Global Variome shared LOVD
PTCH1 (patched 1)
LOVD v.3.0 Build 30b [
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Curators:
Hans Gille
and
Michel van Geel
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Unique variants in the PTCH1 gene
The variants shown are described using the NM_000264.3 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%)
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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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633 entries on 7 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_24_
c.(?_-340192)_(*2301659_?)del
r.0?
p.0?
-
pathogenic
g.(?_95907016)_(98610835_?)dup
-
-
-
PTCH1_000000
-
PubMed: Izumi 2011
-
-
Unknown
-
-
-
-
-
Michel van Geel
+/.
1
_1_24_
c.-107315_*190107dup
r.?
p.?
-
pathogenic
g.98018568_98377958dup
g.95256286_95615676dup
-
-
PTCH1_000448
-
PubMed: Derwinska 2009
-
-
Unknown
-
-
-
-
-
Michel van Geel
?/.
1
-
c.-8459A>G
r.(?)
p.(=)
-
VUS
g.98279102T>C
-
PTCH1(NM_001083603.2):c.1A>G (p.M1?)
-
PTCH1_000732
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+?/.
1
_1
c.-8456delG
-
-
-
likely pathogenic
g.98279100del
g.95516818del
NM_001083603.1:c.4delG p.(Glu2Asnfs*9)
-
PTCH1_000457
-
PubMed: Chassaing 2016
-
-
Germline
-
-
-
-
-
Michel van Geel
?/.
1
-
c.-8446del
r.(?)
p.(=)
-
VUS
g.98279090del
-
PTCH1(NM_001083603.3):c.14del (p.(Asn5Ilefs*6))
-
PTCH1_000747
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
1
-
c.-8375G>C
r.(?)
p.(=)
-
likely benign
g.98279018C>G
g.95516736C>G
PTCH1(NM_001083603.2):c.85G>C (p.G29R)
-
PTCH1_000666
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
2
-
c.-8329A>G
r.(?)
p.(=)
-
likely benign
g.98278972T>C
g.95516690T>C
PTCH1(NM_001083602.1):c.-219A>G (p.(=)), PTCH1(NM_001083603.2):c.131A>G (p.E44G)
-
PTCH1_000664
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
-/.
1
-
c.-8318_-8316dup
r.(?)
p.(=)
-
benign
g.98278972_98278974dup
g.95516690_95516692dup
PTCH1(NM_001083603.2):c.142_144dupGAA (p.E48dup)
-
PTCH1_000665
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.-8297G>C
r.(?)
p.(=)
-
likely benign
g.98278940C>G
g.95516658C>G
PTCH1(NM_001083602.1):c.-187G>C (p.(=))
-
PTCH1_000661
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/+
1
_1_2i
c.(?_-188)_(394+1_395-1)del
r.(?)
p.?
-
pathogenic
g.(98248157_98268688)_(98270831_?)del
-
-
-
PTCH1_000516
-
Reinders et al, accepted in Molecular Genetics & Genomic Medicine
-
-
Unknown
-
-
-
-
-
Michel van Geel
+/+, +/.
15
_1_24_
c.(?_-188)_(*3411_?)del
r.0?
p.(?), p.0?
-
pathogenic
g.(?_98205264)_(98270831_?)del, g.98205264_98270831del
g.95442982_95508549del
deletion entire PTCH gene, whole PTCH1 gene deletion
-
PTCH1_000292
-
Reinders et al, accepted in Molecular Genetics & Genomic Medicine,
PubMed: Aradhya 2012
,
8 more items
-
-
Unknown
-
-
-
-
-
Michel van Geel
?/.
1
-
c.-12_-4del
r.(?)
p.(=)
-
VUS
g.98270661_98270669del
-
PTCH1(NM_000264.5):c.-12_-4delGGCGGCGGC
-
PTCH1_000722
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-?/.
