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%)
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
-
c.1.1kb deletion
r.(?)
p.(?)
-
pathogenic
g.?
-
1.1kb deletion
-
NPHS2_000000
data copied from the Inherited arrhythmogenic diseases and cardiac ion channels database ;
PubMed: Bhuiyan 2007 , PubMed: Medeiros-Domingo 2009
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.3.6kb deletion
r.(?)
p.(?)
-
pathogenic
g.?
-
3.6kb deletion
-
NPHS2_000000
1 more item
PubMed: Medeiros-Domingo 2009
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., -/.
27
-
c.?
r.(?)
p.(?)
-
benign, pathogenic
g.?
-
-
-
NPHS2_000000
data copied from the Inherited arrhythmogenic diseases and cardiac ion channels database ;
PubMed: Beckmann 2008 , PubMed: Beery 2009 , PubMed: Berge 2008 , PubMed: Creigthon 2006 , 9 more items
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
1
-
c.49-172C>T
r.(=)
p.(=)
-
benign
g.237433625C>T
g.237270325C>T
RYR2(NM_001035.3):c.49-172C>T
-
RYR2_000790
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
?/.
1
-
c.79A>G
r.(?)
p.(Thr27Ala)
-
VUS
g.237433827A>G
-
-
-
RYR2_001595
-
-
-
rs1409511801
Unknown
-
-
-
-
-
MobiDetails
?/.
1
-
c.141T>G
r.(?)
p.(Cys47Trp)
-
VUS
g.237433889T>G
g.237270589T>G
RYR2(NM_001035.3):c.141T>G (p.C47W)
-
RYR2_001260
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/.
1
-
c.184C>T
r.(?)
p.(?)
-
pathogenic
g.237494193C>T
g.237330893C>T
C184T
-
RYR2_000771
data copied from the Inherited arrhythmogenic diseases and cardiac ion channels database ;
PubMed: Medeiros-Domingo 2009
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.218T>C
r.(?)
p.(Leu73Pro)
-
VUS
g.237494227T>C
g.237330927T>C
-
-
RYR2_001529
variant definitively linked to disease
Fusco 2042, submitted
-
-
Germline
-
-
-
-
-
Carmela Fusco
+/.
4
-
c.230C>T
r.(?)
p.(?), p.(Ala77Val)
-
pathogenic
g.237494239C>T
g.237330939C>T
C230T, RYR2(NM_001035.3):c.230C>T (p.A77V)
-
RYR2_000772
VKGL data sharing initiative Nederland, 1 more item
PubMed: d'Amati 2005 , PubMed: Medeiros-Domingo 2009
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen , VKGL-NL_AMC
+/., +?/.
2
-
c.241A>C
r.(?)
p.(?), p.(Met81Leu)
-
likely pathogenic, pathogenic
g.237494250A>C
g.237330950A>C
A241C, RYR2(NM_001035.3):c.241A>C (p.M81L)
-
RYR2_000773
VKGL data sharing initiative Nederland, 1 more item
PubMed: Medeiros-Domingo 2009
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen , VKGL-NL_AMC
-?/.
1
-
c.273+3A>G
r.spl?
p.?
-
likely benign
g.237494285A>G
g.237330985A>G
RYR2(NM_001035.3):c.273+3A>G
-
RYR2_000791
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/.
1
-
c.273+9C>T
r.(=)
p.(=)
-
benign
g.237494291C>T
g.237330991C>T
RYR2(NM_001035.3):c.273+9C>T
-
RYR2_000792
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/.
1
-
c.274-23A>G
r.(=)
p.(=)
-
likely benign
g.237519242A>G
-
-
-
RYR2_001589
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
?/.
1
-
c.281A>T
r.(?)
p.(Asp94Val)
-
VUS
g.237519272A>T
g.237355972A>T
-
-
RYR2_001112
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/.
1
-
c.294+7_294+8dup
r.(=)
p.(=)
-
benign
g.237519292_237519293dup
g.237355992_237355993dup
RYR2(NM_001035.3):c.294+6_294+7dupTG
-
RYR2_001113
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
1
-
c.309+1_309+5del
r.spl?
p.?
