Global Variome shared LOVD
SNRNP200 (small nuclear ribonucleoprotein 200kDa (U5))
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Unique variants in the SNRNP200 gene
This database is one of the
"Eye disease"
gene variant databases.
The variants shown are described using the NM_014014.4 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%)
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Operator
Column type
Example
Matches
Text
Arg
all entries containing 'Arg'
space
Text
Arg Ser
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|
Text
Arg|Ser
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!
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'
!=""
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!=""
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'
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178 entries on 2 pages. Showing entries 1 - 100.
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Effect
Reported
Exon
DNA change (cDNA)
RNA change
Protein
Classification method
Clinical classification
DNA change (genomic) (hg19)
DNA change (hg38)
Published as
ISCN
DB-ID
Variant remarks
Reference
ClinVar ID
dbSNP ID
Origin
Segregation
Frequency
Re-site
VIP
Methylation
Owner
+/., ?/.
2
31i
c.?
r.(?), r.?
p.(?), p.?
-
pathogenic (dominant), VUS
g.?
-
Arg681Lys, IVS31-9G>A
-
SNRNP200_000007
no G at 4393-9
PubMed: Daiger 2014
,
PubMed: Neveling 2012
-
-
Germline
-
-
-
-
-
Kornelia Neveling
-/.
1
-
c.51G>T
r.(?)
p.(Ser17=)
-
benign
g.96970601C>A
g.96304863C>A
SNRNP200(NM_014014.5):c.51G>T (p.S17=)
-
SNRNP200_000034
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/.
2
-
c.90G>C
r.(?)
p.(Arg30=)
-
likely benign
g.96970562C>G
g.96304824C>G
SNRNP200(NM_014014.4):c.90G>C (p.R30=), SNRNP200(NM_014014.5):c.90G>C (p.R30=)
-
SNRNP200_000088
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
-/.
1
-
c.210-11A>T
r.(=)
p.(=)
-
benign
g.96969079T>A
g.96303341T>A
SNRNP200(NM_014014.5):c.210-11A>T
-
SNRNP200_000082
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
1
-
c.210-5A>G
r.spl?
p.(?)
ACMG
VUS
g.96969073T>C
g.96303335T>C
SNRNP200 c.210-5A>G, p.(?)
-
SNRNP200_000121
-
PubMed: Jespersgaar 2019
-
-
Germline
?
-
-
-
-
LOVD
-?/.
1
-
c.273G>A
r.(?)
p.(Ser91=)
-
likely benign
g.96969005C>T
g.96303267C>T
SNRNP200(NM_014014.5):c.273G>A (p.S91=)
-
SNRNP200_000081
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
1
-
c.334A>C
r.(?)
p.(Thr112Pro)
-
VUS
g.96968944T>G
-
SNRNP200(NM_014014.4):c.334A>C (p.T112P)
-
SNRNP200_000092
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.381+7G>A
r.(=)
p.(=)
-
likely benign
g.96968890C>T
g.96303152C>T
SNRNP200(NM_014014.4):c.381+7G>A
-
SNRNP200_000087
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.461G>A
r.(?)
p.(Arg154Gln)
-
likely benign
g.96967375C>T
g.96301637C>T
SNRNP200(NM_014014.4):c.461G>A (p.R154Q)
-
SNRNP200_000080
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/.
1
4i
c.575-7G>T
r.(spl?)
p.(?)
-
benign
g.96966798C>A
g.96301060C>A
-
-
SNRNP200_000005
predicted benign
PubMed: Neveling 2012
-
-
Germline
-
-
-
-
-
Kornelia Neveling
-?/.
2
-
c.578A>G
r.(?)
p.(Asp193Gly)
-
likely benign
g.96966788T>C
g.96301050T>C
SNRNP200(NM_014014.4):c.578A>G (p.D193G), SNRNP200(NM_014014.5):c.578A>G (p.D193G)
-
SNRNP200_000079
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
+?/.
1
-
c.583A>G
r.(?)
p.(Ile195Val)
-
likely pathogenic
g.96966783T>C
g.96301045T>C
SNRNP200, variant 1: c.583A>G/p.I195V
-
SNRNP200_000134
possibly solved, heterozygous
PubMed: Weisschuh 2020
-
-
Unknown
?
-
-
-
-
LOVD
+/.
2
-
c.594_597del
r.(?)
p.(Tyr199Valfs*2)
ACMG
pathogenic
g.96966771_96966774del
g.96301033_96301036del
SNRNP200 c.594_597del, p.(Tyr199Valfs*2)
-
SNRNP200_000135
-
PubMed: Rodriguez Munoz 2021
-
-
De novo
yes
-
-
-
-
LOVD
-?/.