1
-
c.-9_-4del
r.(?)
p.(=)
-
likely benign
g.98270664_98270669del
g.95508382_95508387del
-
-
PTCH1_000660
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/.
1
-
c.-9_-4dup
r.(?)
p.(=)
-
likely benign
g.98270664_98270669dup
g.95508382_95508387dup
-
-
PTCH1_000673
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/.
1
-
c.-6_-4del
r.(?)
p.(=)
-
likely benign
g.98270667_98270669del
-
PTCH1(NM_000264.5):c.-6_-4delGGC
-
PTCH1_000784
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-/., -?/., ?/.
6
1
c.-6_-4dup
r.(=), r.(?)
p.(=)
-
benign, likely benign, VUS
g.98270667_98270669dup
g.95508385_95508387dup
CGG 8x,
1 more item
-
PTCH1_000291
VKGL data sharing initiative Nederland
PubMed: Tietze 2013
-
rs71366293
CLASSIFICATION record, Germline, Unknown
-
-
-
-
-
Michel van Geel
,
Andreas Laner
,
VKGL-NL_Groningen
,
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
,
VKGL-NL_NKI
+?/+?
1
2i_12i
c.(394+1_395-1)_(1728+1_1729-1)(2)
r.(?)
p.?
-
likely pathogenic
g.(98224281_98229397)_(98248157_98268688)(2)
-
-
-
PTCH1_000452
-
Reinders et al, accepted in Molecular Genetics & Genomic Medicine
-
-
Unknown
-
-
-
-
-
Michel van Geel
+?/+?
1
2i_16i
c.(394+1_395-1)_(2703+1_2704-1)(2)
r.(?)
p.(?)
-
likely pathogenic
g.(98222066_98224137)_(98248157_98268688)(2)
-
-
-
PTCH1_000454
de novo
Reinders et al, accepted in Molecular Genetics & Genomic Medicine
-
-
De novo
-
-
-
-
-
Michel van Geel
+/.
2
_1
c.?
r.?
p.(?), p.?
-
pathogenic
g.98696759_105723255del, g.?
-
5' adjecent to gene, Inv (9)(q21.2q33.1) untraceable in publication
-
PTCH1_000000
-
PubMed: Endo 2009
,
PubMed: Ibn-Salem 2014
-
-
Unknown
-
-
-
-
-
Michel van Geel
-?/., ?/.
4
-
c.37C>G
r.(?)
p.(Arg13Gly)
-
likely benign, VUS
g.98270607G>C
g.95508325G>C
PTCH1(NM_000264.3):c.37C>G (p.R13G, p.(Arg13Gly)), PTCH1(NM_000264.5):c.37C>G (p.R13G)
-
PTCH1_000614
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
,
VKGL-NL_VUmc
?/.
1
-
c.56G>A
r.(?)
p.(Gly19Asp)
-
VUS
g.98270588C>T
-
-
-
PTCH1_000772
-
-
-
rs587780708
CLASSIFICATION record
-
-
-
-
-
MobiDetails
?/.
1
-
c.92_98dup
r.(?)
p.(Arg34Glufs*58)
-
VUS
g.98270553_98270559dup
-
PTCH1(NM_000264.3):c.92_98dupGGAGGCG (p.(Arg34fs))
-
PTCH1_000769
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-/., -?/.
2
-
c.109G>C
r.(?)
p.(Gly37Arg)
-
benign, likely benign
g.98270535C>G
g.95508253C>G
PTCH1(NM_000264.3):c.109G>C (p.(Gly37Arg)), PTCH1(NM_000264.5):c.109G>C (p.G37R)
-
PTCH1_000613
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_VUmc
-?/., ?/.
3
1
c.113G>A
r.(?)
p.(Gly38Glu)
-
likely benign, VUS
g.98270531C>T
g.95508249C>T
g.13717G>A, PTCH1(NM_000264.3):c.113G>A (p.G38E)
-
PTCH1_000550
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
VKGL-NL_Rotterdam
,
Julia Täubner
,
VKGL-NL_Nijmegen
-?/.
