Global Variome shared LOVD
PCDH19 (protocadherin 19)
LOVD v.3.0 Build 30b [
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Curator:
Christel Depienne
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Unique variants in the PCDH19 gene
Please note
the unique pattern of X-linked inheritance in this gene with male sparing
The variants shown are described using the
NM_001184880.1
NM_020766.2
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
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'
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246 entries on 3 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
_1_3i
c.(?_-1)_(2616+1_2617-1)del
r.0
p.0
-
pathogenic
g.99657522_99663595del
-
-
-
PCDH19_000000
1 more item
PubMed: Depienne 2011
-
-
De novo
-
-
-
-
-
Christel Depienne
+/., -?/.
9
_1_6_
c.(?_-1)_(*1_?)del
r.0, r.o
p.0
-
likely benign, likely pathogenic, NA, pathogenic
g.(98400000_98486801)_(99768045_99800000)dup, g.(98860000_98844724)_(99732138_99750000)del,
3 more items
-
hg18 98731380-99618794del, hg18 g.98373457_99654701dup, hg18 g.99548358_99553797del
-
PCDH19_000000, PCDH19_000094, PCDH19_000096
1.3 Mb duplication, incl. LOC442459, PCDH19, 5.5 Kb deletion,
2 more items
PubMed: Depienne 2009
,
PubMed: Depienne 2011
,
PubMed: Matsuzaki 2009
,
PubMed: Pinto 2007
,
1 more item
-
-
De novo, Germline, Somatic, Unknown
yes
1/776 controls, 4/90 controls
-
-
-
Johan den Dunnen
,
Christel Depienne
,
Xiaoxu Yang
-?/.
1
_1_6_
c.(?_-1)_(*1_?)dup
r.(=)
p.(=)
-
likely benign
g.(99660000_99661702)_(99667141_99668000)dup
-
hg18 g.99548358_99553797dup
-
PCDH19_000095
5.5 Kb duplication
PubMed: Matsuzaki 2009
-
-
Unknown
-
4/90 controls
-
-
-
Johan den Dunnen
+/.
1
3i_6_
c.2617-6811_*4633{0}
r.?
p.?
-
pathogenic
g.91548808_99612513del
g.92293810_100357515del
-
-
PCDH19_000250
-
PubMed: Hamdan 2017
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.3G>A
r.?
p.(Met1?)
-
likely pathogenic (recessive)
g.99663593C>T
g.100408595C>T
-
-
PCDH19_000261
-
-
-
-
Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
-/.
3
1
c.6G>A
r.(=), r.(?)
p.(=), p.(Glu2=), p.(Glu2Glu)
-
benign
g.99663590C>T
g.100408592C>T
(Glu2Glu), PCDH19(NM_001184880.2):c.6G>A (p.E2=)
-
PCDH19_000092
VKGL data sharing initiative Nederland
PubMed: Higurashi 2011
,
PubMed: Hynes 2010
-
-
CLASSIFICATION record, Unknown
-
0.0123, 10/116 patients
-
-
-
Johan den Dunnen
,
VKGL-NL_Utrecht
-?/.
1
-
c.31C>A
r.(?)
p.(Leu11Met)
-
likely benign
g.99663565G>T
g.100408567G>T
PCDH19(NM_001105243.1):c.31C>A (p.(Leu11Met))
-
PCDH19_000206
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
1
-
c.47G>A
r.(?)
p.(Trp16Ter)
-
pathogenic
g.99663549C>T
g.100408551C>T
-
-
PCDH19_000205
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
1
c.64del
r.(?)
p.(Leu22Serfs*8)
-
pathogenic
g.99663534del
g.100408536del
64delC
-
PCDH19_000164
-
-
-
-
De novo
yes
-
-
-
-
Xiaoxu Yang
+/.
1
1
c.74T>C
r.(?)
p.(Leu25Pro)
-
pathogenic
g.99663522A>G
g.100408524A>G
-
-
PCDH19_000059
missense change
PubMed: Dibbens 2011
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
+/.
1
1
c.78del
r.(?)
p.(Lys26Asnfs*4)
-
pathogenic
g.99663518del
g.100408520del
-
-
PCDH19_000037
frameshift change
PubMed: Jamal 2010
-
-
De novo
-
-
-
-
-
Johan den Dunnen
-/.
