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
|
| +/. |
7i_8_ |
c.(612+1_613-1)_*1272{0} |
r.? |
p.? |
- |
pathogenic |
g.(?_120427374)_(120428952_120446602)del |
- |
del ex8 |
- |
TSPAN12_000096 |
- |
PubMed: Huang 2017 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +/. |
_1_8_ |
c.-374_*1272{0} |
r.0? |
c.0? |
- |
pathogenic (dominant) |
g.(?_120427374)_(120498177_?)del |
g.(?_120787320)_(120858123_?)del |
7q22 gene deletion |
- |
TSPAN12_000097 |
- |
PubMed: Miller 2015 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
| +/. |
2 |
c.1A>G |
r.(?) |
p.0? |
- |
pathogenic (dominant) |
g.120496817T>C |
g.120856763T>C |
- |
- |
TSPAN12_000039 |
- |
PubMed: Li 2019 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Jasmine Chen |
| +/. |
2 |
c.1A>G |
r.? |
p.? |
- |
pathogenic |
g.120496817T>C |
g.120856763T>C |
- |
- |
TSPAN12_000039 |
- |
PubMed: Yuan 2019 |
- |
rs1341600790 |
Germline |
yes |
3/120 patients, 0/500 individual controls |
- |
- |
- |
Dong Sun |
| +/. |
2 |
c.1A>G |
r.? |
p.? |
- |
VUS |
g.120496817T>C |
g.120856763T>C |
- |
- |
TSPAN12_000039 |
- |
PubMed: Yuan 2019 |
- |
rs1341600790 |
Germline |
yes |
3/120 patients, 0/500 individual controls |
- |
- |
- |
Dong Sun |
| +/. |
2 |
c.1A>G |
r.? |
p.? |
- |
pathogenic |
g.120496817T>C |
g.120856763T>C |
- |
- |
TSPAN12_000039 |
- |
PubMed: Yuan 2019 |
- |
rs1341600790 |
Germline |
yes |
3/120 patients, 0/500 individual controls |
- |
- |
- |
Dong Sun |
| +/. |
- |
c.2T>C |
r.(?) |
p.(Met1?) |
- |
pathogenic |
g.120496816A>G |
g.120856762A>G |
- |
- |
TSPAN12_000054 |
- |
PubMed: Tang 2017 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +?/. |
2 |
c.2T>C |
r.(?) |
p.(Met1?) |
- |
likely pathogenic |
g.120496816A>G |
g.120856762A>G |
TSPAN12 2T?>?C, Met1Thr |
- |
TSPAN12_000054 |
- |
PubMed: Wang 2019 |
- |
- |
Germline/De novo (untested) |
? |
- |
- |
- |
- |
LOVD |
| +?/. |
- |
c.53A>G |
r.(?) |
p.(Asn18Ser) |
- |
likely pathogenic |
g.120496765T>C |
g.120856711T>C |
461A>C, Gln154Pro |
- |
TSPAN12_000087 |
- |
PubMed: Chen 2019 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
| +?/. |
- |
c.53A>G |
r.(?) |
p.(Asn18Ser) |
- |
likely pathogenic |
g.120496765T>C |
g.120856711T>C |
461A>C, Gln154Pro |
- |
TSPAN12_000087 |
- |
PubMed: Chen 2019 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
| ?/. |
- |
c.56T>G |
r.(?) |
p.(Leu19Arg) |
- |
VUS |
g.120496762A>C |
g.120856708A>C |
- |
- |
TSPAN12_000032 |
- |
PubMed: Seo 2016 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Jasmine Chen |
| +?/. |
- |
c.56T>G |
r.(?) |
p.(Leu19Arg) |
- |
likely pathogenic (dominant) |
g.120496762A>C |
g.120856708A>C |
- |
- |
TSPAN12_000032 |
not in 288 control alleles |
PubMed: Seo 2015 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +?/. |
- |
c.66+1G>A |
r.spl? |
p.? |
- |
likely pathogenic |
g.120496751C>T |
g.120856697C>T |
- |
- |