2
-
c.113G>T
r.(?)
p.(Gly38Val)
-
likely benign
g.98270531C>A
g.95508249C>A
PTCH1(NM_000264.3):c.113G>T (p.G38V), PTCH1(NM_000264.5):c.113G>T (p.G38V)
-
PTCH1_000612
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_VUmc
+/+, +/.
2
1
c.114del
r.(?)
p.(Leu39Cysfs*41), p.(Leu39CysfsTer41)
-
pathogenic
g.98270536del
g.95508254del
-
-
PTCH1_000526
VKGL data sharing initiative Nederland
PubMed: Reinders 2018
PubMed: Gielen 2018
-
-
CLASSIFICATION record, De novo
-
-
-
-
-
Michel van Geel
,
VKGL-NL_Nijmegen
+/.
1
-
c.122del
r.(?)
p.(Arg41Leufs*39)
-
pathogenic
g.98270522del
-
-
-
PTCH1_000708
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/.
1
-
c.127G>A
r.(?)
p.(Ala43Thr)
-
likely benign
g.98270517C>T
g.95508235C>T
PTCH1(NM_000264.5):c.127G>A (p.A43T)
-
PTCH1_000657
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
?/.
1
-
c.148_149del
r.(?)
p.(Leu50AlafsTer39)
-
VUS
g.98270496_98270497del
-
-
-
PTCH1_000696
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/., ?/.
2
-
c.174C>T
r.(?)
p.(Ala58=)
-
likely benign, VUS
g.98270470G>A
g.95508188G>A
PTCH1(NM_000264.3):c.174C>T (p.A58=)
-
PTCH1_000656
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Nijmegen
-?/.
1
-
c.181G>A
r.(?)
p.(Ala61Thr)
-
likely benign
g.98270463C>T
g.95508181C>T
PTCH1(NM_000264.3):c.181G>A (p.(Ala61Thr))
-
PTCH1_000672
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
-
c.201G>A
r.(?)
p.(=)
-
VUS
g.98270443C>T
-
PTCH1(NM_000264.5):c.201G>A (p.K67=)
-
PTCH1_000775
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
+?/.
1
-
c.202-2A>C
r.spl?
p.?
-
likely pathogenic
g.98268883T>G
g.95506601T>G
-
-
PTCH1_000655
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/+?
1
1i
c.202-2A>G
r.spl?
p.?
-
likely pathogenic
g.98268883T>C
g.95506601T>C
-
-
PTCH1_000525
-
Reinders et al, accepted in Molecular Genetics & Genomic Medicine
-
-
Unknown
-
-
-
-
-
Michel van Geel
+/+
1
2
c.205A>T
r.(?)
p.(Lys69*)
-
pathogenic
g.98268878T>A
g.95506596T>A
-
-
PTCH1_000524
-
Reinders et al, accepted in Molecular Genetics & Genomic Medicine
-
-
Unknown
-
-
-
-
-
Michel van Geel
?/.
1
-
c.211A>G
r.(?)
p.(Thr71Ala)
-
VUS
g.98268872T>C
-
-
-
PTCH1_000726
-
-
-
-
Unknown
-
-
-
-
-
MobiDetails
+/.
1
2
c.222del
r.(?)
p.(Ala75Argfs*5)
-
pathogenic
g.98268863del
g.95506581del
-
-
PTCH1_000222
-
PubMed: Savino 2004
-
-
Unknown
-
-
-
-
-
Michel van Geel
?/.
1
-
c.229C>G
r.(?)
p.(Leu77Val)
-
VUS
g.98268854G>C
-
PTCH1(NM_000264.3):c.229C>G (p.(Leu77Val))
-
PTCH1_000746
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
1
2
c.233G>A
r.(?)
p.(Trp78*)
-
pathogenic
g.98268850C>T
g.95506568C>T
-
-
PTCH1_000356
-
PubMed: Guo 2013
-
-
Unknown
-
-
-
-
-
Michel van Geel
+/.
1
2
c.240_241del
r.(?)
p.(Arg80Serfs*9)
-
pathogenic
g.98268845_98268846del
g.95506563_95506564del
240_241delAG
-
PTCH1_000360
-
PubMed: Takahashi 2009
-
-
Unknown
-
-
-
-
-
Michel van Geel
+/.
1
-
c.244_245del
r.(?)
p.(Lys82ValfsTer7)
-
pathogenic
g.98268838_98268839del
g.95506556_95506557del
PTCH1(NM_001083602.3):c.46_47delAA (p.K16Vfs*7)
-
PTCH1_000611
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+/.
1
2
c.250C>T
r.(?)
p.(Gln84*)
-
pathogenic
g.98268833G>A
g.95506551G>A
-
-
PTCH1_000065
-
PubMed: Savino 2004
-
-
Unknown
-
-
-
-
-
Michel van Geel
+/+, +/.
2
2
c.254_255del
r.(?)
p.(Arg85Thrfs*4)
-
pathogenic
g.98268831_98268832del
g.95506549_95506550del
254_255delGA
-
PTCH1_000104, PTCH1_000523
-
Reinders et al, accepted in Molecular Genetics & Genomic Medicine,
PubMed: Marsh 2005
-
-
Germline, Unknown
yes
-
-
-
-
Michel van Geel
+/+, +/.
6
2
c.258_259del
r.(?)
p.(Leu87Ilefs*2), p.(Leu87IlefsTer2)
-
pathogenic
g.98268826_98268827del
g.95506544_95506545del
244delCT
-
PTCH1_000202
VKGL data sharing initiative Nederland,
1 more item
Reinders et al, accepted in Molecular Genetics & Genomic Medicine,
PubMed: Wicking 1997
,
2 more items
-
-
CLASSIFICATION record, De novo, Germline, Unknown
-
-
-
-
-
Michel van Geel
,
VKGL-NL_Nijmegen
+/.
1
2
c.260_264delinsAA
r.(?)
p.(Leu87*)
-
pathogenic
g.98268819_98268823delinsTT
g.95506537_95506541delinsTT
260_264delTATTTinsAA
-
PTCH1_000270
-
PubMed: Boutet 2003
-
-
Unknown
-
-
-
-
-
Michel van Geel
+/.
1
2
c.262_265del
r.(?)
p.(Phe88Asnfs*28)
-
pathogenic
g.98268821_98268824del
g.95506539_95506542del
262_265delTTTA
-
PTCH1_000135
-
PubMed: Klein 2005
-
-
Unknown
-
-
-
-
-
Michel van Geel
+/+, +/.
2
2
c.262_266del
r.(?)
p.(Phe88Thrfs*50)
-
pathogenic
g.98268818_98268822del
g.95506536_95506540del
PTCH1(NM_000264.5):c.262_266delTTTAA (p.F88Tfs*50)
-
PTCH1_000522
VKGL data sharing initiative Nederland
Reinders et al, accepted in Molecular Genetics & Genomic Medicine
-
-
CLASSIFICATION record, Unknown
-
-
-
-
-
Michel van Geel
,
VKGL-NL_Groningen
?/.
1
-
c.271G>T
r.(?)
p.(Gly91Cys)
-
VUS
g.98268812C>A
g.95506530C>A
PTCH1(NM_000264.3):c.271G>T (p.(Gly91Cys))
-
PTCH1_000671
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
1
2
c.271_293del
r.(?)
p.(Gly91Argfs*41)
-
pathogenic
g.98268796_98268818del
g.95506514_95506536del
253del23
-
PTCH1_000234
-
PubMed: Unden 1996
-
-
Germline
yes
-
-
-
-
Michel van Geel
+/.