1
-
c.81C>T
r.(?)
p.(Tyr27=)
-
benign
g.99663515G>A
g.100408517G>A
PCDH19(NM_001184880.2):c.81C>T (p.Y27=)
-
PCDH19_000110
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/.
1
1
c.83C>A
r.(?)
p.(Ser28*)
-
pathogenic
g.99663513G>T
g.100408515G>T
[83C>A;90A>G]
-
PCDH19_000025
nonsense change
PubMed: Marini 2010
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+?/.
1
1
c.90A>G
r.(=)
p.(Glu30=)
-
likely pathogenic
g.99663506T>C
g.100408508T>C
[83C>A;90A>G]
-
PCDH19_000102
nonsense change
PubMed: Marini 2010
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+?/.
1
1
c.94G>T
r.(?)
p.(Glu32Ter)
-
likely pathogenic
g.99663502C>A
g.100408504C>A
-
-
PCDH19_000233
-
PubMed: Ganapathy 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
1
c.134_135del
r.(?)
p.(Asp45Glyfs*43)
-
pathogenic
g.99663461_99663462del
g.100408463_100408464del
134_135delAC
-
PCDH19_000165
-
-
-
-
Germline
yes
-
-
-
-
Xiaoxu Yang
+/.
2
1
c.142G>T
r.(?)
p.(Glu48*)
-
pathogenic
g.99663454C>A
g.100408456C>A
-
-
PCDH19_000015
nonsense change; no variants SCN1A gene; not in 180 control individuals
PubMed: Depienne 2009
,
OMIM:var0006
-
-
Unknown
-
-
-
-
-
Christel Depienne
?/.
2
-
c.154G>A
r.(?)
p.(Ala52Thr)
-
VUS
g.99663442C>T
g.100408444C>T
PCDH19(NM_001184880.1):c.154G>A (p.A52T)
-
PCDH19_000204
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Nijmegen
+/.
1
1
c.158dup
r.(?)
p.(Asp54Glyfs*35)
-
pathogenic
g.99663438dup
g.100408440dup
158dupT
-
PCDH19_000166
-
-
-
-
Somatic
yes
-
-
-
-
Xiaoxu Yang
?/.
1
-
c.172G>T
r.(?)
p.(Ala58Ser)
-
VUS
g.99663424C>A
g.100408426C>A
PCDH19(NM_001105243.1):c.172G>T (p.(Ala58Ser))
-
PCDH19_000154
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
1
1
c.183dup
r.(?)
p.(Arg62Serfs*27)
-
pathogenic
g.99663415dup
g.100408417dup
183_184insT
-
PCDH19_000167
-
-
-
-
De novo
yes
-
-
-
-
Xiaoxu Yang
?/.
1
-
c.194C>T
r.(?)
p.(Ser65Phe)
-
VUS
g.99663402G>A
-
PCDH19(NM_001184880.1):c.194C>T (p.S65F)
-
PCDH19_000227
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.197A>G
r.(?)
p.(Asn66Ser)
-
VUS
g.99663399T>C
g.100408401T>C
PCDH19(NM_001184880.2):c.197A>G (p.N66S)
-
PCDH19_000153
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+/.
1
-
c.202_203insTC
r.(?)
p.(Ala68ValfsTer5)
-
pathogenic
g.99663393_99663394insGA
-
PCDH19(NM_001184880.1):c.202_203insTC (p.A68Vfs*5)
-
PCDH19_000219
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
1
c.215T>G
r.(?)
p.(Val72Gly)
-
pathogenic
g.99663381A>C
g.100408383A>C
-
-
PCDH19_000060
missense change
PubMed: Higurashi 2011
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
+/.
1
1
c.241dup
r.(?)
p.(Leu81Profs*8)
-
pathogenic
g.99663355dup
g.100408357dup
-
-
PCDH19_000073
frameshift change
Depienne, personal communication
-
-
De novo
-
-
-
-
-
Christel Depienne
+/.
1
1
c.242T>G
r.(?)
p.(Leu81Arg)
-
pathogenic
g.99663354A>C
g.100408356A>C
-
-
PCDH19_000044
missense change
PubMed: Depienne 2011
-
-
Unknown
-
-
-
-
-
Christel Depienne
+/.