TSPAN12_000010 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
| -?/. |
- |
c.67-3C>T |
r.spl? |
p.? |
- |
likely benign |
g.120480166G>A |
g.120840112G>A |
TSPAN12(NM_012338.4):c.67-3C>T |
- |
TSPAN12_000003 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_AMC |
| +?/. |
- |
c.67-2A>G |
r.spl |
p.? |
- |
likely pathogenic |
g.120480165T>C |
g.120840111T>C |
- |
- |
TSPAN12_000056 |
- |
PubMed: Iarossi 2017 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +/. |
2i |
c.67-2A>G |
r.spl |
p.? |
- |
pathogenic (dominant) |
g.120480165T>C |
- |
IVS2 c.67-2A>G |
- |
TSPAN12_000056 |
- |
PubMed: Amorelli 2019 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
| +/. |
2i |
c.67-1G>C |
r.spl |
p.Leu23Glyfs*66 |
- |
likely pathogenic (recessive) |
g.120480164C>G |
g.120840110C>G |
- |
- |
TSPAN12_000031 |
- |
PubMed: Poulter 2012 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Jasmine Chen |
| +/. |
2i |
c.67-1G>C |
r.spl |
p.Leu23Glyfs*66 |
- |
likely pathogenic |
g.120480164C>G |
g.120840110C>G |
- |
- |
TSPAN12_000031 |
- |
PubMed: Poulter 2012 |
- |
- |
Germline |
? |
- |
- |
- |
- |
Jasmine Chen |
| +/. |
3 |
c.68T>G |
r.(?) |
p.(Leu23*) |
- |
VUS |
g.120480162A>C |
g.120840108A>C |
- |
- |
TSPAN12_000030 |
- |
PubMed: Poulter 2010 |
- |
- |
De novo |
? |
- |
- |
- |
- |
Jasmine Chen |
| +/. |
3 |
c.125T>C |
r.(?) |
p.(Val42Ala) |
- |
pathogenic |
g.120480105A>G |
g.120840051A>G |
- |
- |
TSPAN12_000061 |
- |
PubMed: Musada 2016 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +/. |
3 |
c.146C>T |
r.(?) |
p.(Thr49Met) |
- |
pathogenic |
g.120480084G>A |
g.120840030G>A |
- |
- |
TSPAN12_000029 |
heterozygous, either germline or de novo |
PubMed: Poulter 2012 |
- |
- |
Germline/De novo (untested) |
? |
0/500 control chromosomes |
- |
- |
- |
Jasmine Chen |
| +/. |
- |
c.146C>T |
r.(?) |
p.(Thr49Met) |
- |
likely pathogenic |
g.120480084G>A |
g.120840030G>A |
- |
- |
TSPAN12_000029 |
- |
PubMed: Yang 2011 |
- |
- |
Germline/De novo (untested) |
? |
- |
- |
- |
- |
Jasmine Chen |
| +?/. |
- |
c.146C>T |
r.(?) |
p.(Thr49Met) |
- |
likely pathogenic |
g.120480084G>A |
g.120840030G>A |
146C>T, Thr49Met |
- |
TSPAN12_000029 |
- |
PubMed: Chen 2020 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
| +/. |
- |
c.149+1G>A |
r.spl |
p.? |
- |
pathogenic |
g.120480080C>T |
g.120840026C>T |
- |
- |
TSPAN12_000068 |
- |
PubMed: Salvo 2015 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +/. |
- |
c.149+1G>A |
r.spl |
p.? |
- |
pathogenic |
g.120480080C>T |
g.120840026C>T |
- |
- |
TSPAN12_000068 |
- |
PubMed: Salvo 2015 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +?/. |
4i |
c.149+3A>G |
r.spl? |
p.? |
- |
likely pathogenic (dominant) |
g.120480078T>C |
g.120840024T>C |
- |
- |
TSPAN12_000028 |
- |
PubMed: Poulter 2010 |
- |
- |
Germline |
yes |
0/500 control chromosomes |
- |
- |
- |
Jasmine Chen |
| +?/. |
- |
c.149+3A>G |
r.spl? |
p.? |
- |
VUS |
g.120480078T>C |
g.120840024T>C |
- |
- |
TSPAN12_000028 |
- |
PubMed: Carroll 2019 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Dong Sun |
| +?/. |
- |
c.149+3A>G |
r.spl? |
p.? |
- |
VUS |
g.120480078T>C |
g.120840024T>C |
- |
- |
TSPAN12_000028 |
- |
PubMed: Carroll 2019 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Dong Sun |
| +?/. |
- |
c.149+3A>G |
r.spl? |
p.? |
- |
VUS |
g.120480078T>C |
g.120840024T>C |
- |
- |
TSPAN12_000028 |
- |
PubMed: Carroll 2019 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Dong Sun |
| +/. |
- |
c.150-1G>A |
r.spl |
p.? |
- |
pathogenic |
g.120478967C>T |
g.120838913C>T |
- |
- |
TSPAN12_000053 |
- |
PubMed: Tang 2017 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +?/. |
3i |
c.150-1G>A |
r.spl |
p.(?) |
- |
likely pathogenic |
g.120478967C>T |
g.120838913C>T |
TSPAN12 150-1G?>?A, IVS3 ds G-A ?1 |
- |
TSPAN12_000053 |
- |
PubMed: Wang 2019 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
| +/. |
- |
c.171_172ins |
r.(?) |
p.(Thr58Tyrfs*59) |
- |
pathogenic |
g.? |
g.? |
c.171_172ins, p.(Thr58Tyrfs*59) |
- |
EZH2_000001 |
error in annotation: inserted nucleotides not written, heterozygous |
PubMed: Wang 2019 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
| ?/. |
- |
c.175T>G |
r.(?) |
p.(Tyr59Asp) |
- |
VUS |
g.120478941A>C |
g.120838887A>C |
- |
- |
TSPAN12_000009 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
| +/. |
4 |
c.177del |
r.(?) |
p.(Tyr59fs*67) |
- |
pathogenic (dominant) |
g.120478939del |
g.120838885del |
177delC |
- |
TSPAN12_000038 |
0/400 control chromosomes |
PubMed: Xu 2014 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Jasmine Chen |
| +?/. |
- |
c.193C>T |
r.(?) |
p.(Pro65Ser) |
- |
likely pathogenic |
g.120478923G>A |
g.120838869G>A |
c.193G>A, p.(Pro65Ser) |
- |
TSPAN12_000086 |
error in annotation: c.193G>A instead of C>T, heterozygous |
PubMed: Wang 2019 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
| ?/. |
- |
c.194C>T |
r.(?) |
p.(Pro65Leu) |
- |
VUS |
g.120478922G>A |
g.120838868G>A |
- |
- |
TSPAN12_000063 |
not in 354 control alleles |
PubMed: Seo 2015 |
- |
- |
Germline |
- |
2/51 patients |
- |
- |
- |
LOVD |
| ?/. |
4 |
c.194C>T |
r.(?) |
p.(Pro65Leu) |
ACMG |
VUS |
g.120478922G>A |
g.120838868G>A |
c.194C>T, p.P65L |
- |
TSPAN12_000063 |
- |
PubMed: Surl 2020 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
| ?/. |
4 |
c.194C>T |
r.(?) |
p.(Pro65Leu) |
ACMG |
VUS |
g.120478922G>A |
g.120838868G>A |
TPSAN12 c.194C>T, p.P65L |
- |
TSPAN12_000063 |
heterozygous |
PubMed: Li 2020 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
| +?/. |
5 |
c.194C>T |
r.(?) |
p.(Pro65Leu) |
- |
likely pathogenic (dominant) |
g.120478922G>A |
- |
c.194C>T |
- |
TSPAN12_000063 |
- |
PubMed: Liu-2020 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| ?/. |
- |
c.194C>T |
r.(?) |
p.(Pro65Leu) |
- |
VUS |
g.120478922G>A |
g.120838868G>A |
- |
- |
TSPAN12_000063 |
variant in unaffected father |
PubMed: Fan 2020 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
| +?/. |
4 |
c.212G>T |
r.(?) |
p.(Cys71Phe) |
- |
likely pathogenic |
g.120478904C>A |
g.120838850C>A |
TSPAN12 212G?>?T, Cys71Phe |
- |
TSPAN12_000085 |
- |
PubMed: Wang 2019 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
| +?/. |
- |
c.212G>T |
r.(?) |
p.(Cys71Phe) |
- |
likely pathogenic |
g.120478904C>A |
g.120838850C>A |
c.212C>A, p.(Cys71Phe) |
- |
TSPAN12_000085 |
error in annotation: c.212C>A instead of G>T, heterozygous |
PubMed: Wang 2019 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
| +/. |
4 |
c.212_218dup |
r.(?) |
p.(Phe73Leufs*46) |
- |
likely pathogenic (dominant) |
g.120478907_120478913dup |
g.120838853_120838859dup |
218_219insGCTCTTT |
- |
TSPAN12_000042 |
figure shows insertion of GCTGTTT not GCTCTTT; premature termination at codon 118 |
PubMed: Poulter 2010 |
- |
- |
Germline |
? |
- |
- |
- |
- |
Jasmine Chen |
| +/. |
4 |
c.212_218dup |
r.(?) |
p.(Phe73Leufs*46) |
- |
likely pathogenic (dominant) |
g.120478907_120478913dup |
g.120838853_120838859dup |
218_219insGCTCTTT |
- |
TSPAN12_000042 |
figure shows insertion of GCTGTTT not GCTCTTT; premature termination at codon 118 |
PubMed: Poulter 2010 |
- |
- |
Germline/De novo (untested) |
? |
- |
- |
- |
- |
Jasmine Chen |
| +/. |
- |
c.225_227del |
r.(?) |
p.(Ile76del) |
- |
pathogenic |
g.120478889_120478891del |
- |
7:120478888AATG>A ENST00000222747.3:c.225_227delCAT (Ile76del) |
- |
TSPAN12_000047 |
- |
PubMed: Carss 2017 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +?/. |
- |
c.225_227del |
r.(?) |
p.(Ile76del) |
- |
likely pathogenic |
g.120478891_120478893del |
g.120838837_120838839del |
TSPAN12 c.225_227delCAT, p.Ile76del |
- |
TSPAN12_000047 |
heterozygous |
PubMed: Turro 2020 |
- |
- |
Germline/De novo (untested) |
? |
- |
- |
- |
- |
LOVD |
| +/. |
- |
c.234del |
r.(?) |
p.(Met79CysfsTer2) |
- |
pathogenic |
g.120478885del |
g.120838831del |
231del |
- |
TSPAN12_000062 |
not in 624 control chromosomes |
PubMed: Sun 2015 |
- |
- |
Germline |
- |
1/596 chromosomes |
- |
- |
- |
LOVD |
| +/. |
- |
c.234del |
r.(?) |
p.(Met79Cysfs*2) |
- |
pathogenic |
g.120478885del |
g.120838831del |
c.234del, p.(Met79Cysfs*2) |
- |
TSPAN12_000062 |
heterozygous |
PubMed: Wang 2019 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
| +?/. |
- |
c.253A>C |
r.(?) |
p.(Thr85Pro) |
- |
likely pathogenic |
g.120478863T>G |
g.120838809T>G |
c.253A>C, p.(Thr85Pro) |
- |
TSPAN12_000084 |
error in annotation: c.253T>G instead of A>C, heterozygous |
PubMed: Wang 2019 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
| +?/. |
- |
c.253A>C |
r.(?) |
p.(Thr85Pro) |
- |
likely pathogenic |
g.120478863T>G |
g.120838809T>G |
c.253T>G, p.(Thr85Pro) |
- |
TSPAN12_000084 |
error in annotation: c.253T>G instead of A>C, heterozygous |
PubMed: Wang 2019 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
| +/. |
- |
c.254C>T |
r.(?) |
p.(Thr85Met) |
- |
pathogenic (dominant) |
g.120478862G>A |
g.120838808G>A |
- |
- |
TSPAN12_000037 |
0/400 control chromosomes |
PubMed: Xu 2014 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Jasmine Chen |
| ?/. |
4 |
c.274C>T |
r.(?) |
p.(Leu92Phe) |
- |
VUS |
g.120478842G>A |
g.120838788G>A |
C274T |
- |
TSPAN12_000069 |
- |
PubMed: Katagiri 2014 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| ?/. |
4_ |
c.285+1G>A |
r.spl |
p.? |
- |
VUS |
g.120478830C>T |
g.120838776C>T |
- |
- |
TSPAN12_000026 |
heterozygous |
PubMed: Poulter 2012 |
- |
- |
Germline/De novo (untested) |
? |
- |
- |
- |
- |
Jasmine Chen |
| +/. |
- |
c.285+1G>A |
r.spl |
p.? |
- |
pathogenic |
g.120478830C>T |
g.120838776C>T |
- |
- |
TSPAN12_000026 |
- |
PubMed: Tang 2017 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +?/. |
4i |
c.285+1G>A |
r.spl |
p.(?) |
- |
likely pathogenic |
g.120478830C>T |
g.120838776C>T |
TSPAN12 285+1G�>�A, IVS4 ds G-A +1 |
- |
TSPAN12_000026 |
- |
PubMed: Wang 2019 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
| +?/. |
2 |
c.286-2A>C |
r.spl |
p.(?) |
- |
likely pathogenic |
g.120455859T>G |
g.120815805T>G |
TSPAN12 286-2A?>?C, - |
- |
TSPAN12_000083 |
- |
PubMed: Wang 2019 |
- |
- |
Germline/De novo (untested) |
? |
- |
- |
- |
- |
LOVD |
| +?/. |
- |
c.286-2A>C |
r.spl |
p.(?) |
- |
likely pathogenic |
g.120455859T>G |
g.120815805T>G |
286-2A>C, - |
- |
TSPAN12_000083 |
- |
PubMed: Chen 2020 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
| +/. |
4i |
c.286-2A>G |
r.spl |
p.? |
- |
pathogenic |
g.120455859T>C |
- |
IVS4-2A>G |
- |
TSPAN12_000095 |
variant description assumes reference transcript intron numbering (not specified in paper) |
PubMed: Keser 2017 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
| +?/. |
5 |
c.286-1G>T |
r.spl |
p.(?) |
- |
likely pathogenic |
g.120455858C>A |
g.120815804C>A |
TSPAN12 286-1G?>?T, - |
- |
TSPAN12_000082 |
- |
PubMed: Wang 2019 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
| +?/. |
5 |
c.286-1G>T |
r.spl |
p.(?) |
- |
likely pathogenic |
g.120455858C>A |
g.120815804C>A |
TSPAN12 286-1G?>?T, - |
- |
TSPAN12_000082 |
- |
PubMed: Wang 2019 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
| +?/. |
5 |
c.302T>A |
r.(?) |
p.(Leu101His) |
- |
likely pathogenic |
g.120455841A>T |
g.120815787A>T |
- |
- |
TSPAN12_000025 |
- |
PubMed: Poulter 2010 |
- |
- |
Germline |
yes |
0/400 control chromosomes |
- |
- |
- |
Jasmine Chen |
| +/. |
- |
c.308T>C |
r.(?) |
p.(Ile103Thr) |
- |
pathogenic |
g.120455835A>G |
g.120815781A>G |
- |
- |
TSPAN12_000067 |
- |
PubMed: Salvo 2015 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +/. |
- |
c.313T>C |
r.(?) |
p.(Cys105Arg) |
- |
likely pathogenic |
g.120455830A>G |
g.120815776A>G |