1
2
c.279C>A
r.(?)
p.(Tyr93*)
-
pathogenic
g.98268804G>T
g.95506522G>T
C279A
-
PTCH1_000375
-
PubMed: Minami 2001
-
-
De novo
-
-
-
-
-
Michel van Geel
+/+
1
2
c.279C>G
r.(?)
p.(Tyr93*)
-
pathogenic
g.98268804G>C
g.95506522G>C
-
-
PTCH1_000521
-
Reinders et al, accepted in Molecular Genetics & Genomic Medicine
-
-
Unknown
-
-
-
-
-
Michel van Geel
+/.
1
2
c.280dup
r.(?)
p.(Ile94Asnfs*46)
-
pathogenic
g.98268803dup
g.95506521dup
-
-
PTCH1_000048
-
PubMed: Boutet 2003
-
-
Unknown
-
-
-
-
-
Michel van Geel
+/.
1
2
c.282dup
r.(?)
p.(Gln95Serfs*45)
-
pathogenic
g.98268802dup
g.95506520dup
269insT
-
PTCH1_000213
-
PubMed: Chidambaram 1996
-
-
Germline
-
-
-
-
-
Michel van Geel
?/.
1
-
c.286A>C
r.(?)
p.(Lys96Gln)
-
VUS
g.98268797T>G
-
PTCH1(NM_000264.3):c.286A>C (p.(Lys96Gln))
-
PTCH1_000768
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
1
2
c.289_290delinsC
r.(?)
p.(Asn97Profs*20)
-
pathogenic
g.98268793_98268794delinsG
g.95506511_95506512delinsG
277AA>C
-
PTCH1_000211
-
PubMed: Chidambaram 1996
-
-
Germline
-
-
-
-
-
Michel van Geel
+/.
2
2
c.290del
r.(?)
p.(Asn97Thrfs*20)
-
pathogenic
g.98268799del
g.95506517del
PTCH1(NM_000264.5):c.290delA (p.N97Tfs*20)
-
PTCH1_000379
VKGL data sharing initiative Nederland
PubMed: Wilson 2006
-
-
CLASSIFICATION record, Unknown
-
-
-
-
-
Michel van Geel
,
VKGL-NL_VUmc
+/.
1
2
c.290dup
r.(?)
p.(Asn97Lysfs*43)
-
pathogenic
g.98268799dup
g.95506517dup
271insA
-
PTCH1_000224
Negative family history but neither parent examined.
PubMed: Wicking 1997
,
PubMed: Wicking 1997
-
-
De novo
-
-
-
-
-
Michel van Geel
+/+
1
2
c.294C>A
r.(?)
p.(Cys98*)
-
pathogenic
g.98268789G>T
g.95506507G>T
-
-
PTCH1_000520
-
Reinders et al, accepted in Molecular Genetics & Genomic Medicine
-
-
Unknown
-
-
-
-
-
Michel van Geel
+/.
1
2
c.305T>A
r.(?)
p.(Leu102*)
-
pathogenic
g.98268778A>T
g.95506496A>T
-
-
PTCH1_000066
-
PubMed: Savino 2004
-
-
Unknown
-
-
-
-
-
Michel van Geel
+/.
1
2
c.317T>G
r.(?)
p.(Leu106Arg)
-
pathogenic
g.98268766A>C
g.95506484A>C
-
-
PTCH1_000110
-
PubMed: Li 2008
-
-
Unknown
-
-
-
-
-
Michel van Geel
-/., -?/.
3
-
c.318C>T
r.(=), r.(?)
p.(=), p.(Leu106=)
-
benign, likely benign
g.98268765G>A
g.95506483G>A
PTCH1(NM_000264.3):c.318C>T (p.(Leu106=)), PTCH1(NM_000264.5):c.318C>T (p.L106=)
-
PTCH1_000610
14 heterozygous, no homozygous;
Clinindb (India)
, VKGL data sharing initiative Nederland
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs1805153
CLASSIFICATION record, Germline
-
14/2795 individuals
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_VUmc
,
Mohammed Faruq
?/.
