Global Variome shared LOVD
ATP2A2 (ATPase, Ca++ transporting, cardiac muscle, ...)
LOVD v.3.0 Build 30b [
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Curator:
Michel van Geel
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Unique variants in the ATP2A2 gene
The variants shown are described using the
NM_001681.3
NM_170665.3
transcript reference sequence.
Legend
Please note that a short description of a certain column can be displayed when you move your mouse cursor over the column's header and hold it still. Below, a more detailed description is shown per column.
Effect
: The variant's effect on the function of the gene/protein, displayed in the format 'R/C'. R is the value reported by the source (publication, submitter) and this classification may vary between records. C is the value concluded by the curator. Note that in some database the curator uses Summary records to give details on the classification of the variant.Values used: '+' indicating the variant affects function, '+?' probably affects function, '-' does not affect function, '-?' probably does not affect function, '?' effect unknown, '.' effect was not classified.
Reported
: The number of times this variant has been reported in the database.
Exon
: number of exon/intron containing variant; 2 = exon 2, 12i = intron 12, 2i_7i = from intron 2 to intron 7, 8i_9 = intron 8/exon 9 boundary, _1 = 5' to exon 1, 18_ = 3' of exon 18, _1_18_ = encompassing the entire 18-exon gene
DNA change (cDNA)
: description of variant at DNA level, based on a coding DNA reference sequence (following HGVS recommendations); e.g. c.123C>T, c.123_145del, c.123_126dup. For deletions/duplications extending beyond the reference transcript resp. {0}/{2} is used to replace del/dup. Extent of the deletion/duplication should be specified using the genomic description (g.). "-" indicates the variant described on genomic level does not affect the coding DNA reference sequence.
RNA change
: description of variant at RNA level (following HGVS recommendations).
r.123c>u
r.? = unknown
r.(?) = RNA not analysed but probably transcribed copy of DNA variant
r.spl? = RNA not analysed but variant probably affects splicing
r.(spl?) = RNA not analysed but variant may affect splicing
r.0? = change expected to abolish transcription
Protein
: description of variant at protein level (following HGVS recommendations).
p.(Arg345Pro) = change predicted from DNA (RNA not analysed)
p.Arg345Pro = change derived from RNA analysis
p.? = unknown effect
p.0? = probably no protein produced
Classification method
: The method used for the clinical classification of this variant.
All options:
ACMG
ACGS
EAHAD-CFDB
ENIGMA
IARC
InSiGHT
kConFab
other
Clinical classification
: Clinical classification of variant, preferably based on standardised criteria (e.g. ACMG), directed on the clinical consequences as published/submitted, indicated using an enriched system including inheritance: e.g. pathogenic, pathogenic (dominant), pathogenic (recessive), pathogenic (!), pathogenic (maternal), pathogenic (paternal). Standard inheritance is covered by dominant/recessive, imprinting by maternal/paternal. A '!' warns for exceptional circumstances to be explained in the 'Remarks' field (low penetrance, variants pathogenic in heterozygous state only, hypomorphic/hypermorphic variants, protective variants, etc.). Non-disease consequences (e.g. drug metabolism (pharmacogenetics), risk factor, blood group, tasting bitter) are indicated using additions to the benign classification; benign (dominant), benign (recessive), benign (!), etc. The value 'association' is used for variants associated with a phenotype and 'NA' for variants from in vitro/in silico records. NOTE: classification may differ from the opinion of the curator as given in a variant SUMMARY-record or the 'Functional effect concluded'). NOTE: pathogenic/likely pathogenic should go together with "variant (probably) affects function" In ClassFunctional.
All options:
pathogenic
pathogenic (dominant)
pathogenic (recessive)
pathogenic (!)
pathogenic (maternal)
pathogenic (paternal)
likely pathogenic
likely pathogenic (dominant)
likely pathogenic (recessive)
likely pathogenic (!)
likely pathogenic (maternal)
likely pathogenic (paternal)
VUS
VUS (!)
likely benign
likely benign (dominant)
likely benign (recessive)
likely benign (!)
likely benign (maternal)
likely benign (paternal)
benign
benign (dominant)
benign (recessive)
benign (!)
benign (maternal)
benign (paternal)
conflicting
association
NA
DNA change (genomic) (hg19)
: HGVS description of variant at DNA level, based on the genomic (chromosomal) DNA reference sequence; e.g. g.12345678C>T, g.12345679del, g.12345678_12345890dup
DNA change (hg38)
: HGVS description of variant at DNA level, based on the hg38 genomic (chromosomal) eference sequence; e.g. g.12345678C>T, g.12345679del, g.12345678_12345890dup
Published as
: listed only when different from "DNA change"; variant as reported originally (e.g. 521delT). Variants seen in animal models, tested in vitro, predicted from RNA analysis, etc. are described between brackets like c.(456C>G)
ISCN
: description of the variant according to ISCN nomenclature
DB-ID
: database ID of variant, grouping multiple observations of the same variant together, starting with the HGNC gene symbol, followed by an underscore (_) and a six digit number (e.g. DMD_012345). _000000 is used for variants where DNA was not analysed (change predicted from RNA analysis), variants seen in animal models or variants not seen in humans but functionally tested in vitro
Variant remarks
: remarks regarding variant described, e.g. germline mosaicism in mother, 345 kb deletion, muscle RNA analysed, not in 200 control chromosomes tested, on founder haplotype, etc.
