Global Variome shared LOVD
SACS (spastic ataxia of Charlevoix-Saguenay (sacsin))
LOVD v.3.0 Build 30b [
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Curators:
Bernard Brais
and
Marie-Josée Dicaire
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Unique variants in the SACS gene
The variants shown are described using the NM_014363.5 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.
410 entries on 5 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
-/.
2
-
c.-13A>G
r.(?)
p.(=)
-
benign
g.23985391T>C
g.23411252T>C
SACS(NM_014363.5):c.-13A>G
-
SACS_000264
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Nijmegen
+?/?
1
_1_10_
c.(?_-1)_(-1_?)dup
r.?
p.?
-
likely pathogenic
g.(?_23495953)_(24927491_?)dup
g.(?_22921814)_(24353353_?)dup
hg18:g.(?_22,393,953)_(23,825,491_?)dup
-
SACS_000170
chromosome 13 duplication
PubMed: McElroy 2013
-
-
De novo
-
-
-
-
-
Bernard Brais
+/?
11
_1_10_
c.0
r.(?), r.0
p.0
-
pathogenic
g.(?_23511581)_(24928468?)del, g.(?_23904274)_(23985379_?)del
-
0.7-Mb deletion, 1.5 Mb macrodeletion, 1.5Mb del on chr13q12.12, 23511581_24928468del, 584 kb deletion,
3 more items
-
SACS_000048
del 1.5 Mb (6 genes), del 584 Kb (SACS and SGCG) chr13: 22,423,000-23,007,000,
3 more items
PubMed: Breckpot 2008
,
PubMed: McMillan 2009
,
PubMed: Pyle 2013
,
PubMed: Romano 2013
,
3 more items
-
-
De novo, Germline, Unknown
?
-
-
-
-
Bernard Brais
+/?
1
8
c.1475ˆ1476G>A
r.?
p.(Trp492*)
-
pathogenic
g.23929275ˆ23929276C>T
g.23355136ˆ23355137C>T
Trp492X, g.56103G>A
-
SACS_000043
-
PubMed: Vermeer 2008
-
-
Germline
-
-
-
-
-
Bernard Brais
-?/.
1
-
c.21-15C>T
r.(=)
p.(=)
-
likely benign
g.23949423G>A
g.23375284G>A
SACS(NM_014363.5):c.21-15C>T
-
SACS_000263
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/?
1
2i_5i
c.(20+1_21-1)_(345+1_346-1)dup
r.(?)
p.fs*
-
pathogenic
g.(23939417_23942540)_(23949409_23985358)dup
-
ex03_05del
-
SACS_000075
-
PubMed: Baets 2010
-
-
Germline
-
-
-
-
-
Bernard Brais
-?/.
1
-
c.90C>T
r.(?)
p.(Thr30=)
-
likely benign
g.23949339G>A
-
SACS(NM_014363.5):c.90C>T (p.T30=)
-
SACS_000387
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.166C>T
r.(?)
p.(Arg56Cys)
-
VUS
g.23949263G>A
-
SACS(NM_014363.6):c.166C>T (p.(Arg56Cys))
-
SACS_000437
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
1
-
c.171+1G>A
r.spl?
p.?
-
pathogenic
g.23949257C>T
g.23375118C>T
-
-
SACS_000295
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/.
2
-
c.171+6C>T
r.(=)
p.(=)
-
benign
g.23949252G>A
g.23375113G>A
SACS(NM_014363.5):c.171+6C>T
-
SACS_000262
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Nijmegen
-?/.
1
-
c.171+13C>T
r.(=)
p.(=)
-
likely benign
g.23949245G>A
g.23375106G>A
SACS(NM_014363.5):c.171+13C>T
-
SACS_000261
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/?
1
4
c.216del
r.(?)
p.(His73Ilefs*4)
-
pathogenic
g.23945260del
g.23371121del
216delT p.Cys72Cysfs*4
-
SACS_000073
-
PubMed: Baets 2010
-
-
Germline
-
-
-
-
-
Bernard Brais
+/?
2
4
c.237dup
r.(?)
p.(Ser80Ilefs*98)
-
pathogenic
g.23945240dup
g.23371101dup
237insA
-
SACS_000090
-
PubMed: Thiffault 2012
-
-
Germline
-
-
-
-
-
Bernard Brais
+/.
1
-
c.262C>T
r.(?)
p.(Arg88Ter)
ACMG
pathogenic
g.23942624G>A
g.23368485G>A
-
-
SACS_000400
-
PubMed: Thomas 2022
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
1
-
c.346-19del
r.(=)
p.(=)
-
benign
g.23939440del
-
SACS(NM_014363.6):c.346-19delT
-
SACS_000407
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/?
2
6
c.414C>G
r.(?)
p.(Tyr138*)
-
pathogenic
g.23939348G>C
g.23365209G>C
414C>G, p.[Y138X]
-
SACS_000121
-
PubMed: Haga 2012
,
PubMed: Shimazaki 2013
-
-
Unknown
-
-
-
-
-
Bernard Brais
+?/.
1
-
c.423_424del
r.(?)
p.(Glu141AspfsTer42)
-
likely pathogenic
g.23939341_23939342del
g.23365202_23365203del
SACS(NM_014363.5):c.423_424delGA (p.E141Dfs*42)
-
SACS_000260
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
2
-
c.432G>T
r.(?)
p.(Trp144Cys)
-
VUS
g.23939330C>A
g.23365191C>A
SACS(NM_014363.5):c.432G>T (p.W144C)
-
SACS_000259
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Nijmegen
+/.
1
-
c.457+1G>A
r.spl?
p.?
-
pathogenic
g.23939304C>T
g.23365165C>T
-
-
SACS_000294
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/?
2
7
c.482del
r.(?)
p.(Asn161Thrfs*15)
-
pathogenic
g.23932597del
g.23358458del
g.482delA, g.482delA N161Fs*175, g.52784delC
-
SACS_000049
-
PubMed: Kamada 2008
-
-
Germline
-
-
-
-
-
Bernard Brais
+/?
1
7
c.502G>T
r.(?)
p.(Asp168Tyr)
-
pathogenic
g.23932576C>A
g.23358437C>A
p.Asp168Tyr
-
SACS_000105
ATPase domain
PubMed: Vermeer 2008
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/?
1
7_7i
c.600_604+1del
r.spl
p.Thr201Cysfs*6
-
pathogenic
g.23932473_23932478del
g.23358334_23358339del
600_604+1delAACAGG (p.I202fsX6)
-
SACS_000056
-
PubMed: Terracciano 2008
-
-
Germline
-
-
-
-
-
Bernard Brais
+/?
1
7
c.602C>A
r.(?)
p.(Thr201Lys)
-
pathogenic
g.23932476G>T
g.23358337G>T
-
-
SACS_000078
-
PubMed: Baets 2010
-
-
Germline
-
-
-
-
-
Bernard Brais
?/.
1
7i
c.605-3C>G
r.spl
p.?
-
VUS
g.23930149G>C
g.23356010G>C
-
-
SACS_000389
-
PubMed: Ganapathy 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
8
c.623G>A
r.(?)
p.(Ser208Asn)
ACMG
likely pathogenic
g.23930128C>T
g.23355989C>T
-
-
SACS_000412
-
-
-
-
Germline
yes
-
-
-
-
Marketa Wayhelova
?/.
1
-
c.639G>T
r.(?)
p.(Gly213=)
-
VUS
g.23930112C>A
g.23355973C>A
SACS(NM_014363.5):c.639G>T (p.G213=)
-
SACS_000351
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.662T>C
r.(?)
p.(Leu221Pro)
-
VUS
g.23930089A>G
g.23355950A>G
SACS(NM_014363.6):c.662T>C (p.L221P)
-
SACS_000367
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
-/.
3
-
c.696T>A
r.(?)
p.(Asn232Lys)
-
benign
g.23930055A>T
g.23355916A>T
SACS(NM_014363.5):c.696T>A (p.N232K), SACS(NM_014363.6):c.696T>A (p.N232K)
-
SACS_000258
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
-?/.
1
-
c.744A>C
r.(?)
p.(Ala248=)
-
likely benign
g.23930007T>G
g.23355868T>G
SACS(NM_014363.5):c.744A>C (p.A248=)
-
SACS_000257
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.810T>G
r.(?)
p.(Phe270Leu)
-
likely benign
g.23929941A>C
g.23355802A>C
SACS(NM_014363.5):c.810T>G (p.F270L)
-
SACS_000366
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/?
10
8
c.814C>T
r.(?)
p.(Arg272Cys)
-
pathogenic
g.23929937G>A
g.23355798G>A
814C>T
-
SACS_000059
-
PubMed: Guernsey 2010
,
PubMed: Thiffault 2012
-
-
Germline
-
-
-
-
-
Bernard Brais
+/?
1
8
c.815G>A
r.(?)
p.(Arg272His)
-
pathogenic
g.23929936C>T
g.23355797C>T
815G>A(p.R272H)
-
SACS_000150
-
PubMed: Romano 2013
-
-
Unknown
?
-
-
-
-
Bernard Brais
+?/?
2
8
c.826C>T
r.(?)
p.(Arg276Cys)
-
likely pathogenic
g.23929925G>A
g.23355786G>A
826C>T
-
SACS_000130
-
PubMed: Prodi 2013
-
-
Germline
?
-
-
-
-
Bernard Brais
+/?
2
8
c.832C>T
r.(?)
p.(Gln278*)
-
pathogenic
g.23929919G>A
g.23355780G>A
832C>T
-
SACS_000139
-
PubMed: Gazulla 2011
-
-
Unknown
?
-
-
-
-
Bernard Brais
-/., -?/.
4
-
c.909A>G
r.(=), r.(?)
p.(=), p.(Ala303=)
-
benign, likely benign
g.23929842T>C
g.23355703T>C
SACS(NM_014363.5):c.909A>G (p.A303=)
-
SACS_000256
136 heterozygous;
Clinindb (India)
, 5 homozygous;
Clinindb (India)
,
1 more item
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs41315020
CLASSIFICATION record, Germline
-
136/2795 individuals, 5/2795 individuals
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Nijmegen
,
Mohammed Faruq
+/?
4
8
c.922C>T
r.(?)
p.(Leu308Phe)
-
pathogenic
g.23929829G>A
g.23355690G>A
922C>T
-
SACS_000051
-
PubMed: Takado 2007
-
-
Germline
-
-
-
-
-
Bernard Brais
?/.
1
-
c.956A>T
r.(?)
p.(Tyr319Phe)
-
VUS
g.23929795T>A
-
SACS(NM_014363.6):c.956A>T (p.(Tyr319Phe))
-
SACS_000436
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/?
3
8
c.961C>T
r.(?)
p.(Arg321*)
-
pathogenic
g.23929790G>A
g.23355651G>A
961C>T, p. Arg321*
-
SACS_000035
no domain
PubMed: Prodi 2013
,
PubMed: Vermeer 2008
-
-
De novo, Germline
-
-
-
-
-
Bernard Brais
?/.
1
-
c.972C>A
r.(?)
p.(Asp324Glu)
-
VUS
g.23929779G>T
g.23355640G>T
SACS(NM_014363.5):c.972C>A (p.D324E)
-
SACS_000371
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.973G>A
r.(?)
p.(Gly325Arg)
-
VUS
g.23929778C>T
-
SACS(NM_014363.6):c.973G>A (p.(Gly325Arg))
-
SACS_000435
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
-
c.991_999del
r.(?)
p.(Phe331_Val333del)
-
VUS
g.23929755_23929763del
g.23355616_23355624del
-
-
SACS_000350
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
-
c.1037C>T
r.(?)
p.(Pro346Leu)
-
VUS
g.23929714G>A
g.23355575G>A
SACS(NM_014363.5):c.1037C>T (p.P346L)
-
SACS_000349
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.1159G>A
r.(?)
p.(Ala387Thr)
-
VUS
g.23929592C>T
g.23355453C>T
SACS(NM_014363.6):c.1159G>A (p.A387T)
-
SACS_000365
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/?
1
8
c.1185_1194del
r.(?)
p.(Cys395Trpfs*13)
-
pathogenic
g.23929562_23929571del
g.23355423_23355432del
g.32627_32636delACACTGTTAC, g.5581255821delgtaacagtgt
-
SACS_000015
in Rpt1/ARM-rpt
PubMed: Ouyang 2006
-
-
Germline
-
-
-
-
-
Bernard Brais
+/?
1
8
c.1189dup
r.(?)
p.(S397Kfs*405)
-
pathogenic
g.23929562dup
g.23355423dup
1190insA
-
SACS_000091
-
PubMed: Thiffault 2012
-
-
Germline
-
-
-
-
-
Bernard Brais
+/.
5
10
c.1201C>T
r.(?)
p.(Arg401*), p.(Arg401Ter)
-
pathogenic (recessive)
g.23929550G>A
g.23355411G>A
NM_001278055:c.760C>T
-
SACS_000396
ACMG PVS1, PM2, PP1, PP3,PP5
PubMed: Saadi 2023
,
PubMed: Salinas 2020
SUB7801298
rs769212398
Germline
yes
-
-
-
-
Johan den Dunnen
,
Sherifa Ahmed Hamed
?/.
2
-
c.1219C>A
r.(?)
p.(Leu407Ile)
-
VUS
g.23929532G>T
-
SACS(NM_014363.5):c.1219C>A (p.L407I), SACS(NM_014363.6):c.1219C>A (p.(Leu407Ile))
-
SACS_000380
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
+/?
1
8
c.1228_1229del
r.(?)
p.(Leu410Serfs*2)
-
pathogenic
g.23929523_23929524del
g.23355384_23355385del
1228_1229delTT
-
SACS_000114
-
PubMed: Prodi 2013
-
-
De novo
-
-
-
-
-
Bernard Brais
+/?
2
8
c.1229del
r.(?)
p.(Leu410*)
-
pathogenic
g.23929524del
g.23355385del
1229delT p.Leu410X
-
SACS_000171
-
PubMed: Chen 2013
-
-
Unknown
?
-
-
-
-
Bernard Brais
-?/.
1
-
c.1273C>A
r.(?)
p.(Pro425Thr)
-
likely benign
g.23929478G>T
g.23355339G>T
SACS(NM_014363.5):c.1273C>A (p.P425T)
-
SACS_000255
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/?, +?/+?, -?/., ?/., ?/?
8
8
c.1373C>T
r.(?)
p.(Thr458Ile)
-
likely benign, likely pathogenic, pathogenic, VUS
g.23929378G>A
g.23355239G>A
1373C>T p.Thr458Ile, 1373C>T p.Thr458Ile, 1373C>T(p.T458I),
1 more item
-
SACS_000152
VKGL data sharing initiative Nederland
PubMed: Romano 2013
,
PubMed: Synofzik 2013
-
-
CLASSIFICATION record, Unknown
?
-
-
-
-
Bernard Brais
,
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
,
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
?/.
1
-
c.1378C>T
r.(?)
p.(Leu460Phe)
-
VUS
g.23929373G>A
g.23355234G>A
SACS(NM_001278055.1):c.937C>T (p.(Leu313Phe))
-
SACS_000347
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/?
1
8
c.1420C>T
r.(?)
p.(Arg474Cys)
-
pathogenic
g.23929331G>A
g.23355192G>A
1420C>T(p.R474C)
-
SACS_000154
-
PubMed: Romano 2013
-
-
Unknown
?
-
-
-
-
Bernard Brais
-?/.
1
-
c.1562G>C
r.(?)
p.(Ser521Thr)
-
likely benign
g.23929189C>G
-
SACS(NM_014363.5):c.1562G>C (p.S521T)
-
SACS_000386
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/+?
1
8
c.1580C>G
r.(?)
p.(Ser527*)
-
likely pathogenic
g.23929171G>C
g.23355032G>C
1580C>G (p.Ser527X)
-
SACS_000188
-
PubMed: Yu-Wai-Man 2014
-
-
Unknown
?
-
-
-
-
Bernard Brais
-?/.
1
-
c.1593C>T
r.(?)
p.(Ile531=)
-
likely benign
g.23929158G>A
g.23355019G>A
SACS(NM_014363.5):c.1593C>T (p.I531=)
-
SACS_000254
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/?, ?/.
3
8
c.1607C>T
r.(?)
p.(Pro536Leu)
-
likely pathogenic, VUS
g.23929144G>A
g.23355005G>A
p.Pro536Leu
-
SACS_000047
-
PubMed: Anheim 2008
,
PubMed: Ganapathy 2019
ClinVar-RCV000523535.1
rs1440541889
Germline
-
-
-
-
-
Johan den Dunnen
,
Bernard Brais
+?/?
1
8
c.1647_1658del
r.(?)
p.(Leu549_Leu552del)
-
likely pathogenic
g.23929095_23929106del
g.23354956_23354967del
1647_1658del p.Leu549_Leu552del
-
SACS_000162
-
PubMed: Synofzik 2013
-
-
Unknown
?
-
-
-
-
Bernard Brais
-/.
3
-
c.1656A>G
r.(?)
p.(Leu552=)
-
benign
g.23929095T>C
g.23354956T>C
SACS(NM_014363.5):c.1656A>G (p.L552=), SACS(NM_014363.6):c.1656A>G (p.L552=)
-
SACS_000253
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
+/?, ?/.
2
8
c.1667T>C
r.(?)
p.(Leu556Pro)
-
pathogenic, VUS
g.23929084A>G
g.23354945A>G
p.Leu556Pro, SACS(NM_014363.5):c.1667T>C (p.L556P)
-
SACS_000104
VKGL data sharing initiative Nederland
PubMed: Baets 2010
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Rotterdam
+?/.
1
8
c.1728C>G
r.(?)
p.(Tyr576*)
ACMG
likely pathogenic
g.23929023G>C
g.23354884G>C
-
-
SACS_000413
-
-
-
-
Germline
yes
-
-
-
-
Marketa Wayhelova
?/.
1
-
c.1762A>G
r.(?)
p.(Lys588Glu)
-
VUS
g.23928989T>C
g.23354850T>C
SACS(NM_014363.6):c.1762A>G (p.K588E)
-
SACS_000252
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-/.
1
-
c.1839G>A
r.(?)
p.(Gln613=)
-
benign
g.23928912C>T
g.23354773C>T
-
-
SACS_000292
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
-
c.1885G>A
r.(?)
p.(Ala629Thr)
-
VUS
g.23928866C>T
-
SACS(NM_014363.6):c.1885G>A (p.A629T)
-
SACS_000411
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+?/?
1
8
c.1894C>T
r.(?)
p.(Arg632Trp)
-
likely pathogenic
g.23928857G>A
g.23354718G>A
1894C>T
-
SACS_000138
-
PubMed: Gazulla 2011
-
-
Unknown
?
-
-
-
-
Bernard Brais
+/.
2
-
c.1925del
r.(?)
p.(Gly642AlafsTer10)
-
pathogenic
g.23928828del
g.23354689del
SACS(NM_014363.5):c.1925delG (p.G642Afs*10)
-
SACS_000346
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Nijmegen
-?/.
1
-
c.2021G>A
r.(?)
p.(Gly674Asp)
-
likely benign
g.23928730C>T
g.23354591C>T
SACS(NM_001278055.1):c.1580G>A (p.(Gly527Asp))
-
SACS_000364
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/?
1
8
c.2060del
r.(?)
p.(Asp687Valfs*27)
-
pathogenic
g.23928691del
g.23354552del
31760 delT, g.56688delA
-
SACS_000016
in ARM-rpt
PubMed: Ouyang 2006
-
-
Germline
-
-
-
-
-
Bernard Brais
+?/+?
2
8
c.2076del
r.(?)
p.(Ser693Glnfs*21)
-
likely pathogenic
g.23928676del
g.23354537del
2076delG (p.Thr692Thrfs*713)
-
SACS_000184
-
PubMed: Pyle 2012
-
-
Germline
-
-
-
-
-
Bernard Brais
-/., -?/.
6
-
c.2080G>A
r.(?)
p.(Ala694Thr)
-
benign, likely benign
g.23928671C>T
g.23354532C>T
SACS(NM_014363.5):c.2080G>A (p.A694T), SACS(NM_014363.6):c.2080G>A (p.A694T)
-
SACS_000251
1 homozygous;
Clinindb (India)
, 30 heterozygous;
Clinindb (India)
,
1 more item
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs17325713
CLASSIFICATION record, Germline
-
1/2795 individuals, 30/2795 individuals
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
,
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
,
Mohammed Faruq
+/?
1
8i
c.2094-2A>G
r.spl
p.?
-
pathogenic
g.23928017T>C
g.23353878T>C
2094-2 A>G, g.56725 A>G
-
SACS_000039
in ARM-rpt-SK
PubMed: Vermeer 2008
-
-
Germline
-
-
-
-
-
Bernard Brais
+/.
1
9
c.2115_2116insC
r.(?)
p.(Gly706Argfs*7)
-
pathogenic
g.23927993_23927994insG
g.23353854_23353855insG
-
-
SACS_000201
homozygosity mapping (3.2 Mb region)
PubMed: Faruq 2014
,
Journal: Faruq 2014
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
-?/.
3
-
c.2146C>T
r.(?)
p.(His716Tyr)
-
likely benign
g.23927963G>A
g.23353824G>A
1 more item
-
SACS_000345
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
+/., +/?
4
9
c.2182C>T
r.(?)
p.(Arg728*), p.(Arg728Ter)
-
pathogenic
g.23927927G>A
g.23353788G>A
NM_014363: c.C2182T; p.R728X, p.Arg728*, p.Arg728X, g.57452C>T
-
SACS_000106
in ARM-rpt, VKGL data sharing initiative Nederland
PubMed: Karaca 2015
,
PubMed: Synofzik 2013
,
PubMed: Vermeer 2008
-
-
CLASSIFICATION record, Germline, Unknown
?
-
-
-
-
Johan den Dunnen
,
Bernard Brais
,
VKGL-NL_Nijmegen
+/?
1
9i
c.2185+1del
r.spl
p.?
-
pathogenic
g.23927924del
g.23353785del
1891+1delG (erratum)
-
SACS_000107
in ARM rpt
PubMed: Vermeer 2008
-
-
Germline
-
-
-
-
-
Bernard Brais
+/?
1
9i_10_
c.2185+7748_9863del
r.spl
p.(Gly729_Trp3278del)
-
pathogenic
g.23908152_23920176del
-
2185+7748_9863del12Kb
-
SACS_000116
1 more item
PubMed: Prodi 2013
-
-
De novo
-
-
-
-
-
Bernard Brais
-?/.
1
-
c.2186-93_2186-91del
r.(=)
p.(=)
-
likely benign
g.23915943_23915945del
-
SACS(NM_014363.6):c.2186-93_2186-91delAAA
-
SACS_000402
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/+
1
9i_10_
c.(2185+1_2186-1)_(*1_?)dup
r.?
p.?
-
pathogenic
g.(?_23904274)_(23915830_23927923)dup
-
exon10 dup
-
SACS_000186
-
PubMed: Mignarri 2014
-
-
Unknown
?
-
-
-
-
Bernard Brais
+/?
2
10
c.2224C>T
r.(?)
p.(Arg742*)
-
pathogenic
g.23915791G>A
g.23341652G>A
2224C>T(p.R472X), p.Arg742X
-
SACS_000061
-
PubMed: Romano 2013
,
PubMed: Terracciano 2010
-
-
Germline, Unknown
?
-
-
-
-
Bernard Brais
?/.
1
-
c.2225G>A
r.(?)
p.(Arg742Gln)
-
VUS
g.23915790C>T
-
SACS(NM_014363.6):c.2225G>A (p.(Arg742Gln))
-
SACS_000434
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
-
c.2234G>A
r.(?)
p.(Arg745His)
-
VUS
g.23915781C>T
g.23341642C>T
SACS(NM_014363.5):c.2234G>A (p.R745H)
-
SACS_000250
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/., ?/.
2
-
c.2294A>G
r.(?)
p.(Tyr765Cys)
-
likely benign, VUS
g.23915721T>C
g.23341582T>C
SACS(NM_014363.5):c.2294A>G (p.Y765C, p.(Tyr765Cys))
-
SACS_000344
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
?/.
1
-
c.2359T>C
r.(?)
p.(Tyr787His)
-
VUS
g.23915656A>G
-
SACS(NM_014363.6):c.2359T>C (p.(Tyr787His))
-
SACS_000410
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/?
2
10
c.2387del
r.(?)
p.(Leu796Tyrfs*13)
-
pathogenic
g.23915630del
g.23341491del
2387delT p.Leu796Tyrfs*13
-
SACS_000159
-
PubMed: Synofzik 2013
-
-
Unknown
?
-
-
-
-
Bernard Brais
+/?
1
10
c.2405T>C
r.(?)
p.(Leu802Pro)
-
pathogenic
g.23915610A>G
g.23341471A>G
g.2405T>C L802P, g.69769T>C
-
SACS_000050
-
PubMed: Kamada 2008
-
-
Germline
-
-
-
-
-
Bernard Brais
+/.