1
-
c.319_321del
r.(?)
p.(Leu107del)
-
VUS
g.98268766_98268768del
-
PTCH1(NM_001083602.1):c.121_123delCTC (p.L41del)
-
PTCH1_000702
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
2
c.323_324insGA
r.(?)
p.(Ile108Metfs*10)
-
pathogenic
g.98268759_98268760insTC
g.95506477_95506478insTC
323_324insGA
-
PTCH1_000391
-
PubMed: Pastorino 2005
-
-
Unknown
-
-
-
-
-
Michel van Geel
+/.
1
2
c.328G>C
r.(?)
p.(Gly110Arg)
-
pathogenic
g.98268755C>G
g.95506473C>G
-
-
PTCH1_000396
-
PubMed: Tanioka 2005
-
-
Unknown
-
-
-
-
-
Michel van Geel
+/.
1
2
c.331del
r.(?)
p.(Ala111Profs*6)
-
pathogenic
g.98268755del
g.95506473del
-
-
PTCH1_000108
-
PubMed: Klein 2005
-
-
Unknown
-
-
-
-
-
Michel van Geel
+/.
1
-
c.343G>T
r.(?)
p.(Gly115*)
-
pathogenic
g.98268740C>A
g.95506458C>A
-
-
PTCH1_000681
1 heterozygous, no homozygous;
Clinindb (India)
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs794727242
Germline
-
1/2795 individuals
-
-
-
Mohammed Faruq
+/.
1
3
c.379G>T
r.(?)
p.(Glu127*)
-
pathogenic
g.98268704C>A
g.95506422C>A
-
-
PTCH1_000125
-
PubMed: Klein 2005
-
-
Unknown
-
-
-
-
-
Michel van Geel
+?/.
1
2
c.383T>C
r.(?)
p.(Leu128Pro)
-
pathogenic (dominant)
g.98268700A>G
-
383C>T
-
PTCH1_000693
-
-
-
-
Germline
-
-
-
-
-
Pascal Soularue
+/+
1
2
c.385_386dup
r.(?)
p.(Trp129Cysfs*9)
-
pathogenic
g.98268699_98268700dup
g.95506417_95506418dup
-
-
PTCH1_000519
-
Reinders et al, accepted in Molecular Genetics & Genomic Medicine
-
-
Unknown
-
-
-
-
-
Michel van Geel
+/.
1
3
c.386G>A
r.(?)
p.(Trp129*)
-
pathogenic
g.98268697C>T
g.95506415C>T
-
-
PTCH1_000407
-
PubMed: Soufir 2006
-
-
Unknown
-
-
-
-
-
Michel van Geel
+/.
1
3
c.387G>A
r.(?)
p.(Trp129*)
-
pathogenic
g.98268696C>T
g.95506414C>T
-
-
PTCH1_000188
-
PubMed: Suzuki 2012
-
-
Unknown
-
-
-
-
-
Michel van Geel
+/+
1
2i_24_
c.(393+1_394-1)_(*3411_?)del
r.(?)
p.?
-
pathogenic
g.(?_98205264)_(98248157_98268688)del
-
-
-
PTCH1_000455
-
Reinders et al, accepted in Molecular Genetics & Genomic Medicine
-
-
Unknown
-
-
-
-
-
Michel van Geel
+?/+?
1
2i
c.394+1G>A
r.spl
p.?
-
likely pathogenic
g.98268688C>T
g.95506406C>T
-
-
PTCH1_000517
-
Reinders et al, accepted in Molecular Genetics & Genomic Medicine
-
-
Germline
-
-
-
-
-
Michel van Geel
+?/+?
1
2i
c.394+1G>C
r.spl?
p.?
-
likely pathogenic
g.98268688C>G
g.95506406C>G
-
-
PTCH1_000518
-
Reinders et al, accepted in Molecular Genetics & Genomic Medicine
-
-
Germline
-
-
-
-
-
Michel van Geel
?/.
1
-
c.395-62C>T
r.(?)
p.(?)
-
VUS
g.98248218G>A
-
-
-
PTCH1_000748
-
-
-
rs1841935827
CLASSIFICATION record
-
-
-
-
-
MobiDetails
+/+, +/.
4
3
c.403C>T
r.(?)
p.(Arg135*)
-
pathogenic
g.98248148G>A
g.95485866G>A
C391T, PTCH1(NM_000264.5):c.403C>T (p.R135*)
-
PTCH1_000447
VKGL data sharing initiative Nederland,
1 more item
Reinders et al accepted in Molecular Genetics & Genomic Medicine,
2 more items
-
-
CLASSIFICATION record, De novo, Germline
-
-
-
-
-
Michel van Geel
,
VKGL-NL_Groningen
?/.
1
-
c.419T>C
r.(?)
p.(Leu140Ser)
-
VUS
g.98248132A>G
g.95485850A>G
PTCH1(NM_000264.3):c.419T>C (p.(Leu140Ser))
-
PTCH1_000654
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
1
3
c.426dup
r.(?)
p.(Thr143Tyrfs*12)
-
pathogenic
g.98248125dup
g.95485843dup
426dupT
-
PTCH1_000410
-
Okamoto 2014 Hum Genome Var 1
-
-
Unknown
-
-
-
-
-
Michel van Geel
+?/.
1
-
c.456_457del
r.(?)
p.(Met152Ilefs*2)
-
likely pathogenic (!)
g.98248095_98248096del
g.95485813_95485814del
delGT
-
PTCH1_000725
candidate disease gene
PubMed: Furey 2018
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+/.
1
3
c.459del
r.(?)
p.(Phe153Leufs*6)
-
pathogenic
g.98248094del
g.95485812del
-
-
PTCH1_000411
-
PubMed: Nagao 2005
-
-
Unknown
-
-
-
-
-
Michel van Geel
+/+
1
3
c.466C>T
r.(?)
p.(Gln156*)
-
pathogenic
g.98248085G>A
g.95485803G>A
-
-
PTCH1_000515
-
Reinders et al accepted in Molecular Genetics & Genomic Medicine
-
-
Germline
-
-
-
-
-
Michel van Geel
+/.
1
3
c.478C>T
r.(?)
p.(Gln160*)
-
pathogenic
g.98248073G>A
g.95485791G>A
-
-
PTCH1_000080
-
PubMed: Marsh 2005
-
-
Unknown
-
-
-
-
-
Michel van Geel
+/.
1
3
c.478_479dup
r.(?)
p.(Gln160Hisfs*12)
-
pathogenic
g.98248073_98248074dup
g.95485791_95485792dup
464insAC
-
PTCH1_000412
-
PubMed: Wicking 1997
-
-
Unknown
-
-
-
-
-
Michel van Geel
+/+, +/.
2
3
c.479_482del
r.(?), r.spl?
p.(Gln160Profs*10)
-
pathogenic
g.98248071_98248074del
g.95485789_95485792del
667delAGAC
-
PTCH1_000413
-
Reinders et al accepted in Molecular Genetics & Genomic Medicine,
PubMed: Abe 2008
-
-
Unknown
-
-
-
-
-
Michel van Geel
?/.
1
-
c.500C>A
r.(?)
p.(Ala167Asp)
-
VUS
g.98248051G>T
-
PTCH1(NM_001083602.1):c.302C>A (p.A101D)
-
PTCH1_000709
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.522G>A
r.(?)
p.(Ala174=)
-
likely benign
g.98248029C>T
g.95485747C>T
PTCH1(NM_001083602.1):c.324G>A (p.A108=)
-
PTCH1_000686
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/.
