Global Variome shared LOVD
PAX3 (paired box 3)
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Curator:
Veronique Pingault
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Unique variants in the PAX3 gene
The variants shown are described using the NM_181457.3 transcript reference sequence.
Legend
Please note that a short description of a certain column can be displayed when you move your mouse cursor over the column's header and hold it still. Below, a more detailed description is shown per column.
Effect
: The variant's effect on the function of the gene/protein, displayed in the format 'R/C'. R is the value reported by the source (publication, submitter) and this classification may vary between records. C is the value concluded by the curator. Note that in some database the curator uses Summary records to give details on the classification of the variant.Values used: '+' indicating the variant affects function, '+?' probably affects function, '-' does not affect function, '-?' probably does not affect function, '?' effect unknown, '.' effect was not classified.
Reported
: The number of times this variant has been reported in the database.
Exon
: number of exon/intron containing variant; 2 = exon 2, 12i = intron 12, 2i_7i = from intron 2 to intron 7, 8i_9 = intron 8/exon 9 boundary, _1 = 5' to exon 1, 18_ = 3' of exon 18, _1_18_ = encompassing the entire 18-exon gene
DNA change (cDNA)
: description of variant at DNA level, based on a coding DNA reference sequence (following HGVS recommendations); e.g. c.123C>T, c.123_145del, c.123_126dup. For deletions/duplications extending beyond the reference transcript resp. {0}/{2} is used to replace del/dup. Extent of the deletion/duplication should be specified using the genomic description (g.). "-" indicates the variant described on genomic level does not affect the coding DNA reference sequence.
RNA change
: description of variant at RNA level (following HGVS recommendations).
r.123c>u
r.? = unknown
r.(?) = RNA not analysed but probably transcribed copy of DNA variant
r.spl? = RNA not analysed but variant probably affects splicing
r.(spl?) = RNA not analysed but variant may affect splicing
r.0? = change expected to abolish transcription
Protein
: description of variant at protein level (following HGVS recommendations).
p.(Arg345Pro) = change predicted from DNA (RNA not analysed)
p.Arg345Pro = change derived from RNA analysis
p.? = unknown effect
p.0? = probably no protein produced
Classification method
: The method used for the clinical classification of this variant.
All options:
ACMG
ACGS
EAHAD-CFDB
ENIGMA
IARC
InSiGHT
kConFab
other
Clinical classification
: Clinical classification of variant, preferably based on standardised criteria (e.g. ACMG), directed on the clinical consequences as published/submitted, indicated using an enriched system including inheritance: e.g. pathogenic, pathogenic (dominant), pathogenic (recessive), pathogenic (!), pathogenic (maternal), pathogenic (paternal). Standard inheritance is covered by dominant/recessive, imprinting by maternal/paternal. A '!' warns for exceptional circumstances to be explained in the 'Remarks' field (low penetrance, variants pathogenic in heterozygous state only, hypomorphic/hypermorphic variants, protective variants, etc.). Non-disease consequences (e.g. drug metabolism (pharmacogenetics), risk factor, blood group, tasting bitter) are indicated using additions to the benign classification; benign (dominant), benign (recessive), benign (!), etc. The value 'association' is used for variants associated with a phenotype and 'NA' for variants from in vitro/in silico records. NOTE: classification may differ from the opinion of the curator as given in a variant SUMMARY-record or the 'Functional effect concluded'). NOTE: pathogenic/likely pathogenic should go together with "variant (probably) affects function" In ClassFunctional.
All options:
pathogenic
pathogenic (dominant)
pathogenic (recessive)
pathogenic (!)
pathogenic (maternal)
pathogenic (paternal)
likely pathogenic
likely pathogenic (dominant)
likely pathogenic (recessive)
likely pathogenic (!)
likely pathogenic (maternal)
likely pathogenic (paternal)
VUS
VUS (!)
likely benign
likely benign (dominant)
likely benign (recessive)
likely benign (!)
likely benign (maternal)
likely benign (paternal)
benign
benign (dominant)
benign (recessive)
benign (!)
benign (maternal)
benign (paternal)
conflicting
association
NA
DNA change (genomic) (hg19)
: HGVS description of variant at DNA level, based on the genomic (chromosomal) DNA reference sequence; e.g. g.12345678C>T, g.12345679del, g.12345678_12345890dup
DNA change (hg38)
: HGVS description of variant at DNA level, based on the hg38 genomic (chromosomal) eference sequence; e.g. g.12345678C>T, g.12345679del, g.12345678_12345890dup
Published as
: listed only when different from "DNA change"; variant as reported originally (e.g. 521delT). Variants seen in animal models, tested in vitro, predicted from RNA analysis, etc. are described between brackets like c.(456C>G)
ISCN
: description of the variant according to ISCN nomenclature
DB-ID
: database ID of variant, grouping multiple observations of the same variant together, starting with the HGNC gene symbol, followed by an underscore (_) and a six digit number (e.g. DMD_012345). _000000 is used for variants where DNA was not analysed (change predicted from RNA analysis), variants seen in animal models or variants not seen in humans but functionally tested in vitro
Variant remarks
: remarks regarding variant described, e.g. germline mosaicism in mother, 345 kb deletion, muscle RNA analysed, not in 200 control chromosomes tested, on founder haplotype, etc.