Reference
: publication describing the variant submitted, incl. links to OMIM, PubMed or other source, e.g. "den Dunnen ASHG2003 P2346"
ClinVar ID
: ID of variant in ClinVar database
dbSNP ID
: the dbSNP ID
Origin
: Origin of variant/record: Germline = in all cells, De novo = in all cells, but not in either parent, Germline/De novo (untested) = in all cells, parents not tested (use only when De novo is likely, e.g. isolated/sporadic cases with dominant disease), Somatic = present in a subset of cells, but not in either parent, Uniparental disomy = from parental disomy (maternal or paternal), CLASSIFICATION record = submitter only sharing variant classification (note another report may share Individual data), SUMMARY record = master summary record from curator (may link to another database), In vitro (cloned) = data resulting from in vitro functional assays, animal model = data from animal model, Artefact = false positive variant call, DUPLICATE record = variant already described on another chromosome (e.g. unbalanced translocation, duplicating transposition, 2nd fusion transcript, etc.)
All options:
Germline
De novo
Germline/De novo (untested)
Somatic
Uniparental disomy
Uniparental disomy, maternal allele
Uniparental disomy, paternal allele
CLASSIFICATION record
SUMMARY record
In vitro (cloned)
In silico
animal model
Artefact
DUPLICATE record
Unknown
Not applicable
Segregation
: Indicates whether the variant segregates with the phenotype (yes), does not segregate with the phenotype (no) or segregation is unknown (?)
All options:
? = unknown
yes = segregates with phenotype
no = does not segregate with phenotype
- = not applicable
Frequency
: frequency in which the variant was found; e.g 5/760 chromosomes (in 5 of 760 chromosomes tested), 1/33 patients (in 1 of 33 patients analysed in study), 0.05 controls (in 5% of control cases tested)
Re-site
: restriction enzyme recognition site created (+) or destroyed (-); e.g. BglII+;BamHI-
VIP
: variant VIP-status was requested for matchmaking - need collaboration(s) to crack the case - please contact the submitter/curator. NOTE: to get VIP status ask the curator.
Methylation
: result of methylation test; GOM (gain of methylation), LOM (loss of methylation), 30% (30% methylated). NOTE: when several tests were done mention the method as well (e.g. MS-PCR 75%)
How to query this table
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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.
307 entries on 4 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
+/.
1
-
c.-85G>A
r.(?)
p.(=)
-
VUS
g.110719510G>A
-
-
-
ATP2A2_000320
pathogenic tendency
-
-
-
Germline/De novo (untested)
-
-
-
-
-
Gemeinschaftspraxis für Humangenetik Dresden
-?/.
1
-
c.-10C>G
r.(?)
p.(=)
-
likely benign
g.110719585C>G
g.110281780C>G
ATP2A2(NM_170665.4):c.-10C>G
-
ATP2A2_000299
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/.
6
1
c.1A>G
r.(?)
p.(?)
-
pathogenic
g.110719595A>G
g.110281790A>G
-
-
ATP2A2_000043
Mutation in start codon
PubMed: Green 2013
,
PubMed: Ikeda 2003
,
PubMed: Nellen 2017
,
PubMed: Onozuka 2004
,
1 more item
-
-
Germline, Unknown
?, yes
-
-
-
-
Johan den Dunnen
,
Michel van Geel
+?/.
2
1
c.1A>T
r.(?)
p.(Met1?)
-
likely pathogenic
g.110719595A>T
g.110281790A>T
-
-
ATP2A2_000286
VKGL data sharing initiative Nederland
PubMed: Nellen 2017
-
-
CLASSIFICATION record, Unknown
-
-
-
-
-
Michel van Geel
,
VKGL-NL_Nijmegen
+/.
1
1
c.2T>C
r.(?)
p.(?)
-
pathogenic
g.110719596T>C
g.110281791T>C
-
-
ATP2A2_000099
Mutation in start codon
PubMed: Ikeda 2003
-
-
Germline
yes
-
-
-
-
Michel van Geel
+/.
2
1
c.32_34del
r.(?)
p.(Glu11del)
-
pathogenic
g.110719626_110719628del
g.110281821_110281823del
32delAGG
-
ATP2A2_000077
-
PubMed: Ikeda 2003
,
PubMed: Ringpfeil 2001
-
-
Unknown
yes
-
-
-
-
Johan den Dunnen
,
Michel van Geel
+/.
2
1
c.35_91dup
r.(?)
p.(Lys30_Lys31ins(19)), p.(Lys30_Lys31insMetLeuGlyHisPheGlyValAsnGluSerThrGlyLeuSerLeuGluGlnValLys)
-
pathogenic
g.110719629_110719685dup
g.110281824_110281880dup
34+57ins, 91ins57
-
ATP2A2_000147
putative protein sequence inserted (MLGHFGVNESTGLSLEQVK)
PubMed: Green 2013
,
PubMed: Sakuntabhai 1999
-
-
Germline, Unknown
?, yes
c.91_92ins57:c.35_91
-
-
-
Johan den Dunnen
+/.
1
1
c.52_65delinsTGAGTGGAGGA
r.(?)
p.(Val18*)
-
pathogenic
g.110719646_110719659delinsTGAGTGGAGGA
g.110281841_110281854delinsTGAGTGGAGGA
48del14/ins11
-
ATP2A2_000189
-
PubMed: Green 2013
-
-
Unknown
?
-
-
-
-
Johan den Dunnen
+/.
6
1
c.68G>A
r.(?)
p.(Gly23Glu)
-
pathogenic
g.110719662G>A
g.110281857G>A
-
-
ATP2A2_000001
Mosaic Darier disease, VKGL data sharing initiative Nederland
PubMed: Harboe 2011
,
PubMed: Ikeda 2003
,
PubMed: Nellen 2017
,
PubMed: Ruiz-Perez 1999
,
OMIM:var0001
,
1 more item
-
-
CLASSIFICATION record, De novo, Unknown
?, yes
-
-
-
-
Johan den Dunnen
,
Michel van Geel
,
VKGL-NL_Nijmegen
+/.
1
1
c.94C>T
r.(?)
p.(Leu32Phe)
-
pathogenic
g.110719688C>T
g.110281883C>T
-
-
ATP2A2_000190
-
PubMed: Green 2013
-
-
Unknown
?
-
-
-
-
Johan den Dunnen
+/.
1
1
c.95T>C
r.(?)
p.(Leu32Pro)
-
pathogenic
g.110719689T>C
g.110281884T>C
-
-
ATP2A2_000170
-
PubMed: Klausegger 2011
-
-
Unknown
yes
-
-
-
-
Johan den Dunnen
+/.
1
1
c.110dup
r.(?)
p.(Ser38Leufs*7)
-
pathogenic
g.110719704dup
g.110281899dup
-
-
ATP2A2_000289
-
PubMed: Dodiuk-Gad 2016
-
-
Unknown
-
-
-
-
-
Michel van Geel
+/.
2
1
c.115A>G
r.(?)
p.(Asn39Asp)
-
pathogenic
g.110719709A>G
g.110281904A>G
-
-
ATP2A2_000072
-
PubMed: Onozuka 2004
,
PubMed: Ringpfeil 2001
-
-
Germline, Unknown
yes
-
-
-
-
Johan den Dunnen
,
Michel van Geel
+/.
2
1
c.116A>C
r.(?)
p.(Asn39Thr)
-
pathogenic
g.110719710A>C
g.110281905A>C
-
-
ATP2A2_000063
-
PubMed: Jacobsen 1999
,
PubMed: Racz 2004
-
-
Unknown
?, yes
-
-
-
-
Johan den Dunnen
,
Michel van Geel
+/.
1
1i
c.118+1G>C
r.spl
p.(?)
-
pathogenic
g.110719713G>C
g.110281908G>C
-
-
ATP2A2_000149
-
PubMed: Jacobsen 1999
-
-
Unknown
?
-
-
-
-
Michel van Geel
+?/.
1
1i
c.118+3A>T
r.spl?
p.(?)
-
likely pathogenic
g.110719715A>T
g.110281910A>T
-
-
ATP2A2_000253
-
PubMed: Nellen 2017
-
-
Germline
yes
-
-
-
-
Michel van Geel
+?/.
1
1i
c.119-2A>G
r.spl
p.(?)
-
likely pathogenic
g.110720407A>G
g.110282602A>G
-
-
ATP2A2_000260
-
PubMed: Nellen 2017
-
-
Germline
yes
-
-
-
-
Michel van Geel
+/.
1
1i
c.119-2A>T
r.119_133del
p.?
-
pathogenic
g.110720407A>T
g.110282602A>T
-
-
ATP2A2_000288
-
PubMed: Akagi 2015
-
-
Unknown
-
-
-
-
-
Michel van Geel
+/.
2
2
c.119_120del
r.(?)
p.(Glu40Valfs*4)
-
pathogenic
g.110720409_110720410del
g.110282604_110282605del
-
-
ATP2A2_000040
-
PubMed: Bchetnia 2009
,
PubMed: Ruiz-Perez 1999
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
,
Michel van Geel
+/.
1
2
c.120_122del
r.(?)
p.(Leu41del)
-
pathogenic
g.110720410_110720412del
g.110282605_110282607del
-
-
ATP2A2_000138
not found in 50 healthy controls
PubMed: Tsuruta 2010
-
-
De novo
-
-
-
-
-
Michel van Geel
+/.
1
2
c.121_123del
r.(?)
p.(Leu41del)
-
pathogenic
g.110720411_110720413del
g.110282606_110282608del
-
-
ATP2A2_000073
-
PubMed: Ringpfeil 2001
-
-
Germline
yes
-
-
-
-
Michel van Geel
+/.
1
2
c.124_126del
r.(?)
p.(Pro42del)
-
pathogenic
g.110720414_110720416del
g.110282609_110282611del
124delCCG
-
ATP2A2_000164
-
PubMed: Ringpfeil 2001
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.126G>T
r.(?)
p.(Pro42=)
-
likely benign
g.110720416G>T
g.110282611G>T
ATP2A2(NM_170665.4):c.126G>T (p.P42=)
-
ATP2A2_000291
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
2
c.130del
r.(?)
p.(Glu44Lysfs*10)
-
pathogenic
g.110720420del
g.110282615del
-
-
ATP2A2_000015
-
PubMed: Sakuntabhai 1999
-
-
Unknown
yes
-
-
-
-
Michel van Geel
+/., +?/.
2
2i
c.136+3A>C
r.spl?
p.(?), p.?
-
likely pathogenic, pathogenic
g.110720429A>C
g.110282624A>C
-
-
ATP2A2_000249
VKGL data sharing initiative Nederland
PubMed: Nellen 2017
-
-
CLASSIFICATION record, Unknown
?
-
-
-
-
Michel van Geel
,
VKGL-NL_Nijmegen
+/.
1
2i
c.137-13_137-12insN[18]
r.spl
p.(Glu45_Gly46insMFLTGK)
-
pathogenic
g.110720505_110720506insN[18]
-
137-12ins18
-
ATP2A2_000035
-
PubMed: Sakuntabhai 1999
,
OMIM:var0007
-
-
Germline
yes
c.137-13_-12ins18
-
-
-
Michel van Geel
-?/.
1
-
c.137-12T>C
r.(=)
p.(=)
-
likely benign
g.110720506T>C
g.110282701T>C
ATP2A2(NM_170665.4):c.137-12T>C
-
ATP2A2_000292
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/.
1
2i_3
c.137-12_142dup
r.spl
p.(?)
-
pathogenic
g.110720506_110720523dup
g.110282701_110282718dup
142ins18
-
ATP2A2_000192
-
PubMed: Green 2013
-
-
Unknown
?
-
-
-
-
Johan den Dunnen
+/.
1
2i
c.137-3C>G
r.spl?
p.(?)
-
pathogenic
g.110720515C>G
g.110282710C>G
-
-
ATP2A2_000191
-
PubMed: Green 2013
-
-
Unknown
?
-
-
-
-
Johan den Dunnen
+/.
1
3
c.142del
r.(?)
p.(Thr48Profs*6)
-
pathogenic
g.110720523del
g.110282718del
-
-
ATP2A2_000177
-
PubMed: Pedace 2011
-
-
Unknown
?
-
-
-
-
Johan den Dunnen
+/.
2
3
c.149_158del
r.(?)
p.(Leu50Argfs*9)
-
pathogenic
g.110720530_110720539del
g.110282725_110282734del
149-158del10
-
ATP2A2_000171
-
PubMed: Klausegger 2011
-
-
Germline, Unknown
yes
-
-
-
-
Johan den Dunnen
+/.
1
3
c.160A>G
r.(?)
p.(Ile54Val)
-
pathogenic
g.110720541A>G
g.110282736A>G
-
-
ATP2A2_000091
de novo, somatic mosaicism
PubMed: Wada 2003
-
-
De novo
-
-
-
-
-
Michel van Geel
?/.
1
-
c.161T>C
r.(?)
p.(Ile54Thr)
-
VUS
g.110720542T>C
-
-
-
ATP2A2_000316
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
3
c.167A>G
r.(?)
p.(Gln56Arg)
-
pathogenic
g.110720548A>G
g.110282743A>G
-
-
ATP2A2_000193
-
PubMed: Green 2013
-
-
Unknown
?
-
-
-
-
Johan den Dunnen
+/.
2
3
c.194T>C
r.(?)
p.(Leu65Ser)
-
pathogenic
g.110720575T>C
g.110282770T>C
294T>C
-
ATP2A2_000159
-
PubMed: Green 2013
,
PubMed: Ruiz-Perez 1999
-
-
Germline, Unknown
?, yes
-
-
-
-
Johan den Dunnen
+/.
1
3
c.203C>A
r.(?)
p.(Ala68Glu)
-
pathogenic
g.110720584C>A
g.110282779C>A
-
-
ATP2A2_000128
-
PubMed: Shi 2009
-
-
Germline
yes
-
-
-
-
Michel van Geel
+/.
1
3
c.215C>A
r.(?)
p.(Ser72Tyr)
-
pathogenic
g.110720596C>A
g.110282791C>A
-
-
ATP2A2_000172
-
PubMed: Klausegger 2011
-
-
Unknown
yes
-
-
-
-
Johan den Dunnen
+/.
1
3
c.218T>C
r.(?)
p.(Phe73Ser)
-
pathogenic
g.110720599T>C
g.110282794T>C
-
-
ATP2A2_000173
-
PubMed: Klausegger 2011
-
-
Unknown
yes
-
-
-
-
Johan den Dunnen
+/.
1
3i
c.219+1G>A
r.137_219del
p.(fs*)
-
pathogenic
g.110720601G>A
g.110282796G>A
-
-
ATP2A2_000163
-
-
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.219+2T>A
r.spl?
p.?
-
likely pathogenic
g.110720602T>A
-
-
-
ATP2A2_000317
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
3i
c.219+5_219+6insAA
r.spl?
p.(?)
-
pathogenic
g.110720605_110720606insAA
g.110282800_110282801insAA
219+5insAA
-
ATP2A2_000194
-
PubMed: Green 2013
-
-
Unknown
?
-
-
-
-
Johan den Dunnen
+/.
1
-
c.284del
r.(?)
p.(Leu95Tyrfs*4)
-
pathogenic
g.110729889del
-
ATP2A2(NM_001681.4):c.284delT (p.L95Yfs*4)
-
ATP2A2_000327
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+?/.
1
4
c.302A>G
r.(?)
p.(Asn101Ser)
-
likely pathogenic
g.110729907A>G
g.110292102A>G
-
-
ATP2A2_000256
-
PubMed: Nellen 2017
-
-
Germline
yes
-
-
-
-
Michel van Geel
+/.
1
4
c.322C>T
r.220_324del
p.(Val74_Gln108del)
-
pathogenic
g.110729927C>T
g.110292122C>T
-
-
ATP2A2_000011
mutation leads to in-frame skipping of exon 4
PubMed: Sakuntabhai 1999
,
OMIM:var0002
-
-
Unknown
?
-
-
-
-
Michel van Geel
+/.
1
4
c.324G>C
r.220_324del
p.(Val74_Gln108del)
-
pathogenic
g.110729929G>C
g.110292124G>C
-
-
ATP2A2_000153
Mutation leads to in-frame skipping of exon 4
PubMed: Ruiz-Perez 1999
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
+/.
2
4i
c.325-2A>G
r.spl, r.spl?
p.(?), p.?
-
pathogenic
g.110734402A>G
g.110296597A>G
-
-
ATP2A2_000195
-
PubMed: Green 2013
,
PubMed: Zhao 2016
-
-
Unknown
?
-
-
-
-
Johan den Dunnen
,
Michel van Geel
-/., -?/.
3
-
c.327A>G
r.(?)
p.(Glu109=)
-
benign, likely benign
g.110734406A>G
g.110296601A>G
ATP2A2(NM_170665.3):c.327A>G (p.(Glu109Glu)), ATP2A2(NM_170665.4):c.327A>G (p.E109=)
-
ATP2A2_000293
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Groningen
,
VKGL-NL_Utrecht
+/.
1
5
c.366T>G
r.(?)
p.(Tyr122*)
-
pathogenic
g.110734445T>G
g.110296640T>G
-
-
ATP2A2_000030
-
PubMed: Sakuntabhai 1999
-
-
Germline
yes
-
-
-
-
Michel van Geel
+/.
4
5
c.391C>T
r.(?)
p.(Arg131*)
-
pathogenic
g.110734470C>T
g.110296665C>T
-
-
ATP2A2_000122
-
PubMed: Amichai 2007
,
PubMed: Dodiuk-Gad 2015
,
PubMed: Nellen 2017
,
PubMed: Noda 2016
-
-
Germline, Unknown
yes
-
-
-
-
Michel van Geel
+/.
8
5
c.392G>A
r.(?)
p.(Arg131Gln)
-
pathogenic
g.110734471G>A
g.110296666G>A
ATP2A2(NM_170665.4):c.392G>A (p.R131Q)
-
ATP2A2_000031
VKGL data sharing initiative Nederland
PubMed: Chao 2002
,
OMIM:var0012
,
PubMed: Fu 2011
,
PubMed: Nellen 2017
,
PubMed: Ringpfeil 2001
,
1 more item
-
-
CLASSIFICATION record, Germline, Unknown
?, yes
-
-
-
-
Johan den Dunnen
,
Michel van Geel
,
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
+/.
1
5
c.392G>T
r.(?)
p.(Arg131Leu)
-
pathogenic
g.110734471G>T
g.110296666G>T
-
-
ATP2A2_000100
-
PubMed: Ikeda 2003
-
-
Unknown
yes
-
-
-
-
Michel van Geel
?/.
1
-
c.404A>G
r.(?)
p.(Lys135Arg)
-
VUS
g.110734483A>G
-
ATP2A2(NM_001681.4):c.404A>G (p.K135R)
-
ATP2A2_000321
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+/.
2
5
c.434_435del
r.(?)
p.(Ile145Serfs*4)
-
pathogenic
g.110734513_110734514del
g.110296708_110296709del
433-434del, 433delAT
-
ATP2A2_000010
-
PubMed: Ruiz-Perez 1999
,
PubMed: Sakuntabhai 1999
-
-
Unknown
?, yes
-
-
-
-
Johan den Dunnen
,
Michel van Geel
+/.
1
-
c.435dup
r.(?)
p.(Val146SerfsTer4)
-
pathogenic
g.110734514dup
g.110296709dup
ATP2A2(NM_170665.4):c.435dupA (p.V146Sfs*4)
-
ATP2A2_000300
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+/.
1
5
c.440del
r.(?)
p.(Pro147Leufs*5)
-
pathogenic
g.110734519del
g.110296714del
439delC
-
ATP2A2_000101
-
PubMed: Ikeda 2003
-
-
Germline
yes
-
-
-
-
Michel van Geel
+/.
1
5
c.445G>A
r.(?)
p.(Asp149Asn)
-
pathogenic
g.110734524G>A
g.110296719G>A
-
-
ATP2A2_000079
-
PubMed: Yang 2001
-
-
Germline
yes
-
-
-
-
Michel van Geel
-/., -?/.
3
-
c.464-70C>A
r.(=)
p.(=)
-
benign, likely benign
g.110760727C>A
g.110322922C>A
ATP2A2(NM_170665.3):c.464-70C>A (p.?), ATP2A2(NM_170665.4):c.464-70C>A
-
ATP2A2_000294
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Groningen
,
VKGL-NL_Utrecht
+/.
1
5i
c.464-6_464-1del
r.spl?
p.?
-
pathogenic
g.110760791_110760796del
g.110322986_110322991del
463-6del6
-
ATP2A2_000290
-
PubMed: Godic 2010
-
-
Unknown
-
-
-
-
-
Michel van Geel
+/.
1
5i
c.464-1A>C
r.spl
p.(?)
-
pathogenic
g.110760796A>C
g.110322991A>C
-
-
ATP2A2_000196
1 more item
PubMed: Green 2013
-
-
Unknown
?
-
-
-
-
Johan den Dunnen
+/.
4
6
c.479C>T
r.(?)
p.(Pro160Leu)
-
pathogenic
g.110760812C>T
g.110323007C>T
-
-
ATP2A2_000032
-
PubMed: Godic 2010
,
PubMed: Nellen 2017
,
PubMed: Ringpfeil 2001
,
PubMed: Sakuntabhai 1999
-
-
Germline, Unknown
?, yes
-
-
-
-
Johan den Dunnen
,
Michel van Geel
+/.
1
6
c.482C>A
r.(?)
p.(Ala161Asp)
-
pathogenic
g.110760815C>A
g.110323010C>A
-
-
ATP2A2_000112
-
PubMed: Racz 2004
-
-
Unknown
yes
-
-
-
-
Michel van Geel
+/.
1
6
c.490A>G
r.(?)
p.(Arg164Gly)
-
pathogenic
g.110760823A>G
g.110323018A>G
-
-
ATP2A2_000197
-
PubMed: Green 2013
-
-
Unknown
?
-
-
-
-
Johan den Dunnen
+/.
1
6
c.492G>C
r.(?)
p.(Arg164Ser)
-
pathogenic
g.110760825G>C
g.110323020G>C
-
-
ATP2A2_000113
-
PubMed: Racz 2004
-
-
Germline
yes
-
-
-
-
Michel van Geel
-?/.
1
-
c.506A>G
r.(?)
p.(Lys169Arg)
-
likely benign
g.110760839A>G
-
ATP2A2(NM_170665.4):c.506A>G (p.K169R)
-
ATP2A2_000314
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
2
6
c.530A>C
r.(?)
p.(Gln177Pro)
-
pathogenic
g.110760863A>C
g.110323058A>C
-
-
ATP2A2_000123
-
PubMed: Amichai 2007
,
PubMed: Dodiuk-Gad 2015
-
-
Germline
yes
-
-
-
-
Michel van Geel
?/.
1
-
c.535A>G
r.(?)
p.(Ile179Val)
-
VUS
g.110760868A>G
-
ATP2A2(NM_001681.4):c.535A>G (p.I179V)
-
ATP2A2_000326
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+/.
1
6
c.539T>G
r.(?)
p.(Leu180Arg)
-
pathogenic
g.110760872T>G
g.110323067T>G
-
-
ATP2A2_000107
-
PubMed: Onozuka 2004
-
-
Unknown
yes
-
-
-
-
Michel van Geel
+/.
1
6
c.542C>G
r.(?)
p.(Thr181Arg)
-
pathogenic
g.110760875C>G
g.110323070C>G
-
-
ATP2A2_000116
-
PubMed: Racz 2005
-
-
Germline
yes
-
-
-
-
Michel van Geel
+/.
1
6
c.543del
r.(?)
p.(Gly182Valfs*43)
-
pathogenic
g.110760876del
g.110323071del
-
-
ATP2A2_000198
-
PubMed: Green 2013
-
-
Unknown
?