3
1
c.253C>T
r.(?)
p.(Gln85*)
-
pathogenic
g.99663343G>A
g.100408345G>A
-
-
PCDH19_000010
nonsense change,
1 more item
Depienne, personal communication,
PubMed: Dibbens 2008
,
OMIM:var0003
-
-
Unknown
yes
-
-
-
-
Johan den Dunnen
,
Christel Depienne
+/.
1
1
c.262G>T
r.(?)
p.(Asp88Tyr)
-
pathogenic
g.99663334C>A
g.100408336C>A
-
-
PCDH19_000168
-
-
-
-
De novo
yes
-
-
-
-
Xiaoxu Yang
+?/.
1
-
c.263A>C
r.(?)
p.(Asp88Ala)
ACMG
likely pathogenic
g.99663333T>G
g.100408335T>G
-
-
PCDH19_000251
ACMG PS2,PM2,PM6,PP3
PubMed: Chuan 2022
-
-
Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.265C>T
r.(?)
p.(Arg89Cys)
-
VUS
g.99663331G>A
-
PCDH19(NM_001184880.2):c.265C>T (p.R89C)
-
PCDH19_000226
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
?/.
1
-
c.301A>G
r.(?)
p.(Ile101Val)
-
VUS
g.99663295T>C
-
PCDH19(NM_001184880.1):c.301A>G (p.(Ile101Val))
-
PCDH19_000269
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
1
1
c.317T>A
r.(?)
p.(Met106Lys)
-
pathogenic
g.99663279A>T
g.100408281A>T
-
-
PCDH19_000173
-
-
-
-
Somatic
yes
-
-
-
-
Xiaoxu Yang
-?/.
1
-
c.321C>T
r.(?)
p.(Ser107=)
-
likely benign
g.99663275G>A
g.100408277G>A
PCDH19(NM_001184880.1):c.321C>T (p.S107=)
-
PCDH19_000152
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
1
c.339C>A
r.(?)
p.(Cys113*)
-
pathogenic
g.99663257G>T
g.100408259G>T
-
-
PCDH19_000174
-
-
-
-
Germline
yes
-
-
-
-
Xiaoxu Yang
+/.
1
1
c.339dup
r.(?)
p.(Val114Argfs*112)
-
pathogenic
g.99663257dup
g.100408259dup
339_340insC
-
PCDH19_000169
-
-
-
-
Germline
yes
-
-
-
-
Xiaoxu Yang
+?/.
1
1
c.339_355delinsTCGT
r.(?)
p.(Val114ArgfsTer5)
-
likely pathogenic
g.99663241_99663257delinsACGA
g.100408243_100408259delinsACGA
-
-
PCDH19_000232
-
PubMed: Ganapathy 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
1
c.352G>T
r.(?)
p.(Glu118*)
-
pathogenic
g.99663244C>A
g.100408246C>A
-
-
PCDH19_000018
missense change; no variants SCN1A gene; not in 180 control individuals
PubMed: Depienne 2009
-
-
De novo
-
-
-
-
-
Christel Depienne
+/.
2
1
c.357del
r.(?)
p.(Lys120Argfs*3)
-
pathogenic
g.99663239del
g.100408241del
-
-
PCDH19_000011
frameshift change; not in 250 male XLMR/750 control chromosomes
PubMed: Dibbens 2008
-
-
Unknown
yes
-
-
-
-
Johan den Dunnen
+/., +?/.
3
1
c.361G>A
r.(?)
p.(Asp121Asn)
-
likely pathogenic, pathogenic
g.99663235C>T
g.100408237C>T
PCDH19(NM_001184880.1):c.361G>A (p.D121N)
-
PCDH19_000019
missense change; no variants SCN1A gene; not in 180 control individuals,
1 more item
PubMed: Depienne 2009
-
-
CLASSIFICATION record, Unknown
-
-
-
-
-
Christel Depienne
,
VKGL-NL_Rotterdam
+/.
1
-
c.369C>A
r.(?)
p.(Asn123Lys)
-
pathogenic
g.99663227G>T
g.100408229G>T
-
-
PCDH19_000252
-
PubMed: Chuan 2022
-
-
Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
+/.