Reference
: publication describing the variant submitted, incl. links to OMIM, PubMed or other source, e.g. "den Dunnen ASHG2003 P2346"
ClinVar ID
: ID of variant in ClinVar database
dbSNP ID
: the dbSNP ID
Origin
: Origin of variant/record: Germline = in all cells, De novo = in all cells, but not in either parent, Germline/De novo (untested) = in all cells, parents not tested (use only when De novo is likely, e.g. isolated/sporadic cases with dominant disease), Somatic = present in a subset of cells, but not in either parent, Uniparental disomy = from parental disomy (maternal or paternal), CLASSIFICATION record = submitter only sharing variant classification (note another report may share Individual data), SUMMARY record = master summary record from curator (may link to another database), In vitro (cloned) = data resulting from in vitro functional assays, animal model = data from animal model, Artefact = false positive variant call, DUPLICATE record = variant already described on another chromosome (e.g. unbalanced translocation, duplicating transposition, 2nd fusion transcript, etc.)
All options:
Germline
De novo
Germline/De novo (untested)
Somatic
Uniparental disomy
Uniparental disomy, maternal allele
Uniparental disomy, paternal allele
CLASSIFICATION record
SUMMARY record
In vitro (cloned)
In silico
animal model
Artefact
DUPLICATE record
Unknown
Not applicable
Segregation
: Indicates whether the variant segregates with the phenotype (yes), does not segregate with the phenotype (no) or segregation is unknown (?)
All options:
? = unknown
yes = segregates with phenotype
no = does not segregate with phenotype
- = not applicable
Frequency
: frequency in which the variant was found; e.g 5/760 chromosomes (in 5 of 760 chromosomes tested), 1/33 patients (in 1 of 33 patients analysed in study), 0.05 controls (in 5% of control cases tested)
Re-site
: restriction enzyme recognition site created (+) or destroyed (-); e.g. BglII+;BamHI-
VIP
: variant VIP-status was requested for matchmaking - need collaboration(s) to crack the case - please contact the submitter/curator. NOTE: to get VIP status ask the curator.
Methylation
: result of methylation test; GOM (gain of methylation), LOM (loss of methylation), 30% (30% methylated). NOTE: when several tests were done mention the method as well (e.g. MS-PCR 75%)
How to query this table
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Operator
Column type
Example
Matches
Text
Arg
all entries containing 'Arg'
space
Text
Arg Ser
all entries containing 'Arg' and 'Ser'
|
Text
Arg|Ser
all entries containing 'Arg' or 'Ser'
!
Text
!fs
all entries not containing 'fs'
^
Text
^p.(Arg
all entries beginning with 'p.(Arg'
$
Text
Ser)$
all entries ending with 'Ser)'
=""
Text
=""
all entries with this field empty
=""
Text
="p.0"
all entries exactly matching 'p.0'
!=""
Text
!=""
all entries with this field not empty
!=""
Text
!="p.0"
all entries not exactly matching 'p.0?'
combination
Text
*|Ter !fs
all entries containing '*' or 'Ter' but not containing 'fs'
Date
2020
all entries matching the year 2020
|
Date
2020-03|2020-04
all entries matching March or April, 2020
!
Date
!2020-03
all entries not matching March, 2020
<
Date
<2020
all entries before the year 2020
<=
Date
<=2020-06
all entries in or before June, 2020
>
Date
>2020-06
all entries after June, 2020
>=
Date
>=2020-06-15
all entries on or after June 15th, 2020
combination
Date
2019|2020 <2020-03
all entries in 2019 or 2020, and before March, 2020
Numeric
23
all entries exactly matching 23
|
Numeric
23|24
all entries exactly matching 23 or 24
!
Numeric
!23
all entries not exactly matching 23
<
Numeric
<23
all entries lower than 23
<=
Numeric
<=23
all entries lower than, or equal to, 23
>
Numeric
>23
all entries higher than 23
>=
Numeric
>=23
all entries higher than, or equal to, 23
combination
Numeric
>=20 <30 !23
all entries with values from 20 to 29, but not equal to 23
Some more advanced examples:
Example
Matches
Asian
all entries containing 'Asian', 'asian', including 'Caucasian', 'caucasian', etc.
Asian !Caucasian
all entries containing 'Asian' but not containing 'Caucasian'
Asian|African !Caucasian
all entries containing 'Asian' or 'African', but not containing 'Caucasian'
"South Asian"
all entries containing 'South Asian', but not containing 'South East Asian'
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179 entries on 2 pages. Showing entries 1 - 100.
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Effect
Reported
Exon
DNA change (cDNA)
RNA change
Protein
Classification method
Clinical classification
DNA change (genomic) (hg19)
DNA change (hg38)
Published as
ISCN
DB-ID
Variant remarks
Reference
ClinVar ID
dbSNP ID
Origin
Segregation
Frequency
Re-site
VIP
Methylation
Owner
?/.