1
-
c.2543_2561del
r.(?)
p.(Gly848Aspfs*2)
-
pathogenic
g.23915454_23915472del
-
SACS(NM_014363.6):c.2543_2561delGAGGGTTTGTCCTTAAAAA (p.G848Dfs*2)
-
SACS_000405
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
-?/.
2
-
c.2580A>G
r.(?)
p.(Gln860=)
-
likely benign
g.23915435T>C
g.23341296T>C
SACS(NM_014363.5):c.2580A>G (p.Q860=), SACS(NM_014363.6):c.2580A>G (p.Q860=)
-
SACS_000249
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
?/.
1
-
c.2708T>C
r.(?)
p.(Leu903Pro)
-
VUS
g.23915307A>G
g.23341168A>G
-
-
SACS_000343
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/.
1
-
c.2721G>A
r.(?)
p.(Leu907=)
-
benign
g.23915294C>T
g.23341155C>T
-
-
SACS_000363
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/.
2
-
c.2776A>G
r.(?)
p.(Ile926Val)
-
likely benign
g.23915239T>C
g.23341100T>C
SACS(NM_014363.5):c.2776A>G (p.I926V), SACS(NM_014363.6):c.2776A>G (p.I926V)
-
SACS_000342
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
?/.
1
-
c.2785C>T
r.(?)
p.(Arg929Cys)
-
VUS
g.23915230G>A
g.23341091G>A
SACS(NM_014363.6):c.2785C>T (p.R929C)
-
SACS_000248
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-?/.
1
-
c.2825C>T
r.(?)
p.(Thr942Ile)
-
likely benign
g.23915190G>A
g.23341051G>A
SACS(NM_014363.5):c.2825C>T (p.T942I)
-
SACS_000362
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
-
c.2829dup
r.(?)
p.(Phe797IlefsTer2)
-
pathogenic (recessive)
g.23915627dup
g.23341488dup
NM_001278055:c.2388dupA
-
SACS_000423
-
PubMed: Salinas 2020
SUB7803129
-
Germline
-
-
-
-
-
Johan den Dunnen
+/?
1
10
c.2881C>T
r.(?)
p.(Arg961*)
-
pathogenic
g.23915134G>A
g.23340995G>A
2881C>T
-
SACS_000132
-
PubMed: Prodi 2013
-
-
Unknown
?
-
-
-
-
Bernard Brais
-?/.
1
-
c.2929C>T
r.(?)
p.(Leu977=)
-
likely benign
g.23915086G>A
g.23340947G>A
SACS(NM_014363.6):c.2929C>T (p.L977=)
-
SACS_000247
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+/?
2
10
c.2938_2939del
r.(?)
p.(Met980Valfs*10)
-
pathogenic
g.23915076_23915077del
g.23340937_23340938del
-
-
SACS_000183
-
-
-
-
Germline
?
-
-
-
-
Bernard Brais
+/?, ?/.
2
10
c.2971T>C
r.(?)
p.(Cys991Arg)
-
pathogenic, VUS
g.23915044A>G
g.23340905A>G
p.Cys991Arg
-
SACS_000101
VKGL data sharing initiative Nederland
PubMed: Baets 2010
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Bernard Brais
,
VKGL-NL_Nijmegen
-?/., ?/?
3
10
c.2983G>T
r.(?)
p.(Val995Phe)
-
likely benign, VUS
g.23915032C>A
g.23340893C>A
2983G>T p.Val995Phe,
1 more item
-
SACS_000169
VKGL data sharing initiative Nederland
PubMed: Synofzik 2013
-
-
CLASSIFICATION record, Unknown
?
-
-
-
-
Bernard Brais
,
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
-?/.
2
-
c.2988A>G
r.(?)
p.(Leu996=)
-
likely benign
g.23915027T>C
g.23340888T>C
SACS(NM_014363.5):c.2988A>G (p.L996=), SACS(NM_014363.6):c.2988A>G (p.L996=)
-
SACS_000341
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
?/.
1
-
c.2996T>C
r.(?)
p.(Ile999Thr)
-
VUS
g.23915019A>G
g.23340880A>G
-
-
SACS_000291
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
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