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.
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
Allele: On which allele is the variant located? Does not necessarily imply inheritance! 'Paternal' (confirmed or inferred), 'Maternal' (confirmed or inferred), 'Parent #1' or #2 for compound heterozygosity without having screened the parents, 'Unknown' for heterozygosity without having screened the parents, 'Both' for homozygozity.
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
|

 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_3i |
c.(?_-1)_(2616+1_2617-1)del |
r.0 |
p.0 |
- |
pathogenic |
g.99657522_99663595del |
- |
- |
- |
PCDH19_000000 |
deletion of exons 1, 2 and 3
Variant Error [EMISMATCH/EUNCERTAIN]: This transcript variant has an error. Please fix this entry and then remove this message. |
PubMed: Depienne 2011 |
- |
- |
De novo |
- |
- |
- |
- |
- |
Christel Depienne |
+/. |
_1_6_ |
c.(?_-1)_(*1_?)del |
r.0 |
p.0 |
- |
pathogenic |
g.99551275_99663595del |
- |
- |
- |
PCDH19_000000 |
whole gene deletion
Variant Error [EMISMATCH/EUNCERTAIN]: This transcript variant has an error. Please fix this entry and then remove this message. |
PubMed: Depienne 2011 |
- |
- |
De novo |
- |
- |
- |
- |
- |
Christel Depienne |
+/. |
_1_6_ |
c.(?_-1)_(*1_?)del |
r.0 |
p.0 |
- |
pathogenic |
g.99551275_99663595del |
- |
- |
- |
PCDH19_000000 |
whole gene deletion
Variant Error [EMISMATCH/EUNCERTAIN]: This transcript variant has an error. Please fix this entry and then remove this message. |
PubMed: Vincent 2011 |
- |
- |
De novo |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_6_ |
c.(?_-1)_(*1_?)del |
r.0 |
p.0 |
- |
NA |
g.(98860000_98844724)_(99732138_99750000)del |
- |
hg18 98731380-99618794del |
- |
PCDH19_000000 |
890 Kb whole gene deletion (PCDH19 gene only), not present in mother, somatic mosaicism (fibroblasts 0.47 del) |
PubMed: Depienne 2009 |
- |
- |
Somatic |
- |
- |
- |
- |
- |
Christel Depienne |
+/. |
_1_6_ |
c.(?_-1)_(*1_?)del |
r.0 |
p.0 |
- |
pathogenic |
g.99551275_99663595del |
- |
- |
- |
PCDH19_000000 |
whole gene deletion
Variant Error [EMISMATCH/EUNCERTAIN]: This transcript variant has an error. Please fix this entry and then remove this message. |
PubMed: Depienne 2011 |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Christel Depienne |
+/. |
_1_6_ |
c.(?_-1)_(*1_?)del |
r.o |
p.0 |
- |
pathogenic |
g.99551275_99663595del |
- |
- |
- |
PCDH19_000000 |
whole gene deletion
Variant Error [EMISMATCH/EUNCERTAIN]: This transcript variant has an error. Please fix this entry and then remove this message. |
PubMed: Vincent 2011 |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
-?/. |
_1_6_ |
c.(?_-1)_(*1_?)del |
r.0 |
p.0 |
- |
likely benign |
g.(98400000_98486801)_(99768045_99800000)dup |
- |
hg18 g.98373457_99654701dup |
- |
PCDH19_000094 |
1.3 Mb duplication, incl. LOC442459, PCDH19 |
PubMed: Pinto 2007 |
- |
- |
Unknown |
- |
1/776 controls |
- |
- |
- |
Johan den Dunnen |
-?/. |
_1_6_ |
c.(?_-1)_(*1_?)del |
r.0 |
p.0 |
- |
likely pathogenic |
g.(99660000_99661702)_(99667141_99668000)del |
- |
hg18 g.99548358_99553797del |
- |
PCDH19_000096 |
5.5 Kb deletion |
PubMed: Matsuzaki 2009 |
- |
- |
Unknown |
- |
