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
|
| ?/. |
- |
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 |
| +/+ |
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 |
| +/+ |
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 |
| +/+ |
_1_8_ |
c.-381_*211{0} |
r.0 |
p.0 |
- |
pathogenic |
g.(?_223066432)_(223163715_?)del |
- |
deletion 2q35-q36,1 or deletion (2)(q35q36.2) |
- |
PAX3_000101 |
whole gene deletion? |
PubMed: Wu 1993, PubMed: Lu-Kuo 1993 |
- |
- |
De novo |
- |
- |
- |
- |
- |
Veronique Pingault |
| +/+ |
_1_8_ |
c.-381_*211{0} |
r.0 |
p.0 |
- |
pathogenic |
g.(?_223066432)_(223163715_?)del |
- |
del (2)(q35q36) |
- |
PAX3_000102 |
whole gene deletion? |
PubMed: Pasteris 1993 |
- |
- |
De novo |
- |
- |
- |
- |
- |
Veronique Pingault |
| +/+ |
_1_8_ |
c.-381_*211{0} |
r.0 |
p.0 |
- |
pathogenic |
g.(?_223066432)_(223163715_?)del |
- |
del (2)(q33q35) |
- |
PAX3_000103 |
whole gene deletion? |
PubMed: Pasteris 1993 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Veronique Pingault |
| +/+ |
_1_8_ |
c.-381_*211{0} |
r.0 |
p.0 |
- |
pathogenic |
g.(?_223066432)_(223163715_?)del |
- |
deletion 2q34-q36,2 |
- |
PAX3_000104 |
whole gene deletion |
PubMed: Tassabehji 1994 |
- |
- |
De novo |
- |
- |
- |
- |
- |
Veronique Pingault |
| +/+ |
_1_8_ |
c.-381_*211{0} |
r.0 |
p.0 |
- |
pathogenic |
g.(?_223066432)_(223163715_?)del |
- |
whole gene deletion |
- |
PAX3_000105 |
- |
PubMed: Milunsky 2007 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Veronique Pingault |
| +/+ |
_1_8_ |
c.-381_*211{0} |
r.0 |
p.0 |
- |
pathogenic |
g.(?_223066432)_(223163715_?)del |
- |
whole gene deletion |
- |
PAX3_000106 |
- |
PubMed: Milunsky 2007 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Veronique Pingault |
| +/+ |
_1_8_ |
c.-381_*211{0} |
r.0 |
p.0 |
- |
pathogenic |
g.(?_223066432)_(223163715_?)del |
- |
whole gene deletion |
- |
PAX3_000107 |
- |
PubMed: Milunsky 2007 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Veronique Pingault |
| +/+ |
_1_8_ |
c.-381_*211{0} |
r.0 |
p.0 |
- |
pathogenic |
g.(?_223066432)_(223163715_?)del |
- |
- |
- |
PAX3_000111 |
whole gene deletion, 1.8-2.6 Mb |
PubMed: Matsunaga 2013 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Veronique Pingault |
| +/+ |
_1_8_ |
c.-381_*211{0} |
r.0 |
p.0 |
- |
pathogenic |
g.(?_223066432)_(223163715_?)del |
- |
(?_-61)_(1452-33_?) |
- |
PAX3_000123 |
whole gene deletion |
PubMed: Wildhardt 2013 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Veronique Pingault |
| +/+ |
_1_8_ |
c.-381_*211{0} |
r.0 |
p.0 |
- |
pathogenic |
g.(?_223066432)_(223163715_?)del |
- |
(?_-61)_(1452-33_?) |
- |
PAX3_000126 |
whole gene deletion |
PubMed: Wildhardt 2013 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Veronique Pingault |
| +/+ |
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 |
| +/. |
- |
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 |
| +/. |
- |
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 |
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 |
| +/. |
- |
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 |
| +/+ |
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 |
| +/+ |
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 |
| +/+ |
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 |
| +/+ |
2 |
c.111dup |
r.(?) |
p.(Val38Argfs*76) |
- |
pathogenic |
g.223161907dup |
g.222297188dup |
111dupC |
- |
PAX3_000113 |
- |
PubMed: Wildhardt 2013 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Veronique Pingault |
| +?/+? |
2 |
c.115A>T |
r.(?) |
p.(Asn39Tyr) |
- |
likely pathogenic |
g.223161903T>A |
g.222297184T>A |
- |
- |
PAX3_000004 |
- |
PubMed: Pingault 2010 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Veronique Pingault |
| +?/. |
- |
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 |
| +/. |
- |
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 |
| +?/. |
- |
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 |
| -/. |
- |
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 |
| -/. |
- |
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_Nijmegen |
| +/+ |
2 |
c.133T>C |
r.(?) |
p.(Phe45Leu) |
- |
pathogenic |
g.223161885A>G |
g.222297166A>G |
- |
- |
PAX3_000005 |
- |
MORL Deafness Variation Database, PubMed: Tassabehji 1994, PubMed: Fortin 1997 |
- |
- |
SUMMARY record |
- |
- |
- |
- |
- |
Global Variome, with Curator vacancy |
| +?/+? |
2 |
c.133T>C |
r.(?) |
p.(Phe45Leu) |
- |
likely pathogenic |
g.223161885A>G |
g.222297166A>G |
F45L |
- |
PAX3_000005 |
- |
PubMed: Tassabehji 1994 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Veronique Pingault |
| +/+ |
2 |
c.139A>C |
r.(?) |
p.(Asn47His) |
- |
pathogenic |
g.223161879T>G |
g.222297160T>G |
- |
- |
PAX3_000006 |
- |
MORL Deafness Variation Database, PubMed: Sheffer 1992, PubMed: Goodman 1982, PubMed: Hoth 1993, PubMed: Fortin 1997 |
- |
- |
SUMMARY record |
- |
- |
- |
- |
- |
Global Variome, with Curator vacancy |
| +?/+? |
2 |
c.139A>C |
r.(?) |
p.(Asn47His) |
- |
likely pathogenic |
g.223161879T>G |
g.222297160T>G |
352A>C; Asn>His at 47 |
- |
PAX3_000006 |
- |
PubMed: Hoth 1993 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Veronique Pingault |
| +/+ |
2 |
c.141C>G |
r.(?) |
p.(Asn47Lys) |
- |
pathogenic |
g.223161877G>C |
g.222297158G>C |
- |
- |
PAX3_000007 |
- |
MORL Deafness Variation Database, PubMed: Sommer 1983 |
- |
- |
SUMMARY record |
- |
- |
- |
- |
- |
Global Variome, with Curator vacancy |
| +?/+? |
2 |
c.141C>G |
r.(?) |
p.(Asn47Lys) |
- |
likely pathogenic |
g.223161877G>C |
g.222297158G>C |
- |
- |
PAX3_000007 |
- |
PubMed: Asher 1996 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Veronique Pingault |
| +?/+? |
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 |
| +/+ |
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 |
| +?/+? |
2 |
c.143G>A |
r.(?) |
p.(Gly48Asp) |
- |
likely pathogenic |
g.223161875C>T |
g.222297156C>T |
- |
- |
PAX3_000114 |
- |
PubMed: Wildhardt 2013 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Veronique Pingault |
| +/+ |
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 |
c.149C>T |
r.(?) |
p.(Pro50Leu) |
- |
pathogenic |
g.223161869G>A |
g.222297150G>A |
- |
- |
PAX3_000010 |
- |
MORL Deafness Variation Database, PubMed: Baldwin 1992, PubMed: Milunsky 1993, PubMed: Alford 2014, PubMed: da-Silva 1991, PubMed: Fortin 1997 |
- |
- |
SUMMARY record |
- |
- |
- |
- |
- |
Global Variome, with Curator vacancy |
| +?/+? |
2 |
c.149C>T |
r.(?) |
p.(Pro50Leu) |
- |
likely pathogenic |
g.223161869G>A |
g.222297150G>A |
Pro (CCG) to Leu (CTG) in exon 2 |
- |
PAX3_000010 |
- |
PubMed: Baldwin 1992 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Veronique Pingault |
| -/. |
- |
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 |
| +/+ |
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 |
| +/. |
- |
c.166C>T |
r.(?) |
p.(Arg56Cys) |
ACMG |
pathogenic |
g.223161852G>A |
- |
- |
- |
PAX3_000191 |
- |
- |
- |
- |
Germline |
- |
- |
- |
- |
- |
Anju Shukla |
| ?/. |
- |
c.166C>T |
r.(?) |
p.(Arg56Cys) |
- |
VUS |
g.223161852G>A |
- |
- |
- |
PAX3_000191 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
| +?/+? |
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 |
c.167G>T |
r.(?) |
p.(Arg56Leu) |
- |
pathogenic |
g.223161851C>A |
g.222297132C>A |
- |
- |
PAX3_000011 |
- |
MORL Deafness Variation Database, PubMed: Carezani-Gavin 1992, PubMed: Milunsky 1993, PubMed: Alford 2014, PubMed: Fortin 1997, PubMed: Armoni 2002 |
- |
- |
SUMMARY record |
- |
- |
- |
- |
- |
Global Variome, with Curator vacancy |
| +?/+? |
2 |
c.167G>T |
r.(?) |
p.(Arg56Leu) |
- |
likely pathogenic |
g.223161851C>A |
g.222297132C>A |
380G>T; Arg>Leu at 56 |
- |
PAX3_000011 |
- |
PubMed: Hoth 1993 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Veronique Pingault |
| +/+ |
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 |
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: Soejima 1997 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Veronique Pingault |
| +?/+? |
2 |
c.175A>T |
r.(?) |
p.(Ile59Phe) |
- |
likely pathogenic |
g.223161843T>A |
g.222297124T>A |
- |
- |
PAX3_000014 |
- |
PubMed: Matsunaga 2013 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Veronique Pingault |
| +/+ |
2 |
c.176T>A |
r.(?) |
p.(Ile59Asn) |
- |
pathogenic |
g.223161842A>T |
g.222297123A>T |
- |
- |
PAX3_000094 |
- |
MORL Deafness Variation Database |
- |
- |
SUMMARY record |
- |
- |
- |
- |
- |
Global Variome, with Curator vacancy |
| +?/+? |
2 |
c.176T>A |
r.(?) |
p.(Ile59Asn) |
- |
likely pathogenic |
g.223161842A>T |
g.222297123A>T |
t176t->A, I59N |
- |
PAX3_000094 |
- |
Markova 1998 Human Mutation, Mutation and polymorphism report#34 online |
- |
- |
Germline |
- |
- |
- |
- |
- |
Veronique Pingault |
| +?/+? |
2 |
c.178G>A |
r.(?) |
p.(Val60Met) |
- |
likely pathogenic |
g.223161840C>T |
g.222297121C>T |
V60M |
- |
PAX3_000015 |
- |
PubMed: Baldwin 1995 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Veronique Pingault |
| +?/+? |
2 |
c.178G>A |
r.(?) |
p.(Val60Met) |
- |
likely pathogenic |
g.223161840C>T |
g.222297121C>T |
G178A (V60M) |
- |
PAX3_000015 |
- |
PubMed: Wollnik 2003 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Veronique Pingault |
| +?/. |
- |
c.184A>G |
r.(?) |
p.(Met62Val) |
- |
likely pathogenic |
g.223161834T>C |
g.222297115T>C |
PAX3(NM_181458.3):c.184A>G (p.M62V) |
- |
PAX3_000017 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
| +/+ |
2 |
c.184A>G |
r.(?) |
p.(Met62Val) |
- |
pathogenic |
g.223161834T>C |
g.222297115T>C |
- |
- |
PAX3_000017 |
- |
MORL Deafness Variation Database, PubMed: Pierpont 1994 |
- |
- |
SUMMARY record |
- |
- |
- |
- |
- |
Global Variome, with Curator vacancy |
| +?/+? |
2 |
c.184A>G |
r.(?) |
p.(Met62Val) |
- |
likely pathogenic |
g.223161834T>C |
g.222297115T>C |
M62V |
- |
PAX3_000017 |
- |
PubMed: Hol 1998 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Veronique Pingault |
| +?/+ |
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 |
| +?/+? |
2 |
c.186G>A |
r.(?) |
p.(Met62Ile) |
- |
likely pathogenic |
g.223161832C>T |
g.222297113C>T |
- |
- |
PAX3_000115 |
- |
PubMed: Wildhardt 2013 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Veronique Pingault |
| +/+ |
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 |
| +?/+? |
2 |
c.202C>G |
r.(?) |
p.(Arg68Gly) |
- |
likely pathogenic |
g.223161816G>C |
g.222297097G>C |
- |
- |
PAX3_000020 |
- |
PubMed: Pingault 2010 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Veronique Pingault |
| +?/. |
- |
c.214A>G |