1
-
c.-5466C>T
r.(?)
p.(=)
-
VUS
g.223168800G>A
-
CCDC140(NM_153038.1):c.179G>A (p.(Arg60Lys))
-
CCDC140_000010
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/+
1
7i_8_
c.(1173+1_1174-1)_*211{0}
r.?
p.?
-
pathogenic
g.(?_223066432)_(223066910_223084858)del
-
1173+?_(1452-33_?)del
-
PAX3_000125
Deletion of exons 8 and 9
PubMed: Wildhardt 2013
-
-
Germline
-
-
-
-
-
Veronique Pingault
+/+
1
4i_8_
c.(586+1_587-1_*211{0}
r.?
p.?
-
pathogenic
g.(?_223066432)_(223097003_223158885)del
-
Exon 5-9 deletion
-
PAX3_000109
-
PubMed: Milunsky 2007
-
-
Germline
-
-
-
-
-
Veronique Pingault
+/+
10
_1_8_
c.-381_*211{0}
r.0
p.0
-
pathogenic
g.(?_223066432)_(223163715_?)del
-
(?_-61)_(1452-33_?), del (2)(q33q35), del (2)(q35q36), deletion 2q34-q36,2, whole gene deletion,
1 more item
-
PAX3_000101, PAX3_000102, PAX3_000103, PAX3_000104, PAX3_000105, PAX3_000106, PAX3_000107, PAX3_000111,
2 more items
whole gene deletion, whole gene deletion, 1.8-2.6 Mb, whole gene deletion?
PubMed: Matsunaga 2013
,
PubMed: Milunsky 2007
,
PubMed: Pasteris 1993
,
PubMed: Tassabehji 1994
,
2 more items
-
-
De novo, Germline
-
-
-
-
-
Veronique Pingault
+/+
1
1
c.1-?_?
r.0?
p.0?
-
pathogenic
g.?
-
Exon 1 deletion
-
PAX3_000108
4.07kb deletion including exon1 and promoter
PubMed: Milunsky 2007
-
-
Germline
-
-
-
-
-
Veronique Pingault
+/.
1
-
c.52C>T
r,(?)
p.(Gln18Ter)
-
pathogenic (dominant)
g.223163283G>A
g.222298564G>A
-
-
PAX3_000201
combination of alleles not reported
PubMed: Wu 2019
-
-
Germline
-
1/1291 cases hearing loss
-
-
-
Johan den Dunnen
+/.
1
-
c.60C>G
r.(?)
p.(Tyr20Ter)
-
pathogenic
g.223163275G>C
g.222298556G>C
-
-
PAX3_000170
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/+
1
1
c.64C>A
r.(?)
p.(Arg22Ser)
-
pathogenic
g.223163271G>T
g.222298552G>T
-
-
PAX3_000190
-
MORL Deafness Variation Database
,
PubMed: Ptok 2006
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
+/.
1
-
c.86-3C>G
r.spl?
p.?
-
pathogenic
g.223161935G>C
g.222297216G>C
-
-
PAX3_000156
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/+
1
1i
c.86-2A>G
r.spl
p.?
-
pathogenic
g.223161934T>C
g.222297215T>C
86-2 A>G
-
PAX3_000001
-
PubMed: Attaie 1997
-
-
Germline
-
-
-
-
-
Veronique Pingault
+/+
1
1i
c.86-2A>T
r.spl
p.?
-
pathogenic
g.223161934T>A
g.222297215T>A
86>2A>T
-
PAX3_000002
-
PubMed: Soejima 1997
-
-
Germline
-
-
-
-
-
Veronique Pingault
+/+
1
2
c.101dup
r.(?)
p.(Gln35Profs*79)
-
pathogenic
g.223161917dup
g.222297199dup
ins at codon 63 ; 1bpâ–¼34;FS113TER
-
PAX3_000003
-
PubMed: Morell 1993
-
-
Germline
-
-
-
-
-
Veronique Pingault
+/+
1
2
c.111dup
r.(?)
p.(Val38Argfs*76)
-
pathogenic
g.223161907dup
g.222297188dup
111dupC
-
PAX3_000113
-
PubMed: Wildhardt 2013
-
-
Germline
-
-
-
-
-
Veronique Pingault
+?/+?
1
2
c.115A>T
r.(?)
p.(Asn39Tyr)
-
likely pathogenic
g.223161903T>A
g.222297184T>A
-
-
PAX3_000004
-
PubMed: Pingault 2010
-
-
Germline
-
-
-
-
-
Veronique Pingault
+?/.
1
-
c.117C>G
r.(?)
p.(Asn39Lys)
-
likely pathogenic
g.223161901G>C
g.222297182G>C
-
-
PAX3_000155
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
-
c.118C>T
r.(?)
p.(Gln40Ter)
-
pathogenic
g.223161900G>A
g.222297181G>A
-
-
PAX3_000154
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/.
1
-
c.127G>A
r.(?)
p.(Gly43Ser)
-
likely pathogenic
g.223161891C>T
g.222297172C>T
-
-
PAX3_000153
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/.
2
-
c.129T>C
r.(?)
p.(Gly43=)
-
benign
g.223161889A>G
g.222297170A>G
PAX3(NM_181458.4):c.129T>C (p.G43=)
-
PAX3_000134
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
+/+, +?/+?
2
2
c.133T>C
r.(?)
p.(Phe45Leu)
-
likely pathogenic, pathogenic
g.223161885A>G
g.222297166A>G
F45L
-
PAX3_000005
-
MORL Deafness Variation Database
,
PubMed: Tassabehji 1994
,
PubMed: Fortin 1997
,
1 more item
-
-
Germline, SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
,
Veronique Pingault
+/+, +?/+?
2
2
c.139A>C
r.(?)
p.(Asn47His)
-
likely pathogenic, pathogenic
g.223161879T>G
g.222297160T>G
352A>C; Asn>His at 47
-
PAX3_000006
-
PubMed: Hoth 1993
,
1 more item
-
-
Germline, SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
,
Veronique Pingault
+/+, +?/+?
2
2
c.141C>G
r.(?)
p.(Asn47Lys)
-
likely pathogenic, pathogenic
g.223161877G>C
g.222297158G>C
-
-
PAX3_000007
-
MORL Deafness Variation Database
,
PubMed: Sommer 1983
,
PubMed: Asher 1996
-
-
Germline, SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
,
Veronique Pingault
+?/+?
1
2
c.142G>C
r.(?)
p.(Gly48Arg)
-
likely pathogenic
g.223161876C>G
g.222297157C>G
Gly48>Arg
-
PAX3_000008
-
PubMed: Pandya 1996
-
-
Germline
-
-
-
-
-
Veronique Pingault
+/+
1
2
c.142G>T
r.(?)
p.(Gly48Cys)
-
pathogenic
g.223161876C>A
g.222297157C>A
-
-
PAX3_000189
-
MORL Deafness Variation Database
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
+?/+?
1
2
c.143G>A
r.(?)
p.(Gly48Asp)
-
likely pathogenic
g.223161875C>T
g.222297156C>T
-
-
PAX3_000114
-
PubMed: Wildhardt 2013
-
-
Germline
-
-
-
-
-
Veronique Pingault
+/+
1
2
c.145_148dup
r.(?)
p.(Pro50Glnfs*65)
-
pathogenic
g.223161870_223161873dup
g.222297154_222297157dup
Ins146+4 (R49fsX160) dupGGCA
-
PAX3_000009
-
PubMed: Wollnik 2003
-
-
Germline
-
-
-
-
-
Veronique Pingault
+/+, +?/+?
2
2
c.149C>T
r.(?)
p.(Pro50Leu)
-
likely pathogenic, pathogenic
g.223161869G>A
g.222297150G>A
Pro (CCG) to Leu (CTG) in exon 2
-
PAX3_000010
-
PubMed: Baldwin 1992
,
1 more item
-
-
Germline, SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
,
Veronique Pingault
-/.
1
-
c.156C>G
r.(?)
p.(Pro52=)
-
benign
g.223161862G>C
g.222297143G>C
-
-
PAX3_000151
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/+
1
2
c.164T>A
r.(?)
p.(Ile55Asn)
-
pathogenic
g.223161854A>T
g.222297135A>T
-
-
PAX3_000188
-
MORL Deafness Variation Database
,
PubMed: Ptok 2006
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
+/., ?/.
2
-
c.166C>T
r.(?)
p.(Arg56Cys)
ACMG
pathogenic, VUS
g.223161852G>A
-
-
-
PAX3_000191
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
VKGL-NL_Nijmegen
,
Anju Shukla
+?/+?
1
2
c.167G>A
r.(?)
p.(Arg56His)
-
likely pathogenic
g.223161851C>T
g.222297132C>T
-
-
PAX3_000012
-
PubMed: Pingault 2010
-
-
De novo
-
-
-
-
-
Veronique Pingault
+/+, +?/+?
2
2
c.167G>T
r.(?)
p.(Arg56Leu)
-
likely pathogenic, pathogenic
g.223161851C>A
g.222297132C>A
380G>T; Arg>Leu at 56
-
PAX3_000011
-
PubMed: Hoth 1993
,
1 more item
-
-
Germline, SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
,
Veronique Pingault
+/+
1
2
c.169del
r.(?)
p.(His57Thrfs*53)
-
pathogenic
g.223161849del
g.222297131del
1bp del of (C)169
-
PAX3_000013
-
PubMed: Pandya 1996
-
-
Germline
-
-
-
-
-
Veronique Pingault
+?/+?
2
2
c.175A>T
r.(?)
p.(Ile59Phe)
-
likely pathogenic
g.223161843T>A
g.222297124T>A
A to T, Ile to Phe at 59
-
PAX3_000014
-
PubMed: Matsunaga 2013
,
PubMed: Soejima 1997
-
-
Germline
-
-
-
-
-
Veronique Pingault
+/+, +?/+?
2
2
c.176T>A
r.(?)
p.(Ile59Asn)
-
likely pathogenic, pathogenic
g.223161842A>T
g.222297123A>T
t176t->A, I59N
-
PAX3_000094
-
Markova 1998 Human Mutation, Mutation and polymorphism report#34 online,
1 more item
-
-
Germline, SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
,
Veronique Pingault
+?/+?
2
2
c.178G>A
r.(?)
p.(Val60Met)
-
likely pathogenic
g.223161840C>T
g.222297121C>T
G178A (V60M), V60M
-
PAX3_000015
-
PubMed: Baldwin 1995
,
PubMed: Wollnik 2003
-
-
Germline
-
-
-
-
-
Veronique Pingault
+/+, +?/+?, +?/.
3
2
c.184A>G
r.(?)
p.(Met62Val)
-
likely pathogenic, pathogenic
g.223161834T>C
g.222297115T>C
M62V, PAX3(NM_181458.3):c.184A>G (p.M62V)
-
PAX3_000017
VKGL data sharing initiative Nederland
MORL Deafness Variation Database
,
PubMed: Pierpont 1994
,
PubMed: Hol 1998
-
-
CLASSIFICATION record, Germline, SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
,
VKGL-NL_Rotterdam
,
Veronique Pingault
+?/+
1
2
c.185_202del
r.(?)
p.(Met62_Ile67del)
-
likely pathogenic
g.223161816_223161833del
g.222297097_222297114del
185_202del18
-
PAX3_000018
-
PubMed: Tassabehji 1992
-
-
Germline
-
-
-
-
-
Veronique Pingault
+?/+?
1
2
c.186G>A
r.(?)
p.(Met62Ile)
-
likely pathogenic
g.223161832C>T
g.222297113C>T
-
-
PAX3_000115
-
PubMed: Wildhardt 2013
-
-
Germline
-
-
-
-
-
Veronique Pingault
+/+
1
2
c.191_207del
r.(?)
p.(His64Leufs*44)
-
pathogenic
g.223161811_223161827del
g.222297097_222297113del
nt 191del(17)
-
PAX3_000019
-
PubMed: Tassabehji 1995
-
-
Germline
-
-
-
-
-
Veronique Pingault
+?/+?
1
2
c.202C>G
r.(?)
p.(Arg68Gly)
-
likely pathogenic
g.223161816G>C
g.222297097G>C
-
-
PAX3_000020
-
PubMed: Pingault 2010
-
-
Germline
-
-
-
-
-
Veronique Pingault
+?/.
1
-
c.214A>G
r.(?)
p.(Ile72Val)
ACMG
VUS
g.223161804T>C
-
-
-
PAX3_000196
-
-
-
-
Germline/De novo (untested)
-
-
-
-
-
Jian Song
+/., +?/+?
2
2
c.218C>T
r.(?)
p.(Ser73Leu)
-
likely pathogenic, pathogenic
g.223161800G>A
g.222297081G>A
S73L
-
PAX3_000021
VKGL data sharing initiative Nederland
PubMed: Sotirova 2000
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
VKGL-NL_Nijmegen
,
Veronique Pingault
-?/.
1
-
c.219G>A
r.(?)
p.(Ser73=)
-
likely benign
g.223161799C>T
g.222297080C>T
PAX3(NM_181458.3):c.219G>A (p.S73=)
-
PAX3_000133
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
-
c.220C>T
r.(?)
p.(Arg74Cys)
-
pathogenic
g.223161798G>A
g.222297079G>A
-
-
PAX3_000175
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/+
1
2
c.223C>T
r.(?)
p.(Gln75*)
-
pathogenic
g.223161795G>A
g.222297076G>A
Gln75>Term
-
PAX3_000022
-
PubMed: Pandya 1996
-
-
Germline
-
-
-
-
-
Veronique Pingault
+?/+?
1
2
c.232G>A
r.(?)
p.(Val78Met)
-
likely pathogenic
g.223161786C>T
g.222297067C>T
V78M
-
PAX3_000023
-
PubMed: Tassabehji 1995
-
-
Germline
-
-
-
-
-
Veronique Pingault
+/+, +/+?
2
2
c.238C>G
r.(?)
p.(His80Asp)
-
pathogenic
g.223161780G>C
g.222297061G>C
-
-
PAX3_000096
inherited from an unaffected father
MORL Deafness Variation Database
,
PubMed: Alford 2014
,
PubMed: Milunsky 1993
,
PubMed: Zhang 2012
,
1 more item
-
-
SUMMARY record, Unknown
-
-
-
-
-
Global Variome, with Curator vacancy
,
Veronique Pingault
+?/+?
1
2
c.241G>C
r.(?)
p.(Gly81Arg)
-
likely pathogenic
g.223161777C>G
g.222297058C>G
-
-
PAX3_000024
-
PubMed: Pingault 2010
-
-
Germline
-
-
-
-
-
Veronique Pingault
?/?
1
2
c.241G>T
r.(?)
p.(Gly81Cys)
-
VUS
g.223161777C>A
g.222297058C>A
-
-
PAX3_000187
-
MORL Deafness Variation Database
,
PubMed: Milunsky 1993
,
PubMed: Alford 2014
,
PubMed: Tang 2015
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
+/+, +?/+?
2
2
c.242G>C
r.(?)
p.(Gly81Ala)
-
likely pathogenic, pathogenic
g.223161776C>G
g.222297057C>G
Gly to Ala in exon 2
-
PAX3_000025
-
PubMed: Tassabehji 1993
,
1 more item
-
-
Germline, SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
,
Veronique Pingault
+?/.
1
-
c.242G>T
r.(?)
p.(Gly81Val)
-
likely pathogenic
g.223161776C>A
g.222297057C>A
-
-
PAX3_000168
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/+, +?/+?
3
2
c.251C>T
r.(?)
p.(Ser84Phe)
-
likely pathogenic, pathogenic
g.223161767G>A
g.222297048G>A
S84F
-
PAX3_000026
-
PubMed: Zlotogora 1995
,
1 more item
-
-
Germline, SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
,
Veronique Pingault
+?/+?
1
2
c.253A>G
r.(?)
p.(Lys85Glu)
-
likely pathogenic
g.223161765T>C
g.222297046T>C
K85E
-
PAX3_000027
-
PubMed: Baldwin 1995
-
-
Germline
-
-
-
-
-
Veronique Pingault
+/+
1
2
c.260del
r.(?)
p.(Leu87Argfs*23)
-
pathogenic
g.223161758del
g.222297039del
260delT
-
PAX3_000092
-
PubMed: Pingault 2010
-
-
Germline
-
-
-
-
-
Veronique Pingault
+/+
1
2
c.266_279del
r.(?)
p.(Tyr90Leufs*19)
-
pathogenic
g.223161739_223161752del
g.222297020_222297033del
14 bp deletion in the paired domain
-
PAX3_000028
-
PubMed: Morell 1992
-
-
Germline
-
-
-
-
-
Veronique Pingault
+?/.
1
-
c.267G>T
r.(?)
p.(Arg89Ser)
-
likely pathogenic
g.223161751C>A
g.222297032C>A
-
-
PAX3_000202
-
-
-
-
Germline/De novo (untested)
-
-
-
-
-
Gemeinschaftspraxis für Humangenetik Dresden
+?/+?
2
2
c.268T>C
r.(?)
p.(Tyr90His)
-
likely pathogenic
g.223161750A>G
g.222297031A>G
T268C (Y90H)
-
PAX3_000029
-
PubMed: Wollnik 2003
-
-
Germline
-
-
-
-
-
Veronique Pingault
+/+
1
2
c.268_281del
r.(?)
p.(Tyr90Leufs*19)
-
pathogenic
g.223161739_223161752del
g.222297020_222297033del
-
-
PAX3_000028
-
MORL Deafness Variation Database
,
PubMed: Morell 1992
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
+?/., ?/.
4
-
c.281G>T
r.(?)
p.(Gly94Val)
ACMG
likely pathogenic (dominant), VUS
g.223161737C>A
g.222297018C>A
-
-
PAX3_000160
VKGL data sharing initiative Nederland
PubMed: Batissoco 2021
ClinVar-SCV001792232
-
CLASSIFICATION record, Germline
yes
-
-
-
-
VKGL-NL_Nijmegen
,
Karina Lezirovitz Mandelbaum
+?/+?
1
2
c.284_289dup
r.(?)
p.(Ile96_Arg97insProIle)
-
likely pathogenic
g.223161729_223161734dup
g.222297010_222297015dup
duplication of 6bp (ccatc) at codon 96
-
PAX3_000095
-
PubMed: Kohli 2010
-
-
Germline
-
-
-
-
-
Veronique Pingault
+/+
1
2
c.288del
r.(?)
p.(Arg97Valfs*13)
-
pathogenic
g.223161730del
g.222297011del
288delA
-
PAX3_000030
-
PubMed: Tassabehji 1993
-
-
Germline
-
-
-
-
-
Veronique Pingault
+/+
1
2
c.291dup
r.(?)
p.(Pro98Serfs*16)
-
pathogenic
g.223161727dup
g.222297008dup
Insertion (T) in position 292
-
PAX3_000031
-
PubMed: Wildhardt 1996
-
-
Germline
-
-
-
-
-
Veronique Pingault
+/+, +?/+?
2
2
c.295G>A
r.(?)
p.(Gly99Ser)
-
likely pathogenic, pathogenic
g.223161723C>T
g.222297004C>T
[Gly99Ser]+[Arg270Cys]
-
PAX3_000032
-
Bottani 1999 Am J Hum Genet 65:A143,
MORL Deafness Variation Database
,
PubMed: Pingault 2010
-
-
Germline, SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
,
Veronique Pingault
+/+, +?/+?
2
2
c.296G>A
r.(?)
p.(Gly99Asp)
-
likely pathogenic, pathogenic
g.223161722C>T
g.222297003C>T
G99D
-
PAX3_000033
-
MORL Deafness Variation Database
,
PubMed: Fortin 1997
,
PubMed: Tassabehji 1994
-
-
Germline, SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
,
Veronique Pingault
+/+
1
2
c.297_321+3del
r.spl
p.?
-
pathogenic
g.223161694_223161721del
-
297_321+3del28
-
PAX3_000034
-
PubMed: Baldwin 1994
-
-
Germline
-
-
-
-
-
Veronique Pingault
?/.