4/90 controls |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_6_ |
c.(?_-1)_(*1_?)del |
r.0 |
p.0 |
- |
pathogenic |
g.(?_99551274)_(99663596_?)del |
- |
- |
- |
PCDH19_000000 |
- |
- |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Xiaoxu Yang |
+/. |
_1_6_ |
c.(?_-1)_(*1_?)del |
r.0 |
p.0 |
- |
pathogenic |
g.(?_99551274)_(99663596_?)del |
- |
- |
- |
PCDH19_000000 |
- |
- |
- |
- |
De novo |
yes |
- |
- |
- |
- |
Xiaoxu Yang |
-?/. |
_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 |
+/. |
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 |
+?/. |
- |
c.3G>A |
r.? |
p.(Met1?) |
- |
likely pathogenic (recessive) |
g.99663593C>T |
g.100408595C>T |
- |
- |
PCDH19_000261 |
- |
- |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
-/. |
1 |
c.6G>A |
r.(=) |
p.(Glu2Glu) |
- |
benign |
g.99663590C>T |
g.100408592C>T |
- |
- |
PCDH19_000092 |
- |
PubMed: Higurashi 2011 |
- |
- |
Unknown |
- |
10/116 patients |
- |
- |
- |
Johan den Dunnen |
-/. |
1 |
c.6G>A |
r.(=) |
p.(=) |
- |
benign |
g.99663590C>T |
g.100408592C>T |
(Glu2Glu) |
- |
PCDH19_000092 |
- |
PubMed: Hynes 2010 |
- |
- |
Unknown |
- |
0.0123 |
- |
- |
- |
Johan den Dunnen |
-/. |
- |
c.6G>A |
r.(?) |
p.(Glu2=) |
- |
benign |
g.99663590C>T |
g.100408592C>T |
PCDH19(NM_001184880.2):c.6G>A (p.E2=) |
- |
PCDH19_000092 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Utrecht |
-?/. |
- |
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 |
+/. |
- |
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 |
c.64del |
r.(?) |
p.(Leu22Serfs*8) |
- |
pathogenic |
g.99663534del |
g.100408536del |
64delC |
- |
PCDH19_000164 |
- |
- |
- |
- |
De novo |
yes |
- |
- |
- |
- |
Xiaoxu Yang |
+/. |
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 |
c.78del |
r.(?) |
p.(Lys26Asnfs*4) |
- |
pathogenic |
g.99663518del |
g.100408520del |
- |
- |
PCDH19_000037 |
frameshift change |
PubMed: Jamal 2010 |
- |
- |
De novo |
- |
- |
- |
- |
- |
Johan den Dunnen |
-/. |
- |
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 |
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 |
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 |
c.94G>T |
r.(?) |
p.(Glu32Ter) |
- |
likely pathogenic |
g.99663502C>A |
g.100408504C>A |
- |
- |
PCDH19_000233 |
- |
PubMed: Ganapathy 2019 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
c.134_135del |
r.(?) |
p.(Asp45Glyfs*43) |
- |
pathogenic |
g.99663461_99663462del |
g.100408463_100408464del |
134_135delAC |
- |
PCDH19_000165 |
- |
- |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Xiaoxu Yang |
+/. |
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 |
+/. |
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 |
?/. |
- |
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 |
?/. |
- |
c.154G>A |
r.(?) |
p.(Ala52Thr) |
- |
VUS |
g.99663442C>T |
- |
PCDH19(NM_001184880.1):c.154G>A (p.A52T) |
- |
PCDH19_000204 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
+/. |
1 |
c.158dup |
r.(?) |
p.(Asp54Glyfs*35) |
- |
pathogenic |
g.99663438dup |
g.100408440dup |
158dupT |
- |
PCDH19_000166 |
- |
- |
- |
- |
Somatic |
yes |
- |
- |
- |
- |
Xiaoxu Yang |
?/. |
- |
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 |
c.183dup |
r.(?) |
p.(Arg62Serfs*27) |
- |
pathogenic |
g.99663415dup |
g.100408417dup |