r.(?) |
p.(Ile72Val) |
ACMG |
VUS |
g.223161804T>C |
- |
- |
- |
PAX3_000196 |
- |
- |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Jian Song |
| +/. |
- |
c.218C>T |
r.(?) |
p.(Ser73Leu) |
- |
pathogenic |
g.223161800G>A |
g.222297081G>A |
- |
- |
PAX3_000021 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
| +?/+? |
2 |
c.218C>T |
r.(?) |
p.(Ser73Leu) |
- |
likely pathogenic |
g.223161800G>A |
g.222297081G>A |
S73L |
- |
PAX3_000021 |
- |
PubMed: Sotirova 2000 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Veronique Pingault |
| -?/. |
- |
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 |
| +/. |
- |
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 |
| +/+ |
2 |
c.223C>T |
r.(?) |
p.(Gln75*) |
- |
pathogenic |
g.223161795G>A |
g.222297076G>A |
Gln75>Term |
- |
PAX3_000022 |
- |
PubMed: Pandya 1996 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Veronique Pingault |
| +?/+? |
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 |
c.238C>G |
r.(?) |
p.(His80Asp) |
- |
pathogenic |
g.223161780G>C |
g.222297061G>C |
- |
- |
PAX3_000096 |
- |
MORL Deafness Variation Database, PubMed: Alford 2014, PubMed: Milunsky 1993, PubMed: Zhang 2012 |
- |
- |
SUMMARY record |
- |
- |
- |
- |
- |
Global Variome, with Curator vacancy |
| +/+? |
2 |
c.238C>G |
r.(?) |
p.(His80Asp) |
- |
pathogenic |
g.223161780G>C |
g.222297061G>C |
- |
- |
PAX3_000096 |
inherited from an unaffected father |
PubMed: Chen 2010 |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Veronique Pingault |
| +?/+? |
2 |
c.241G>C |
r.(?) |
p.(Gly81Arg) |
- |
likely pathogenic |
g.223161777C>G |
g.222297058C>G |
- |
- |
PAX3_000024 |
- |
PubMed: Pingault 2010 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Veronique Pingault |
| ?/? |
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 |
c.242G>C |
r.(?) |
p.(Gly81Ala) |
- |
pathogenic |
g.223161776C>G |
g.222297057C>G |
- |
- |
PAX3_000025 |
- |
MORL Deafness Variation Database, PubMed: Tassabehji 1995, PubMed: Alford 2014, PubMed: Hoth 1993, PubMed: Milunsky 1993, PubMed: Reynolds 1995, PubMed: Fortin 1997 |
- |
- |
SUMMARY record |
- |
- |
- |
- |
- |
Global Variome, with Curator vacancy |
| +?/+? |
2 |
c.242G>C |
r.(?) |
p.(Gly81Ala) |
- |
likely pathogenic |
g.223161776C>G |
g.222297057C>G |
Gly to Ala in exon 2 |
- |
PAX3_000025 |
- |
PubMed: Tassabehji 1993 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Veronique Pingault |
| +?/. |
- |
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 |
| +/+ |
2 |
c.251C>T |
r.(?) |
p.(Ser84Phe) |
- |
pathogenic |
g.223161767G>A |
g.222297048G>A |
- |
- |
PAX3_000026 |
- |
MORL Deafness Variation Database, PubMed: Milunsky 1993, PubMed: Zlotogora 1995, PubMed: Alford 2014, PubMed: Fortin 1997 |
- |
- |
SUMMARY record |
- |
- |
- |
- |
- |
Global Variome, with Curator vacancy |
| +?/+? |
2 |
c.251C>T |
r.(?) |
p.(Ser84Phe) |
- |
likely pathogenic |
g.223161767G>A |
g.222297048G>A |
S84F |
- |
PAX3_000026 |
- |
PubMed: Zlotogora 1995 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Veronique Pingault |
| +?/+? |
2 |
c.251C>T |
r.(?) |
p.(Ser84Phe) |
- |
likely pathogenic |
g.223161767G>A |
g.222297048G>A |
S84F |
- |
PAX3_000026 |
- |
PubMed: Zlotogora 1995 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Veronique Pingault |
| +?/+? |
2 |
c.253A>G |
r.(?) |
p.(Lys85Glu) |
- |
likely pathogenic |
g.223161765T>C |
g.222297046T>C |
K85E |
- |
PAX3_000027 |
- |
PubMed: Baldwin 1995 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Veronique Pingault |