-
VUS
g.237527673_237527677del
g.237364373_237364377del
RYR2(NM_001035.2):c.307_309+2delAAGGT
-
RYR2_001261
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.309+7C>T
r.(=)
p.(=)
-
likely benign
g.237527679C>T
g.237364379C>T
RYR2(NM_001035.2):c.309+7C>T
-
RYR2_001114
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/.
1
-
c.309+20A>C
r.(=)
p.(=)
-
benign
g.237527692A>C
-
RYR2(NM_001035.3):c.309+20A>C
-
RYR2_001490
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/., -?/.
2
-
c.310-23_310-21del
r.(=)
p.(=)
-
benign, likely benign
g.237532811_237532813del
g.237369511_237369513del
RYR2(NM_001035.3):c.310-23_310-21delCCT
-
RYR2_000794
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen , VKGL-NL_Nijmegen
-?/.
1
-
c.322G>A
r.(?)
p.(Gly108Ser)
-
likely benign
g.237532846G>A
g.237369546G>A
-
-
RYR2_001262
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/.
1
-
c.365G>A
r.(?)
p.(Arg122His)
-
likely pathogenic
g.237532889G>A
g.237369589G>A
RYR2(NM_001035.3):c.365G>A (p.R122H)
-
RYR2_000795
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
1
-
c.371C>T
r.(?)
p.(Ser124Phe)
-
VUS
g.237532895C>T
-
-
-
RYR2_001509
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/.
1
-
c.375T>C
r.(?)
p.(Tyr125=)
-
likely benign
g.237532899T>C
g.237369599T>C
RYR2(NM_001035.2):c.375T>C (p.Y125=)
-
RYR2_001115
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.385-9A>G
r.(=)
p.(=)
-
likely benign
g.237538008A>G
g.237374708A>G
RYR2(NM_001035.2):c.385-9A>G
-
RYR2_001116
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.463+3A>G
r.spl?
p.?
-
likely benign
g.237538098A>G
g.237374798A>G
RYR2(NM_001035.3):c.463+3A>G
-
RYR2_000796
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-/.
1
-
c.463+18C>T
r.(=)
p.(=)
-
benign
g.237538113C>T
-
RYR2(NM_001035.3):c.463+18C>T
-
RYR2_001460
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/.
2
-
c.464-8A>C
r.(=)
p.(=)
-
benign
g.237540615A>C
g.237377315A>C
RYR2(NM_001035.3):c.464-8A>C
-
RYR2_000797
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen , VKGL-NL_AMC
-/., -?/.
2
-
c.464-8A>T
r.(=)
p.(=)
-
benign, likely benign
g.237540615A>T
g.237377315A>T
RYR2(NM_001035.3):c.464-8A>T
-
RYR2_000798
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen , VKGL-NL_AMC
?/.
2
-
c.473G>A
r.(?)
p.(Cys158Tyr)
-
VUS
g.237540632G>A
g.237377332G>A
RYR2(NM_001035.3):c.473G>A (p.C158Y)
-
RYR2_000799
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht , VKGL-NL_AMC
+/.
2
-
c.490C>T
r.(?)
p.(?)
-
pathogenic
g.237540649C>T
g.237377349C>T
C490T
-
RYR2_000774
data copied from the Inherited arrhythmogenic diseases and cardiac ion channels database ;
PubMed: Choi 2004 , PubMed: Tester 2006
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
-
c.493C>T
r.(?)
p.(?)
-
pathogenic
g.237540652C>T
g.237377352C>T
C493T
-
RYR2_000775
1 more item
PubMed: Choi 2004 , PubMed: Medeiros-Domingo 2009
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., +?/.
4
-
c.506G>A
r.(?)
p.(?), p.(Arg169Gln)
-
likely pathogenic, pathogenic
g.237540665G>A
g.237377365G>A
G506A
-
RYR2_000776
1 heterozygous, no homozygous; Clinindb (India) , 1 more item
PubMed: Hsueh 2006 , PubMed: Medeiros-Domingo 2009 , PubMed: Narang 2020 , Journal: Narang 2020
-
rs397516539
Germline
-
1/2795 individuals
-
-
-
Johan den Dunnen , Mohammed Faruq
+/.
3
-
c.527G>A
r.(?)
p.(?)
-
pathogenic
g.237540686G>A
g.237377386G>A
G527A
-
RYR2_000777
data copied from the Inherited arrhythmogenic diseases and cardiac ion channels database ;
PubMed: Haugaa 2010 , PubMed: Tester 2005 , PubMed: Tiso 2001
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.556G>A
r.(?)
p.(?)
-
pathogenic
g.237540715G>A
g.237377415G>A
G556A
-
RYR2_000046
data copied from the Inherited arrhythmogenic diseases and cardiac ion channels database ;
PubMed: Tester 2006
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
3
-
c.567A>T
r.(?)
p.(?)
-
pathogenic
g.237540726A>T
g.237377426A>T
A567T
-
RYR2_000778
data copied from the Inherited arrhythmogenic diseases and cardiac ion channels database ;
PubMed: Davis 2006 , PubMed: Jones 2010 , PubMed: Medeiros-Domingo 2009
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.569G>C
r.(?)
p.(Arg190Thr)
-
likely pathogenic
g.237540728G>C
-
-
-
RYR2_001579
-
-
-
-
Unknown
-
-
-
-
-
MobiDetails
-/., -?/.
4
-
c.576+7G>C
r.(=)
p.(=)
-
benign, likely benign
g.237540742G>C
g.237377442G>C
RYR2(NM_001035.2):c.576+7G>C, RYR2(NM_001035.3):c.576+7G>C
-
RYR2_000800
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam , VKGL-NL_Groningen , VKGL-NL_Utrecht , VKGL-NL_AMC
-?/.
2
-
c.577-14G>A
r.(=)
p.(=)
-
likely benign
g.237550567G>A
g.237387267G>A
RYR2(NM_001035.3):c.577-14G>A
-
RYR2_001263
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen , VKGL-NL_AMC
-?/.
1
-
c.577-5_577-4insTT
r.spl?
p.?
-
likely benign
g.237550576_237550577insTT
g.237387276_237387277insTT
-
-
RYR2_001264
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
-
c.577-4_577-2delinsTTATG
r.spl?
p.?
-
VUS
g.237550577_237550579delinsTTATG
g.237387277_237387279delinsTTATG
-
-
RYR2_001265
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/.
1
-
c.577-3C>T
r.spl?
p.?
-
likely benign
g.237550578C>T
g.237387278C>T
-
-
RYR2_001266
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
-
c.577-2A>G
r.spl?
p.?
-
VUS
g.237550579A>G
g.237387279A>G
-
-
RYR2_001267
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/.
1
-
c.615C>T
r.(?)
p.(=)
-
likely benign
g.237550619C>T
-
RYR2(NM_001035.3):c.615C>T (p.A205=)
-
RYR2_001559
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-/.
1
-
c.639C>T
r.(?)
p.(Ser213=)
-
benign
g.237550643C>T
g.237387343C>T
RYR2(NM_001035.3):c.639C>T (p.S213=)
-
RYR2_001117
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/., ?/.
3
-
c.649A>G
r.(?)
p.(Ile217Val)
-
likely benign, VUS
g.237550653A>G
g.237387353A>G
RYR2(NM_001035.2):c.649A>G (p.I217V), RYR2(NM_001035.3):c.649A>G (p.I217V)
-
RYR2_000801
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam , VKGL-NL_Utrecht , VKGL-NL_AMC
-?/.
1
-
c.651C>T
r.(?)
p.(Ile217=)
-
likely benign
g.237550655C>T
g.237387355C>T
RYR2(NM_001035.3):c.651C>T (p.I217=)
-
RYR2_000802
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-/.
1
-
c.676+13_676+17del
r.(=)
p.(=)
-
benign
g.237550693_237550697del
-
RYR2(NM_001035.3):c.676+13_676+17delCTTCA
-
RYR2_001483
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/.
1
-
c.676+18A>G
r.(=)
p.(=)
-
benign
g.237550698A>G
-
RYR2(NM_001035.3):c.676+18A>G
-
RYR2_001429
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/.
1
-
c.677-11T>A
r.(=)
p.(=)
-
benign
g.237551376T>A
g.237388076T>A
RYR2(NM_001035.3):c.677-11T>A
-
RYR2_000803
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/.
1
-
c.684C>T
r.(?)
p.(=)
-
likely benign
g.237551394C>T
-
RYR2(NM_001035.3):c.684C>T (p.L228=)
-
RYR2_001510
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
?/.
1
-
c.688G>A
r.(?)
p.(Gly230Ser)
-
VUS
g.237551398G>A
-
RYR2(NM_001035.3):c.688G>A (p.G230S)
-
RYR2_001496
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
+?/.
1
-
c.689G>A
r.(?)
p.(Gly230Asp)
-
likely pathogenic
g.237551399G>A
g.237388099G>A
RYR2(NM_001035.3):c.689G>A (p.G230D)
-
RYR2_000804
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+?/.
1
-
c.704G>A
r.(?)
p.(Arg235Lys)
-
likely pathogenic
g.237551414G>A
g.237388114G>A
-
-
RYR2_001329
1 heterozygous, no homozygous; Clinindb (India)
PubMed: Narang 2020 , Journal: Narang 2020
-
rs794728711
Germline
-
1/2795 individuals
-
-
-
Mohammed Faruq
-?/.
1
-
c.705G>A
r.(?)
p.(Arg235=)
-
likely benign
g.237551415G>A
-
RYR2(NM_001035.2):c.705G>A (p.R235=)
-
RYR2_001348
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/.
1
-
c.724G>C
r.(?)
p.(Asp242His)
-
likely pathogenic
g.237551434G>C
g.237388134G>C
RYR2(NM_001035.3):c.724G>C (p.D242H)
-
RYR2_000805
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/., -?/.
2
-
c.726C>T
r.(?)
p.(Asp242=)
-
benign, likely benign
g.237551436C>T
g.237388136C>T
RYR2(NM_001035.2):c.726C>T (p.D242=), RYR2(NM_001035.3):c.726C>T (p.D242=)
-
RYR2_001118
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam , VKGL-NL_AMC
+/., ?/.
2
-
c.727G>A
r.(?)
p.(?), p.(Glu243Lys)
-
pathogenic, VUS
g.237551437G>A
g.237388137G>A
G727A, RYR2(NM_001035.3):c.727G>A (p.E243K)
-
RYR2_000743
VKGL data sharing initiative Nederland, 1 more item
PubMed: Medeiros-Domingo 2009
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen , VKGL-NL_AMC
?/.
1
-
c.745T>C
r.(?)
p.(Ser249Pro)
-
VUS
g.237551455T>C
g.237388155T>C
RYR2(NM_001035.2):c.745T>C (p.S249P)
-
RYR2_001119
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.760G>A
r.(?)
p.(Glu254Lys)
-
VUS
g.237551470G>A
-
RYR2(NM_001035.3):c.760G>A (p.E254K)
-
RYR2_001388
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
?/.
1
-
c.769C>T
r.(?)
p.(Arg257Trp)
-
VUS
g.237551479C>T
-
RYR2(NM_001035.3):c.769C>T (p.R257W)
-
RYR2_001430
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-/.
1
-
c.773+141C>T
r.(=)
p.(=)
-
benign
g.237551624C>T
g.237388324C>T
RYR2(NM_001035.3):c.773+141C>T
-
RYR2_000806
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-/.
1
-
c.774-9_774-8insG
r.(=)
p.(=)
-
benign
g.237580340_237580341insG
g.237417040_237417041insG
RYR2(NM_001035.3):c.774-9_774-8insG
-
RYR2_001120
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
2
-
c.782A>G
r.(?)
p.(His261Arg)
-
VUS
g.237580357A>G
g.237417057A>G
RYR2(NM_001035.3):c.782A>G (p.H261R)
-
RYR2_000808
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen , VKGL-NL_AMC
?/.
1
-
c.817T>A
r.(?)
p.(Ser273Thr)
-
VUS
g.237580392T>A
-
RYR2(NM_001035.2):c.817T>A (p.S273T)
-
RYR2_001431
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.828A>G
r.(?)
p.(Arg276=)
-
likely benign
g.237580403A>G
g.237417103A>G
RYR2(NM_001035.2):c.828A>G (p.R276=)
-
RYR2_001121
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
3
-
c.836C>T
r.(?)
p.(Thr279Met)
-
VUS
g.237580411C>T
g.237417111C>T
RYR2(NM_001035.3):c.836C>T (p.T279M)
-
RYR2_001122
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen , VKGL-NL_Utrecht , VKGL-NL_AMC
-?/.
1
-
c.848+8G>C
r.(=)
p.(=)
-
likely benign
g.237580431G>C
g.237417131G>C
RYR2(NM_001035.3):c.848+8G>C
-
RYR2_001337
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-/.
1
-
c.849-8T>C
r.(=)
p.(=)
-
benign
g.237586384T>C
g.237423084T>C
RYR2(NM_001035.3):c.849-8T>C
-
RYR2_001123
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
2
-
c.892C>T
r.(?)
p.(Arg298Cys)
-
VUS
g.237586435C>T
g.237423135C>T
RYR2(NM_001035.3):c.892C>T (p.R298C)
-
RYR2_000809
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen , VKGL-NL_AMC
?/.
1
-
c.893G>A
r.(?)
p.(Arg298His)
-
VUS
g.237586436G>A
g.237423136G>A
RYR2(NM_001035.3):c.893G>A (p.R298H)
-
RYR2_001124
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
1
-
c.904A>G
r.(?)
p.(Thr302Ala)
-
VUS
g.237586447A>G
-
-
-
RYR2_001564
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
-
c.914A>G
r.(?)
p.(Tyr305Cys)
-
VUS
g.237586457A>G
g.237423157A>G
-
-
RYR2_001530
variant definitively linked to disease
Fusco 2042, submitted
-
-
Germline
-
-
-
-
-
Carmela Fusco
?/.
1
-
c.950T>C
r.(?)
p.(Met317Thr)
-
VUS
g.237586493T>C
g.237423193T>C
RYR2(NM_001035.3):c.950T>C (p.M317T)
-
RYR2_000810
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
1
-
c.985T>C
r.(?)
p.(?)
-
pathogenic
g.237586528T>C
g.237423228T>C
T985C
-
RYR2_000744
data copied from the Inherited arrhythmogenic diseases and cardiac ion channels database ;
PubMed: Medeiros-Domingo 2009
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., ?/.
3
-
c.994C>T
r.(?)
p.(?), p.(Arg332Trp)
ACMG
pathogenic, VUS
g.237586537C>T
g.237423237C>T
C994T
-
RYR2_000745
ACMG grading: PM2,PP3
secondary finding, ACMG: PM2,PP3, 1 more item
PubMed: Medeiros-Domingo 2009
-
rs876657995
Germline
-
-
-
-
-
Johan den Dunnen , Andreas Laner
-?/.
1
-
c.1005+6A>G
r.(=)
p.(=)
-
likely benign
g.237586554A>G
g.237423254A>G
RYR2(NM_001035.3):c.1005+6A>G
-
RYR2_001338
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-/., -?/.
3
-
c.1005+13A>G
r.(=)
p.(=)
-
benign, likely benign
g.237586561A>G
g.237423261A>G
RYR2(NM_001035.3):c.1005+13A>G
-
RYR2_000811
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen , VKGL-NL_Utrecht , VKGL-NL_AMC
-/., -?/.
3
-
c.1006-12C>T
r.(=)
p.(=)
-
benign, likely benign
g.237604607C>T
g.237441307C>T
RYR2(NM_001035.3):c.1006-12C>T
-
RYR2_001125
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht , VKGL-NL_Nijmegen , VKGL-NL_AMC
+?/.
1
-
c.1006-3T>G
r.spl?
p.?
-
likely pathogenic
g.237604616T>G
g.237441316T>G
RYR2(NM_001035.3):c.1006-3T>G
-
RYR2_000812
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/.
1
-
c.1038A>G
r.(?)
p.(Val346=)
-
likely benign
g.237604651A>G
g.237441351A>G
RYR2(NM_001035.3):c.1038A>G (p.V346=)
-
RYR2_000813
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-/.
1
-
c.1053A>G
r.(?)
p.(Thr351=)
-
benign
g.237604666A>G
g.237441366A>G
RYR2(NM_001035.2):c.1053A>G (p.T351=)
-
RYR2_000814
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
4
-
c.1069G>A
r.(?)
p.(Gly357Ser)
-
pathogenic
g.237604682G>A
g.237441382G>A
RYR2(NM_001035.2):c.1069G>A (p.G357S), RYR2(NM_001035.3):c.1069G>A (p.G357S)
-
RYR2_001126
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam , VKGL-NL_Groningen , VKGL-NL_Utrecht , VKGL-NL_AMC
+/.
1
-
c.1072G>A
r.(?)
p.(?)
-
pathogenic
g.237604685G>A
g.237441385G>A
G1072A
-
RYR2_000746
data copied from the Inherited arrhythmogenic diseases and cardiac ion channels database ;
PubMed: Medeiros-Domingo 2009
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.1081T>C
r.(?)
p.(Cys361Arg)
-
VUS
g.237604694T>C
g.237441394T>C
RYR2(NM_001035.3):c.1081T>C (p.C361R)
-
RYR2_000815
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/., -?/.
4
-
c.1108C>T
r.(?)
p.(Leu370=)
-
benign, likely benign
g.237604721C>T
g.237441421C>T
RYR2(NM_001035.2):c.1108C>T (p.L370=), RYR2(NM_001035.3):c.1108C>T (p.L370=)
-
RYR2_001127
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam , VKGL-NL_Utrecht , VKGL-NL_Nijmegen , VKGL-NL_AMC
-?/.
2
-
c.1110A>G
r.(?)
p.(Leu370=)
-
likely benign
g.237604723A>G
g.237441423A>G
RYR2(NM_001035.3):c.1110A>G (p.L370=)
-
RYR2_000816
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht , VKGL-NL_Nijmegen
-/.
1
-
c.1129G>A
r.(?)
p.(?)
-
benign
g.237604742G>A
g.237441442G>A
G1129A
-
RYR2_000747
data copied from the Inherited arrhythmogenic diseases and cardiac ion channels database ;
PubMed: Medeiros-Domingo 2009
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/., -?/.
5
-
c.1134C>T
r.(?)
p.(Asp378=)
-
benign, likely benign
g.237604747C>T
g.237441447C>T
RYR2(NM_001035.2):c.1134C>T (p.D378=), RYR2(NM_001035.3):c.1134C>T (p.D378=)
-
RYR2_000817
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam , VKGL-NL_Groningen , VKGL-NL_Utrecht , VKGL-NL_Nijmegen , VKGL-NL_AMC
-?/.
3
-
c.1135G>A
r.(?)
p.(Val379Met)
-
likely benign
g.237604748G>A
g.237441448G>A
RYR2(NM_001035.2):c.1135G>A (p.V379M), RYR2(NM_001035.3):c.1135G>A (p.V379M)
-
RYR2_001128
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam , VKGL-NL_Groningen , VKGL-NL_Utrecht
-?/.
2
-
c.1143C>T
r.(?)
p.(Ser381=)
-
likely benign
g.237604756C>T
g.237441456C>T
RYR2(NM_001035.2):c.1143C>T (p.S381=), RYR2(NM_001035.3):c.1143C>T (p.S381=)
-
RYR2_000818
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam , VKGL-NL_Groningen
-?/., ?/.
4
-
c.1144G>A
r.(?)
p.(Val382Met)
-
likely benign, VUS
g.237604757G>A
g.237441457G>A
RYR2(NM_001035.3):c.1144G>A (p.V382M, p.(Val382Met))
-
RYR2_000819
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden , VKGL-NL_Groningen , VKGL-NL_Utrecht , VKGL-NL_AMC
?/.
2
-
c.1154G>C
r.(?)
p.(Gly385Ala)
-
VUS
g.237604767G>C
-
RYR2(NM_001035.2):c.1154G>C (p.(Gly385Ala))
-
RYR2_001539
VKGL data sharing initiative Nederland
-
-
rs373148638
CLASSIFICATION record, Unknown
-
-
-
-
-
VKGL-NL_Leiden , MobiDetails
?/.
1
-
c.1159A>C
r.(?)
p.(Ile387Leu)
-
VUS
g.237604772A>C
-
RYR2(NM_001035.3):c.1159A>C (p.I387L)
-
RYR2_001511
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/.
1
-
c.1170+10T>C
r.(=)
p.(=)
-
benign
g.237604793T>C
g.237441493T>C
RYR2(NM_001035.3):c.1170+10T>C
-
RYR2_000820
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+?/.
1
-
c.1195A>G
r.(?)
p.(Met399Val)
-
likely pathogenic
g.237608725A>G
g.237445425A>G
-
-
RYR2_001531
variant definitively linked to disease
Fusco 2042, submitted
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Germline
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Carmela Fusco
-?/.
1
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c.1203T>C
r.(?)
p.(Asp401=)
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likely benign
g.237608733T>C
g.237445433T>C
RYR2(NM_001035.2):c.1203T>C (p.D401=)
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RYR2_001268
VKGL data sharing initiative Nederland
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CLASSIFICATION record
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VKGL-NL_Rotterdam
-/., -?/.
4
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c.1218G>A
r.(?)
p.(Ser406=)
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benign, likely benign
g.237608748G>A
g.237445448G>A
RYR2(NM_001035.2):c.1218G>A (p.S406=), RYR2(NM_001035.3):c.1218G>A (p.S406=)
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RYR2_000821
VKGL data sharing initiative Nederland
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CLASSIFICATION record
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VKGL-NL_Rotterdam , VKGL-NL_Groningen , VKGL-NL_Utrecht , VKGL-NL_AMC
+/., ?/.
5
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c.1240C>T
r.(?)
p.(?), p.(Arg414Cys)
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pathogenic, VUS
g.237608770C>T
g.237445470C>T
C1240T, RYR2(NM_001035.3):c.1240C>T (p.R414C)
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RYR2_000748
4 heterozygous, no homozygous; Clinindb (India) , VKGL data sharing initiative Nederland, 1 more item
PubMed: Medeiros-Domingo 2009 , PubMed: Narang 2020 , Journal: Narang 2020 , PubMed: Tester 2005
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rs764698152
CLASSIFICATION record, Germline
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4/2795 individuals
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Johan den Dunnen , VKGL-NL_Groningen , VKGL-NL_AMC , Mohammed Faruq
?/.
2
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c.1241G>A
r.(?)
p.(Arg414His)
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VUS
g.237608771G>A
g.237445471G>A
RYR2(NM_001035.2):c.1241G>A (p.R414H), RYR2(NM_001035.3):c.1241G>A (p.R414H)
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RYR2_000822
VKGL data sharing initiative Nederland
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CLASSIFICATION record
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VKGL-NL_Rotterdam , VKGL-NL_AMC
+/.
2
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c.1241G>T
r.(?)
p.(?)
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pathogenic
g.237608771G>T
g.237445471G>T
G1241T
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RYR2_000749
data copied from the Inherited arrhythmogenic diseases and cardiac ion channels database ;
PubMed: Choi 2004 , PubMed: Tester 2005
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Germline
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Johan den Dunnen