183_184insT |
- |
PCDH19_000167 |
- |
- |
- |
- |
De novo |
yes |
- |
- |
- |
- |
Xiaoxu Yang |
?/. |
- |
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 |
?/. |
- |
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 |
+/. |
- |
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 |
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 |
c.241dup |
r.(?) |
p.(Leu81Profs*8) |
- |
pathogenic |
g.99663355dup |
g.100408357dup |
- |
- |
PCDH19_000073 |
frameshift change |
Depienne, personal communication |
- |
- |
De novo |
- |
- |
- |
- |
- |
Christel Depienne |
+/. |
1 |
c.242T>G |
r.(?) |
p.(Leu81Arg) |
- |
pathogenic |
g.99663354A>C |
g.100408356A>C |
- |
- |
PCDH19_000044 |
missense change |
PubMed: Depienne 2011 |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Christel Depienne |
+/. |
1 |
c.253C>T |
r.(?) |
p.(Gln85*) |
- |
pathogenic |
g.99663343G>A |
g.100408345G>A |
- |
- |
PCDH19_000010 |
nonsense change; not in 250 male XLMR/750 control chromosomes; fibroblast mRNA shows NMD |
PubMed: Dibbens 2008, OMIM:var0003 |
- |
- |
Unknown |
yes |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
c.253C>T |
r.(?) |
p.(Gln85*) |
- |
pathogenic |
g.99663343G>A |
g.100408345G>A |
- |
- |
PCDH19_000010 |
nonsense change |
Depienne, personal communication |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Christel Depienne |
+/. |
1 |
c.253C>T |
r.(?) |
p.(Gln85*) |
- |
pathogenic |
g.99663343G>A |
g.100408345G>A |
- |
- |
PCDH19_000010 |
nonsense change; not in 250 male XLMR/750 control chromosomes; fibroblast mRNA shows NMD |
PubMed: Dibbens 2008, OMIM:var0003 |
- |
- |
Unknown |
yes |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
c.262G>T |
r.(?) |
p.(Asp88Tyr) |
- |
pathogenic |
g.99663334C>A |
g.100408336C>A |
- |
- |
PCDH19_000168 |
- |
- |
- |
- |
De novo |
yes |
- |
- |
- |
- |
Xiaoxu Yang |
+?/. |
- |
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 |
?/. |
- |
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 |
?/. |
- |
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 |
c.317T>A |
r.(?) |
p.(Met106Lys) |
- |
pathogenic |
g.99663279A>T |
g.100408281A>T |
- |
- |
PCDH19_000173 |
- |
- |
- |
- |
Somatic |
yes |
- |
- |
- |
- |
Xiaoxu Yang |
-?/. |
- |
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 |
c.339C>A |
r.(?) |
p.(Cys113*) |
- |
pathogenic |
g.99663257G>T |
g.100408259G>T |
- |
- |
PCDH19_000174 |
- |
- |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Xiaoxu Yang |
+/. |
1 |
c.339dup |
r.(?) |
p.(Val114Argfs*112) |
- |
pathogenic |
g.99663257dup |
g.100408259dup |
339_340insC |
- |
PCDH19_000169 |
- |
- |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Xiaoxu Yang |
+?/. |
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 |
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 |
+/. |
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 |
+/. |
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 |
+/. |
1 |
c.361G>A |
r.(?) |
p.(Asp121Asn) |
- |
pathogenic |
g.99663235C>T |
g.100408237C>T |
- |
- |
PCDH19_000019 |
missense change; no variants SCN1A gene; not in 180 control individuals |
PubMed: Depienne 2009 |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Christel Depienne |
+/. |
1 |
c.361G>A |
r.(?) |
p.(Asp121Asn) |
- |
pathogenic |
g.99663235C>T |
g.100408237C>T |
- |
- |
PCDH19_000019 |
missense change; no variants SCN1A gene; not in 180 control individuals |
PubMed: Depienne 2009 |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Christel Depienne |
+?/. |
- |
c.361G>A |
r.(?) |
p.(Asp121Asn) |
- |
likely pathogenic |
g.99663235C>T |
- |
PCDH19(NM_001184880.1):c.361G>A (p.D121N) |
- |
PCDH19_000019 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
+/. |
- |
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 |
c.370G>A |
r.(?) |
p.(Asp124Asn) |
- |
pathogenic |
g.99663226C>T |
g.100408228C>T |
- |
- |
PCDH19_000188 |
- |
PubMed: Liu 2017 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Xiaoxu Yang |
?/. |
- |
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 |
-/. |
1 |
c.402C>A |
r.(=) |
p.(Ile134=) |
- |
benign |
g.99663194G>T |
g.100408196G>T |
- |
- |
PCDH19_000085 |
- |
PubMed: Higurashi 2011 |
- |
rs41300169 |
Unknown |
- |
52/116 patients |
- |
- |
- |
Johan den Dunnen |
-/. |
1 |
c.402C>A |
r.(=) |
p.(Ile134=) |
- |
benign |
g.99663194G>T |
g.100408196G>T |
- |
- |
PCDH19_000085 |
- |
PubMed: Depienne 2011 |
- |
rs41300169 |
Unknown |
- |
0.07 |
- |
- |
- |
Christel Depienne |
-/. |
1 |
c.402C>A |
r.(=) |
p.(Ile134=) |
- |
benign |
g.99663194G>T |
g.100408196G>T |
- |
- |
PCDH19_000085 |
- |
PubMed: Hynes 2010 |
- |
rs41300169 |
Unknown |
- |
0.0780 |
- |
- |
- |
Johan den Dunnen |
+/. |
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 |
+/. |
- |
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 |
c.424del |
r.(?) |
p.(Ala142Profs*70) |
- |
pathogenic |
g.99663172del |
g.100408174del |
- |
- |
PCDH19_000047 |
frameshift change |
PubMed: Depienne 2011 |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Christel Depienne |
+/. |
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 |
c.445C>T |
r.(?) |
p.(Pro149Ser) |
- |
pathogenic |
g.99663151G>A |
g.100408153G>A |
- |
- |
PCDH19_000097 |
- |
PubMed: Leonardi 2014 |
- |
- |
De novo |
- |
- |
- |
- |
- |
Emanuela Leonardi |
+/. |
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 |
c.462C>A |
r.(?) |
p.(Tyr154*) |
- |
pathogenic |
g.99663134G>T |
g.100408136G>T |
- |
- |
PCDH19_000049 |
nonsense change |
PubMed: Depienne 2011 |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Christel Depienne |
+/. |
- |
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 |
+/. |
- |
c.462C>G |
r.(?) |
p.(Tyr154Ter) |
- |
pathogenic |
g.99663134G>C |
- |
PCDH19(NM_001184880.2):c.462C>G (p.Y154*) |
- |
PCDH19_000203 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_VUmc |
+?/. |
- |
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 |
c.471C>A |
r.(?) |
p.(Asp157Glu) |
- |
pathogenic |
g.99663125G>T |
g.100408127G>T |
- |
- |
PCDH19_000175 |
- |
- |
- |
- |
De novo |
yes |
- |
- |
- |
- |
Xiaoxu Yang |
+/. |
1 |
c.471C>G |
r.(?) |
p.(Asp157Glu) |
- |
pathogenic |
g.99663125G>C |
g.100408127G>C |
- |
- |
PCDH19_000176 |
- |
- |
- |
- |
De novo |
yes |
- |
- |
- |
- |
Xiaoxu Yang |
+?/. |
1 |
c.488T>G |
r.(?) |
p.(Val163Gly) |
- |
likely pathogenic |
g.99663108A>C |
g.100408110A>C |
- |
- |
PCDH19_000159 |
- |
PubMed: Liu 2017 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Xiaoxu Yang |
+/. |
- |