| +/+ |
2 |
c.260del |
r.(?) |
p.(Leu87Argfs*23) |
- |
pathogenic |
g.223161758del |
g.222297039del |
260delT |
- |
PAX3_000092 |
- |
PubMed: Pingault 2010 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Veronique Pingault |
| +/+ |
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 |
| +?/. |
- |
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 |
c.268T>C |
r.(?) |
p.(Tyr90His) |
- |
likely pathogenic |
g.223161750A>G |
g.222297031A>G |
T268C (Y90H) |
- |
PAX3_000029 |
- |
PubMed: Wollnik 2003 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Veronique Pingault |
| +?/+? |
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 |
| +/+ |
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 |
| ?/. |
- |
c.281G>T |
r.(?) |
p.(Gly94Val) |
- |
VUS |
g.223161737C>A |
g.222297018C>A |
- |
- |
PAX3_000160 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
| +?/. |
- |
c.281G>T |
r.(?) |
p.(Gly94Val) |
ACMG |
likely pathogenic (dominant) |
g.223161737C>A |
- |
- |
- |
PAX3_000160 |
- |
PubMed: Batissoco 2021 |
ClinVar-SCV001792232 |
- |
Germline |
yes |
- |
- |
- |
- |
Karina Lezirovitz Mandelbaum |
| +?/. |
- |
c.281G>T |
r.(?) |
p.(Gly94Val) |
ACMG |
likely pathogenic (dominant) |
g.223161737C>A |
- |
- |
- |
PAX3_000160 |
- |
PubMed: Batissoco 2021 |
ClinVar-SCV001792232 |
- |
Germline |
yes |
- |
- |
- |
- |
Karina Lezirovitz Mandelbaum |
| +?/. |
- |
c.281G>T |
r.(?) |
p.(Gly94Val) |
ACMG |
likely pathogenic (dominant) |
g.223161737C>A |
- |
- |
- |
PAX3_000160 |
- |
PubMed: Batissoco 2021 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Karina Lezirovitz Mandelbaum |
| +?/+? |
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 |
| +/+ |
2 |
c.288del |
r.(?) |
p.(Arg97Valfs*13) |
- |
pathogenic |
g.223161730del |
g.222297011del |
288delA |
- |
PAX3_000030 |
- |
PubMed: Tassabehji 1993 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Veronique Pingault |
| +/+ |
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 |
c.295G>A |
r.(?) |
p.(Gly99Ser) |
- |
pathogenic |
g.223161723C>T |
g.222297004C>T |
- |
- |
PAX3_000032 |
- |
MORL Deafness Variation Database, PubMed: Pingault 2010 |
- |
- |
SUMMARY record |
- |
- |
- |
- |
- |
Global Variome, with Curator vacancy |
| +?/+? |
2 |
c.295G>A |
r.(?) |
p.(Gly99Ser) |
- |
likely pathogenic |
g.223161723C>T |
g.222297004C>T |
[Gly99Ser]+[Arg270Cys] |
- |
PAX3_000032 |
- |
Bottani 1999 Am J Hum Genet 65:A143 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Veronique Pingault |
| +/+ |
2 |
c.296G>A |
r.(?) |
p.(Gly99Asp) |
- |
pathogenic |
g.223161722C>T |
g.222297003C>T |
- |
- |
PAX3_000033 |
- |
MORL Deafness Variation Database, PubMed: Fortin 1997 |
- |
- |
SUMMARY record |
- |
- |
- |
- |
- |
Global Variome, with Curator vacancy |
| +?/+? |
2 |
c.296G>A |
r.(?) |
p.(Gly99Asp) |
- |
likely pathogenic |
g.223161722C>T |
g.222297003C>T |
G99D |
- |
PAX3_000033 |
- |
PubMed: Tassabehji 1994 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Veronique Pingault |
| +/+ |
2 |
c.297_321+3del |
r.spl |
p.? |
- |
pathogenic |
g.223161694_223161721del |
- |
297_321+3del28 |
- |
PAX3_000034 |
- |
PubMed: Baldwin 1994 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Veronique Pingault |
| ?/. |
- |
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 |
| +/. |
- |
c.306del |
r.(?) |
p.(Gly103Alafs*7) |
- |
pathogenic |
g.223161712del |
- |
- |
- |
CCDC140_000004 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
| -?/. |
- |
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 |