1
-
c.705C>T
r.(?)
p.(Val235=)
-
likely benign
g.96965091G>A
-
SNRNP200(NM_014014.5):c.705C>T (p.V235=)
-
SNRNP200_000153
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
1
-
c.728A>G
r.(?)
p.(Asn243Ser)
-
VUS
g.96965068T>C
g.96299330T>C
-
-
SNRNP200_000059
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
-
Germline
-
1/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
-/.
1
-
c.732C>T
r.(?)
p.(Leu244=)
-
benign
g.96964703G>A
g.96298965G>A
SNRNP200(NM_014014.5):c.732C>T (p.L244=)
-
SNRNP200_000033
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
1
-
c.733G>A
r.(?)
p.(Val245Ile)
-
VUS
g.96964702C>T
g.96298964C>T
-
-
SNRNP200_000111
-
PubMed: Xu 2014
-
-
Germline
-
1/314 case chromosomes
-
-
-
LOVD
-?/.
1
-
c.1071C>T
r.(?)
p.(Ser357=)
-
likely benign
g.96964070G>A
g.96298332G>A
SNRNP200(NM_014014.4):c.1071C>T (p.S357=)
-
SNRNP200_000032
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.1082A>G
r.(?)
p.(Tyr361Cys)
ACMG
VUS
g.96964059T>C
g.96298321T>C
-
-
SNRNP200_000159
ACMG PM2, PP2
PubMed: Weisschuh 2024
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.1118G>A
r.(?)
p.(Arg373Gln)
-
VUS
g.96964023C>T
g.96298285C>T
-
-
SNRNP200_000058
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
rs745786225
Germline
-
1/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
-/., -?/.
3
10
c.1159A>G
r.(?)
p.(Met387Val)
-
benign, likely benign
g.96963419T>C
g.96297681T>C
SNRNP200(NM_014014.4):c.1159A>G (p.M387V), SNRNP200(NM_014014.5):c.1159A>G (p.M387V)
-
SNRNP200_000001
predicted unknown effect on function, present at significant fraction in Exome Variant Server,
1 more item
PubMed: Neveling 2012
-
-
CLASSIFICATION record, Germline
yes
-
-
-
-
Kornelia Neveling
,
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
-/., -?/.
2
-
c.1203+9C>T
r.(=)
p.(=)
-
benign, likely benign
g.96963366G>A
g.96297628G>A
SNRNP200(NM_014014.4):c.1203+9C>T, SNRNP200(NM_014014.5):c.1203+9C>T
-
SNRNP200_000078
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
?/.
1
-
c.1367G>C
r.(?)
p.(Gly456Ala)
-
VUS
g.96963111C>G
g.96297373C>G
SNRNP200(NM_014014.5):c.1367G>C (p.G456A)
-
SNRNP200_000031
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/., ?/.
2
11
c.1376A>T
r.(?)
p.(Glu459Val)
-
pathogenic (dominant), VUS
g.96963102T>A
g.96297364T>A
c.1376A>T
-
SNRNP200_000057
-
PubMed: Colombo-2020
,
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
-
Germline
-
1/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
+?/.
1
-
c.1462C>T
r.(?)
p.(Leu488Phe)
-
likely pathogenic
g.96962724G>A
g.96296986G>A
SNRNP200, variant 1: c.1462C>T/p.L488F
-
SNRNP200_000133
possibly solved, heterozygous
PubMed: Weisschuh 2020
-
-
Unknown
?
-
-
-
-
LOVD
?/.
1
-
c.1469G>A
r.(?)
p.(Arg490His)
-
VUS
g.96962717C>T
g.96296979C>T
SNRNP200(NM_014014.4):c.1469G>A (p.R490H)
-
SNRNP200_000077
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.1484C>T
r.(?)
p.(Thr495Met)
-
likely benign
g.96962702G>A
-
SNRNP200(NM_014014.5):c.1484C>T (p.T495M)
-
SNRNP200_000157
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+?/.
1
12
c.1504T>C
r.(?)
p.(Cys502Arg)
-
likely pathogenic
g.96962682A>G
g.96296944A>G
SNRNP200 c.1504T>C, (p.C502R)
-
SNRNP200_000150
heterozygous
PubMed: Zhang 2013
-
-
Germline
yes
0/178 controls
-
-
-
LOVD
+/., ?/.