1
-
c.301A>G
r.(?)
p.(Ile101Val)
-
VUS
g.223161717T>C
-
PAX3(NM_181458.4):c.301A>G (p.(Ile101Val))
-
CCDC140_000016
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
1
-
c.306del
r.(?)
p.(Gly103Alafs*7)
-
pathogenic
g.223161712del
-
-
-
CCDC140_000004
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/.
1
-
c.321+3G>A
r.spl?
p.?
-
likely benign
g.223161694C>T
-
PAX3(NM_181458.4):c.321+3G>A
-
CCDC140_000017
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+?/.
1
-
c.322-57_322-8del
r.(321_322ins[322-117_322-58;cuuaaag])
p.?
-
VUS
g.223160384_223160433del
g.222295665_222295714del
-
-
PAX3_000197
effect on splicing predicted from mini-gene splicing assay
PubMed: Chen 2021
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.353T>C
r.(?)
p.(Ile118Thr)
-
likely pathogenic (dominant)
g.223160345A>G
g.222295626A>G
-
-
PAX3_000198
-
PubMed: Boucher 2020
-
-
Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
+/+
1
3
c.358del
r.(?)
p.(Glu120Asnfs*32)
-
pathogenic
g.223160340del
g.222295622del
nt 358del(G)
-
PAX3_000035
-
PubMed: Tassabehji 1995
-
-
Germline
-
-
-
-
-
Veronique Pingault
+/+
1
3
c.365_369del
r.(?)
p.(Lys122Argfs*21)
-
pathogenic
g.223160329_223160333del
g.222295611_222295615del
nt 364del(5)
-
PAX3_000036
-
PubMed: Tassabehji 1995
-
-
Germline
-
-
-
-
-
Veronique Pingault
+/+
1
3
c.386_398del
r.(?)
p.(Phe129Serfs*19)
-
pathogenic
g.223160301_223160313del
g.222295582_222295594del
384del(13)
-
PAX3_000037
-
PubMed: Tekin 2001
-
-
Germline
-
-
-
-
-
Veronique Pingault
+/+
1
3
c.400C>T
r.(?)
p.(Arg134*)
-
pathogenic
g.223160298G>A
g.222295579G>A
-
-
PAX3_000116
-
PubMed: Wildhardt 2013
-
-
Germline
-
-
-
-
-
Veronique Pingault
+/+
1
3
c.434_449del
r.(?)
p.(Arg145Glnfs*2)
-
pathogenic
g.223160249_223160264del
g.222295532_222295547del
434del16
-
PAX3_000038
-
PubMed: Read 1997
-
-
Germline
-
-
-
-
-
Veronique Pingault
+/+
1
3
c.446del
r.(?)
p.(Pro149Argfs*3)
-
pathogenic
g.223160252del
g.222295534del
446delC
-
PAX3_000039
-
PubMed: Pingault 2010
-
-
Unknown
-
-
-
-
-
Veronique Pingault
+/.
1
3
c.449C>G
r.(?)
p.(Ser150*)
-
pathogenic
g.223160249G>C
g.222295530G>C
-
-
PAX3_000131
-
-
-
-
Germline/De novo (untested)
-
-
-
-
-
Gemeinschaftspraxis für Humangenetik Dresden
?/.
1
-
c.450A>G
r.(?)
p.(=)
-
VUS
g.223160248T>C
g.222295529T>C
-
-
PAX3_000157
classification tendency pathogenic
-
-
-
Germline/De novo (untested)
-
-
-
-
-
Gemeinschaftspraxis für Humangenetik Dresden
+/+
2
3i
c.451+1G>A
r.spl, r.spl?
p.?
-
pathogenic
g.223160246C>T
g.222295527C>T
G to A at the exon3-intron 3 boundary
-
PAX3_000041
-
MORL Deafness Variation Database
,
PubMed: Xiong 2015
,
PubMed: Butt 1994
-
-
Germline, SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
,
Veronique Pingault
+/+
2
3i
c.451+1G>T
r.spl, r.spl?
p.?
-
pathogenic
g.223160246C>A
g.222295527C>A
-
-
PAX3_000040
-
MORL Deafness Variation Database
,
PubMed: Xiong 2015
,
PubMed: Tassabehji 1994
-
-
Germline, SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
,
Veronique Pingault
+/+, +/.
5
3i
c.452-2A>G
r.spl, r.spl?
p.?
ACMG
pathogenic, pathogenic (dominant)
g.223159022T>C
g.222294303T>C
-
-
PAX3_000042
-
MORL Deafness Variation Database
,
PubMed: Xiong 2015
,
PubMed: Li 2019
,
PubMed: Tassabehji 1994
,
1 more item
-
-
Germline, SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
,
Johan den Dunnen
,
Veronique Pingault
,
Jian Song
+/+
1
4
c.455_458dup
r.(?)
p.(Ile154Phefs*9)
-
pathogenic
g.223159014_223159017dup
g.222294295_222294298dup
456_459dupTTCC, p.Ile154PhefsX162
-
PAX3_000098
455_458dupGTTC confirmed by authors
PubMed: Wang 2010
-
-
De novo
-
-
-
-
-
Veronique Pingault
+/+
1
4
c.456_459dup
r.(?)
p.(Ile154Phefs*9)
-
pathogenic
g.223159013_223159016dup
g.222294294_222294297dup
-
-
PAX3_000098
-
MORL Deafness Variation Database
,
PubMed: Wang 2010
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
-?/.
1
-
c.525G>C
r.(?)
p.(Lys175Asn)
-
likely benign
g.223158947C>G
g.222294228C>G
PAX3(NM_181458.3):c.525G>C (p.K175N)
-
PAX3_000167
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/+
1
4
c.556del
r.(?)
p.(His186Thrfs*7)
-
pathogenic
g.223158916del
g.222294197del
556delC
-
PAX3_000097
-
PubMed: Chen 2010
-
-
Unknown
-
-
-
-
-
Veronique Pingault
+/+, +/.
2
4
c.558_559del
r.(?)
p.(His186Glnfs*17)
-
pathogenic
g.223158913_223158914del, g.223158916_223158917del
g.222294197_222294198del
558_559delCA, PAX3(NM_181458.4):c.558_559del (p.(His186Glnfs*17))
-
PAX3_000043
VKGL data sharing initiative Nederland
PubMed: Tassabehji 1993
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
VKGL-NL_Leiden
,
Veronique Pingault
?/.
2
-
c.561C>G
r.(?)
p.(Ser187Arg)
-
VUS
g.223158911G>C
-
PAX3(NM_181457.4):c.561C>G (p.S187R), PAX3(NM_181458.4):c.561C>G (p.(Ser187Arg))
-
CCDC140_000011
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Groningen
+/+
1
4
c.567_586+17del
r.spl
p.?
-
pathogenic
g.223158869_223158905del
-
567_586+17del37
-
PAX3_000099
-
PubMed: Wang 2010
-
-
Unknown
-
-
-
-
-
Veronique Pingault
-?/.
1
-
c.579C>A
r.(?)
p.(Ser193Arg)
-
likely benign
g.223158893G>T
-
PAX3(NM_181459.3):c.579C>A (p.(Ser193Arg))
-
CCDC140_000009
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
-
c.580G>A
r.(?)
p.(Glu194Lys)
-
VUS
g.223158892C>T
g.222294173C>T
-
-
PAX3_000172
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/+
2
4
c.583C>T
r.(?)
p.(Arg195*)
-
pathogenic
g.223158889G>A
g.222294170G>A
R195X
-
PAX3_000044
-
MORL Deafness Variation Database
,
PubMed: Xiong 2015
,
PubMed: Read 1997
-
-
Germline, SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
,
Veronique Pingault
+?/+?
2
4
c.586G>A
r.spl?
p.?
-
likely pathogenic
g.223158886C>T
g.222294167C>T
A196T, G586A (A196T)
-
PAX3_000045
splice or missense variant?
PubMed: Tassabehji 1995
,
PubMed: Wollnik 2003
-
-
Germline, Unknown
-
-
-
-
-
Veronique Pingault
+?/.
2
-
c.586+2T>A
r.spl
p.?
ACMG
pathogenic (dominant)
g.223158884A>T
g.222294165A>T
-
-
PAX3_000194
-
PubMed: Batissoco 2021
ClinVar-SCV001792234
-
Germline, Unknown
yes
-
-
-
-
Karina Lezirovitz Mandelbaum
-?/.
1
-
c.586+57A>G
r.(=)
p.(=)
-
likely benign
g.223158829T>C
-
PAX3(NM_000438.5):c.643A>G (p.S215G)
-
CCDC140_000002
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.586+408A>C
r.(=)
p.(=)
-
likely benign
g.223158478T>G
g.222293759T>G
PAX3(NM_000438.5):c.*346A>C (p.(=))
-
PAX3_000166
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
-
c.586+425C>T
r.(=)
p.(=)
-
VUS
g.223158461G>A
-
PAX3(NM_013942.5):c.602C>T (p.(Ser201Phe))
-
CCDC140_000015
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/., ?/.
2
-
c.586+434C>T
r.(=)
p.(=)
-
likely benign, VUS
g.223158452G>A
g.222293733G>A
PAX3(NM_013942.4):c.611C>T (p.S204F), PAX3(NM_013942.5):c.611C>T (p.(Ser204Phe))
-
PAX3_000165
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
+/.
1
-
c.587-2A>G
r.spl
p.?
-
pathogenic (dominant)
g.223097004T>C
g.222232285T>C
-
-
PAX3_000200
combination of alleles not reported
PubMed: Wu 2019
-
-
Germline
-
1/1291 cases hearing loss
-
-
-
Johan den Dunnen
+/+
1
5
c.589del
r.(?)
p.(Ser197Glnfs*19)
-
pathogenic
g.223097000del
g.222232281del
589delT
-
PAX3_000117
-
PubMed: Wildhardt 2013
-
-
Germline
-
-
-
-
-
Veronique Pingault
+/+
2
5
c.598C>T
r.(?)
p.(Gln200*)
-
pathogenic
g.223096991G>A
g.222232272G>A
Q200X
-
PAX3_000046
-
MORL Deafness Variation Database
,
PubMed: Xiong 2015
,
PubMed: Baldwin 1995
-
-
Germline, SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
,
Veronique Pingault
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