Global Variome shared LOVD
EXT2 (exostosin 2)
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Curator:
Wim Wuyts
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Unique variants in the EXT2 gene
A Multiple Osteochondroma Mutation Database
The variants shown are described using the NM_207122.1 transcript reference sequence.
Legend
Please note that a short description of a certain column can be displayed when you move your mouse cursor over the column's header and hold it still. Below, a more detailed description is shown per column.
Effect
: The variant's effect on the function of the gene/protein, displayed in the format 'R/C'. R is the value reported by the source (publication, submitter) and this classification may vary between records. C is the value concluded by the curator. Note that in some database the curator uses Summary records to give details on the classification of the variant.Values used: '+' indicating the variant affects function, '+?' probably affects function, '-' does not affect function, '-?' probably does not affect function, '?' effect unknown, '.' effect was not classified.
Reported
: The number of times this variant has been reported in the database.
Exon
: number of exon/intron containing variant; 2 = exon 2, 12i = intron 12, 2i_7i = from intron 2 to intron 7, 8i_9 = intron 8/exon 9 boundary, _1 = 5' to exon 1, 18_ = 3' of exon 18, _1_18_ = encompassing the entire 18-exon gene
DNA change (cDNA)
: description of variant at DNA level, based on a coding DNA reference sequence (following HGVS recommendations); e.g. c.123C>T, c.123_145del, c.123_126dup. For deletions/duplications extending beyond the reference transcript resp. {0}/{2} is used to replace del/dup. Extent of the deletion/duplication should be specified using the genomic description (g.). "-" indicates the variant described on genomic level does not affect the coding DNA reference sequence.
RNA change
: description of variant at RNA level (following HGVS recommendations).
r.123c>u
r.? = unknown
r.(?) = RNA not analysed but probably transcribed copy of DNA variant
r.spl? = RNA not analysed but variant probably affects splicing
r.(spl?) = RNA not analysed but variant may affect splicing
r.0? = change expected to abolish transcription
Protein
: description of variant at protein level (following HGVS recommendations).
p.(Arg345Pro) = change predicted from DNA (RNA not analysed)
p.Arg345Pro = change derived from RNA analysis
p.? = unknown effect
p.0? = probably no protein produced
Classification method
: The method used for the clinical classification of this variant.
All options:
ACMG
ACGS
EAHAD-CFDB
ENIGMA
IARC
InSiGHT
kConFab
other
Clinical classification
: Clinical classification of variant, preferably based on standardised criteria (e.g. ACMG), directed on the clinical consequences as published/submitted, indicated using an enriched system including inheritance: e.g. pathogenic, pathogenic (dominant), pathogenic (recessive), pathogenic (!), pathogenic (maternal), pathogenic (paternal). Standard inheritance is covered by dominant/recessive, imprinting by maternal/paternal. A '!' warns for exceptional circumstances to be explained in the 'Remarks' field (low penetrance, variants pathogenic in heterozygous state only, hypomorphic/hypermorphic variants, protective variants, etc.). Non-disease consequences (e.g. drug metabolism (pharmacogenetics), risk factor, blood group, tasting bitter) are indicated using additions to the benign classification; benign (dominant), benign (recessive), benign (!), etc. The value 'association' is used for variants associated with a phenotype and 'NA' for variants from in vitro/in silico records. NOTE: classification may differ from the opinion of the curator as given in a variant SUMMARY-record or the 'Functional effect concluded'). NOTE: pathogenic/likely pathogenic should go together with "variant (probably) affects function" In ClassFunctional.
All options:
pathogenic
pathogenic (dominant)
pathogenic (recessive)
pathogenic (!)
pathogenic (maternal)
pathogenic (paternal)
likely pathogenic
likely pathogenic (dominant)
likely pathogenic (recessive)
likely pathogenic (!)
likely pathogenic (maternal)
likely pathogenic (paternal)
VUS
VUS (!)
likely benign
likely benign (dominant)
likely benign (recessive)
likely benign (!)
likely benign (maternal)
likely benign (paternal)
benign
benign (dominant)
benign (recessive)
benign (!)
benign (maternal)
benign (paternal)
conflicting
association
NA
DNA change (genomic) (hg19)
: HGVS description of variant at DNA level, based on the genomic (chromosomal) DNA reference sequence; e.g. g.12345678C>T, g.12345679del, g.12345678_12345890dup
DNA change (hg38)
: HGVS description of variant at DNA level, based on the hg38 genomic (chromosomal) eference sequence; e.g. g.12345678C>T, g.12345679del, g.12345678_12345890dup
Published as
: listed only when different from "DNA change"; variant as reported originally (e.g. 521delT). Variants seen in animal models, tested in vitro, predicted from RNA analysis, etc. are described between brackets like c.(456C>G)
ISCN
: description of the variant according to ISCN nomenclature
DB-ID
: database ID of variant, grouping multiple observations of the same variant together, starting with the HGNC gene symbol, followed by an underscore (_) and a six digit number (e.g. DMD_012345). _000000 is used for variants where DNA was not analysed (change predicted from RNA analysis), variants seen in animal models or variants not seen in humans but functionally tested in vitro
Variant remarks
: remarks regarding variant described, e.g. germline mosaicism in mother, 345 kb deletion, muscle RNA analysed, not in 200 control chromosomes tested, on founder haplotype, etc.
Reference
: publication describing the variant submitted, incl. links to OMIM, PubMed or other source, e.g. "den Dunnen ASHG2003 P2346"
ClinVar ID
: ID of variant in ClinVar database
dbSNP ID
: the dbSNP ID
Origin
: Origin of variant/record: Germline = in all cells, De novo = in all cells, but not in either parent, Germline/De novo (untested) = in all cells, parents not tested (use only when De novo is likely, e.g. isolated/sporadic cases with dominant disease), Somatic = present in a subset of cells, but not in either parent, Uniparental disomy = from parental disomy (maternal or paternal), CLASSIFICATION record = submitter only sharing variant classification (note another report may share Individual data), SUMMARY record = master summary record from curator (may link to another database), In vitro (cloned) = data resulting from in vitro functional assays, animal model = data from animal model, Artefact = false positive variant call, DUPLICATE record = variant already described on another chromosome (e.g. unbalanced translocation, duplicating transposition, 2nd fusion transcript, etc.)
All options:
Germline
De novo
Germline/De novo (untested)
Somatic
Uniparental disomy
Uniparental disomy, maternal allele
Uniparental disomy, paternal allele
CLASSIFICATION record
SUMMARY record
In vitro (cloned)
In silico
animal model
Artefact
DUPLICATE record
Unknown
Not applicable
Segregation
: Indicates whether the variant segregates with the phenotype (yes), does not segregate with the phenotype (no) or segregation is unknown (?)
All options:
? = unknown
yes = segregates with phenotype
no = does not segregate with phenotype
- = not applicable
Frequency
: frequency in which the variant was found; e.g 5/760 chromosomes (in 5 of 760 chromosomes tested), 1/33 patients (in 1 of 33 patients analysed in study), 0.05 controls (in 5% of control cases tested)
Re-site
: restriction enzyme recognition site created (+) or destroyed (-); e.g. BglII+;BamHI-
VIP
: variant VIP-status was requested for matchmaking - need collaboration(s) to crack the case - please contact the submitter/curator. NOTE: to get VIP status ask the curator.
Methylation
: result of methylation test; GOM (gain of methylation), LOM (loss of methylation), 30% (30% methylated). NOTE: when several tests were done mention the method as well (e.g. MS-PCR 75%)
How to query this table
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Operator
Column type
Example
Matches
Text
Arg
all entries containing 'Arg'
space
Text
Arg Ser
all entries containing 'Arg' and 'Ser'
|
Text
Arg|Ser
all entries containing 'Arg' or 'Ser'
!
Text
!fs
all entries not containing 'fs'
^
Text
^p.(Arg
all entries beginning with 'p.(Arg'
$
Text
Ser)$
all entries ending with 'Ser)'
=""
Text
=""
all entries with this field empty
=""
Text
="p.0"
all entries exactly matching 'p.0'
!=""
Text
!=""
all entries with this field not empty
!=""
Text
!="p.0"
all entries not exactly matching 'p.0?'
combination
Text
*|Ter !fs
all entries containing '*' or 'Ter' but not containing 'fs'
Date
2020
all entries matching the year 2020
|
Date
2020-03|2020-04
all entries matching March or April, 2020
!
Date
!2020-03
all entries not matching March, 2020
<
Date
<2020
all entries before the year 2020
<=
Date
<=2020-06
all entries in or before June, 2020
>
Date
>2020-06
all entries after June, 2020
>=
Date
>=2020-06-15
all entries on or after June 15th, 2020
combination
Date
2019|2020 <2020-03
all entries in 2019 or 2020, and before March, 2020
Numeric
23
all entries exactly matching 23
|
Numeric
23|24
all entries exactly matching 23 or 24
!
Numeric
!23
all entries not exactly matching 23
<
Numeric
<23
all entries lower than 23
<=
Numeric
<=23
all entries lower than, or equal to, 23
>
Numeric
>23
all entries higher than 23
>=
Numeric
>=23
all entries higher than, or equal to, 23
combination
Numeric
>=20 <30 !23
all entries with values from 20 to 29, but not equal to 23
Some more advanced examples:
Example
Matches
Asian
all entries containing 'Asian', 'asian', including 'Caucasian', 'caucasian', etc.
Asian !Caucasian
all entries containing 'Asian' but not containing 'Caucasian'
Asian|African !Caucasian
all entries containing 'Asian' or 'African', but not containing 'Caucasian'
"South Asian"
all entries containing 'South Asian', but not containing 'South East Asian'
To sort on a certain column, click on the column header or on the arrows. If that column is already selected to sort on, the sort order will be swapped. The column currently sorted on has a darker blue background color than the other columns. The up and down arrows next to the column name indicate the current sorting direction. When sorting on any field other than the default, LOVD will sort secondarily on the default sort column.
298 entries on 3 pages. Showing entries 1 - 100.
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Effect
Reported
Exon
DNA change (cDNA)
RNA change
Protein
Classification method
Clinical classification
DNA change (genomic) (hg19)
DNA change (hg38)
Published as
ISCN
DB-ID
Variant remarks
Reference
ClinVar ID
dbSNP ID
Origin
Segregation
Frequency
Re-site
VIP
Methylation
Owner
+/.
3
9i_14_
c.(1495+1_1495-1)_*1143{0}
r.?
p.0?
-
pathogenic
g.(44219569_44228342)_(44266980_?)del
g.(44198019_44206792)_(44245430_?)del
c.(1495+1_1495-1)_(*1_?)del
-
EXT2_000447
-
PubMed: Fusco 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/-
1
1
c.-484T>C
r.(=)
p.(=)
-
benign
g.44116949T>C
g.44095399T>C
c.-331T>C
-
EXT2_000023
SNP rs10458911 T/C
-
-
-
Unknown
-
346
-
-
-
Ivy Jennes
+?/+?
5
_1_14_
c.-334_*1143{0}
r.spl?
p.(?)
-
likely pathogenic
g.(?_44117099)_(44266980_?)del
g.(?_44095549)_(44245430_?)del
-
-
EXT2_000010
-
-
-
-
Germline, Unknown
-
-
-
-
-
Bert Bakker
,
Ivy Jennes
-/-
1
1
c.-311G>A
r.(=)
p.(=)
-
benign
g.44117122G>A
g.44095572G>A
c.-464G>A
-
EXT2_000022
SNP rs3923808 G/A
-
-
-
Unknown
-
538
-
-
-
Ivy Jennes
-/-
1
1
c.-214C>G
r.(=)
p.(=)
-
benign
g.44117219C>G
g.44095669C>G
c.-61C>G
-
EXT2_000021
SNP rs12800404 C/G
-
-
-
Unknown
-
115
-
-
-
Ivy Jennes
-/-, -/.
2
1i
c.-31+7G>T
r.(=)
p.(=)
-
benign
g.44117409G>T
g.44095859G>T
c.-184+7G>T, EXT2(NM_001178083.1):c.-31+7G>T (p.(=))
-
EXT2_000019
SNP rs58861092 G/T, VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record, Unknown
-
38
-
-
-
VKGL-NL_Leiden
,
Ivy Jennes
-/-
1
1i
c.-31+22G>A
r.(=)
p.(=)
-
benign
g.44117424G>A
g.44095874G>A
c.-184+22G>A
-
EXT2_000015
SNP rs12365753 G/A
-
-
-
Unknown
-
462
-
-
-
Ivy Jennes
-/-
1
1i
c.-31+42A>G
r.(=)
p.(=)
-
benign
g.44117444A>G
g.44095894A>G
c.-184+42A>G
-
EXT2_000016
SNP rs12362775 A/G
-
-
-
Unknown
-
346
-
-
-
Ivy Jennes
-/-
1
1i
c.-31+54G>T
r.(=)
p.(=)
-
benign
g.44117456G>T
g.44095906G>T
c.-30-3884G>T
-
EXT2_000014
-
-
-
-
Unknown
-
154
-
-
-
Ivy Jennes
?/.
1
-
c.-31+403G>A
r.(=)
p.(=)
-
VUS
g.44117805G>A
g.44096255G>A
EXT2(NM_000401.3):c.3G>A (p.M1?)
-
EXT2_000407
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/-, -/., -?/.
3
1i
c.-31+474G>A
r.(=)
p.(=)
-
benign, likely benign
g.44117876G>A
g.44096326G>A
c.-87+5G>A, EXT2(NM_000401.3):c.69+5G>A (p.?)
-
EXT2_000017
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record, Unknown
-
38
-
-
-
VKGL-NL_Leiden
,
Ivy Jennes
,
VKGL-NL_VUmc
-/-
1
1i
c.-31+496T>G
r.(=)
p.(=)
-
benign
g.44117898T>G
g.44096348T>G
c.-87+27T>G
-
EXT2_000018
SNP rs11037860 T/G
-
-
-
Unknown
-
385
-
-
-
Ivy Jennes
+?/+?
1
1i_2
c.-30-10_441del
r.spl?
p.?
-
likely pathogenic
g.44129223_44129703del
g.44107673_44108153del
-
-
EXT2_000013
-
-
-
-
Unknown
-
-
-
-
-
Ivy Jennes
+?/+?
1
1i_2i
c.(-31+1_-30-1)_(536+1_537-1)del
r.spl?
p.?
-
likely pathogenic
g.44129232_44129799del
-
-
-
EXT2_000005
1 more item
-
-
-
Germline
-
-
-
-
-
Ivy Jennes
+?/+?
1
1i_8i
c.(-31+1_-30-1)_(1305+1_1306-1)del
r.spl?
p.?
-
likely pathogenic
g.44129232_44193293del
-
-
-
EXT2_000006
1 more item
-
-
-
Germline
-
-
-
-
-
Ivy Jennes
?/?
1
2
c.-28G>A
r.(=)
p.(=)
-
VUS
g.44129235G>A
g.44107685G>A
c.1-28G>A
-
EXT2_000024
Regulatory?
-
-
-
Unknown
-
-
-
-
-
Ivy Jennes
+/.
1
9i_14_
c.(1495+1_1495-1)_*1143{0}
r.?
p.0?
-
pathogenic
g.(44219569_44228342)_(44266980_?)del
g.(44198019_44206792)_(44245430_?)del
c.(1495+1_1495-1)_(*1_?)del
-
EXT2_000447
-
PubMed: Fusco 2019
-
-
Germline
-
-
-
-
-
Carmela Fusco
+?/+?
1
1_10i
c.-334_(1662+1_1663-1){0}
r.0?
p.0?
-
likely pathogenic
g.(?_44114092)_(44228510_44253902)del
g.(?_44092542)_(44206960_44232352)del
c.(-3341)_(1662+1)del
-
EXT2_000004
-
-
-
-
Germline
-
-
-
-
-
Ivy Jennes
-/-
1
_1_14_
c.?
r.(?)
p.(?)
-
benign
g.44117100_44266981del
g.44095550_44245431del
c.-833C>T
-
EXT2_000010
-
-
-
-
Unknown
-
38
-
-
-
Ivy Jennes
?/.
1
-
c.8C>T
r.(?)
p.(Ala3Val)
-
VUS
g.44129270C>T
g.44107720C>T
EXT2(NM_001178083.1):c.8C>T (p.A3V)
-
EXT2_000420
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/., ?/.
2
-
c.11C>T
r.(?)
p.(Ser4Leu)
-
likely pathogenic, VUS
g.44129273C>T
g.44107723C>T
EXT2(NM_207122.1):c.11C>T (p.(Ser4Leu)), NM_000401.3:c.110C>T; p.Ser37Leu
-
EXT2_000434
VKGL data sharing initiative Nederland
PubMed: Nair 2018
-
rs527624522
CLASSIFICATION record, Germline
?
-
-
-
-
VKGL-NL_Leiden
-/-, -/., -?/.
3
2
c.28C>A
r.(=)
p.(=)
-
benign, likely benign
g.44129290C>A
g.44107740C>A
(Protein: R10R, CGG>AGG)
-
EXT2_000025
1 more item
PubMed: Chen 2020
,
PubMed: Guo 2021
-
-
Germline, Unknown
-
0,03
-
-
-
Johan den Dunnen
,
Ivy Jennes
+?/+?
1
2
c.63del
r.(?)
p.(Lys21Asnfs*38)
-
likely pathogenic
g.44129325del
g.44107775del
63delG
-
EXT2_000026
-
-
-
-
Germline
-
-
-
-
-
Ivy Jennes
+/., +?/+?
33
2
c.67C>T
r.(?)
p.(Arg23*), p.(Arg23Ter)
-
likely pathogenic, pathogenic, pathogenic (dominant)
g.44129329C>T
g.44107779C>T
EXT2(NM_207122.1):c.67C>T (p.(Arg23Ter))
-
EXT2_000027
VKGL data sharing initiative Nederland
PubMed: Fusco 2019
,
PubMed: Ruan 2018
-
-
CLASSIFICATION record, Germline, Unknown
yes
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Leiden
,
Ivy Jennes
,
Elena Pedrini
,
Elena Pedrini
,
Carmela Fusco
+?/+?
1
2
c.78dup
r.(?)
p.(Ile27Tyrfs*29)
-
likely pathogenic
g.44129340dup
g.44107790dup
77- or 78insT
-
EXT2_000036
-
-
-
-
Germline
-
-
-
-
-
Ivy Jennes
+/., +?/+?
2
2
c.81del
r.(?)
p.(Thr28Profs*31), p.(Thr28ProfsTer31)
-
likely pathogenic, pathogenic
g.44129343del
g.44107793del
c.81delC
-
EXT2_000236
-
PubMed: Fusco 2019
-
-
Germline
-
-
-
-
-
Elena Pedrini
,
Carmela Fusco
+?/+?
1
2
c.112_113del
r.(?)
p.(Ile38Cysfs*17)
-
likely pathogenic
g.44129374_44129375del
g.44107824_44107825del
112delAT
-
EXT2_000037
-
-
-
-
Germline
-
-
-
-
-
Ivy Jennes
-/.
1
-
c.124A>G
r.(?)
p.(Met42Val)
-
benign
g.44129386A>G
g.44107836A>G
EXT2(NM_000401.3):c.223A>G (p.M75V)
-
EXT2_000392
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
+/., +?/+?
2
2
c.124del
r.(?)
p.(Met42Cysfs*17), p.(Met42CysfsTer17)
-
likely pathogenic, pathogenic
g.44129386del
g.44107836del
c.124delA
-
EXT2_000237
-
-
-
-
Germline, Unknown
-
-
-
-
-
Elena Pedrini
,
MobiDetails
+/., +?/+?
5
2
c.151G>T
r.(?)
p.(Glu51*), p.(Glu51Ter)
-
likely pathogenic, pathogenic
g.44129413G>T
g.44107863G>T
-
-
EXT2_000038
-
PubMed: Fusco 2019
-
-
Germline, Unknown
-
-
-
-
-
Ivy Jennes
,
Elena Pedrini
,
Elena Pedrini
,
Carmela Fusco
+/.
1
2
c.173del
r.(?)
p.(Val58GlufsTer54)
-
pathogenic
g.44129435del
g.44107885del
-
-
EXT2_000442
-
PubMed: Fusco 2019
-
-
Germline
-
-
-
-
-
Carmela Fusco
+?/+?
1
2
c.178A>T
r.(?)
p.(Lys60*)
-
likely pathogenic
g.44129440A>T
g.44107890A>T
-
-
EXT2_000040
-
-
-
-
Germline
-
-
-
-
-
Ivy Jennes
+?/+?
1
2
c.197_202delinsC
r.(?)
p.(Val66Alafs*3)
-
likely pathogenic
g.44129459_44129464delinsC
g.44107909_44107914delinsC
197-202delTGCCGGinsC
-
EXT2_000041
-
-
-
-
Germline
-
-
-
-
-
Ivy Jennes
+/., +?/+?
2
2
c.200dup
r.(?)
p.(Val68Glyfs*3)
-
likely pathogenic, pathogenic
g.44129462dup
g.44107912dup
201_202insC, EXT2(NM_207122.1):c.200dup (p.(Val68GlyfsTer3))
-
EXT2_000042
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record, Unknown
-
-
-
-
-
VKGL-NL_Leiden
,
Ivy Jennes
-?/.
1
-
c.201G>A
r.(?)
p.(Pro67=)
-
likely benign
g.44129463G>A
g.44107913G>A
EXT2(NM_001178083.1):c.201G>A (p.P67=)
-
EXT2_000408
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/+?
1
2
c.201_202insC
r.(?)
p.(Val68Argfs*3)
-
likely pathogenic
g.44129463_44129464insC
g.44107913_44107914insC
-
-
EXT2_000043
-
-
-
-
Germline
-
-
-
-
-
Ivy Jennes
?/.
1
-
c.232C>G
r.(?)
p.(Pro78Ala)
-
VUS
g.44129494C>G
-
EXT2(NM_000401.3):c.331C>G (p.(Pro111Ala))
-
EXT2_000437
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+?/+?
1
2
c.233del
r.(?)
p.(Pro78Glnfs*34)
-
likely pathogenic
g.44129495del
g.44107945del
233delC
-
EXT2_000044
-
-
-
-
Germline
-
-
-
-
-
Ivy Jennes
+/., +?/+?
2
2
c.244del
r.(?)
p.(Asp82Ilefs*30), p.(Asp82IlefsTer30)
-
likely pathogenic, pathogenic
g.44129506del
g.44107956del
244delG
-
EXT2_000318
-
PubMed: Fusco 2019
-
-
Germline
-
-
-
-
-
Ivy Jennes
,
Carmela Fusco
+?/+?
2
2
c.244dup
r.(?)
p.(Asp82Glyfs*11)
-
likely pathogenic
g.44129506dup
g.44107956dup
239-244insG
-
EXT2_000045
-
-
-
-
Germline, Unknown
-
-
-
-
-
Ivy Jennes
+?/+?
1
2
c.244_245insC
r.(?)
p.(Asp82Alafs*11)
-
likely pathogenic
g.44129506_44129507insC
g.44107956_44107957insC
-
-
EXT2_000239
-
-
-
-
Germline
-
-
-
-
-
Elena Pedrini
+?/+?
1
2
c.245del
r.(?)
p.(Asp82Valfs*30)
-
likely pathogenic
g.44129507del
g.44107957del
245delA
-
EXT2_000046
-
-
-
-
Germline
-
-
-
-
-
Ivy Jennes
+?/+
1
2
c.253T>C
r.(?)
p.(Cys85Arg)
-
likely pathogenic
g.44129515T>C
g.44107965T>C
-
-
EXT2_000047
This variant is reported as pathogenic by Uniprot.
-
-
-
Germline
-
-
-
-
-
Ivy Jennes
?/.
1
-
c.260T>G
r.(?)
p.(Met87Arg)
-
VUS
g.44129522T>G
-
-
-
EXT2_000435
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/., -?/.
3
-
c.264C>T
r.(?)
p.(His88=)
-
benign, likely benign
g.44129526C>T
g.44107976C>T
EXT2(NM_000401.3):c.363C>T (p.(His121=), p.H121=), EXT2(NM_207122.1):c.264C>T (p.H88=)
-
EXT2_000409
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Utrecht
,
VKGL-NL_VUmc
+?/+?
1
2
c.269_271delinsAAA
r.(?)
p.(Cys90*)
-
likely pathogenic
g.44129531_44129533delinsAAA
g.44107981_44107983delinsAAA
269_271GTT>AAA
-
EXT2_000048
-
-
-
-
Germline
-
-
-
-
-
Ivy Jennes
?/.
2
-
c.283C>T
r.(?)
p.(Arg95Cys)
-
VUS
g.44129545C>T
-
EXT2(NM_000401.3):c.382C>T (p.(Arg128Cys))
-
EXT2_000426
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Nijmegen
?/.
2
-
c.284G>A
r.(?)
p.(Arg95His)
-
VUS
g.44129546G>A
g.44107996G>A
EXT2(NM_207122.2):c.284G>A (p.(Arg95His))
-
EXT2_000419
conflicting interpretations of pathogenicity; 1 heterozygous, no homozygous;
Clinindb (India)
,
1 more item
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs143703574
CLASSIFICATION record, Germline
-
1/2795 individuals
-
-
-
VKGL-NL_Leiden
,
Mohammed Faruq
+?/+?
1
2
c.302_357del
r.(?)
p.(Lys101Serfs*12)
-
likely pathogenic
g.44129564_44129619del
g.44108014_44108069del
302del56
-
EXT2_000049
-
-
-
-
Unknown
-
-
-
-
-
Ivy Jennes
+/., +?/.
17
2
c.310del
r.(?)
p.(Ile104Serfs*8)
ACMG
likely pathogenic (dominant), pathogenic (dominant)
g.44129572del
g.44108022del
c.409delA, ENST00000395673:c.409delA
-
EXT2_000459
The variant has been assessed by the VEP tool on 06 August 2024,
1 more item
10.2991/ahsr.k.220103.050,
Journal: Yakovleva 2022
-
-
Germline
?, yes
17/48 cases, 17/48 patients patients
-
-
-
Leonid R. Zhozhikov
,
Yakovleva Alexandra Eremeevna
+?/+?
1
2
c.313A>T
r.(?)
p.(Lys105*)
-
likely pathogenic
g.44129575A>T
g.44108025A>T
-
-
EXT2_000050
-
-
-
-
Germline
-
-
-
-
-
Ivy Jennes
+?/+?
1
2
c.314del
r.(?)
p.(Lys105Argfs*7)
-
likely pathogenic
g.44129576del
g.44108026del
c.314delA
-
EXT2_000240
-
-
-
-
Germline
-
-
-
-
-
Elena Pedrini
+/.
1
2
c.315del
r.(?)
p.(Val106CysfsTer6)
-
pathogenic
g.44129578del
g.44108028del
-
-
EXT2_000446
-
PubMed: Fusco 2019
-
-
Germline
-
-
-
-
-
Carmela Fusco
+?/+?
1
2
c.316dup
r.(?)
p.(Val106Glyfs*12)
-
likely pathogenic
g.44129578dup
g.44108028dup
315-316insG
-
EXT2_000051
-
-
-
-
Germline
-
-
-
-
-
Ivy Jennes
+?/+?
1
2
c.317dup
r.(?)
p.(Tyr107Valfs*11)
-
likely pathogenic
g.44129579dup
g.44108029dup
c.317_318insT
-
EXT2_000364
-
-
-
-
Germline
-
-
-
-
-
Elena Pedrini
+?/+?
1
2
c.318_319dup
r.(?)
p.(Tyr107Cysfs*6)
-
likely pathogenic
g.44129580_44129581dup
g.44108030_44108031dup
319insGT
-
EXT2_000052
-
-
-
-
Unknown
-
-
-
-
-
Ivy Jennes
+?/+?
1
2
c.342C>A
r.(?)
p.(Tyr114*)
-
likely pathogenic
g.44129604C>A
g.44108054C>A
-
-
EXT2_000053
-
-
-
-
Unknown
-
-
-
-
-
Ivy Jennes
+/., +?/+?
2
2
c.342C>G
r.(?)
p.(Tyr114*), p.(Tyr114Ter)
-
likely pathogenic, pathogenic
g.44129604C>G
g.44108054C>G
-
-
EXT2_000054
-
-
-
rs763623546
CLASSIFICATION record, Germline
-
-
-
-
-
Ivy Jennes
,
MobiDetails
+?/+?
1
2
c.362_363del
r.(?)
p.(Ser121Cysfs*10)
-
likely pathogenic
g.44129624_44129625del
g.44108074_44108075del
c.362_363delCT
-
EXT2_000241
-
-
-
-
Germline
-
-
-
-
-
Elena Pedrini
+?/+?
1
2
c.372del
r.(?)
p.(Asn124Lysfs*146)
-
likely pathogenic
g.44129634del
g.44108084del
c.372delC
-
EXT2_000349
-
-
-
-
Germline
-
-
-
-
-
Ivy Jennes
+?/+?
1
2
c.376_445del
r.(?)
p.(Ile126Glyfs*121)
-
likely pathogenic
g.44129638_44129707del
g.44108088_44108157del
374-443del70
-
EXT2_000055
-
-
-
-
Unknown
-
-
-
-
-
Ivy Jennes
?/.
1
-
c.380C>G
r.(?)
p.(Ser127Cys)
-
VUS
g.44129642C>G
-
EXT2(NM_207122.2):c.380C>G (p.(Ser127Cys))
-
EXT2_000465
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
1
2
c.380del
r.(?)
p.(Arg128GlyfsTer142)
-
pathogenic
g.44129644del
g.44108094del
-
-
EXT2_000393
-
PubMed: Fusco 2019
-
-
Germline
-
-
-
-
-
Carmela Fusco
+?/+?, -?/.
2
2
c.382C>T
r.(?)
p.(Arg128Trp)
-
likely benign, likely pathogenic
g.44129644C>T
g.44108094C>T
EXT2(NM_207122.1):c.382C>T (p.(Arg128Trp))
-
EXT2_000056
VKGL data sharing initiative Nederland,
1 more item
-
-
-
CLASSIFICATION record, Unknown
-
14
-
-
-
VKGL-NL_Leiden
,
Ivy Jennes
+/.
1
-
c.382del
r.(?)
p.(Arg128GlyfsTer142)
-
pathogenic
g.44129644del
g.44108094del
EXT2(NM_000401.3):c.480delT (p.R161Gfs*142)
-
EXT2_000393
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.383G>A
r.(?)
p.(Arg128Gln)
-
VUS
g.44129645G>A
-
EXT2(NM_207122.2):c.383G>A (p.(Arg128Gln))
-
EXT2_000466
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/., +?/+?
4
2
c.390T>A
r.(?)
p.(Tyr130*), p.(Tyr130Ter)
-
likely pathogenic, pathogenic
g.44129652T>A
g.44108102T>A
-
-
EXT2_000338
-
PubMed: Fusco 2019
-
-
Germline
-
-
-
-
-
Elena Pedrini
,
Carmela Fusco
+/.
1
-
c.395_396del
r.(?)
p.(Glu132AlafsTer9)
-
pathogenic
g.44129657_44129658del
g.44108107_44108108del
EXT2(NM_000401.3):c.494_495delTG (p.E165Afs*9)
-
EXT2_000394
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/+?
1
2
c.398T>G
r.(?)
p.(Leu133Arg)
-
likely pathogenic
g.44129660T>G
g.44108110T>G
-
-
EXT2_000345
1 more item
-
-
-
Unknown
-
-
-
-
-
Ivy Jennes
-?/.
2
-
c.404T>C
r.(?)
p.(Met135Thr)
-
likely benign
g.44129666T>C
g.44108116T>C
EXT2(NM_000401.3):c.503T>C (p.(Met168Thr)), EXT2(NM_001178083.1):c.404T>C (p.M135T)
-
EXT2_000410
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
?/.
1
-
c.425A>T
r.(?)
p.(Tyr142Phe)
-
VUS
g.44129687A>T
-
EXT2(NM_001178083.1):c.425A>T (p.Y142F)
-
EXT2_000436
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/+?
1
2
c.427dup
r.(?)
p.(Tyr143Leufs*3)
-
likely pathogenic
g.44129689dup
g.44108139dup
-
-
EXT2_000219
-
-
-
-
Unknown
-
-
-
-
-
Bert Bakker
+/?
1
2
c.429C>A
r.(?)
p.(Tyr143*)
-
pathogenic
g.44129691C>A
g.44108141C>A
-
-
EXT2_000387
Refseq NM_207122.1
-
-
-
Unknown
-
-
-
-
-
Caroline Janel
+?/+?
1
2
c.445_454del
r.(?)
p.(Arg149Cysfs*118)
-
likely pathogenic
g.44129707_44129716del
g.44108157_44108166del
-
-
EXT2_000220
-
-
-
-
Unknown
-
-
-
-
-
Bert Bakker
+/.
1
2
c.448del
r.(?)
p.(Ala150ProfsTer120)
-
pathogenic
g.44129710del
g.44108160del
-
-
EXT2_000443
-
PubMed: Fusco 2019
-
-
Germline
-
-
-
-
-
Carmela Fusco
+?/+?
1
2
c.448G>A
r.(?)
p.(Ala150Thr)
-
likely pathogenic
g.44129710G>A
g.44108160G>A
-
-
EXT2_000057
1 more item
-
-
-
Unknown
-
-
-
-
-
Ivy Jennes
+?/+?
1
2
c.452_462dup
r.(?)
p.(Pro155Valfs*119)
-
likely pathogenic
g.44129714_44129724dup
g.44108164_44108174dup
-
-
EXT2_000058
-
-
-
-
Germline
-
-
-
-
-
Ivy Jennes
+/., +?/+?, ?/+?
8
2
c.454_457del
r.(?)
p.(Val154Profs*115), p.(Val154ProfsTer115)
-
likely pathogenic, pathogenic
g.44129716_44129719del
g.44108166_44108169del
450del4, 784-787del4, c.454_457delCTGT
-
EXT2_000059
-
PubMed: Fusco 2019
-
-
Germline, Unknown
-
-
-
-
-
Ivy Jennes
,
Elena Pedrini
,
Zhou Yongan
,
Carmela Fusco
+?/+
1
2
c.455T>G
r.(?)
p.(Leu152Arg)
-
likely pathogenic
g.44129717T>G
g.44108167T>G
T455G
-
EXT2_000062
This variant is reported as pathogenic by Uniprot.
-
-
-
Germline
-
-
-
-
-
Ivy Jennes
+?/+?
1
2
c.459del
r.(?)
p.(Phe153Leufs*117)
-
likely pathogenic
g.44129721del
g.44108171del
457-459delT
-
EXT2_000063
-
-
-
-
Germline
-
-
-
-
-
Ivy Jennes
+/., +?/+?
5
2
c.459_462del
r.(?)
p.(Val154Profs*115)
-
likely pathogenic, pathogenic
g.44129721_44129724del
g.44108171_44108174del
455del4, 459_462delTGTT, c.455-458delTGTT, EXT2(NM_207122.1):c.459_462del (p.(Val154ProfsTer115))
-
EXT2_000064
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record, Germline, Unknown
-
-
-
-
-
VKGL-NL_Leiden
,
Ivy Jennes
-?/.
1
-
c.462T>A
r.(?)
p.(=)
-
likely benign
g.44129724T>A
-
EXT2(NM_000401.3):c.561T>A (p.V187=)
-
EXT2_000464
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
+?/+?
1
2
c.468del
r.(?)
p.(Ile157Serfs*113)
-
likely pathogenic
g.44129730del
g.44108180del
c.468delC
-
EXT2_000319
-
-
-
-
Unknown
-
-
-
-
-
Ivy Jennes
+?/+?
1
2
c.484del
r.(?)
p.(Gln162Argfs*108)
-
likely pathogenic
g.44129746del
g.44108196del
484delC
-
EXT2_000065
-
-
-
-
Germline
-
-
-
-
-
Ivy Jennes
+?/+?
1
2
c.494_495del
r.(?)
p.(Leu165Profs*14)
-
likely pathogenic
g.44129756_44129757del
g.44108206_44108207del
494delTG
-
EXT2_000066
-
-
-
-
Germline
-
-
-
-
-
Ivy Jennes
+?/+?
1
2
c.495del
r.(?)
p.(Arg166Alafs*104)
-
likely pathogenic
g.44129757del
g.44108207del
495delG
-
EXT2_000067
-
-
-
-
Germline
-
-
-
-
-
Ivy Jennes
?/.
1
-
c.497G>A
r.(?)
p.(Arg166His)
-
VUS
g.44129759G>A
-
-
-
EXT2_000463
-
-
-
rs772821141
CLASSIFICATION record
-
-
-
-
-
MobiDetails
+?/+?
4
2
c.505G>T
r.(?)
p.(Glu169*)
-
likely pathogenic
g.44129767G>T
g.44108217G>T
-
-
EXT2_000068
-
-
-
-
Germline, Unknown
-
-
-
-
-
Ivy Jennes
,
Brett Casey
?/.
1
-
c.509C>T
r.(?)
p.(Thr170Ile)
-
VUS
g.44129771C>T
g.44108221C>T
EXT2(NM_001178083.1):c.509C>T (p.T170I)
-
EXT2_000421
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/+?
12
2
c.514C>T
r.(?)
p.(Gln172*)
-
likely pathogenic
g.44129776C>T
g.44108226C>T
-
-
EXT2_000070
-
-
-
-
Germline, Unknown
-
-
-
-
-
Ivy Jennes
,
Elena Pedrini
,
Brett Casey
-/-, -/., -?/., ?/?
6
2
c.519G>C
r.(=), r.(?)
p.(=), p.(Ala173=)
-
benign, likely benign, VUS
g.44129781G>C
g.44108231G>C
EXT2(NM_000401.3):c.618G>C (p.(Ala206=), p.A206=)
-
EXT2_000076
VKGL data sharing initiative Nederland,
2 more items
-
-
-
CLASSIFICATION record, Unknown
-
14
-
-
-
VKGL-NL_Leiden
,
Ivy Jennes
,
VKGL-NL_VUmc
+?/+?, -?/.
2
2
c.520A>C
r.(?)
p.(Met174Leu)
-
likely benign, likely pathogenic
g.44129782A>C
g.44108232A>C
EXT2(NM_000401.3):c.619A>C (p.(Met207Leu))
-
EXT2_000080
VKGL data sharing initiative Nederland,
1 more item
-
-
-
CLASSIFICATION record, Unknown
-
-
-
-
-
VKGL-NL_Leiden
,
Ivy Jennes
-?/.
1
-
c.520A>T
r.(?)
p.(Met174Leu)
-
likely benign
g.44129782A>T
g.44108232A>T
EXT2(NM_001178083.1):c.520A>T (p.M174L)
-
EXT2_000422
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/., +?/+?
2
2
c.526C>T
r.(?)
p.(Gln176*)
-
likely pathogenic, pathogenic (dominant)
g.44129788C>T
g.44108238C>T
-
-
EXT2_000342
-
PubMed: Chen 2020
-
-
Germline, Unknown
yes
-
-
-
-
Johan den Dunnen
,
Elena Pedrini
+?/+?
1
2
c.536G>A
r.(?)
p.(Arg179Lys)
-
likely pathogenic
g.44129798G>A
g.44108248G>A
-
-
EXT2_000353
1 more item
-
-
-
Germline
-
-
-
-
-
Ivy Jennes
+?/+?
1
2
c.536G>T
r.(?)
p.(Arg179Met)
-
likely pathogenic
g.44129798G>T
g.44108248G>T
-
-
EXT2_000285
1 more item
-
-
-
Unknown
-
-
-
-
-
Brett Casey
+?/+?
5
2i
c.536+1G>A
r.spl?
p.?
-
likely pathogenic
g.44129799G>A
g.44108249G>A
-
-
EXT2_000081
1 more item
-
-
-
Germline, Unknown
-
-
-
-
-
Bert Bakker
,
Ivy Jennes
+?/+?
4
2i
c.536+1G>T
r.spl?
p.?
-
likely pathogenic
g.44129799G>T
g.44108249G>T
IVS2+1G>A
-
EXT2_000084
1 more item
-
-
-
Germline, Unknown
-
-
-
-
-
Ivy Jennes
?/?
1
2i
c.537-84C>T
r.(=)
p.(=)
-
VUS
g.44130660C>T
g.44109110C>T
-
-
EXT2_000090
Substitution in intron 2. The consequence of this change is not predictable.
-
-
-
Unknown
-
0,03
-
-
-
Ivy Jennes
+?/+?
1
2i_3
c.537-17_560del
r.spl?
p.?
-
likely pathogenic
g.44130727_44130767del
g.44109177_44109217del
c.537-18_559del41
-
EXT2_000321
1 more item
-
-
-
Unknown
-
-
-
-
-
Ivy Jennes
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