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 |
c.497dup |
r.(?) |
p.(Tyr166*) |
- |
pathogenic |
g.99663099dup |
g.100408101dup |
497_498insA |
- |
PCDH19_000170 |
- |
- |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Xiaoxu Yang |
+/. |
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 |
-?/. |
- |
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 |
c.514dup |
r.(?) |
p.(Glu172Glyfs*54) |
- |
pathogenic |
g.99663082dup |
g.100408084dup |
- |
- |
PCDH19_000050 |
frameshift change |
PubMed: Depienne 2011 |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Christel Depienne |
-/. |
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 |
?/. |
- |
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 |
c.569T>G |
r.(?) |
p.(Leu190Arg) |
- |
pathogenic |
g.99663027A>C |
g.100408029A>C |
- |
- |
PCDH19_000076 |
missense change |
Depienne, personal communication |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Christel Depienne |
+/. |
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 |
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 |
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 |
+?/. |
- |
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 |
c.617T>A |
r.(?) |
p.(Phe206Tyr) |
- |
pathogenic |
g.99662979A>T |
g.100407981A>T |
- |
- |
PCDH19_000051 |
missense change |
PubMed: Depienne 2011 |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Christel Depienne |
+?/. |
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 |
-/. |
1 |
c.655C>T |
r.(=) |
p.(=) |
- |
benign |
g.99662941G>A |
g.100407943G>A |
(Leu219Leu) |
- |
PCDH19_000091 |
- |
PubMed: Hynes 2010 |
- |
- |
Unknown |
- |
0.0044 |
- |
- |
- |
Johan den Dunnen |
-?/. |
- |
c.655C>T |
r.(?) |
p.(Leu219=) |
- |
likely benign |
g.99662941G>A |
- |
PCDH19(NM_001184880.1):c.655C>T (p.L219=) |
- |
PCDH19_000091 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
?/. |
- |
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 |
-?/. |
- |
c.684G>A |
r.(?) |
p.(=) |
- |
likely benign |
g.99662912C>T |
- |
- |
- |
PCDH19_000257 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
+/. |
1 |
c.695A>G |
r.(?) |
p.(Asn232Ser) |
- |
pathogenic |
g.99662901T>C |
g.100407903T>C |
- |
- |
PCDH19_000077 |
missense change |
Depienne, personal communication |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Christel Depienne |
+/. |
- |
c.695A>G |
r.(?) |
p.(Asn232Ser) |
- |
pathogenic |
g.99662901T>C |
g.100407903T>C |
PCDH19(NM_001184880.2):c.695A>G (p.N232S) |
- |
PCDH19_000077 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Utrecht |
+/. |
1 |
c.695A>G |
r.(?) |
p.(Asn232Ser) |
- |
pathogenic |
g.99662901T>C |
g.100407903T>C |
- |
- |
PCDH19_000077 |
- |
PubMed: Liu 2017 |
- |
- |
De novo |
yes |
- |
- |
- |
- |
Xiaoxu Yang |
+/. |
- |
c.695A>G |
r.(?) |
p.(Asn232Ser) |
- |
pathogenic |
g.99662901T>C |
g.100407903T>C |
- |
- |
PCDH19_000077 |
- |
PubMed: Chuan 2022 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
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 |
c.701A>G |
r.(?) |
p.(Asn234Ser) |
- |
pathogenic |
g.99662895T>C |
g.100407897T>C |
- |
- |
PCDH19_000078 |
missense change |
Depienne, personal communication |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Christel Depienne |