-
-
-
-
Johan den Dunnen
+/.
3
6i
c.544+1G>A
r.spl
p.(?)
-
pathogenic
g.110760878G>A
g.110323073G>A
IVS6+1G>A
-
ATP2A2_000146
-
PubMed: Ruiz-Perez 1999
,
PubMed: Sakuntabhai 1999
,
OMIM:var0003
-
-
Germline, Unknown
?, yes
-
-
-
-
Johan den Dunnen
+/.
1
6i
c.544+1_544+6del
r.464_544del
p.(Val155_Thr181del)
-
pathogenic
g.110760878_110760883del
g.110323073_110323078del
544+1del6bp
-
ATP2A2_000144
Mutation leads to in-frame skipping of exon 6
PubMed: Godic 2010
-
-
Germline
yes
-
-
-
-
Michel van Geel
+?/.
1
6i
c.545-10A>G
r.spl?
p.(?)
-
likely pathogenic
g.110764185A>G
g.110326380A>G
-
-
ATP2A2_000265
-
PubMed: Nellen 2017
-
-
Unknown
?
-
-
-
-
Michel van Geel
+/.
1
7
c.545G>A
r.(?)
p.(Gly182Asp)
-
pathogenic
g.110764195G>A
g.110326390G>A
-
-
ATP2A2_000178
Not found in 300 control chromosomes
PubMed: Pedace 2011
-
-
Unknown
?
-
-
-
-
Johan den Dunnen
+/.
1
7
c.547G>A
r.(?)
p.(Glu183Lys)
-
pathogenic
g.110764197G>A
g.110326392G>A
-
-
ATP2A2_000102
-
PubMed: Ikeda 2003
-
-
Unknown
yes
-
-
-
-
Michel van Geel
+/.
2
7
c.548A>T
r.(?)
p.(Glu183Val)
-
pathogenic
g.110764198A>T
g.110326393A>T
-
-
ATP2A2_000103
-
PubMed: Ikeda 2003
,
PubMed: Noda 2016
-
-
Germline, Unknown
yes
-
-
-
-
Michel van Geel
+/.
1
7
c.552_553insCAT
r.(?)
p.(Ser184_Val185insHis)
-
pathogenic
g.110764202_110764203insCAT
g.110326397_110326398insCAT
553insCAT
-
ATP2A2_000104
-
PubMed: Ikeda 2003
-
-
Unknown
yes
-
-
-
-
Michel van Geel
+/.
1
7
c.557C>T
r.(?)
p.(Ser186Phe)
-
pathogenic
g.110764207C>T
g.110326402C>T
-
-
ATP2A2_000033
-
PubMed: Sakuntabhai 1999
-
-
Germline
yes
-
-
-
-
Michel van Geel
+/.
1
7
c.558dup
r.(?)
p.(Val187Cysfs*6)
-
pathogenic
g.110764208dup
g.110326403dup
558insT
-
ATP2A2_000114
-
PubMed: Racz 2004
-
-
Unknown
yes
-
-
-
-
Michel van Geel
+/.
2
7
c.569delAinsN[34]
r.(?)
p.(p.H190delinsLTSSLGEAVSVI)
-
pathogenic
g.130672702delinsN[34]
-
-
-
ATP2A2_000182
Patient with digenic mutations in ATP2A2 and PAX6
PubMed: Castori 2009
,
PubMed: Pedace 2011
-
-
De novo, Unknown
?
-
-
-
-
Johan den Dunnen
+/., +?/.
2
7
c.579_590del
r.(?)
p.(Val194_Pro197del)
-
likely pathogenic, pathogenic
g.110764229_110764240del
g.110326424_110326435del
579-590del
-
ATP2A2_000057
-
PubMed: Nellen 2017
,
PubMed: Ruiz-Perez 1999
-
-
Germline, Unknown
yes
-
-
-
-
Michel van Geel
+/.
1
7_7i
c.629_630+1del
r.spl
p.?
-
pathogenic
g.110764279_110764281del
g.110326474_110326476del
-
-
ATP2A2_000279
-
PubMed: Dodiuk-Gad 2015
-
-
Germline
-
-
-
-
-
Michel van Geel
+/.
1
7i
c.630+1G>C
r.spl?
p.(?)
-
pathogenic
g.110764281G>C
g.110326476G>C
IVS7+1G>C
-
ATP2A2_000154
-
PubMed: Ruiz-Perez 1999
-
-
Unknown
?
-
-
-
-
Johan den Dunnen
+?/.
1
7i
c.630+1G>T
r.spl
p.(?)
-
likely pathogenic
g.110764281G>T
g.110326476G>T
-
-
ATP2A2_000186
-
PubMed: Shi 2012
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
+/.
3
8
c.632G>A
r.(?)
p.(Gly211Asp)
-
pathogenic
g.110765359G>A
g.110327554G>A
-
-
ATP2A2_000058
-
PubMed: Lu 2011
,
PubMed: Nellen 2017
,
PubMed: Ruiz-Perez 1999
-
-
De novo, Germline
yes
-
-
-
-
Johan den Dunnen
,
Michel van Geel
+/.
1
8
c.632G>T
r.(?)
p.(Gly211Val)
-
pathogenic
g.110765359G>T
g.110327554G>T
-
-
ATP2A2_000273
-
PubMed: Dodiuk-Gad 2015
-
-
Germline
-
-
-
-
-
Michel van Geel
?/.
1
-
c.637A>G
r.(?)
p.(Asn213Asp)
-
VUS
g.110765364A>G
g.110327559A>G
ATP2A2(NM_170665.4):c.637A>G (p.N213D)
-
ATP2A2_000301
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+/.
1
8
c.650G>C
r.(?)
p.(Gly217Ala)
-
pathogenic
g.110765377G>C
g.110327572G>C
-
-
ATP2A2_000137
-
PubMed: Bchetnia 2009
-
-
Germline
yes
-
-
-
-
Michel van Geel
+/.
1
8
c.667G>A
r.(?)
p.(Val223Met)
-
pathogenic
g.110765394G>A
g.110327589G>A
-
-
ATP2A2_000059
-
PubMed: Ruiz-Perez 1999
-
-
Unknown
?
-
-
-
-
Michel van Geel
+/.
1
8
c.697G>C
r.(?)
p.(Gly233Arg)
-
pathogenic
g.110765424G>C
g.110327619G>C
-
-
ATP2A2_000108
-
PubMed: Onozuka 2004
-
-
Germline
yes
-
-
-
-
Michel van Geel
+/.
2
8
c.698G>T
r.(?)
p.(Gly233Val)
-
pathogenic
g.110765425G>T
g.110327620G>T
-
-
ATP2A2_000133
-
PubMed: Green 2013
,
PubMed: Huo 2009
-
-
Unknown
?, yes
-
-
-
-
Johan den Dunnen
,
Michel van Geel
+/.
1
8
c.742C>A
r.(?)
p.(Pro248Thr)
-
pathogenic
g.110765469C>A
g.110327664C>A
-
-
ATP2A2_000241
not in 150 healthy controls
PubMed: Liang 2015
-
-
Unknown
yes
-
-
-
-
Johan den Dunnen
+/.
2
8
c.761A>G
r.(?)
p.(Asp254Gly)
-
pathogenic
g.110765488A>G
g.110327683A>G
-
-
ATP2A2_000126
Not present in 50 Japanese controls
PubMed: Li 2011
,
PubMed: Moriuchi 2008
-
-
Unknown
?
-
-
-
-
Johan den Dunnen
,
Michel van Geel
+?/.
1
8
c.775_780dup
r.(?)
p.(Gln259_Leu260dup)
-
likely pathogenic
g.110765502_110765507dup
g.110327697_110327702dup
-
-
ATP2A2_000254
-
PubMed: Nellen 2017
-
-
Germline
yes
-
-
-
-
Michel van Geel
+/.
1
8
c.803G>A
r.(?)
p.(Cys268Tyr)
-
pathogenic
g.110765530G>A
g.110327725G>A
-
-
ATP2A2_000274
-
PubMed: Dodiuk-Gad 2015
-
-
Germline
-
-
-
-
-
Michel van Geel
+/.
1
8
c.803G>T
r.(?)
p.(Cys268Phe)
-
pathogenic
g.110765530G>T
g.110327725G>T
-
-
ATP2A2_000061
-
PubMed: Ruiz-Perez 1999
,
OMIM:var0005
-
-
Unknown
?
-
-
-
-
Michel van Geel
+/.
1
8
c.820A>G
r.(?)
p.(Ile274Val)
-
pathogenic
g.110765547A>G
g.110327742A>G
-
-
ATP2A2_000082
-
PubMed: Takahashi 2001
-
-
Unknown
yes
-
-
-
-
Michel van Geel
+/.
1
8
c.826_840del
r.(?)
p.(Ile276_Asn280del)
-
pathogenic
g.110765553_110765567del
g.110327748_110327762del
826del15
-
ATP2A2_000199
-
PubMed: Green 2013
-
-
Unknown
?
-
-
-
-
Johan den Dunnen
?/.
1
-
c.919A>G
r.(?)
p.(Ile307Val)
-
VUS
g.110765646A>G
g.110327841A>G
ATP2A2(NM_170665.4):c.919A>G (p.I307V)
-
ATP2A2_000295
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+/.
1
8
c.923C>A
r.(?)
p.(Pro308His)
-
pathogenic
g.110765650C>A
g.110327845C>A
-
-
ATP2A2_000200
-
PubMed: Green 2013
-
-
Unknown
?
-
-
-
-
Johan den Dunnen
+/.
1
8
c.925G>A
r.(?)
p.(Glu309Lys)
-
pathogenic
g.110765652G>A
g.110327847G>A
-
-
ATP2A2_000201
-
PubMed: Green 2013
-
-
Unknown
?
-
-
-
-
Johan den Dunnen
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