1
1
c.370G>A
r.(?)
p.(Asp124Asn)
-
pathogenic
g.99663226C>T
g.100408228C>T
-
-
PCDH19_000188
-
PubMed: Liu 2017
-
-
Germline
yes
-
-
-
-
Xiaoxu Yang
?/.
1
-
c.380C>T
r.(?)
p.(Pro127Leu)
-
VUS
g.99663216G>A
g.100408218G>A
-
-
PCDH19_000212
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/.
3
1
c.402C>A
r.(=)
p.(Ile134=)
-
benign
g.99663194G>T
g.100408196G>T
-
-
PCDH19_000085
-
PubMed: Depienne 2011
,
PubMed: Higurashi 2011
,
PubMed: Hynes 2010
-
rs41300169
Unknown
-
0.07, 0.0780, 52/116 patients
-
-
-
Johan den Dunnen
,
Christel Depienne
+/.
1
1
c.415_423dup
r.(?)
p.(Ser139_Ala141dup)
-
pathogenic
g.99663173_99663181dup
g.100408175_100408183dup
-
-
PCDH19_000046
frameshift change
PubMed: Depienne 2011
-
-
Unknown
-
-
-
-
-
Christel Depienne
+/.
1
-
c.416C>A
r.(?)
p.(Ser139Ter)
-
pathogenic
g.99663180G>T
g.100408182G>T
PCDH19(NM_001184880.2):c.416C>A (p.S139*)
-
PCDH19_000151
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/.
1
1
c.424del
r.(?)
p.(Ala142Profs*70)
-
pathogenic
g.99663172del
g.100408174del
-
-
PCDH19_000047
frameshift change
PubMed: Depienne 2011
-
-
Unknown
-
-
-
-
-
Christel Depienne
+/.
1
1
c.437C>G
r.(?)
p.(Thr146Arg)
-
pathogenic
g.99663159G>C
g.100408161G>C
-
-
PCDH19_000048
missense change
PubMed: Depienne 2011
-
-
Unknown
-
-
-
-
-
Christel Depienne
+/.
1
1
c.445C>T
r.(?)
p.(Pro149Ser)
-
pathogenic
g.99663151G>A
g.100408153G>A
-
-
PCDH19_000097
-
PubMed: Leonardi 2014
-
-
De novo
-
-
-
-
-
Emanuela Leonardi
+/.
1
1
c.457G>A
r.(?)
p.(Ala153Thr)
-
pathogenic
g.99663139C>T
g.100408141C>T
-
-
PCDH19_000075
missense change
Depienne, personal communication
-
-
Unknown
-
-
-
-
-
Christel Depienne
+/.
1
1
c.462C>A
r.(?)
p.(Tyr154*)
-
pathogenic
g.99663134G>T
g.100408136G>T
-
-
PCDH19_000049
nonsense change
PubMed: Depienne 2011
-
-
Unknown
-
-
-
-
-
Christel Depienne
+/.
2
-
c.462C>G
r.(?)
p.(Tyr154Ter)
-
pathogenic
g.99663134G>C
g.100408136G>C
PCDH19(NM_001184880.2):c.462C>G (p.Y154*)
-
PCDH19_000203
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
,
VKGL-NL_VUmc
+?/.
1
-
c.469G>A
r.(?)
p.(Asp157Asn)
-
likely pathogenic
g.99663127C>T
-
PCDH19(NM_001184880.2):c.469G>A (p.D157N)
-
PCDH19_000268
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+/.
1
1
c.471C>A
r.(?)
p.(Asp157Glu)
-
pathogenic
g.99663125G>T
g.100408127G>T
-
-
PCDH19_000175
-
-
-
-
De novo
yes
-
-
-
-
Xiaoxu Yang
+/.
1
1
c.471C>G
r.(?)
p.(Asp157Glu)
-
pathogenic
g.99663125G>C
g.100408127G>C
-
-
PCDH19_000176
-
-
-
-
De novo
yes
-
-
-
-
Xiaoxu Yang
+?/.
1
1
c.488T>G
r.(?)
p.(Val163Gly)
-
likely pathogenic
g.99663108A>C
g.100408110A>C
-
-
PCDH19_000159
-
PubMed: Liu 2017
-
-
Germline
yes
-
-
-
-
Xiaoxu Yang
+/.
1
-
c.496del
r.(?)
p.(Tyr166Thrfs*46)
-
pathogenic
g.99663101del
-
PCDH19(NM_001184880.2):c.496delT (p.Y166Tfs*46)
-
PCDH19_000245
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/.
1
1
c.497dup
r.(?)
p.(Tyr166*)
-
pathogenic
g.99663099dup
g.100408101dup
497_498insA
-
PCDH19_000170
-
-
-
-
Germline
yes
-
-
-
-
Xiaoxu Yang
+/.
1
1
c.506del
r.(?)
p.(Thr169Serfs*43)
-
pathogenic
g.99663090del
g.100408092del
-
-
PCDH19_000020
frameshift change; no variants SCN1A gene; not in 180 control individuals
PubMed: Depienne 2009
-
-
De novo
-
-
-
-
-
Christel Depienne
-?/.
1
-
c.513C>T
r.(?)
p.(Asn171=)
-
likely benign
g.99663083G>A
g.100408085G>A
PCDH19(NM_001184880.2):c.513C>T (p.N171=)
-
PCDH19_000202
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/.
1
1
c.514dup
r.(?)
p.(Glu172Glyfs*54)
-
pathogenic
g.99663082dup
g.100408084dup
-
-
PCDH19_000050
frameshift change
PubMed: Depienne 2011
-
-
Unknown
-
-
-
-
-
Christel Depienne
-/.
1
1
c.531G>A
r.(=)
p.(Glu177Glu)
-
benign
g.99663065C>T
g.100408067C>T
-
-
PCDH19_000090
-
PubMed: Higurashi 2011
-
-
Unknown
-
4/116 patients
-
-
-
Johan den Dunnen
?/.
1
-
c.554C>T
r.(?)
p.(Ser185Phe)
-
VUS
g.99663042G>A
g.100408044G>A
-
-
PCDH19_000211
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
1
c.569T>G
r.(?)
p.(Leu190Arg)
-
pathogenic
g.99663027A>C
g.100408029A>C
-
-
PCDH19_000076
missense change
Depienne, personal communication
-
-
Unknown
-
-
-
-
-
Christel Depienne
+/.
1
1
c.571G>C
r.(?)
p.(Val191Leu)
-
pathogenic
g.99663025C>G
g.100408027C>G
-
-
PCDH19_000062
missense change
PubMed: Higurashi 2011
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
+/.
1
1
c.595G>C
r.(?)
p.(Glu199Gln)
-
pathogenic
g.99663001C>G
g.100408003C>G
-
-
PCDH19_000021
missense change; no variants SCN1A gene; not in 180 control individuals
PubMed: Depienne 2009
-
-
Unknown
-
-
-
-
-
Christel Depienne
+?/.
1
1
c.608A>C
r.(?)
p.(His203Pro)
-
likely pathogenic
g.99662988T>G
g.100407990T>G
[608A>C;617T>G]
-
PCDH19_000024
missense change
PubMed: Marini 2010
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.610T>G
r.(?)
p.(Tyr204Asp)
-
likely pathogenic
g.99662986A>C
g.100407988A>C
PCDH19(NM_001184880.2):c.610T>G (p.Y204D)
-
PCDH19_000210
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+/.
1
1
c.617T>A
r.(?)
p.(Phe206Tyr)
-
pathogenic
g.99662979A>T
g.100407981A>T
-
-
PCDH19_000051
missense change
PubMed: Depienne 2011
-
-
Unknown
-
-
-
-
-
Christel Depienne
+?/.
1
1
c.617T>G
r.(?)
p.(Phe206Cys)
-
likely pathogenic
g.99662979A>C
g.100407981A>C
[608A>C;617T>G]
-
PCDH19_000103
missense change
PubMed: Marini 2010
-
-
De novo
-
-
-
-
-
Johan den Dunnen
-/., -?/.
2
1
c.655C>T
r.(=), r.(?)
p.(=), p.(Leu219=)
-
benign, likely benign
g.99662941G>A
g.100407943G>A
(Leu219Leu), PCDH19(NM_001184880.1):c.655C>T (p.L219=)
-
PCDH19_000091
VKGL data sharing initiative Nederland
PubMed: Hynes 2010
-
-
CLASSIFICATION record, Unknown
-
0.0044
-
-
-
Johan den Dunnen
,
VKGL-NL_Rotterdam
?/.