1
-
c.524T>C
r.(?)
p.(Leu175Pro)
-
likely pathogenic
g.98248027A>G
g.95485745A>G
-
-
PTCH1_000620
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
3
c.531_533del
r.(?)
p.(Gln177del)
-
pathogenic
g.98248021_98248023del
g.95485739_95485741del
531_533delACA
-
PTCH1_000049
-
PubMed: Boutet 2003
-
-
Unknown
-
-
-
-
-
Michel van Geel
+/.
2
3
c.534_545del
r.(?)
p.(His178_Ala182delinsGln)
-
pathogenic
g.98248006_98248017del
g.95485724_95485735del
534_545del12
-
PTCH1_000414
-
PubMed: Guo 2013
-
-
Unknown
-
-
-
-
-
Michel van Geel
+/.
1
3
c.550C>T
r.(?)
p.(Gln184*)
-
pathogenic
g.98248001G>A
g.95485719G>A
-
-
PTCH1_000415
-
PubMed: Endo 2009
-
-
Unknown
-
-
-
-
-
Michel van Geel
-?/.
1
-
c.560G>A
r.(?)
p.(Arg187His)
-
likely benign
g.98247991C>T
-
PTCH1(NM_000264.5):c.560G>A (p.(Arg187His))
-
PTCH1_000745
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+?/+?
1
4i
c.566_584+8del
r.spl
p.?
-
likely pathogenic
g.98247961_98247987del
g.95485679_95485705del
-
-
PTCH1_000513
-
Reinders et al accepted in Molecular Genetics & Genomic Medicine
-
-
Unknown
-
-
-
-
-
Michel van Geel
-?/.
1
-
c.567T>C
r.(?)
p.(=)
-
likely benign
g.98247984A>G
-
PTCH1(NM_000264.5):c.567T>C (p.H189=)
-
PTCH1_000783
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
+/+, +?/.
2
3
c.572_575dup
r.(?)
p.(Met192Ilefs*61), p.(Met192IlefsTer61)
-
likely pathogenic, pathogenic
g.98247978_98247981dup
g.95485696_95485699dup
-
-
PTCH1_000514
VKGL data sharing initiative Nederland
Reinders et al accepted in Molecular Genetics & Genomic Medicine
-
-
CLASSIFICATION record, Unknown
-
-
-
-
-
Michel van Geel
,
VKGL-NL_Nijmegen
+/.
1
3
c.584G>A
r.584_585ins584+1_584+37
p.(Arg195Lys)
-
pathogenic
g.98247967C>T
g.95485685C>T
-
-
PTCH1_000417
-
PubMed: Nagao 2005
-
-
Unknown
-
-
-
-
-
Michel van Geel
+/.
1
3i
c.584+1G>A
r.spl?
p.?
-
pathogenic
g.98247966C>T
g.95485684C>T
-
-
PTCH1_000416
-
PubMed: Guo 2013
-
-
Unknown
-
-
-
-
-
Michel van Geel
+/.
1
3i
c.584+2T>G
r.spl?
p.?
-
pathogenic
g.98247965A>C
g.95485683A>C
-
-
PTCH1_000176
-
PubMed: Fujii 2011
-
-
Unknown
-
-
-
-
-
Michel van Geel
?/.
1
-
c.584+3A>T
r.(?)
p.(?)
-
VUS
g.98247964T>A
-
-
-
PTCH1_000771
-
-
-
-
CLASSIFICATION record
-
-
-
-
-
MobiDetails
+/.
1
3i
c.585-1G>A
r.spl?
p.?
-
pathogenic
g.98244486C>T
g.95482204C>T
-
-
PTCH1_000418
-
PubMed: Pastorino 2012
-
-
Unknown
-
-
-
-
-
Michel van Geel
+/.
1
3i_24_
c.(584+1_585-1)_(*3411_?)del
r.?
p.(?)
-
pathogenic
g.98205264_98244486del
g.95442982_95482204del
-
-
PTCH1_000297
-
PubMed: Soufir 2006
-
-
Unknown
-
-
-
-
-
Michel van Geel
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