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.-403C>G
r.(?)
p.(=)
-
likely benign
g.127864922G>C
g.127107346G>C
-
-
BIN1_000048
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/.
-
c.-402G>C
r.(?)
p.(=)
-
likely benign
g.127864921C>G
g.127107345C>G
-
-
BIN1_000047
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/.
1
c.-27C>T
r.(?)
p.(=)
-
benign
g.127864546G>A
g.127106970G>A
1-27C>T
-
BIN1_000017
-
PubMed: Nicot 2007
-
-
Germline
-
0.07
-
-
-
Johan den Dunnen
+/.
1
c.53T>A
r.(?)
p.(Val18Glu)
-
pathogenic
g.127864467A>T
g.127106891A>T
-
-
BIN1_000039
-
PubMed: Kouwenberg 2017
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
+?/.
-
c.53T>A
r.(?)
p.(Val18Glu)
-
likely pathogenic
g.127864467A>T
g.127106891A>T
-
-
BIN1_000039
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
2
c.105G>T
r.(?)
p.(Lys35Asn)
-
pathogenic
g.127834262C>A
g.127076686C>A
-
-
BIN1_000001
not in 560 control chromosomes; normal DNM2, MTM1
PubMed: Nicot 2007 , OMIM:var0001
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
c.105G>T
r.(?)
p.(Lys35Asn)
-
pathogenic
g.127834262C>A
g.127076686C>A
-
-
BIN1_000001
not in 560 control chromosomes; normal DNM2, MTM1
PubMed: Nicot 2007 , OMIM:var0001
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
c.105G>T
r.(?)
p.(Lys35Asn)
-
pathogenic
g.127834262C>A
g.127076686C>A
-
-
BIN1_000001
not in 560 control chromosomes; normal DNM2, MTM1
PubMed: Nicot 2007 , OMIM:var0001
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
c.105G>T
r.(?)
p.Lys35Asn
-
NA
g.127834262C>A
g.127076686C>A
-
-
BIN1_000001
expression cloning COS-1 cells abolished membrane tubulation
PubMed: Nicot 2007 , OMIM:var0001
-
-
In vitro (cloned)
-
-
-
-
-
Johan den Dunnen
+?/.
2i_3i
c.(165+1_166-1)_(220+1_221-1)del
r.?
p.?
ACMG
likely pathogenic
g.(127828224_127828337)_(127828393_127834201)del
g.(127070648_127070761)_(127070817_127076625)del
del ex3, Chr2:127828335-127828394del
-
BIN1_000065
ACMG 1A, 2B, 2E, 3A, 4L
PubMed: Molaei 2025
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
-
c.175A>G
r.(?)
p.(Thr59Ala)
-
likely benign
g.127828383T>C
g.127070807T>C
-
-
BIN1_000046
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/.
4i
c.315+70C>T
r.(?)
p.(=)
-
benign
g.127828059G>A
g.127070483G>A
318+70C>T?
-
BIN1_000018
-
PubMed: Nicot 2007
-
-
Germline
-
0.02
-
-
-
Johan den Dunnen
-/.
4i
c.316-19G>A
r.(?)
p.(=)
-
benign
g.127827685C>T
g.127070109C>T
319-19C>A
-
BIN1_000019
-
PubMed: Nicot 2007
-
-
Germline
-
0.01
-
-
-
Johan den Dunnen
?/.
4i
c.316-3C>G
r.(=)
p.(=)
-
VUS
g.127827669G>C
g.127070093G>C
-
-
BIN1_000036
-
-
-
rs113742431
Germline
-
?
-
-
-
Johan den Dunnen
-/.
5i
c.412-25T>C
r.(?)
p.(=)
-
benign
g.127826632A>G
g.127069056A>G
-
-
BIN1_000020
-
PubMed: Nicot 2007
-
-
Germline
-
0.19
-
-
-
Johan den Dunnen
?/.
5i
c.412-25T>C
r.(?)
p.(=)
-
VUS
g.127826632A>G
g.127069056A>G
-
-
BIN1_000020
-
-
-
rs67327804
Germline
-
-
-
-
-
Johan den Dunnen
?/.
-
c.434G>C
r.(?)
p.(Arg145Pro)
-
VUS
g.127826585C>G
-
-
-
BIN1_000056
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
6
c.451G>A
r.451g>a
p.Asp151Asn
-
pathogenic
g.127826568C>T
g.127068992C>T
-
-
BIN1_000002
not in 560 control chromosomes; normal DNM2, MTM1
PubMed: Nicot 2007 , PubMed: Toussaint 2010 , OMIM:var0002
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
6
c.451G>A
r.(?)
p.Asp151Asn
-
NA
g.127826568C>T
g.127068992C>T
-
-
BIN1_000002
expression cloning COS-1 cells abolished membrane tubulation
PubMed: Nicot 2007 , OMIM:var0002
-
-
In vitro (cloned)
-
-
-
-
-
Johan den Dunnen
+/.
6
c.461G>A
r.461g>a
p.Arg154Gln
-
pathogenic
g.127826558C>T
g.127068982C>T
-
-
BIN1_000035
not in 560 control chromosomes
PubMed: Claeys 2010 , PubMed: Toussaint 2010
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
6
c.486T>C
r.(?)
p.(=)
-
benign
g.127826533A>G
g.127068957A>G
487T>C?
-
BIN1_000021
-
PubMed: Nicot 2007
-
rs1060743
Germline
-
0.27
-
-
-
Johan den Dunnen
-/.
-
c.486T>C
r.(?)
p.(Thr162=)
-
benign
g.127826533A>G
g.127068957A>G
-
-
BIN1_000021
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/.
6i
c.520-110G>A
r.(?)
p.(=)
-
benign
g.127825941C>T
g.127068365C>T
-
-
BIN1_000022
-
PubMed: Nicot 2007
-
-
Germline
-
0.01
-
-
-
Johan den Dunnen
?/.
-
c.544G>A
r.(?)
p.(Ala182Thr)
-
VUS
g.127825807C>T
-
BIN1(NM_139343.2):c.544G>A (p.(Ala182Thr))
-
BIN1_000060
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
-
c.696C>A
r.(?)
p.(Asn232Lys)
-
VUS
g.127821511G>T
-
BIN1(NM_001320632.1):c.603C>A (p.(Asn201Lys))
-
BIN1_000053
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-/.
8i
c.698+10A>G
r.(?)
p.(=)
-
benign
g.127821499T>C
g.127063923T>C
699+10A>G
-
BIN1_000023
-
PubMed: Nicot 2007
-
rs72481904
Germline
-
0.36
-
-
-
Johan den Dunnen
-/.
-
c.698+10A>G
r.(=)
p.(=)
-
benign
g.127821499T>C
g.127063923T>C
BIN1(NM_139343.3):c.698+10A>G
-
BIN1_000023
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-/.
-
c.698+10A>G
r.(=)
p.(=)
-
benign
g.127821499T>C
g.127063923T>C
BIN1(NM_139343.3):c.698+10A>G
-
BIN1_000023
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/.
8i
c.698+56C>T
r.(?)
p.(=)
-
benign
g.127821453G>A
g.127063877G>A
699+56C>T?
-
BIN1_000024
-
PubMed: Nicot 2007
-
rs78935905
Germline
-
0.08
-
-
-
Johan den Dunnen
-/.
8i
c.698+57G>A
r.(?)
p.(=)
-
benign
g.127821452C>T
g.127063876C>T
699+57G>A?
-
BIN1_000025
-
PubMed: Nicot 2007
-
rs2071267
Germline
-
0.08
-
-
-
Johan den Dunnen
?/.
-
c.699-1G>C
r.spl?
p.?
-
VUS
g.127821223C>G
g.127063647C>G
BIN1(NM_139343.3):c.699-1G>C
-
BIN1_000044
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
?/.
-
c.701G>A
r.(?)
p.(Arg234His)
-
VUS
g.127821220C>T
g.127063644C>T
-
-
BIN1_000043
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/.
9
c.714C>T
r.(?)
p.(=)
-
benign
g.127821207G>A
g.127063631G>A
716C>T?
-
BIN1_000026
-
PubMed: Nicot 2007
-
rs1137845
Germline
-
0.06
-
-
-
Johan den Dunnen
-/.
-
c.714C>T
r.(?)
p.(Tyr238=)
-
benign
g.127821207G>A
g.127063631G>A
-
-
BIN1_000026
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/.
9i
c.775-4G>A
r.(?)
p.(=)
-
benign
g.127819777C>T
g.127062201C>T
775-4A>A?
-
BIN1_000027
-
PubMed: Nicot 2007
-
-
Germline
-
0.10
-
-
-
Johan den Dunnen
-/.
-
c.775-4G>A
r.spl?
p.?
-
benign
g.127819777C>T
g.127062201C>T
BIN1(NM_139343.3):c.775-4G>A
-
BIN1_000027
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
?/.
-
c.839C>T
r.(?)
p.(Thr280Met)
-
VUS
g.127819709G>A
-
BIN1(NM_139343.2):c.839C>T (p.(Thr280Met))
-
BIN1_000059
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
10i
c.857+4C>G
r.(?)
p.(=)
-
VUS
g.127819687G>C
g.127062111G>C
-
-
BIN1_000004
-
-
-
rs61748156
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
-
c.857+8C>T
r.(=)
p.(=)
-
likely benign
g.127819683G>A
-
BIN1(NM_139343.3):c.857+8C>T
-
BIN1_000057
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
-
c.858-1462G>A
r.(=)
p.(=)
-
likely benign
g.127818193C>T
g.127060617C>T
-
-
BIN1_000042
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/.
10i
c.858-12C>A
r.(?)
p.(=)
-
benign
g.127816743G>T
g.127059167G>T
856-12C>A
-
BIN1_000029
-
PubMed: Nicot 2007
-
rs6720741
Germline
-
0.18
-
-
-
Johan den Dunnen
-/.
-
c.858-12C>A
r.(=)
p.(=)
-
benign
g.127816743G>T
g.127059167G>T
BIN1(NM_139343.3):c.858-12C>A
-
BIN1_000029
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-/.
-
c.858-12C>A
r.(=)
p.(=)
-
benign
g.127816743G>T
g.127059167G>T
BIN1(NM_139343.3):c.858-12C>A
-
BIN1_000029
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/.
11
c.894G>A
r.(?)
p.(=)
-
benign
g.127816695C>T
g.127059119C>T
893G>A?
-
BIN1_000030
-
PubMed: Nicot 2007
-
rs78936238
Germline
-
0.10
-
-
-
Johan den Dunnen
-/.
-
c.894G>A
r.(?)
p.(Ser298=)
-
benign
g.127816695C>T
g.127059119C>T
-
-
BIN1_000030
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/.
11
c.957C>T
r.(?)
p.(=)
-
benign
g.127816632G>A
g.127059056G>A
956C>T?
-
BIN1_000006
-
PubMed: Nicot 2007
-
rs2276579
Germline
-
0.13
-
-
-
Johan den Dunnen
-/.
-
c.957C>T
r.(?)
p.(Ala319=)
-
benign
g.127816632G>A
g.127059056G>A
-
-
BIN1_000006
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
-
c.969_980del
r.(?)
p.(Pro324_Thr327del)
-
VUS
g.127816618_127816629del
g.127059042_127059053del
BIN1(NM_004305.3):c.921_932del (p.(Pro308_Thr311del))
-
BIN1_000051
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
11i
c.1002+51C>T
r.(?)
p.(=)
-
VUS
g.127816536G>A
g.127058960G>A
-
-
BIN1_000033
-
-
-
rs2276580
Germline
-
0.00-0.15
-
-
-
Johan den Dunnen
?/.
11i
c.1002+112T>C
r.(?)
p.(=)
-
VUS
g.127816475A>G
g.127058899A>G
-
-
BIN1_000031
-
-
-
rs2276581
Germline
-
0.00-0.17
-
-
-
Johan den Dunnen
?/.
11i
c.1002+163T>C
r.(?)
p.(=)
-
VUS
g.127816424A>G
g.127058848A>G
-
-
BIN1_000032
-
-
-
rs2276582
Germline
-
0.39-0.50
-
-
-
Johan den Dunnen
-/.
11i
c.1003-60C>T
r.(?)
p.(=)
-
benign
g.127815237G>A
g.127057661G>A
1004-61C>T?
-
BIN1_000009
-
PubMed: Nicot 2007
-
rs3754615
Germline
-
0.08
-
-
-
Johan den Dunnen
-/.
11i
c.1003-41G>A
r.(?)
p.(=)
-
benign
g.127815218C>T
g.127057642C>T
1004-41G>A?
-
BIN1_000008
-
PubMed: Nicot 2007
-
rs3754616
Germline
-
0.04
-
-
-
Johan den Dunnen
-/.
11i
c.1003-15G>A
r.(?)
p.(=)
-
benign
g.127815192C>T
g.127057616C>T
1004-15G>A?
-
BIN1_000007
-
PubMed: Nicot 2007
-
-
Germline
-
0.02
-
-
-
Johan den Dunnen
-?/.
-
c.1131+9C>T
r.(=)
p.(=)
-
likely benign
g.127815040G>A
-
BIN1(NM_139343.3):c.1131+9C>T
-
BIN1_000062
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-/.
12i
c.1132-91C>G
r.(?)
p.(=)
-
benign
g.127811679G>C
g.127054103G>C
1134-92C>G?
-
BIN1_000040
-
PubMed: Nicot 2007
-
-
Germline
-
0.06
-
-
-
Johan den Dunnen
-?/.
-
c.1132-7T>C
r.(=)
p.(=)
-
likely benign
g.127811595A>G
-
BIN1(NM_001320642.1):c.1051-7T>C
-
BIN1_000054
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-/.
13i
c.1239+50A>C
r.(?)
p.(=)
-
benign
g.127811431T>G
g.127053855T>G
-
-
BIN1_000010
-
PubMed: Nicot 2007
-
rs79410526
Germline
-
0.01
-
-
-
Johan den Dunnen
-/.
13i
c.1240-21C>T
r.(?)
p.(=)
-
benign
g.127811042G>A
g.127053466G>A
-
-
BIN1_000011
-
PubMed: Nicot 2007
-
rs76414720
Germline
-
0.03
-
-
-
Johan den Dunnen
-/.
-
c.1263+11C>T
r.(=)
p.(=)
-
benign
g.127810987G>A
g.127053411G>A
BIN1(NM_139343.3):c.1263+11C>T
-
BIN1_000041
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-/.
-
c.1362G>T
r.(?)
p.(=)
-
benign
g.127809840C>A
-
BIN1(NM_139343.3):c.1362G>T (p.(Gly454=))
-
BIN1_000061
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
16
c.1461C>T
r.(spl?)
p.(?)
-
VUS
g.127808730G>A
g.127051154G>A
-
-
BIN1_000034
-
-
-
rs34647988
Germline
-
0.01
-
-
-
Johan den Dunnen
-?/.
-
c.1462-3C>T
r.spl?
p.?
-
likely benign
g.127808491G>A
-
BIN1(NM_139343.3):c.1462-3C>T
-
BIN1_000064
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
-
c.1541G>A
r.(?)
p.(Arg514His)
-
VUS
g.127808409C>T
-
BIN1(NM_139343.3):c.1541G>A (p.(Arg514His))
-
BIN1_000063
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-/.
17i
c.1573-72C>T
r.(?)
p.(=)
-
benign
g.127808170G>A
g.127050594G>A
-
-
BIN1_000015
-
PubMed: Nicot 2007
-
rs7558001
Germline
-
0.21
-
-
-
Johan den Dunnen
-/.
17i
c.1573-63C>T
r.(?)
p.(=)
-
benign
g.127808161G>A
g.127050585G>A
-
-
BIN1_000014
-
PubMed: Nicot 2007
-
rs7558000
Germline
-
0.14
-
-
-
Johan den Dunnen
-/.
17i
c.1573-29G>C
r.(?)
p.(=)
-
benign
g.127808127C>G
g.127050551C>G
-
-
BIN1_000013
-
PubMed: Nicot 2007
-
rs12466914
Germline
-
0.05
-
-
-
Johan den Dunnen
-/.
17i
c.1573-18G>C
r.(?)
p.(=)
-
benign
g.127808116C>G
g.127050540C>G
-
-
BIN1_000012
-
PubMed: Nicot 2007
-
rs12466912
Germline
-
0.04
-
-
-
Johan den Dunnen
-/.
-
c.1573-18G>C
r.(=)
p.(=)
-
benign
g.127808116C>G
g.127050540C>G
-
-
BIN1_000012
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/.
-
c.1595C>T
r.(?)
p.(Thr532Met)
-
likely benign
g.127808076G>A
g.127050500G>A
-
-
BIN1_000050
13 heterozygous, no homozygous; Clinindb (India)
PubMed: Narang 2020 , Journal: Narang 2020
-
rs112318500
Germline
-
13/2795 individuals
-
-
-
Mohammed Faruq
-?/.
-
c.1625A>G
r.(?)
p.(Lys542Arg)
-
likely benign
g.127808046T>C
-
BIN1(NM_001320632.1):c.1118A>G (p.(Lys373Arg))
-
BIN1_000052
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-/.
18i
c.1675-88G>A
r.(?)
p.(=)
-
benign
g.127806297C>T
g.127048721C>T
-
-
BIN1_000016
-
PubMed: Nicot 2007
-
-
Germline
-
0.01
-
-
-
Johan den Dunnen
+/.
-
c.1713G>A
r.(?)
p.(Trp571*)
-
pathogenic
g.127806171C>T
g.127048595C>T
-
-
BIN1_000049
1 heterozygous, no homozygous; Clinindb (India)
PubMed: Narang 2020 , Journal: Narang 2020
-
rs587783343
Germline
-
1/2795 individuals
-
-
-
Mohammed Faruq
+/.
19
c.1717C>T
r.(?)
p.(Gln573*)
-
pathogenic
g.127806167G>A
g.127048591G>A
Gln573Stop
-
BIN1_000038
-
PubMed: Toussaint 2010
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
19
c.1723A>T
r.(?)
p.(Lys575*)
-
pathogenic
g.127806161T>A
g.127048585T>A
1723A>T
-
BIN1_000003
not in 560 control chromosomes; normal DNM2, MTM1
PubMed: Nicot 2007 , OMIM:var0003
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
19
c.1723A>T
r.(?)
p.(Lys575*)
-
pathogenic
g.127806161T>A
g.127048585T>A
-
-
BIN1_000003
-
PubMed: Mejaddam 2009
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
19
c.1723A>T
r.(?)
p.Lys575*
-
NA
g.127806161T>A
g.127048585T>A
-
-
BIN1_000003
expression cloning COS-1 cells no effect on membrane tubulation
PubMed: Nicot 2007 , OMIM:var0003
-
-
In vitro (cloned)
-
-
-
-
-
Johan den Dunnen
+/.
19
c.1723A>T
r.(?)
p.Lys575*
-
NA
g.127806161T>A
g.127048585T>A
-
-
BIN1_000003
expression cloning COS-1 cells reduced DNM2 binding CoIP
PubMed: Nicot 2007 , OMIM:var0003
-
-
In vitro (cloned)
-
-
-
-
-
Johan den Dunnen
-?/.
-
c.1747G>A
r.(?)
p.(Val583Ile)
-
likely benign
g.127806137C>T
-
BIN1(NM_139343.2):c.1747G>A (p.(Val583Ile))
-
BIN1_000058
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden