Legend
Please note that a short description of a certain column can be displayed when you move your mouse cursor over the column's header and hold it still. Below, a more detailed description is shown per column.
Effect : The variant's effect on the function of the gene/protein, displayed in the format 'R/C'. R is the value reported by the source (publication, submitter) and this classification may vary between records. C is the value concluded by the curator. Note that in some database the curator uses Summary records to give details on the classification of the variant.Values used: '+' indicating the variant affects function, '+?' probably affects function, '-' does not affect function, '-?' probably does not affect function, '?' effect unknown, '.' effect was not classified.
Exon : number of exon/intron containing variant; 2 = exon 2, 12i = intron 12, 2i_7i = from intron 2 to intron 7, 8i_9 = intron 8/exon 9 boundary, _1 = 5' to exon 1, 18_ = 3' of exon 18, _1_18_ = encompassing the entire 18-exon gene
DNA change (cDNA) : description of variant at DNA level, based on a coding DNA reference sequence (following HGVS recommendations); e.g. c.123C>T, c.123_145del, c.123_126dup. For deletions/duplications extending beyond the reference transcript resp. {0}/{2} is used to replace del/dup. Extent of the deletion/duplication should be specified using the genomic description (g.). "-" indicates the variant described on genomic level does not affect the coding DNA reference sequence.
RNA change : description of variant at RNA level (following HGVS recommendations).
r.123c>u
r.? = unknown
r.(?) = RNA not analysed but probably transcribed copy of DNA variant
r.spl? = RNA not analysed but variant probably affects splicing
r.(spl?) = RNA not analysed but variant may affect splicing
r.0? = change expected to abolish transcription
Protein : description of variant at protein level (following HGVS recommendations).
p.(Arg345Pro) = change predicted from DNA (RNA not analysed)
p.Arg345Pro = change derived from RNA analysis
p.? = unknown effect
p.0? = probably no protein produced
Allele : On which allele is the variant located? Does not necessarily imply inheritance! 'Paternal' (confirmed or inferred), 'Maternal' (confirmed or inferred), 'Parent #1' or #2 for compound heterozygosity without having screened the parents, 'Unknown' for heterozygosity without having screened the parents, 'Both' for homozygozity.
Classification method : The method used for the clinical classification of this variant.
All options:
ACMG
ACGS
EAHAD-CFDB
ENIGMA
IARC
InSiGHT
kConFab
other
Clinical classification : Clinical classification of variant, preferably based on standardised criteria (e.g. ACMG), directed on the clinical consequences as published/submitted, indicated using an enriched system including inheritance: e.g. pathogenic, pathogenic (dominant), pathogenic (recessive), pathogenic (!), pathogenic (maternal), pathogenic (paternal). Standard inheritance is covered by dominant/recessive, imprinting by maternal/paternal. A '!' warns for exceptional circumstances to be explained in the 'Remarks' field (low penetrance, variants pathogenic in heterozygous state only, hypomorphic/hypermorphic variants, protective variants, etc.). Non-disease consequences (e.g. drug metabolism (pharmacogenetics), risk factor, blood group, tasting bitter) are indicated using additions to the benign classification; benign (dominant), benign (recessive), benign (!), etc. The value 'association' is used for variants associated with a phenotype and 'NA' for variants from in vitro/in silico records. NOTE: classification may differ from the opinion of the curator as given in a variant SUMMARY-record or the 'Functional effect concluded'). NOTE: pathogenic/likely pathogenic should go together with "variant (probably) affects function" In ClassFunctional.
All options:
pathogenic
pathogenic (dominant)
pathogenic (recessive)
pathogenic (!)
pathogenic (maternal)
pathogenic (paternal)
likely pathogenic
likely pathogenic (dominant)
likely pathogenic (recessive)
likely pathogenic (!)
likely pathogenic (maternal)
likely pathogenic (paternal)
VUS
VUS (!)
likely benign
likely benign (dominant)
likely benign (recessive)
likely benign (!)
likely benign (maternal)
likely benign (paternal)
benign
benign (dominant)
benign (recessive)
benign (!)
benign (maternal)
benign (paternal)
conflicting
association
NA
DNA change (genomic) (hg19) : HGVS description of variant at DNA level, based on the genomic (chromosomal) DNA reference sequence; e.g. g.12345678C>T, g.12345679del, g.12345678_12345890dup
DNA change (hg38) : HGVS description of variant at DNA level, based on the hg38 genomic (chromosomal) eference sequence; e.g. g.12345678C>T, g.12345679del, g.12345678_12345890dup
Published as : listed only when different from "DNA change"; variant as reported originally (e.g. 521delT). Variants seen in animal models, tested in vitro, predicted from RNA analysis, etc. are described between brackets like c.(456C>G)
ISCN : description of the variant according to ISCN nomenclature
DB-ID : database ID of variant, grouping multiple observations of the same variant together, starting with the HGNC gene symbol, followed by an underscore (_) and a six digit number (e.g. DMD_012345). _000000 is used for variants where DNA was not analysed (change predicted from RNA analysis), variants seen in animal models or variants not seen in humans but functionally tested in vitro
Variant remarks : remarks regarding variant described, e.g. germline mosaicism in mother, 345 kb deletion, muscle RNA analysed, not in 200 control chromosomes tested, on founder haplotype, etc.
Reference : publication describing the variant submitted, incl. links to OMIM, PubMed or other source, e.g. "den Dunnen ASHG2003 P2346"
ClinVar ID : ID of variant in ClinVar database
dbSNP ID : the dbSNP ID
Origin : Origin of variant/record: Germline = in all cells, De novo = in all cells, but not in either parent, Germline/De novo (untested) = in all cells, parents not tested (use only when De novo is likely, e.g. isolated/sporadic cases with dominant disease), Somatic = present in a subset of cells, but not in either parent, Uniparental disomy = from parental disomy (maternal or paternal), CLASSIFICATION record = submitter only sharing variant classification (note another report may share Individual data), SUMMARY record = master summary record from curator (may link to another database), In vitro (cloned) = data resulting from in vitro functional assays, animal model = data from animal model, Artefact = false positive variant call, DUPLICATE record = variant already described on another chromosome (e.g. unbalanced translocation, duplicating transposition, 2nd fusion transcript, etc.)
All options:
Germline
De novo
Germline/De novo (untested)
Somatic
Uniparental disomy
Uniparental disomy, maternal allele
Uniparental disomy, paternal allele
CLASSIFICATION record
SUMMARY record
In vitro (cloned)
In silico
animal model
Artefact
DUPLICATE record
Unknown
Not applicable
Segregation : Indicates whether the variant segregates with the phenotype (yes), does not segregate with the phenotype (no) or segregation is unknown (?)
All options:
? = unknown
yes = segregates with phenotype
no = does not segregate with phenotype
- = not applicable
Frequency : frequency in which the variant was found; e.g 5/760 chromosomes (in 5 of 760 chromosomes tested), 1/33 patients (in 1 of 33 patients analysed in study), 0.05 controls (in 5% of control cases tested)
Re-site : restriction enzyme recognition site created (+) or destroyed (-); e.g. BglII+;BamHI-
VIP : variant VIP-status was requested for matchmaking - need collaboration(s) to crack the case - please contact the submitter/curator.
NOTE: to get VIP status ask the curator.
Methylation : result of methylation test; GOM (gain of methylation), LOM (loss of methylation), 30% (30% methylated). NOTE: when several tests were done mention the method as well (e.g. MS-PCR 75%)
Effect
Exon
DNA change (cDNA)
RNA change
Protein
Classification method
Clinical classification
DNA change (genomic) (hg19)
DNA change (hg38)
Published as
ISCN
DB-ID
Variant remarks
Reference
ClinVar ID
dbSNP ID
Origin
Segregation
Frequency
Re-site
VIP
Methylation
Owner
-/.
1
c.-42T>C
r.(?)
p.(=)
-
benign
g.26126680T>C
g.25800189T>C
-
-
SEPN1_000102
also found in controls
-
-
rs12121707
Germline
-
-
-
-
-
Andreas Laner
-/.
1
c.-42T>C
r.(?)
p.(=)
-
benign
g.26126680T>C
g.25800189T>C
-
-
SEPN1_000102
-
from website {DBsub-Emory}
-
-
Unknown
-
-
-
-
-
Madhuri Hegde
-/.
-
c.-42T>C
r.(?)
p.(=)
-
benign
g.26126680T>C
g.25800189T>C
-
-
SEPN1_000102
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
c.-30_64del
r.(?)
p.?
-
pathogenic (recessive)
g.26126692_26126785del
g.25800201_25800294del
1-34_60del94
-
SEPN1_000074
-
-
-
-
Germline
-
-
-
-
-
Shu Yau
+/.
1
c.-30_64del
r.(?)
p.?
-
pathogenic (recessive)
g.26126692_26126785del
g.25800201_25800294del
1-34_60del94
-
SEPN1_000074
-
-
-
-
Germline
-
-
-
-
-
Shu Yau
+/.
1
c.-30_64del
r.(?)
p.0?
-
pathogenic
g.26126692_26126785del
g.25800201_25800294del
-
-
SEPN1_000074
-
-
-
-
Unknown
-
-
-
-
-
Tom Winder
-/.
1
c.-22_72del
r.(?)
p.0?
-
benign
g.26126700_26126793del
g.25800209_25800302del
1-25_69del
-
SEPN1_000050
-
PubMed: Maiti 2009
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
c.-22_72del
r.(?)
p.0?
-
likely pathogenic (recessive)
g.26126700_26126793del
g.25800209_25800302del
-25_69del
-
SEPN1_000050
combination of variants not reported
PubMed: Villar-Quiles 2020
-
-
Germline
-
1/132 cases
-
-
-
Johan den Dunnen
?/.
1
c.-14_-5dup
r.(?)
p.(=)
-
VUS
g.26126708_26126717dup
g.25800217_25800226dup
-14_-5dup10
-
SEPN1_000037
-
-
-
-
Germline
-
-
-
-
-
Tom Winder
?/.
1
c.-12C>G
r.(?)
p.(=)
-
VUS
g.26126710C>G
g.25800219C>G
-
-
SEPN1_000077
-
-
-
-
Germline
-
-
-
-
-
Shu Yau
?/.
1
c.-12C>G
r.(?)
p.(=)
-
VUS
g.26126710C>G
g.25800219C>G
-
-
SEPN1_000077
-
-
-
-
Germline
-
-
-
-
-
Shu Yau
+/.
1
c.-11_81del
r.(?)
p.0?
-
pathogenic (recessive)
g.26126711_26126802del
g.25800220_25800311del
-19_73del
-
SEPN1_000001
-
PubMed: Clarke 2006
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
c.-11_81del
r.(?)
p.0?
-
pathogenic (recessive)
g.26126711_26126802del
g.25800220_25800311del
-19_73del
-
SEPN1_000001
linked to 1p36
PubMed: Ferreiro 2004
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
c.-11_81del
r.(?)
p.0?
-
pathogenic (recessive)
g.26126711_26126802del
g.25800220_25800311del
-11_81del92
-
SEPN1_000001
-
-
-
-
Germline
-
-
-
-
-
Tom Winder
+/.
1
c.-11_81del
r.(?)
p.0?
-
pathogenic
g.26126711_26126802del
g.25800220_25800311del
1-11_81del92
-
SEPN1_000001
-
-
-
-
Germline
-
-
-
-
-
Shu Yau
+?/.
1
c.-11_81del
r.(?)
p.0?
-
likely pathogenic (recessive)
g.26126711_26126802del
g.25800220_25800311del
-19_73del
-
SEPN1_000001
combination of variants not reported
PubMed: Villar-Quiles 2020
-
-
Germline
-
11/132 cases
-
-
-
Johan den Dunnen
+?/.
-
c.-11_81del
r.(?)
p.?
ACMG
pathogenic (recessive)
g.26126711_26126802del
g.25800220_25800311del
-
-
SEPN1_000001
-
PubMed: Lin 2023 , Journal: Lin 2023
-
-
Germline
?
-
-
-
-
Barbara Vona
+?/.
1
c.-10_135del
r.(?)
p.0?
-
likely pathogenic
g.26126712_26126856del
g.25800221_25800365del
-
-
SEPN1_000101
deletion of the terminal 10 nucleotides of the 5' UTR and the first 135 nucleotides of exon 1
-
-
-
Germline
-
-
-
-
-
Tom Winder
-/-
1_13
c.=
r.=
p.=
-
benign
g.?
-
-
-
SEPN1_000000
no variants 2nd chromosome
-
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/-
1_13
c.=
r.=
p.=
-
benign
g.?
-
-
-
SEPN1_000000
no variants 2nd chromosome
-
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
c.1A>G
r.(?)
p.0?
-
pathogenic (recessive)
g.26126722A>G
g.25800231A>G
-
-
SEPN1_000002
-
PubMed: Clarke 2006
-
rs121908184
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
c.1A>G
r.(?)
p.0?
-
pathogenic (recessive)
g.26126722A>G
g.25800231A>G
-
-
SEPN1_000002
mRNA level near normal
PubMed: Ferreiro 2002 , OMIM:var0003 , PubMed: Allamand 2006
-
rs121908184
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
c.1A>G
r.(?)
p.0?
-
pathogenic (recessive)
g.26126722A>G
g.25800231A>G
-
-
SEPN1_000002
-
PubMed: Ferreiro 2002 , OMIM:var0003 , PubMed: Allamand 2006
-
rs121908184
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
c.1A>G
r.(?)
p.0?
-
pathogenic (recessive)
g.26126722A>G
g.25800231A>G
-
-
SEPN1_000002
-
PubMed: Ferreiro 2002 , OMIM:var0003 , PubMed: Allamand 2006
-
rs121908184
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
c.1A>G
r.(?)
p.0?
-
pathogenic (recessive)
g.26126722A>G
g.25800231A>G
-
-
SEPN1_000002
-
PubMed: Ferreiro 2002 , OMIM:var0003 , PubMed: Allamand 2006
-
rs121908184
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
c.1A>G
r.(?)
p.0?
-
pathogenic
g.26126722A>G
g.25800231A>G
-
-
SEPN1_000002
-
-
-
rs121908184
Germline
-
-
-
-
-
Shu Yau
+/.
1
c.1A>G
r.(?)
p.0?
-
pathogenic
g.26126722A>G
g.25800231A>G
-
-
SEPN1_000002
-
-
-
rs121908184
Germline
-
-
-
-
-
Tom Winder
-/.
1
c.1A>G
r.(?)
p.0?
-
benign
g.26126722A>G
g.25800231A>G
-
-
SEPN1_000002
-
PubMed: Maiti 2009
-
rs121908184
Germline
-
-
-
-
-
Johan den Dunnen
-/.
1
c.1A>G
r.(?)
p.0?
-
benign
g.26126722A>G
g.25800231A>G
-
-
SEPN1_000002
-
PubMed: Maiti 2009
-
rs121908184
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
c.1A>G
r.(?)
p.0?
-
likely pathogenic
g.26126722A>G
g.25800231A>G
-
-
SEPN1_000002
-
-
-
rs121908184
Germline
-
-
-
-
-
Tom Winder
+/.
1
c.1A>G
r.(?)
p.0?
-
pathogenic
g.26126722A>G
g.25800231A>G
(Met1Val)
-
SEPN1_000002
-
-
-
rs121908184
Germline
-
-
-
-
-
Tom Winder
+/.
1
c.1A>G
r.(?)
p.(0?)
-
pathogenic
g.26126722A>G
g.25800231A>G
Met1Val
-
SEPN1_000002
-
-
-
rs121908184
Germline
-
-
-
-
-
Wolfram Kress
+/.
1
c.1A>G
r.(?)
p.(Met1Val)
-
pathogenic
g.26126722A>G
g.25800231A>G
M1V
-
SEPN1_000002
-
-
-
rs121908184
Germline
-
-
-
-
-
Lab Müller-Reible
+/.
1
c.1A>G
r.(?)
p.(Met1Val)
-
pathogenic
g.26126722A>G
g.25800231A>G
M1V
-
SEPN1_000002
-
-
-
rs121908184
Germline
-
-
-
-
-
Lab Müller-Reible
+/.
1
c.1A>G
r.(?)
p.0?
-
pathogenic (recessive)
g.26126722A>G
g.25800231A>G
(Met1Val)
-
SEPN1_000002
-
-
-
rs121908184
Germline
-
-
-
-
-
Tom Winder
+/.
1
c.1A>G
r.(?)
p.0?
-
pathogenic
g.26126722A>G
g.25800231A>G
-
-
SEPN1_000002
-
-
-
rs121908184
Germline
-
-
-
-
-
Shu Yau
+/.
1
c.1A>G
r.(?)
p.0?
-
pathogenic
g.26126722A>G
g.25800231A>G
-
-
SEPN1_000002
-
-
-
rs121908184
Germline
-
-
-
-
-
Shu Yau
+/.
-
c.1A>G
r.(?)
p.(Met1?)
-
pathogenic
g.26126722A>G
g.25800231A>G
-
-
SEPN1_000002
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/.
1
c.1A>G
r.(?)
p.0?
-
likely pathogenic (recessive)
g.26126722A>G
g.25800231A>G
-
-
SEPN1_000002
combination of variants not reported
PubMed: Villar-Quiles 2020
-
rs121908184
Germline
-
20/132 cases
-
-
-
Johan den Dunnen
+/.
-
c.1A>G
r.(?)
p.(Met1?)
-
pathogenic
g.26126722A>G
-
-
-
SEPN1_000002
-
-
-
rs121908184
CLASSIFICATION record
-
-
-
-
-
MobiDetails
+/.
-
c.1A>G
r.(?)
p.(Met1?)
ACMG
pathogenic (recessive)
g.26126722A>G
g.25800231A>G
-
-
SEPN1_000002
-
PubMed: Bouman 2023
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
c.2dup
r.2dup
p.0?
-
pathogenic (recessive)
g.26126723dup
g.25800232dup
1_2insT
-
SEPN1_000004
mRNA level 0.6-0.7; potential secondary translation initiation
PubMed: Okamoto 2006
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
c.2dup
r.2dup
p.0?
-
pathogenic (recessive)
g.26126723dup
g.25800232dup
1_2insT
-
SEPN1_000004
mRNA level 0.6-0.7; potential secondary translation initiation
PubMed: Okamoto 2006
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
c.2dup
r.(?)
p.0?
-
pathogenic
g.26126723dup
g.25800232dup
1_2insT
-
SEPN1_000004
-
PubMed: Okamoto 2006
-
-
Germline
-
0/100
-
-
-
Johan den Dunnen
+/.
1
c.2T>G
r.(?)
p.0?
-
pathogenic (recessive)
g.26126723T>G
g.25800232T>G
(Met1Val)
-
SEPN1_000073
-
-
-
-
Germline
-
-
-
-
-
Tom Winder
+/.
1
c.2T>G
r.(?)
p.0?
-
pathogenic (recessive)
g.26126723T>G
g.25800232T>G
(Met1Val)
-
SEPN1_000073
-
-
-
-
Germline
-
-
-
-
-
Tom Winder
+?/.
1
c.8_12dup
r.(?)
p.(Gln8Argfs*60)
-
likely pathogenic (recessive)
g.26126729_26126733dup
g.25800238_25800242dup
-
-
SEPN1_000153
combination of variants not reported
PubMed: Villar-Quiles 2020
-
-
Germline
-
1/132 cases
-
-
-
Johan den Dunnen
+?/.
1
c.9_33del
r.(?)
p.(Ala4Profs*54)
-
likely pathogenic
g.26126730_26126754del
g.25800239_25800263del
9_33del25
-
SEPN1_000060
-
-
-
-
Germline
-
-
-
-
-
Tom Winder
+/.
1
c.9_33del
r.(?)
p.(Ala4fs*54)
-
pathogenic
g.26126730_26126754del
g.25800239_25800263del
9_33del24
-
SEPN1_000060
-
-
-
-
Germline
-
-
-
-
-
Shu Yau
+/.
1
c.9_33del
r.(?)
p.(Arg4Profs*54)
-
pathogenic
g.26126730_26126754del
g.25800239_25800263del
9_33del24
-
SEPN1_000060
-
-
-
-
Germline
-
-
-
-
-
Shu Yau
+/.
1
c.13_22dup
r.(?)
p.(Gln8Profs*78)
-
pathogenic (recessive)
g.26126734_26126743dup
g.25800243_25800252dup
22dup10
-
SEPN1_000003
-
PubMed: Moghadaszadeh 2001
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
c.13_22dup
r.(?)
p.(Gln8Profs*78)
-
pathogenic (recessive)
g.26126734_26126743dup
g.25800243_25800252dup
22dup10
-
SEPN1_000003
-
PubMed: Moghadaszadeh 2001
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
c.13_22dup
r.(?)
p.(Gln8Profs*78)
-
pathogenic (recessive)
g.26126734_26126743dup
g.25800243_25800252dup
22dup10
-
SEPN1_000003
-
PubMed: Ferreiro 2002 , PubMed: Allamand 2006
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
c.13_22dup
r.(?)
p.(Gln8Profs*78)
-
pathogenic
g.26126734_26126743dup
g.25800243_25800252dup
-
-
SEPN1_000003
-
-
-
-
Germline
-
-
-
-
-
Tom Winder
+/.
1
c.13_22dup
r.(?)
p.(Gln8Profs*78)
-
pathogenic
g.26126734_26126743dup
g.25800243_25800252dup
13_22dup10
-
SEPN1_000003
-
-
-
-
Germline
-
-
-
-
-
Shu Yau
+/.
1
c.13_22dup
r.(?)
p.(Gln8Profs*78)
-
pathogenic
g.26126734_26126743dup
g.25800243_25800252dup
13_22dup10
-
SEPN1_000003
-
-
-
-
Germline
-
-
-
-
-
Shu Yau
+/.
1
c.13_22dup
r.(?)
p.(Gln8Profs*78)
-
pathogenic (recessive)
g.26126734_26126743dup
g.25800243_25800252dup
12_21dup10
-
SEPN1_000003
-
PubMed: Schara 2008
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
c.13_22dup
r.(?)
p.(Gln8Profs*78)
-
pathogenic
g.26126734_26126743dup
g.25800243_25800252dup
-
-
SEPN1_000003
-
-
-
-
Germline
-
-
-
-
-
Lab Müller-Reible
+/.
1
c.13_22dup
r.(?)
p.(Gln8Profs*78)
-
pathogenic
g.26126734_26126743dup
g.25800243_25800252dup
-
-
SEPN1_000003
-
-
-
-
Germline
-
-
-
-
-
Lab Müller-Reible
+/.
1
c.13_22dup
r.(?)
p.(Gln8Profs*78)
-
pathogenic
g.26126734_26126743dup
g.25800243_25800252dup
-
-
SEPN1_000003
-
-
-
-
Germline
-
-
-
-
-
Lab Müller-Reible
+/.
1
c.13_22dup
r.(?)
p.(Gln8Profs*78)
-
pathogenic
g.26126734_26126743dup
g.25800243_25800252dup
-
-
SEPN1_000003
-
-
-
-
Germline
-
-
-
-
-
Lab Müller-Reible
+/.
1
c.13_22dup
r.(?)
p.(Gln8Profs*78)
-
pathogenic
g.26126734_26126743dup
g.25800243_25800252dup
-
-
SEPN1_000003
-
-
-
-
Germline
-
-
-
-
-
Lab Müller-Reible
+/.
1
c.13_22dup
r.(?)
p.(Gln8Profs*78)
-
pathogenic
g.26126734_26126743dup
g.25800243_25800252dup
-
-
SEPN1_000003
-
-
-
-
Germline
-
-
-
-
-
Lab Müller-Reible
+/.
1
c.13_22dup
r.(?)
p.(Gln8Profs*78)
-
pathogenic (recessive)
g.26126734_26126743dup
g.25800243_25800252dup
13_22dup10
-
SEPN1_000003
-
-
-
-
Germline
-
-
-
-
-
Shu Yau
+/.
1
c.13_22dup
r.(?)
p.(Gln8Profs*78)
-
pathogenic (recessive)
g.26126734_26126743dup
g.25800243_25800252dup
13_22dup10
-
SEPN1_000003
-
-
-
-
Germline
-
-
-
-
-
Shu Yau
+/.
1
c.13_22dup
r.(?)
p.(Gln8Profs*78)
-
pathogenic
g.26126734_26126743dup
g.25800243_25800252dup
13_22dupCGGCCGGGCC
-
SEPN1_000003
-
from website {DBsub-Emory}
-
-
Unknown
-
-
-
-
-
Madhuri Hegde
+/.
1
c.13_22dup
r.(?)
p.(Gln8Profs*78)
-
pathogenic
g.26126734_26126743dup
g.25800243_25800252dup
-
-
SEPN1_000003
-
-
-
-
Germline
-
-
-
-
-
Tom Winder
+/.
1
c.13_22dup
r.(?)
p.(Gln8Profs*78)
-
pathogenic
g.26126734_26126743dup
g.25800243_25800252dup
-
-
SEPN1_000003
-
-
-
-
Germline
-
-
-
-
-
Tom Winder
+?/.
1
c.13_22dup
r.(?)
p.(Gln8Profs*78)
-
likely pathogenic (recessive)
g.26126734_26126743dup
g.25800243_25800252dup
8_17dup
-
SEPN1_000003
combination of variants not reported
PubMed: Villar-Quiles 2020
-
rs970951421
Germline
-
12/132 cases
-
-
-
Johan den Dunnen
+/.
-
c.13_22dup
r.(?)
p.(Gln8fs)
ACMG
pathogenic (recessive)
g.26126734_26126743dup
g.25800243_25800252dup
-
-
SEPN1_000003
-
PubMed: Bouman 2023
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
c.23_32dup
r.(?)
p.(Pro12Thrfs*75)
-
pathogenic (recessive)
g.26126744_26126753dup
g.25800253_25800262dup
23_32dup10
-
SEPN1_000038
variant identical to 13_22dup10?
PubMed: Clarke 2006
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
c.42C>T
r.(?)
p.(=)
-
VUS
g.26126763C>T
g.25800272C>T
-
-
SEPN1_000036
-
-
-
-
Germline
-
-
-
-
-
Tom Winder
?/.
1
c.42C>T
r.(?)
p.(=)
-
VUS
g.26126763C>T
g.25800272C>T
-
-
SEPN1_000036
-
from website {DBsub-Emory}
-
-
Unknown
-
-
-
-
-
Madhuri Hegde
-/.
-
c.42C>T
r.(?)
p.?
-
benign
g.26126763C>T
-
-
-
SEPN1_000036
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
c.44_72dup
r.(?)
p.(Arg25Alafs*51)
-
pathogenic
g.26126765_26126793dup
g.25800274_25800302dup
44_72dup29
-
SEPN1_000059
-
-
-
-
Germline
-
-
-
-
-
Tom Winder
+?/.
1
c.44_72dup
r.(?)
p.(Arg25Alafs*51)
-
likely pathogenic
g.26126765_26126793dup
g.25800274_25800302dup
44_72dup29
-
SEPN1_000059
-
-
-
-
Germline
-
-
-
-
-
Tom Winder
+?/.
1
c.44_72dup
r.(?)
p.(Arg25Alafs*51)
-
likely pathogenic (recessive)
g.26126765_26126793dup
g.25800274_25800302dup
-
-
SEPN1_000059
combination of variants not reported
PubMed: Villar-Quiles 2020
-
rs797044620
Germline
-
2/132 cases
-
-
-
Johan den Dunnen
+/.
1
c.56_83dup
r.(?)
p.(Arg29Alafs*63)
-
pathogenic
g.26126777_26126804dup
g.25800286_25800313dup
56_83dup28
-
SEPN1_000095
-
-
-
-
Germline
-
-
-
-
-
Shu Yau
+/.
1
c.60_79dup
r.60_79dup
p.Arg27Leufs*62
-
pathogenic (recessive)
g.26126781_26126800dup
g.25800290_25800309dup
-
-
SEPN1_000005
mRNA level 0.3; potential secondary translation initiation
PubMed: Okamoto 2006
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
c.60_79dup
r.60_79dup
p.Arg27Leufs*62
-
pathogenic (recessive)
g.26126781_26126800dup
g.25800290_25800309dup
-
-
SEPN1_000005
mRNA level 0.3; potential secondary translation initiation
PubMed: Okamoto 2006
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
c.60_79dup
r.?
p.Arg27Leufs*62
-
pathogenic
g.26126781_26126800dup
g.25800290_25800309dup
-
-
SEPN1_000005
-
PubMed: Okamoto 2006
-
-
Germline
-
0/100
-
-
-
Johan den Dunnen
+?/.
1
c.69_76dup
r.(?)
p.(Arg26Hisfs*43)
-
likely pathogenic (recessive)
g.26126790_26126797dup
g.25800299_25800306dup
66_73dup
-
SEPN1_000154
combination of variants not reported
PubMed: Villar-Quiles 2020
-
rs911937146
Germline
-
2/132 cases
-
-
-
Johan den Dunnen
+?/.
1
c.77_84dup
r.(?)
p.(Arg29Alafs*40)
-
likely pathogenic (recessive)
g.26126798_26126805dup
g.25800307_25800314dup
-
-
SEPN1_000155
combination of variants not reported
PubMed: Villar-Quiles 2020
-
-
Germline
-
2/132 cases
-
-
-
Johan den Dunnen
?/.
1
c.103G>C
r.(?)
p.(Gly35Arg)
-
VUS
g.26126824G>C
g.25800333G>C
-
-
SEPN1_000093
-
-
-
-
Germline
-
-
-
-
-
Shu Yau
?/.
1
c.103G>C
r.(?)
p.(Gly35Arg)
-
VUS
g.26126824G>C
g.25800333G>C
-
-
SEPN1_000093
-
-
-
-
Germline
-
-
-
-
-
Shu Yau
+?/.
1
c.125_136dup
r.(?)
p.(Ala42_Ala45dup)
-
likely pathogenic
g.26126846_26126857dup
g.25800355_25800366dup
-
-
SEPN1_000113
-
-
-
-
Germline
-
-
-
-
-
Tom Winder
+?/.
1
c.142del
r.(?)
p.(Val48Serfs*18)
-
likely pathogenic (recessive)
g.26126863del
g.25800372del
-
-
SEPN1_000156
combination of variants not reported
PubMed: Villar-Quiles 2020
-
-
Germline
-
1/132 cases
-
-
-
Johan den Dunnen
+?/.
1
c.163dup
r.(?)
p.(Ala55Glyfs*28)
-
likely pathogenic
g.26126884dup
g.25800393dup
163dupG
-
SEPN1_000100
-
-
-
-
Germline
-
-
-
-
-
Tom Winder
+/.
1
c.166C>T
r.(?)
p.(Gln56*)
-
pathogenic (recessive)
g.26126887C>T
g.25800396C>T
-
-
SEPN1_000080
-
-
-
-
Germline
-
-
-
-
-
Shu Yau
+/.
1
c.166C>T
r.(?)
p.(Gln56*)
-
pathogenic (recessive)
g.26126887C>T
g.25800396C>T
-
-
SEPN1_000080
-
-
-
-
Germline
-
-
-
-
-
Shu Yau
-/.
-
c.183+116G>A
r.(=)
p.(=)
-
benign
g.26127020G>A
-
SELENON(NM_020451.3):c.183+116G>A
-
SEPN1_000186
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+?/.
-
c.234del
r.(?)
p.?
-
likely pathogenic (recessive)
g.26127584del
g.25801093del
233delC
-
SEPN1_000144
no variant 2nd allele
PubMed: Park 2017
-
-
Germline
-
1/209 cases
-
-
-
Johan den Dunnen
+?/.
2
c.249_250dup
r.(?)
p.(Asp84Glyfs*17)
-
likely pathogenic
g.26127599_26127600dup
g.25801108_25801109dup
249_250dupGG
-
SEPN1_000056
-
-
-
-
Germline
-
-
-
-
-
Tom Winder
+?/.
2
c.249_250dup
r.(?)
p.(Asp84Glyfs*17)
-
likely pathogenic
g.26127599_26127600dup
g.25801108_25801109dup
249_250dupGG
-
SEPN1_000056
-
-
-
-
Germline
-
-
-
-
-
Tom Winder
+?/.
2
c.249_250dup
r.(?)
p.(Asp84Glyfs*17)
-
likely pathogenic (recessive)
g.26127599_26127600dup
g.25801108_25801109dup
-
-
SEPN1_000056
combination of variants not reported
PubMed: Villar-Quiles 2020
-
-
Germline
-
1/132 cases
-
-
-
Johan den Dunnen
+/.
2
c.249_250dup
r.(?)
p.(Asp84GlyfsTer17)
-
pathogenic
g.26127599_26127600dup
g.25801108_25801109dup
c.249_250dupGG
-
SEPN1_000056
-
PubMed: Ganapathy 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
2
c.253A>G
r.(?)
p.(Met85Val)
-
VUS
g.26127603A>G
g.25801112A>G
-
-
SEPN1_000027
-
-
-
-
Germline
-
-
-
-
-
Tom Winder
+?/.
-
c.272_273insCTGAT
r.(?)
p.(Glu91Aspfs*2)
-
likely pathogenic
g.26127622_26127623insCTGAT
g.25801131_25801132insCTGAT
-
-
SEPN1_000151
combination of variants not reported
PubMed: Topf 2020
-
-
Germline
-
2/1001 cases
-
-
-
Johan den Dunnen
+?/.
2
c.279dup
r.(?)
p.(Pro94Thrfs*4)
-
likely pathogenic (recessive)
g.26127629dup
g.25801138dup
277dupA
-
SEPN1_000157
combination of variants not reported
PubMed: Villar-Quiles 2020
-
-
Germline
-
1/132 cases
-
-
-
Johan den Dunnen
+?/.
-
c.301G>C
r.spl?
p.(?,Gly101Arg)
ACMG
likely pathogenic (recessive)
g.26127651G>C
g.25801160G>C
-
-
SEPN1_000197
-
PubMed: Bouman 2023
-
-
Germline
-
-
-
-
-
Johan den Dunnen