sergouniotis 2011
,
2 more items
-
-
Germline, Unknown
yes
-
-
-
-
Johan den Dunnen
,
James Hejtmancik
?/.
1
-
c.916G>A
r.(?)
p.(Glu306Lys)
-
VUS
g.234243717G>A
g.233335071G>A
SAG(NM_000541.4):c.916G>A (p.(Glu306Lys))
-
SAG_000043
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
1
11
c.916G>T
r.(?)
p.(Glu306*)
-
pathogenic (recessive)
g.234243717G>T
g.233335071G>T
-
-
SAG_000038
-
PubMed: Waheed 2012
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
+/.
2
-
c.923del
r.(?)
p.(Thr308Lysfs*13)
-
pathogenic
g.234243724del
g.233335078del
-
-
SAG_000032
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
rs754158163
Germline
-
6/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
+/., +?/., ?/.
32
11
c.926del
r.(?)
p.(Asn309Thrfs*12), p.(Asn309ThrfsTer12)
-
likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive), VUS
g.234243726delA, g.234243727del
g.233335081del
1147Adel, 1147de1A, 1147delA, 924delA, 926delA, c.924delA:p.T308fs, c.925delA (p.Asn309ThfsX12),
3 more items
-
SAG_000027
homozygous, c.924delA automapped to NM_000541.4:c.926delA, variant on other allel not excluded
PubMed: Fuchs 1995
,
PubMed: Fujinami 2011
,
PubMed: Hayashi 2007
,
PubMed: Katagiri 2014
,
13 more items
-
-
Germline, Unknown
yes
-
-
-
-
Johan den Dunnen
?/.
1
-
c.944+3G>A
r.(=)
p.(=)
-
VUS
g.234243748G>A
g.233335102G>A
-
-
SAG_000057
-
PubMed: Xu 2014
-
-
Germline
-
1/314 case chromosomes
-
-
-
LOVD
?/.
1
-
c.944+5G>A
r.spl?
p.?
-
VUS
g.234243750G>A
-
SAG(NM_000541.5):c.944+5G>A
-
SAG_000070
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/.
1
-
c.945-9T>C
r.(=)
p.(=)
-
likely benign
g.234247313T>C
g.233338667T>C
SAG(NM_000541.4):c.945-9T>C
-
SAG_000044
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.945-6G>A
r.(=)
p.(=)
-
likely benign
g.234247316G>A
g.233338670G>A
SAG(NM_000541.4):c.945-6G>A
-
SAG_000019
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.965G>A
r.(?)
p.(Arg322Gln)
-
VUS
g.234247342G>A
g.233338696G>A
-
-
SAG_000033
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
rs776736329
Germline
-
1/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
-/.
1
-
c.1022+11C>T
r.(=)
p.(=)
-
benign
g.234247410C>T
g.233338764C>T
SAG(NM_000541.5):c.1022+11C>T
-
SAG_000020
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/.
1
-
c.1022+109C>G
r.(=)
p.(=)
-
benign
g.234247508C>G
g.233338862C>G
SAG(NM_000541.5):c.1022+109C>G
-
SAG_000021
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-/., -?/.
2
-
c.1091C>T
r.(?)
p.(Pro364Leu)
-
benign, likely benign
g.234250961C>T
g.233342315C>T
SAG(NM_000541.4):c.1091C>T (p.P364L), SAG(NM_000541.5):c.1091C>T (p.P364L)
-
SAG_000022
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
+?/.
1
-
c.1103-2A>C
r.spl
p.(?)
-
likely pathogenic
g.234255047A>C
g.233346401A>C
SAG, variant 1: c.1103-2A>C/p.?, variant 2: c.1103-2A>C/p.?
-
SAG_000066
solved, homozygous
PubMed: Weisschuh 2020
-
-
Unknown
?
-
-
-
-
LOVD
+?/.
1
-
c.(1102+1_1103-1)_(*143_?)dup
r.spl
p.(?)
-
likely pathogenic
g.?
g.?
SAG, variant 1: c.682G>A/p.V228M, variant 2 :Duplication exon 15-16
-
SNRNP200_000007
possibly solved, compound heterozygous
PubMed: Weisschuh 2020
-
-
Unknown
?
-
-
-
-
LOVD
-?/.
1
-
c.1112+19T>C
r.(=)
p.(=)
-
likely benign
g.234255077T>C
-
SAG(NM_000541.5):c.1112+19T>C
-
SAG_000073
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
1
16
c.1147del
r.(?)
p.(Ala383Leufs*5)
-
pathogenic
g.234255487del
-
1147delA
-
SAG_000069
-
PubMed: Hayashi 2011
-
-
Germline
-
-
-
-
-
LOVD
-?/.
2
-
c.1167T>A
r.(?)
p.(Asp389Glu)
-
likely benign
g.234255507T>A
g.233346861T>A
SAG(NM_000541.4):c.1167T>A (p.D389E), SAG(NM_000541.5):c.1167T>A (p.D389E)
-
SAG_000023
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
-/.
3
16
c.1207G>A
r.(?)
p.(Val403Ile)
-
benign
g.234255547G>A
g.233346901G>A
(V430I)
-
SAG_000034
-
PubMed: Huang 2012
,
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
rs1046974
Germline
-
108/1204 cases with retinitis pigmentosa, 486/1204 cases with retinitis pigmentosa
-
-
-
Johan den Dunnen
,
Yoshito Koyanagi
-/.
2
-
c.1208T>C
r.(?)
p.(Val403Ala)
-
benign
g.234255548T>C
g.233346902T>C
-
-
SAG_000035
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
rs1046976
Germline
-
14/1204 cases with retinitis pigmentosa, 212/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
-/.
1
-
c.*8G>A
r.(=)
p.(=)
-
benign
g.234255566G>A
g.233346920G>A
SAG(NM_000541.5):c.*8G>A
-
SAG_000024
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
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