- |
- |
TSPAN12_000024 |
- |
PubMed: Yang 2011 |
- |
- |
Germline/De novo (untested) |
? |
- |
- |
- |
- |
Jasmine Chen |
| +/. |
- |
c.315T>A |
r.(?) |
p.(Cys105Ter) |
- |
pathogenic |
g.120455828A>T |
g.120815774A>T |
- |
- |
TSPAN12_000008 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
| +/. |
5 |
c.334G>A |
r.(?) |
p.(Val112Ile) |
- |
pathogenic |
g.120455809C>T |
g.120815755C>T |
- |
- |
TSPAN12_000060 |
- |
PubMed: Musada 2016 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +/. |
6 |
c.345T>G |
r.(?) |
p.(Tyr115*) |
- |
pathogenic |
g.120455798A>C |
- |
c.345T>G |
- |
TSPAN12_000092 |
- |
PubMed: Rao 2017 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +/. |
6 |
c.345T>G |
r.(?) |
p.(Tyr115*) |
- |
pathogenic |
g.120455798A>C |
- |
c.345T>G |
- |
TSPAN12_000092 |
- |
PubMed: Rao 2017 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| ?/. |
5 |
c.346G>A |
r.(?) |
p.(Glu116Lys) |
- |
VUS |
g.120455797C>T |
g.120815743C>T |
954G>A |
- |
TSPAN12_000094 |
- |
PubMed: Zhang 2019 |
- |
- |
Germline/De novo (untested) |
? |
1/29 |
- |
- |
- |
Jasmine Chen |
| +?/. |
- |
c.352G>T |
r.(?) |
p.(Glu118*) |
- |
likely pathogenic |
g.120455791C>A |
g.120815737C>A |
352G>T, Glu118X |
- |
TSPAN12_000090 |
- |
PubMed: Chen 2020 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
| +/. |
- |
c.360+4A>G |
r.spl? |
p.(?) |
- |
pathogenic |
g.120455779T>C |
g.120815725T>C |
c.360+4T>C, p.? |
- |
TSPAN12_000081 |
error in annotation: c.360+4T>C instead of A>G, heterozygous |
PubMed: Wang 2019 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
| -/. |
- |
c.361-34C>T |
r.(=) |
p.(=) |
- |
benign |
g.120450658G>A |
g.120810604G>A |
- |
- |
TSPAN12_000007 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
| -?/. |
- |
c.361-15_361-9del |
r.(=) |
p.(=) |
- |
likely benign |
g.120450637_120450643del |
g.120810583_120810589del |
TSPAN12(NM_012338.3):c.361-15_361-9delGCTTTTT |
- |
TSPAN12_000002 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
| +/. |
5i |
c.361-5_361-1del |
r.spl |
p.? |
- |
likely pathogenic |
g.120450625_120450629del |
g.120810571_120810575del |
361-1_361-5delACCAG |
- |
TSPAN12_000023 |
mutation removes splice acceptor site including consensus AG, possible outcomes of mutation: deletion of exon 6, retention of intron 5, activation of cryptic splice site |
PubMed: Poulter 2010 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Jasmine Chen |
| +?/. |
- |
c.361-2A>G |
r.spl |
p.(?) |
- |
likely pathogenic |
g.120450626T>C |
g.120810572T>C |
c.361-2A-->G; p.? |
- |
TSPAN12_000071 |
no Sanger sequencing; homozygous |
PubMed: Patel 2019 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
| -?/. |
- |
c.367G>C |
r.(?) |
p.(Val123Leu) |
- |
likely benign |
g.120450618C>G |
g.120810564C>G |
TSPAN12(NM_012338.3):c.367G>C (p.V123L), TSPAN12(NM_012338.4):c.367G>C (p.V123L) |
- |