1
-
c.676A>G
r.(?)
p.(Ile226Val)
ACMG
VUS
g.99662920T>C
g.100407922T>C
-
-
PCDH19_000217
ACMG grading: PM1,PM2,PP3 13-month-old female patient with early childhood absences
-
-
-
Germline
-
-
-
-
-
Andreas Laner
-?/.
1
-
c.684G>A
r.(?)
p.(=)
-
likely benign
g.99662912C>T
-
-
-
PCDH19_000257
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
4
1
c.695A>G
r.(?)
p.(Asn232Ser)
-
pathogenic
g.99662901T>C
g.100407903T>C
PCDH19(NM_001184880.2):c.695A>G (p.N232S)
-
PCDH19_000077
missense change, VKGL data sharing initiative Nederland
Depienne, personal communication,
PubMed: Chuan 2022
,
PubMed: Liu 2017
-
-
CLASSIFICATION record, De novo, Germline/De novo (untested), Unknown
yes
-
-
-
-
Johan den Dunnen
,
Christel Depienne
,
VKGL-NL_Utrecht
,
Xiaoxu Yang
+/.
1
1
c.697_700delinsTAAC
r.(?)
p.(Asp233*)
-
pathogenic
g.99662896_99662899delinsGTTA
g.100407898_100407901delinsGTTA
-
-
PCDH19_000052
nonsense change
PubMed: Depienne 2011
-
-
Unknown
-
-
-
-
-
Christel Depienne
+/.
1
1
c.701A>G
r.(?)
p.(Asn234Ser)
-
pathogenic
g.99662895T>C
g.100407897T>C
-
-
PCDH19_000078
missense change
Depienne, personal communication
-
-
Unknown
-
-
-
-
-
Christel Depienne
+/.
1
1
c.706C>T
r.(?)
p.(Pro236Ser)
-
pathogenic
g.99662890G>A
g.100407892G>A
-
-
PCDH19_000057
missense change
PubMed: Specchio 2011
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.707C>T
r.(?)
p.(Pro236Leu)
-
pathogenic
g.99662889G>A
g.100407891G>A
-
-
PCDH19_000117
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
1
c.729C>A
r.(?)
p.(Tyr243*)
-
pathogenic
g.99662867G>T
g.100407869G>T
-
-
PCDH19_000036
nonsense change
PubMed: Jamal 2010
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+/.
1
1
c.730dup
r.(?)
p.(Ala244Glyfs*76)
-
pathogenic
g.99662866dup
g.100407868dup
-
-
PCDH19_000079
frameshift change
Depienne, personal communication
-
-
De novo
-
-
-
-
-
Christel Depienne
+/.
1
1
c.747A>T
r.(?)
p.(Glu249Asp)
-
pathogenic
g.99662849T>A
g.100407851T>A
-
-
PCDH19_000053
missense change
PubMed: Depienne 2011
-
-
Unknown
-
-
-
-
-
Christel Depienne
?/.
2
-
c.754C>T
r.(?)
p.(Pro252Ser)
-
VUS
g.99662842G>A
-
PCDH19(NM_001184880.1):c.754C>T (p.P252S), PCDH19(NM_001184880.2):c.754C>T (p.P252S)
-
PCDH19_000238
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
-?/., ./.
5
-
c.769G>C
r.(?)
p.(Val257Leu)
-
likely benign, VUS
g.99662827C>G
g.100407829C>G
PCDH19(NM_001184880.1):c.769G>C (p.V257L), PCDH19(NM_001184880.2):c.769G>C (p.V257L)
-
PCDH19_000124
VKGL data sharing initiative Nederland
PubMed: Bobbili 2018
,
Journal: Bobbili 2018
-
rs201713027
CLASSIFICATION record, Germline
-
1/567 controls
-
-
-
VKGL-NL_Rotterdam
,
Dheeraj Bobbili
,
VKGL-NL_Groningen
,
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
+/.
1
1
c.772_773del
r.(?)
p.(Ile258Profs*61)
-
pathogenic
g.99662823_99662824del
g.100407825_100407826del
772_773delAT
-
PCDH19_000063
frameshift change
PubMed: Higurashi 2011
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.775C>T
r.(?)
p.(Arg259Cys)
-
VUS
g.99662821G>A
g.100407823G>A
PCDH19(NM_001184880.1):c.775C>T (p.R259C)
-
PCDH19_000201
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
1
c.785C>A
r.(?)
p.(Ala262Asp)
-
pathogenic
g.99662811G>T
g.100407813G>T
-
-
PCDH19_000080
missense change
Depienne, personal communication
-
-
Unknown
-
-
-
-
-
Christel Depienne
?/.
1
-
c.787A>G
r.(?)
p.(Ser263Gly)
-
VUS
g.99662809T>C
-
PCDH19(NM_001184880.1):c.787A>G (p.S263G)
-
PCDH19_000244
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
1
c.790G>C
r.(?)
p.(Asp264His)
-
pathogenic
g.99662806C>G
g.100407808C>G
-
-
PCDH19_000177
-
-
-
-
Germline
yes
-
-
-
-
Xiaoxu Yang
+/.
1
-
c.790G>T
r.(?)
p.(Asp264Tyr)
-
pathogenic
g.99662806C>A
g.100407808C>A
-
-
PCDH19_000200
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/.
1
-
c.798C>T
r.(?)
p.(Asp266=)
-
likely benign
g.99662798G>A
-
PCDH19(NM_001184880.1):c.798C>T (p.D266=)
-
PCDH19_000222
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
1
c.826T>C
r.(?)
p.(Ser276Pro)
-
pathogenic
g.99662770A>G
g.100407772A>G
-
-
PCDH19_000023
missense change
PubMed: Hynes 2010
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+/.
2
1
c.840C>G
r.(?)
p.(Tyr280*), p.(Tyr280Ter)
-
pathogenic
g.99662756G>C
g.100407758G>C
PCDH19(NM_001184880.2):c.840C>G (p.Y280*)
-
PCDH19_000064
nonsense change, VKGL data sharing initiative Nederland
PubMed: Higurashi 2011
-
-
CLASSIFICATION record, De novo
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Groningen
?/.
1
-
c.850C>T
r.(?)
p.(Arg284Cys)
-
VUS
g.99662746G>A
-
PCDH19(NM_001184880.2):c.850C>T (p.R284C)
-
PCDH19_000260
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
?/.
1
-
c.854C>A
r.(?)
p.(Thr285Lys)
-
VUS
g.99662742G>T
g.100407744G>T
PCDH19(NM_001184880.1):c.854C>A (p.T285K)
-
PCDH19_000149
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
3
1
c.859G>T
r.(?)
p.(Glu287*)
-
pathogenic
g.99662737C>A
g.100407739C>A
-
-
PCDH19_000022
missense change; no variants SCN1A gene; not in 180 control individuals
PubMed: Depienne 2009
-
-
De novo, Unknown
-
-
-
-
-
Christel Depienne
+/.
1
-
c.878del
r.(?)
p.(Pro293Argfs*12)
-
pathogenic
g.99662720del
-
PCDH19(NM_001184880.2):c.878delC (p.P293Rfs*12)
-
PCDH19_000225
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+/.
1
1
c.919G>A
r.(?)
p.(Glu307Lys)
-
pathogenic
g.99662677C>T
g.100407679C>T
-
-
PCDH19_000099
-
PubMed: Leonardi 2014
-
-
Germline
-
-
-
-
-
Emanuela Leonardi
+/.
1
1
c.937G>A
r.(?)
p.(Glu313Lys)
-
pathogenic
g.99662659C>T
g.100407661C>T
-
-
PCDH19_000098
-
PubMed: Leonardi 2014
-
-
De novo
-
-
-
-
-
Emanuela Leonardi
+/.
1
1
c.949C>T
r.(?)
p.(Gln317*)
-
pathogenic
g.99662647G>A
g.100407649G>A
-
-
PCDH19_000065
nonsense change
PubMed: Higurashi 2011
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.950A>C
r.(?)
p.(Gln317Pro)
-
likely pathogenic
g.99662646T>G
-
PCDH19(NM_001184880.2):c.950A>C (p.Q317P)
-
PCDH19_000243
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
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