2
13
c.1520C>T
r.(?)
p.(Ala507Val)
ACMG
pathogenic, VUS
g.96962425G>A
g.96296687G>A
SNRNP200 c.1520C>T, p.(A507V)
-
SNRNP200_000056
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
,
PubMed: Xiao-2021
-
-
Germline, Unknown
yes
1/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
?/.
1
13
c.1532A>C
r.(?)
p.(Asn511Thr)
-
VUS
g.96962413T>G
-
c.1532A>C:p.N511T
-
SNRNP200_000144
-
PubMed: Numa-2020
-
-
Unknown
-
-
-
-
-
LOVD
+?/.
3
13
c.1547G>T
r.(?)
p.(Cys516Phe)
ACMG
likely pathogenic
g.96962398C>A
g.96296660C>A
c.1547G>T, p.Cys516Phe, SNRNP200 c.1547G>T, p.Cys516Phe
-
SNRNP200_000120
heterozygous
PubMed: Birtel 2018
,
PubMed: Yusuf 2019
-
-
Germline
?, yes
-
-
-
-
LOVD
+?/.
1
-
c.1614T>G
r.(?)
p.(Ile538Met)
-
likely pathogenic
g.96962331A>C
g.96296593A>C
-
-
SNRNP200_000113
-
PubMed: Huang 2015
-
-
Germline
yes
-
-
-
-
LOVD
+?/., -?/., ?/.
6
-
c.1625C>T
r.(?)
p.(Ala542Val)
-
likely benign, likely pathogenic, VUS
g.96962320G>A
g.96296582G>A
Ala542Val, SNRNP200 c.1625C>T, p.Ala542Val
-
SNRNP200_000095
heterozygous, VKGL data sharing initiative Nederland
PubMed: Bowne 2013
,
PubMed: Daiger 2014
,
PubMed: Zampaglione 2020
-
-
CLASSIFICATION record, Germline, Unknown
?, yes
-
-
-
-
VKGL-NL_Nijmegen
+?/., ?/.
3
13
c.1627C>T
r.(?)
p.(Pro543Ser)
-
likely pathogenic, VUS
g.96962318G>A
g.96296580G>A
c.1627C>T, SNRNP200, variant 1: c.1627C>T/p.P543S
-
SNRNP200_000110
solved, heterozygous
PubMed: Eisenberger-2013
,
PubMed: Fernandez-San Jose 2015
,
PubMed: Weisschuh 2020
-
-
Germline, Unknown
?
-
-
-
-
LOVD
+?/.
1
-
c.1631T>C
r.(?)
p.(Met544Thr)
-
likely pathogenic
g.96962314A>G
g.96296576A>G
-
-
SNRNP200_000112
-
PubMed: Huang 2015
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
3
13
c.1634G>A
r.(?)
p.(Arg545His)
-
likely pathogenic
g.96962311C>T
g.96296573C>T
c.1634G>A, SNRNP200 c.1634G>A, p.(Arg545His)
-
SNRNP200_000132
homozygous
PubMed: Gerth-Kahlert 2019
,
PubMed: Maggi_2021
-
-
Germline
yes
-
-
-
-
LOVD
?/.
1
-
c.1662C>G
r.(?)
p.(Ser554Arg)
-
VUS
g.96962283G>C
g.96296545G>C
SNRNP200 c.1662C>G, p.Ser554Arg
-
SNRNP200_000129
heterozygous
PubMed: Zampaglione 2020
-
-
Unknown
?
-
-
-
-
LOVD
-/.
1
-
c.1671+18T>C
r.(=)
p.(=)
-
benign
g.96962256A>G
g.96296518A>G
SNRNP200(NM_014014.5):c.1671+18T>C
-
SNRNP200_000076
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
1
-
c.1673G>A
r.(?)
p.(Arg558His)
-
VUS
g.96961395C>T
g.96295657C>T
-
-
SNRNP200_000055
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
rs761340019
Germline
-
1/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
?/.
1
-
c.1703A>G
r.(?)
p.(Glu568Gly)
-
VUS
g.96961365T>C
g.96295627T>C
-
-
SNRNP200_000054
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
-
Germline
-
1/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
?/.
1
-
c.1704A>C
r.(?)
p.(Glu568Asp)
-
VUS
g.96961364T>G
-
-
-
SNRNP200_000160
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/.
1
14
c.1708A>G
r.(?)
p.(Thr570Ala)
ACMG
likely pathogenic
g.96961360T>C
g.96295622T>C
SNRNP200 c.1708A > G, p.(T570A)
-
SNRNP200_000152
heterozygous
PubMed: Wang 2022
-
-
Germline/De novo (untested)
?
-
-
-
-
LOVD
+?/.
1
14
c.1724T>G
r.(?)
p.(Leu575Arg)
ACMG
likely pathogenic
g.96961344A>C
g.96295606A>C
SNRNP200 c.1724 T > G, p.(L575R)
-
SNRNP200_000151
heterozygous
PubMed: Wang 2022
-
-
Germline/De novo (untested)
?
-
-
-
-
LOVD
?/.
1
-
c.1763G>T
r.(?)
p.(Cys588Phe)
-
VUS
g.96961305C>A
g.96295567C>A
SNRNP200(NM_014014.5):c.1763G>T (p.C588F)
-
SNRNP200_000075
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
4
-
c.1792C>T
r.(?)
p.(Arg598Cys)
-
VUS
g.96961276G>A
g.96295538G>A
c.1792C>T, p.Arg598Cys, SNRNP200 c.1792C>T, p.Arg598Cys,
1 more item
-
SNRNP200_000037
heterozygous, homozygous, VKGL data sharing initiative Nederland
PubMed: Zampaglione 2020
-
-
CLASSIFICATION record, Unknown
?
-
-
-
-
VKGL-NL_Nijmegen
,
VKGL-NL_AMC
+?/., ?/.
2
-
c.1871G>A
r.(?)
p.(Arg624Lys)
-
likely pathogenic, VUS
g.96959219C>T
g.96293481C>T
-
-
SNRNP200_000103
-
PubMed: Ellingford 2016
,
PubMed: Oishi 2014
-
-
Germline
-
-
-
-
-
LOVD
?/.
1
-
c.1873G>A
r.(?)
p.(Gly625Ser)
-
VUS
g.96959217C>T
g.96293479C>T
-
-
SNRNP200_000089
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
-
c.1910G>A
r.(?)
p.(Arg637Gln)
-
VUS
g.96959180C>T
-
SNRNP200(NM_014014.4):c.1910G>A (p.R637Q)
-
SNRNP200_000125
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
15
c.1951C>T
r.(?)
p.(Leu651Phe)
ACMG
pathogenic
g.96959139G>A
g.96293401G>A
SNRNP200 c.1951C>T, p.(L651F)
-
SNRNP200_000138
-
PubMed: Xiao-2021
-
-
Unknown
yes
-
-
-
-
LOVD
?/.
1
-
c.1961C>A
r.(?)
p.(Thr654Asn)
-
VUS
g.96959129G>T
-
SNRNP200(NM_014014.5):c.1961C>A (p.T654N)
-
SNRNP200_000163
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+?/.
1
15
c.1981G>T
r.(?)
p.(Val661Leu)
ACMG
likely pathogenic (dominant)
g.96959109C>A
g.96293371C>A
-
-
SNRNP200_000101
-
PubMed: Van Cauwenbergh 2017
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
1
-
c.2036+1G>A
r.spl
p.?
-
likely pathogenic (dominant)
g.96959053C>T
g.96293315C>T
2036+1C>A
-
SNRNP200_000104
-
PubMed: Huang 2016
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.2036+1G>T
r.spl
p.?
-
pathogenic (dominant)
g.96959053C>A
g.96293315C>A
-
-
SNRNP200_000109
-
PubMed: Xu 2014
-
-
Germline/De novo (untested)
-
-
-
-
-
LOVD
-?/.
1
-
c.2040C>T
r.(?)
p.(Phe680=)
-
likely benign
g.96958830G>A
g.96293092G>A
SNRNP200(NM_014014.4):c.2040C>T (p.F680=)
-
SNRNP200_000074
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/., +?/., ?/.
61
16
c.2041C>T
p.R681C, r.(?)
p.(Arg681Cys)
ACMG
likely pathogenic, pathogenic, pathogenic (dominant), VUS
g.96958829G>A
g.96293091G>A
2041G>T, c.2041C>T, c.2041C>T, p.Arg681Cys, SNRNP200 c.2041C > T, p.(R681C),
8 more items
-
SNRNP200_000008
ACMG PP3, PM2, PM5, PM1_SUPPORTING, PP2, PP5_STRONG, heterozygous, marked as causative, heterozygous,
3 more items
PubMed: Benaglio 2011
,
PubMed: Birtel 2018
,
PubMed: Bowne 2013
,
PubMed: Colombo-2020
,
PubMed: Ma 2021
,
16 more items
-
rs959069360
CLASSIFICATION record, Germline, Germline/De novo (untested), Unknown
?, yes
1/1204 cases with retinitis pigmentosa, 1/60 cases, 2/258
-
-
-
Johan den Dunnen
,
Feng Wang
,
VKGL-NL_Rotterdam
,
VKGL-NL_Nijmegen
,
Yoshito Koyanagi
,
Daan Panneman
+/., +?/.
19
16
c.2042G>A
r.(?)
p.(Arg681His)
ACMG
likely pathogenic, pathogenic, pathogenic (dominant), VUS
g.17470G>A, g.96958828C>T
g.96293090C>T
c.2042G>A, c.2042G>A, (p.Arg681His), c.2042G>A, p.Arg681His, c.2042G>A, p.R681H,
4 more items
-
SNRNP200_000053
heterozygous, only abstract available, solved, heterozygous
PubMed: Benaglio 2011
,
PubMed: Birtel 2018
,
PubMed: Bowne 2013
,
PubMed: Colombo-2020
,
PubMed: Liu 2020
,
12 more items
-
rs527236113
De novo, Germline, Germline/De novo (untested), Unknown
?, yes
1/258, 2/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
,
Jinu Han
+/., +?/.
3
16
c.2042G>T
r.(?)
p.(Arg681Leu)
ACMG
likely pathogenic, pathogenic
g.96958828C>A
g.96293090C>A
c.2042G>T
-
SNRNP200_000009
-
PubMed: De Castro-Miró 2014
,
PubMed: de Castro-Miró-2014
,
PubMed: Sharon 2019
-
-
Germline
-
1/2420 IRD families
HpyAV+
-
-
Global Variome, with Curator vacancy
,
Marta de Castro-Miró
+/., +?/.
2
-
c.2044C>T
r.(?)
p.(Pro682Ser)
-
likely pathogenic, pathogenic
g.96958826G>A
g.96293088G>A
SNRNP200 c.2044C>T, p.Pro682Ser
-
SNRNP200_000052
heterozygous
PubMed: Bowne 2013
,
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
-
Germline, Germline/De novo (untested)
?
1/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
+/., ?/.
2
16
c.2047G>T
r.(?)
p.(Val683Leu)
-
pathogenic, VUS
g.96958823C>A
g.96293085C>A
SNRNP200 c.2047G>T, p.V683L
-
SNRNP200_000114
heterozygous
PubMed: Benaglio 2011
,
PubMed: Oishi 2014
-
-
Germline, Germline/De novo (untested)
?
-
-
-
-
LOVD
+/., +?/.
4
16
c.2066A>G
r.(?)
p.(Tyr689Cys)
-
likely pathogenic, pathogenic (dominant)
g.96958804T>C
g.96293066T>C
c.2066A>G, SNRNP200 c.2066A>G, p.Y689C
-
SNRNP200_000141
heterozygous
PubMed: Benaglio 2011
,
PubMed: Colombo-2020
-
-
Germline
yes
-
-
-
-
LOVD
+/., ?/.
2
16
c.2122G>A
r.(?)
p.(Val708Ile)
-
pathogenic (dominant), VUS
g.96958748C>T
g.96293010C>T
SNRNP200(NM_014014.4):c.2122G>A (p.V708I)
-
SNRNP200_000030
not in 192 controls, VKGL data sharing initiative Nederland
PubMed: Coussa 2015
-
-
CLASSIFICATION record, Germline
-
1/60 cases
-
-
-
VKGL-NL_Rotterdam
-/.
1
-
c.2310+19G>A
r.(=)
p.(=)
-
benign
g.96957470C>T
g.96291732C>T
SNRNP200(NM_014014.5):c.2310+19G>A
-
SNRNP200_000073
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/., +?/.
12
18
c.2359G>A
r.(?)
p.(Ala787Thr)
ACMG
likely pathogenic, likely pathogenic (dominant), pathogenic, pathogenic (dominant)
g.96957192C>T
g.96291454C>T
c.2359G>A, SNRNP200 c.2359G > A, p.(A787T), SNRNP200 c.2359G>A, p.(A787T),
3 more items
-
SNRNP200_000100
heterozygous, solved, heterozygous
PubMed: Costa 2017
,
PubMed: Liu-2020
,
PubMed: Martin-Merida 2018
,
PubMed: Wang 2014
,
PubMed: Wang 2022
,
5 more items
-
-
Germline, Germline/De novo (untested), Unknown
?, yes
1/258
-
-
-
LOVD
?/.
1
-
c.2443C>G
r.(?)
p.(Leu815Val)
-
VUS
g.96956532G>C
g.96290794G>C
-
-
SNRNP200_000051
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
-
Germline
-
1/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
?/.
1
-
c.2579A>G
r.(?)
p.(Gln860Arg)
-
VUS
g.96956227T>C
g.96290489T>C
-
-
SNRNP200_000108
-
PubMed: Fernandez-San Jose 2015
-
-
Germline
-
-
-
-
-
LOVD
+/.
1
-
c.2580G>C
r.(?)
p.(Gln860His)
ACMG
pathogenic
g.96956226C>G
-
-
-
SNRNP200_000155
-
Villafuerte-de la Cruz RA, et al., 2023. Submitted
ClinVar-13170
-
Germline
yes
-
-
-
-
Rocio Villafuerte-de la Cruz
+/., +?/.
2
-
c.2593G>A
r.(?)
p.(Gly865Ser)
-
likely pathogenic, pathogenic
g.96956213C>T
g.96290475C>T
-
-
SNRNP200_000050
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
,
PubMed: Patel 2016
-
rs786205529
Germline
-
1/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
?/.
1
20
c.2594G>A
r.(?)
p.(Gly865Asp)
-
VUS
g.96956212C>T
-
c.2594G>A:p.G865D
-
SNRNP200_000143
-
PubMed: Numa-2020
-
-
Unknown
-
-
-
-
-
LOVD
+/.
1
20
c.2599G>A
r.(?)
p.(Gly867Ser)
ACMG
pathogenic
g.96956207C>T
g.96290469C>T
SNRNP200 c.2599G>A, p.(G867S)
-
SNRNP200_000137
-
PubMed: Xiao-2021
-
-
Unknown
?
-
-
-
-
LOVD
+?/., ?/.
6
20
c.2653C>G
r.(?)
p.(Gln885Glu)
ACMG
likely pathogenic, VUS
g.96956153G>C
g.96290415G>C
SNRNP200 c.2653C>G, p.(Q885E), SNRNP200 c.C2653G, p.Q885E
-
SNRNP200_000136
heterozygous, marked as causative, heterozygous
PubMed: Liu 2012
,
PubMed: Ma 2021
-
-
Germline, Unknown
?, yes
-
-
-
-
LOVD
+?/.
1
-
c.2684C>G
r.(?)
p.(Ser895*)
-
likely pathogenic
g.96956122G>C
-
-
-
SNRNP200_000165
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
-
c.2743-3T>C
r.spl?
p.?
-
VUS
g.96955737A>G
g.96289999A>G
-
-
SNRNP200_000049
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
rs759342154
Germline
-
1/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
?/.
1
21
c.2758C>A
r.(?)
p.(Leu920Met)
-
VUS
g.96955719G>T
-
c.2758C>A
-
SNRNP200_000119
-
PubMed: Wang-2014
-
-
Germline
-
-
-
-
-
LOVD
?/.
1
-
c.2800A>C
r.(?)
p.(Thr934Pro)
-
VUS
g.96955677T>G
g.96289939T>G
-
-
SNRNP200_000048
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
-
Germline
-
1/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
-/., ?/.
3
-
c.2800A>G
r.(?)
p.(Thr934Ala)
-
benign, VUS
g.96955677T>C
g.96289939T>C
SNRNP200(NM_014014.4):c.2800A>G (p.T934A), SNRNP200(NM_014014.5):c.2800A>G (p.T934A)
-
SNRNP200_000072
VKGL data sharing initiative Nederland
PubMed: Wang 2014
-
rs149616320
CLASSIFICATION record, Germline
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
+?/.
1
21
c.2816C>T
r.(?)
p.(Ser939Phe)
-
likely pathogenic (dominant)
g.96955661G>A
-
c.2816C>T
-
SNRNP200_000140
-
PubMed: Liu-2020
-
-
Germline
-
-
-
-
-
LOVD
+?/.
1
-
c.2837A>T
r.(?)
p.(Asp946Val)
-
likely pathogenic
g.96955640T>A
g.96289902T>A
c.2837T>A, p.(Asp946Val)
-
SNRNP200_000127
error in annotation: c.2837T>A instead of A>T, heterozygous
PubMed: Wang 2019
-
-
Germline
?
-
-
-
-
LOVD
?/.
1
-
c.2879C>T
r.(?)
p.(Ala960Val)
-
VUS
g.96955598G>A
g.96289860G>A
-
-
SNRNP200_000102
-
PubMed: Ellingford 2016
-
-
Germline
-
-
-
-
-
LOVD
-?/., ?/.
2
-
c.2940+4C>T
r.(=), r.spl?
p.(=), p.?
-
likely benign, VUS
g.96955533G>A
g.96289795G>A
SNRNP200(NM_014014.4):c.2940+4C>T
-
SNRNP200_000029
VKGL data sharing initiative Nederland
PubMed: Xu 2014
-
-
CLASSIFICATION record, Germline
-
1/314 case chromosomes
-
-
-
VKGL-NL_Rotterdam
+/., +?/.
2
21i
c.2941-2A>G
r.spl, r.spl?
p.?
ACMG
likely pathogenic (dominant), pathogenic
g.96955119T>C
g.96289381T>C
c.2941-2A>G, NM_014014.4:c.2941-2A>G, NP_054733.2:p.?, NC_000002.11:g.96955119T>C
-
SNRNP200_000118
-
PubMed: Liu-2020
,
PubMed: Wang 2018
-
-
Germline
?
-
-
-
-
LOVD
?/.
1
-
c.2951T>A
r.(?)
p.(Leu984Gln)
-
VUS
g.96955107A>T
g.96289369A>T
SNRNP200(NM_014014.5):c.2951T>A (p.L984Q)
-
SNRNP200_000071
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+?/.
2
-
c.2956C>T
r.(?)
p.(Arg986Cys)
ACMG
likely pathogenic
g.96955102G>A
g.96289364G>A
SNRNP200 c.[2956C>T];[2956=], V1: c.2956C>T, (p.Arg986Cys),
1 more item
-
SNRNP200_000142
heterozygous
PubMed: Chen 2021
,
PubMed: Chen 2021
-
-
Germline, Unknown
?, yes
Taiwan Biobank: 0; GnomAD_exome_East: 0; GnomAD_All: 0
-
-
-
LOVD
?/.
1
-
c.2984A>G
r.(?)
p.(Asn995Ser)
-
VUS
g.96955074T>C
g.96289336T>C
-
-
SNRNP200_000047
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
rs143494993
Germline
-
1/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
+?/., -/., ?/.
3
22
c.3005A>G
r.(?)
p.(Asn1002Ser)
-
benign, likely pathogenic, VUS
g.96955053T>C
g.96289315T>C
SNRNP200(NM_014014.4):c.3005A>G (p.N1002S), SNRNP200(NM_014014.5):c.3005A>G (p.N1002S)
-
SNRNP200_000002
predicted to affect function, but insufficient evidence for definite conclusion,
1 more item
PubMed: Neveling 2012
-
-
CLASSIFICATION record, Germline
no
-
-
-
-
Kornelia Neveling
,
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
?/.
1
-
c.3047G>A
r.(?)
p.(Arg1016Lys)
-
VUS
g.96955011C>T
g.96289273C>T
SNRNP200(NM_014014.5):c.3047G>A (p.R1016K)
-
SNRNP200_000070
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
1
-
c.3062C>T
r.(?)
p.(Ser1021Phe)
-
VUS
g.96954996G>A
-
SNRNP200(NM_014014.5):c.3062C>T (p.S1021F)
-
SNRNP200_000124
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
1
-
c.3074A>G
r.(?)
p.(Lys1025Arg)
-
VUS
g.96954984T>C
g.96289246T>C
SNRNP200(NM_014014.5):c.3074A>G (p.K1025R)
-
SNRNP200_000069
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
3
-
c.3130G>T
r.(?)
p.(Val1044Leu)
ACMG
VUS
g.96954819C>A
g.96289081C>A
SNRNP200(NM_014014.4):c.3130G>T (p.V1044L)
-
SNRNP200_000028
ACMG PM2, PP2; no variant 2nd chromosome, VKGL data sharing initiative Nederland
PubMed: Weisschuh 2024
-
-
CLASSIFICATION record, Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Rotterdam
,
VKGL-NL_Nijmegen
+/., +?/.
2
23
c.3133C>A
r.(?)
p.(Pro1045Thr)
-
likely pathogenic, pathogenic
g.96954816G>T
g.96289078G>T
c.3133C>A
-
SNRNP200_000099
-
PubMed: Stone 2017
,
PubMed: Wang-2013
-
-
Germline, Unknown
-
-
-
-
-
LOVD
?/.
1
-
c.3193G>A
r.(?)
p.(Ala1065Thr)
-
VUS
g.96954466C>T
-
SNRNP200(NM_014014.4):c.3193G>A (p.A1065T)
-
SNRNP200_000147
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/.
2
24
c.3250G>T
r.(?)
p.(Val1084Phe)
-
likely pathogenic, likely pathogenic (dominant)
g.96954409C>A
g.96288671C>A
c.3250G>T
-
SNRNP200_000094
-
PubMed: Zhou 2018
,
PubMed: Zhou-2011
-
-
Germline, Unknown
-
-
-
-
-
LOVD
+/., +?/., -?/.
71
25
c.3260C>T
r.(?)
p.(Ser1087Leu)
ACMG
likely pathogenic, pathogenic, pathogenic (dominant)
g.96953706G>A
g.96287968G>A
2:96953706G>A ENST00000323853.5:c.3260C>T (Ser1087Leu), c.3260C>T, c.3260C>T:p.S1087L,
8 more items
-
SNRNP200_000036
heterozygous, not in 192 controls, only abstract available, solved, heterozygous,
3 more items
PubMed: Benaglio 2011
,
PubMed: Bowne 2013
,
PubMed: Carss 2017
,
PubMed: Chen 2013
,
PubMed: Coussa 2015
,
20 more items
-
rs267607077
CLASSIFICATION record, Germline, Germline/De novo (untested), In vitro (cloned), Unknown
?, yes
1/1204 cases with retinitis pigmentosa, 1/143 cases, 2/258, 2/60 cases
-
-
-
Johan den Dunnen
,
VKGL-NL_Nijmegen
,
Yoshito Koyanagi
,
Daan Panneman
?/.
1
-
c.3261G>A
r.(?)
p.(=)
-
VUS
g.96953705C>T
-
SNRNP200(NM_014014.5):c.3261G>A (p.(Ser1087=))
-
SNRNP200_000162
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/., +?/.
3
-
c.3269G>A
r.(?)
p.(Arg1090Gln)
ACMG
likely pathogenic, pathogenic (recessive)
g.96953697C>T
g.96287959C>T
SNRNP200 c.[3269G>A];[3269=], V1: c.3269G>A, (p.Arg1090Gln),
1 more item
-
SNRNP200_000096
heterozygous
PubMed: Astuti 2018
,
PubMed: Chen 2021
,
PubMed: Chen 2021
-
-
Germline, Unknown
?, yes
Taiwan Biobank: 0; GnomAD_exome_East: 0.000109; GnomAD_All: 0.0000119
-
-
-
LOVD
+?/., -?/.
14
-
c.3269G>T
r.(?)
p.(Arg1090Leu)
-
likely pathogenic, pathogenic
g.96953697C>A
g.96287959C>A
ASCC3L1 c.3269G->T, R1090L, SNRNP200 c.3269G->T, R1090L,
1 more item
-
SNRNP200_000149
heterozygous, VKGL data sharing initiative Nederland,
1 more item
PubMed: Cvackova 2014
,
PubMed: Li 2010
-
-
CLASSIFICATION record, Germline, In vitro (cloned)
?, yes
-
-
-
-
VKGL-NL_Rotterdam
-?/., ?/.
4
25
c.3315A>G
r.(?)
p.(Ala1105=)
-
likely benign, VUS
g.96953651T>C
g.96287913T>C
SNRNP200 c.3315A>G, p.A1105,
1 more item
-
SNRNP200_000068
heterozygous, VKGL data sharing initiative Nederland
PubMed: Benaglio 2011
-
-
CLASSIFICATION record, Germline/De novo (untested)
?
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
?/.
1
-
c.3326A>C
r.(?)
p.(Asp1109Ala)
-
VUS
g.96953640T>G
g.96287902T>G
-
-
SNRNP200_000067
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/.
1
-
c.3454C>T
r.(?)
p.(Arg1152Cys)
-
likely pathogenic
g.96953207G>A
g.96287469G>A
-
-
SNRNP200_000098
-
PubMed: Huang 2017
-
-
Unknown
-
-
-
-
-
LOVD
-/.
1
-
c.3550T>C
r.(?)
p.(Leu1184=)
-
benign
g.96952833A>G
g.96287095A>G
SNRNP200(NM_014014.5):c.3550T>C (p.L1184=)
-
SNRNP200_000039
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
?/.
2
-
c.3572A>T
r.(?)
p.(Gln1191Leu)
ACMG
VUS
g.18122506G>A, g.96952811T>A
g.18122275G>A, g.96287073T>A
SNRNP200:NM_014014 c.A3572T, p.Q1191L
-
NHLRC1_000001
heterozygous, individual unsolved, causality of variants unknown
PubMed: Rodriguez-Munoz 2020
-
-
Germline
?
-
-
-
-
Gerard C.P. Schaafsma
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