TSPAN12_000017 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
| -?/. |
- |
c.367G>C |
r.(?) |
p.(Val123Leu) |
- |
likely benign |
g.120450618C>G |
- |
TSPAN12(NM_012338.3):c.367G>C (p.V123L), TSPAN12(NM_012338.4):c.367G>C (p.V123L) |
- |
TSPAN12_000017 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_AMC |
| +/. |
- |
c.375G>A |
r.(?) |
p.(Trp125*) |
- |
pathogenic |
g.120450610C>T |
g.120810556C>T |
c.375C>T, p.(Trp125*) |
- |
TSPAN12_000080 |
error in annotation: c.375C>T instead of G>A, heterozygous |
PubMed: Wang 2019 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
| +/. |
6 |
c.413A>G |
r.(?) |
p.(Tyr138Cys) |
- |
pathogenic |
g.120450572T>C |
g.120810518T>C |
- |
- |
TSPAN12_000022 |
mild phenotype, heterozygous |
PubMed: Poulter 2012 |
- |
- |
Germline |
yes |
0/500 control chromosomes |
- |
- |
- |
Jasmine Chen |
| +/. |
6 |
c.413A>G |
r.(?) |
p.(Tyr138Cys) |
- |
pathogenic (recessive) |
g.120450572T>C |
g.120810518T>C |
- |
- |
TSPAN12_000022 |
- |
PubMed: Poulter 2012 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Jasmine Chen |
| +/. |
- |
c.419T>A |
r.(?) |
p.(Leu140*) |
- |
pathogenic |
g.120450566A>T |
g.120810512A>T |
- |
- |
TSPAN12_000015 |
- |
PubMed: Kondo 2011 |
- |
- |
De novo |
? |
0/380 control alleles |
- |
- |
- |
Jasmine Chen |
| +/. |
- |
c.419T>A |
r.(?) |
p.(Leu140*) |
- |
pathogenic (dominant) |
g.120450566A>T |
g.120810512A>T |
- |
- |
TSPAN12_000015 |
- |
PubMed: Kondo 2011 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Jasmine Chen |
| +/. |
6 |
c.419T>A |
r.(?) |
p.(Leu140*) |
- |
likely pathogenic (dominant) |
g.120450566A>T |
g.120810512A>T |
- |
- |
TSPAN12_000015 |
truncated protein (305 to 139 aa) |
PubMed: Poulter 2010 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Jasmine Chen |
| +/. |
- |
c.423delT |
r.(?) |
p.(Arg142Aspfs*21) |
- |
pathogenic |
g.120450562del |
g.120810508del |
423del T |
- |
TSPAN12_000058 |
- |
PubMed: Keser 2017 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
| +/. |
7 |
c.434G>A |
r.(?) |
p.(Trp145*) |
- |
pathogenic |
g.120450551C>T |
- |
c.434G>A:Trp145X |
- |
TSPAN12_000093 |
- |
PubMed: Chan 2016 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +/. |
- |
c.438dup |
r.(?) |
p.(Thr147Tyrfs*12) |
- |
pathogenic |
g.120450548dup |
g.120810494dup |
438_439insT |
- |
TSPAN12_000052 |
- |
PubMed: Tang 2017 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +/. |
- |
c.440C>A |
r.(?) |
p.(Thr147Asn) |
- |
pathogenic |
g.120450545G>T |
g.120810491G>T |
- |
- |
TSPAN12_000066 |
- |
PubMed: Salvo 2015 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +?/. |
- |
c.449G>C |
r.(?) |
p.(Trp150Ser) |
- |
likely pathogenic |
g.120450536C>G |
g.120810482C>G |
53A>G, Asn18Ser |
- |
TSPAN12_000079 |
- |
PubMed: Chen 2019 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
| ?/. |
- |
c.449G>C |
r.(?) |
p.(Trp150Ser) |
- |
VUS |
g.120450536C>G |
g.120810482C>G |
c.449C>G, p.(Trp150Ser) |
- |
TSPAN12_000079 |
error in annotation: c.449C>G instead of G>C, heterozygous |
PubMed: Wang 2019 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
| +?/. |
- |
c.461A>C |
r.(?) |
p.(Gln154Pro) |
- |
likely pathogenic |
g.120450524T>G |
g.120810470T>G |
716T>C, Leu239Pro |
- |
TSPAN12_000078 |
- |
PubMed: Chen 2019 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
| +?/. |
- |
c.461A>C |
r.(?) |
p.(Gln154Pro) |
- |
likely pathogenic |
g.120450524T>G |
g.120810470T>G |
716T>C, Leu239Pro |
- |
TSPAN12_000078 |
- |
PubMed: Chen 2019 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
| +?/. |
- |
c.461A>C |
r.(?) |
p.(Gln154Pro) |
- |
likely pathogenic |
g.120450524T>G |
g.120810470T>G |
c.461T>G, p.(Gln154Pro) |
- |
TSPAN12_000078 |
error in annotation: c.461T>G instead of A>C, heterozygous |
PubMed: Wang 2019 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
| +/. |
- |
c.464G>C |
r.(?) |
p.(Arg155Thr) |
- |
pathogenic |
g.120450521C>G |
g.120810467C>G |
- |
- |
TSPAN12_000051 |
- |
PubMed: Tang 2017 |
- |
rs768053082 |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +/. |
- |
c.469-1G>A |
r.spl |
p.? |
- |
pathogenic |
g.120446747C>T |
g.120806693C>T |
- |
- |
TSPAN12_000050 |
- |
PubMed: Tang 2017 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +?/. |
6i |
c.469-1G>A |
r.spl |
p.(?) |
- |
likely pathogenic |
g.120446747C>T |
g.120806693C>T |
TSPAN12 469-1G?>?A, IVS6 ds G-A ?1 |
- |
TSPAN12_000050 |
- |
PubMed: Wang 2019 |
- |
- |
Germline/De novo (untested) |
? |
- |
- |
- |
- |
LOVD |
| +?/. |
7 |
c.476G>A |
r.(?) |
p.(Cys159Tyr) |
- |
likely pathogenic |
g.120446739C>T |
g.120806685C>T |
TSPAN12 476G?>?A, Cys159Tyr |
- |
TSPAN12_000077 |
- |
PubMed: Wang 2019 |
- |
- |
Germline/De novo (untested) |
? |
- |
- |
- |
- |
LOVD |
| +?/. |
- |
c.476G>A |
r.(?) |
p.(Cys159Tyr) |
- |
likely pathogenic |
g.120446739C>T |
g.120806685C>T |
c.476C>T, p.(Cys159Tyr) |
- |
TSPAN12_000077 |
error in annotation: c.476C>T instead of G>A, heterozygous |
PubMed: Wang 2019 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
| +/. |
7 |
c.479G>A |
r.(?) |
p.(Cys160Tyr) |
- |
pathogenic |
g.120446736C>T |
g.120806682C>T |
- |
- |
TSPAN12_000059 |
- |
PubMed: Musada 2016 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +/. |
7 |
c.479G>A |
r.(?) |
p.(Cys160Tyr) |
- |
pathogenic |
g.120446736C>T |
g.120806682C>T |
- |
- |
TSPAN12_000059 |
- |
PubMed: Musada 2016 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +/. |
7 |
c.479G>A |
r.(?) |
p.(Cys160Tyr) |
- |
pathogenic |
g.120446736C>T |
g.120806682C>T |
- |
- |
TSPAN12_000059 |
- |
PubMed: Musada 2016 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +/. |
7 |
c.479G>A |
r.(?) |
p.(Cys160Tyr) |
- |
pathogenic |
g.120446736C>T |
g.120806682C>T |
- |
- |
TSPAN12_000059 |
- |
PubMed: Musada 2016 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |