Global Variome shared LOVD
MKS1 (Meckel syndrome, type 1)
LOVD v.3.0 Build 30b [
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Curator:
Jonna Tallila
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Unique variants in the MKS1 gene
The variants shown are described using the NM_017777.3 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'
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134 entries on 2 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
-?/.
1
-
c.-267T>A
r.(?)
p.(=)
-
likely benign
g.56296858A>T
g.58219497A>T
MKS1(NM_001165927.1):c.15T>A (p.V5=)
-
MKS1_000060
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.-251C>G
r.(?)
p.(=)
-
VUS
g.56296842G>C
-
MKS1(NM_001165927.1):c.31C>G (p.(Arg11Gly))
-
MKS1_000120
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
1
-
c.-216A>G
r.(?)
p.(=)
-
likely benign
g.56296807T>C
-
MKS1(NM_017777.4):c.-216A>G
-
MKS1_000136
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/.
1
-
c.-18C>G
r.(?)
p.(=)
-
benign
g.56296609G>C
g.58219248G>C
MKS1(NM_017777.3):c.-18C>G
-
MKS1_000059
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
1
c.-17C>G
r.(?)
p.?
-
VUS
g.56296608G>C
-
c.-17C>G
-
MKS1_000115
C not found at position 59, found G instead.
PubMed: Abu-Safieh-2012
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/.
2
_1_1i
c.-75_(80+1_81-1){0}
r.0?
p.0?
-
likely pathogenic (recessive), pathogenic (recessive)
g.(56296091_56296511)_(56296666_?)del
g.(58218730_58219150)_(58219305_?)del
del ex1, deletion exon 1
-
MKS1_000091
0/96 ethnically matched controls
PubMed: Abu-Safieh-2012
,
PubMed: Maddirevula 2018
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/.
4
8
c.?
r.(?), r.?
p.?
-
likely pathogenic, pathogenic
g.?
-
496C>T (Arg166Cys), c.857T/C, V339M
-
MYH2_000008
-
PubMed: Costa 2017
,
PubMed: Leitch-2008
,
PubMed: Otto 2011
-
-
Germline
-
0/96 ethnically matched controls
-
-
-
LOVD
?/.
1
-
c.10A>T
r.(?)
p.(Thr4Ser)
-
VUS
g.56296582T>A
g.58219221T>A
MKS1(NM_017777.3):c.10A>T (p.(Thr4Ser))
-
MKS1_000058
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
2
-
c.12C>T
r.(?)
p.(Thr4=)
-
likely benign
g.56296580G>A
g.58219219G>A
MKS1(NM_017777.3):c.12C>T (p.T4=), MKS1(NM_017777.4):c.12C>T (p.T4=)
-
MKS1_000057
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
?/.
1
-
c.25G>C
r.(?)
p.(Asp9His)
-
VUS
g.56296567C>G
-
MKS1(NM_017777.3):c.25G>C (p.(Asp9His))
-
MKS1_000110
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
3
-
c.27C>T
r.(?)
p.(Asp9=)
-
likely benign
g.56296565G>A
g.58219204G>A
MKS1(NM_017777.3):c.27C>T (p.D9=), MKS1(NM_017777.4):c.27C>T (p.D9=)
-
MKS1_000085
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
,
VKGL-NL_AMC
+/.
2
2
c.51_55dup
r.(51_55dup)
p.(Asp19Alafs*36), p.(Asp19AlafsTer36)
-
pathogenic (recessive)
g.56296537_56296541dup
g.58219176_58219180dup
50insCCGGG
-
MKS1_000005
-
PubMed: Kyttala 2006
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Anne Polvi
+/., +?/.
3
1
c.55G>T
r.(?)
p.(Asp19Tyr)
-
likely pathogenic, pathogenic
g.56296537C>A
g.58219176C>A
MKS1 c.55G>T, p.D19Y, NM_017777.3:c.55G>T
-
MKS1_000027
homozygous
PubMed: Bachmann-Gagescu 2015
,
PubMed: Slaats 2016
-
-
Germline
yes
-
-
-
-
Hester Y. Kroes
+/., +?/.
2
2
c.80+2T>C
r.sp, r.spl
p.?
-
likely pathogenic (recessive), pathogenic (recessive)
g.56296510A>G
g.58219149A>G
IVS1+2T>C
-
MKS1_000006
affects splicing
PubMed: Kyttala 2006
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Anne Polvi
-/.
1
-
c.81-40T>G
r.(?)
p.(=)
-
benign
g.56296130A>C
g.58218769A>C
-
-
MKS1_000135
-
PubMed: Khaddour 2007
-
-
Germline
-
1/202
-
-
-
Johan den Dunnen
?/.
1
-
c.83T>C
r.(?)
p.(Val28Ala)
-
VUS
g.56296088A>G
-
MKS1(NM_017777.4):c.83T>C (p.(Val28Ala))
-
MKS1_000141
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-/.
1
-
c.118C>T
r.(?)
p.(His40Tyr)
-
benign
g.56296053G>A
g.58218692G>A
MKS1(NM_017777.3):c.118C>T (p.H40Y)
-
MKS1_000056
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
?/.
1
-
c.139C>T
r.(?)
p.(Leu47Phe)
-
VUS
g.56296032G>A
g.58218671G>A
MKS1(NM_017777.3):c.139C>T (p.L47F)
-
MKS1_000097
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
2
c.157dup
r.(?)
p.(Asp53Glyfs*6)
-
pathogenic
g.56296014dup
g.58218653dup
-
-
MKS1_000026
-
-
-
-
Germline
-
-
-
-
-
Hester Y. Kroes
+?/.
1
-
c.157dupG
r.(?)
p.(Asp53Glyfs*6)
-
likely pathogenic
g.56296014dup
g.58218653dup
MKS1 c.157dupG, p.D53Gfs*6
-
MKS1_000026
heterozygous
PubMed: Slaats 2016
-
-
Germline
yes
-
-
-
-
LOVD
?/.
1
-
c.167C>G
r.(?)
p.(Thr56Ser)
-
VUS
g.56296004G>C
g.58218643G>C
-
-
MKS1_000064
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/?
1
2
c.184A>G
r.(?)
p.(Thr62Ala)
-
pathogenic
g.56295987T>C
g.58218626T>C
-
-
MKS1_000003
-
PubMed: Abu-Safieh-2013
-
-
Germline
yes
-
-
-
-
Leen Abu Safieh
+/.
2
3
c.184_190del
r.spl?, r.[(184_190del, spl?)]
p.(Thr62ValfsTer14), p.[(Thr62Valfs*14, spl?])
-
pathogenic (recessive)
g.56295985_56295991del
g.58218624_58218630del
184_190del7, c.184_190del7
-
MKS1_000013
deletion last nucleotides exon 3
PubMed: Khaddour 2007
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Anne Polvi
+/.
1
-
c.191-1G>A
r.191delG
p.(Ser64Metfs*12)
ACMG
pathogenic
g.56294098C>T
g.58216737C>T
MKS1 c.191-1G>A, p.(S64Mfs*12)
-
MKS1_000129
heterozygous, confirmed on mRNA level
PubMed: Luo 2020
-
-
Germline
yes
-
-
-
-
LOVD
?/.
1
-
c.199C>T
r.(?)
p.(Arg67Cys)
-
VUS
g.56294089G>A
g.58216728G>A
-
-
MKS1_000082
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
-
c.200G>A
r.(?)
p.(Arg67His)
-
VUS
g.56294088C>T
g.58216727C>T
MKS1(NM_017777.3):c.200G>A (p.R67H)
-
MKS1_000096
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/., -?/.
4
-
c.213C>G
r.(?)
p.(Asp71Glu)
-
benign, likely benign
g.56294075G>C
g.58216714G>C
1 more item
-
MKS1_000055
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
,
VKGL-NL_AMC
+?/.
1
-
c.240G>T
r.(?)
p.(Trp80Cys)
-
likely pathogenic
g.56294048C>A
g.58216687C>A
MKS1 c.240G>T, p.(Trp80Cys)
-
MKS1_000126
heterozygous
PubMed: Bader 2016
-
-
Germline/De novo (untested)
?
-
-
-
-
LOVD
-?/.
2
-
c.261+7C>T
r.(=)
p.(=)
-
likely benign
g.56294020G>A
g.58216659G>A
MKS1(NM_017777.3):c.261+7C>T
-
MKS1_000081
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
+/., +?/.
6
3i
c.262-179_262-37del
r.(=), r.262_417del, r.[262_417del,262_417delins418-60_418-1]
p.(=), p.(Phe88_Glu139del), p.[Phe88_Glu139del,Phe88fs]
-
likely pathogenic, pathogenic, pathogenic (recessive)
g.56293641_56293783del
g.58216280_58216422del
262-37_179del, MKS1 c.262-179_262-37del, p.(Phe88_Glu139del), MKS1 c.262-37_179del, p.F88_E139del,
1 more item
-
MKS1_000019
heterozygous, homozygous
PubMed: Bachmann-Gagescu 2015
,
PubMed: Bader 2016
,
PubMed: Frank 2007
,
PubMed: Slaats 2016
-
-
Germline, SUMMARY record
?, yes
-
-
-
-
Anne Polvi
,
Hester Y. Kroes
?/.
1
3
c.262-27A>G
r.(?)
p.(=)
-
VUS
g.56293631T>C
g.58216270T>C
232-27A>G
-
MKS1_000113
-
PubMed: Abu-Safieh-2012
-
-
Germline
-
-
-
-
-
LOVD
+/.
2
3i
c.262-2A>G
r.spl?
p.?
-
pathogenic
g.56293606T>C
g.58216245T>C
c.262-2A>G (p.?)
-
MKS1_000031
-
PubMed: Watson 2016
,
PubMed: Watson 2016
,
Journal: Watson 2016
-
-
Germline
-
-
-
-
-
Christopher Watson
?/.
1
-
c.281A>C
r.(?)
p.(Gln94Pro)
-
VUS
g.56293585T>G
g.58216224T>G
MKS1(NM_017777.3):c.281A>C (p.(Gln94Pro))
-
MKS1_000080
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+?/.
1
-
c.323G>A
r.(?)
p.(Arg108His)
-
likely pathogenic
g.56293543C>T
g.58216182C>T
MKS1(NM_017777.3): c.1601G>A(p.R534Q)/c.323G>A(p.R108H)
-
MKS1_000121
-
PubMed: Sun 2018
-
-
Germline/De novo (untested)
?
228
-
-
-
LOVD
+/.
1
-
c.367C>T
r.(?)
p.(Arg123*)
-
likely pathogenic (recessive)
g.56293499G>A
g.58216138G>A
NM_017777.3:c.367C>T:p.(Arg123*)
-
MKS1_000095
-
PubMed: Maddirevula 2018
-
-
Germline
-
-
-
-
-
LOVD
+?/., -?/., ?/.
6
4
c.368G>A
r.(?)
p.(Arg123Gln)
-
likely benign, likely pathogenic, VUS
g.56293498C>T
g.58216137C>T
MKS1(NM_017777.3):c.368G>A (p.R123Q), MKS1(NM_017777.4):c.368G>A (p.R123Q), R123Q
-
MKS1_000054
VKGL data sharing initiative Nederland
PubMed: Leitch-2008
-
-
CLASSIFICATION record, Germline
-
0/96 ethnically matched controls
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
,
VKGL-NL_VUmc
,
VKGL-NL_AMC
+/.
1
-
c.370C>T
r.(?)
p.(Arg124Ter)
ACMG
pathogenic (recessive)
g.56293496G>A
g.58216135G>A
-
-
MKS1_000127
ACMG PVS1, PM2, PM3, PP3
PubMed: Marinakis 2021
-
-
Germline
-
-
-
-
-
Jan Traeger-Synodinos
+/., +?/.
4
4
c.381del
r.(?)
p.(Tyr128Thrfs*17)
-
likely pathogenic, pathogenic
g.56293486del
g.58216125del
MKS1 c.381delC, p.Y128Tfs*17, NM_017777.3:c.381delC
-
MKS1_000025
heterozygous
PubMed: Bachmann-Gagescu 2015
,
PubMed: Slaats 2016
-
-
De novo, Germline
-, yes
-
-
-
-
Hester Y. Kroes
+/.
2
5
c.392_393del
r.(392_393del), r.(?)
p.(Ser131*)
-
pathogenic (recessive)
g.56293475_56293476del
g.58216114_58216115del
392_393delCT, 392_393delCT (Ser131X)
-
MKS1_000020
-
PubMed: Tallila 2008
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Anne Polvi
?/.
1
-
c.397A>G
r.(?)
p.(Arg133Gly)
-
VUS
g.56293469T>C
g.58216108T>C
MKS1(NM_017777.3):c.397A>G (p.R133G)
-
MKS1_000079
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/., +?/., ?/.
19
4
c.417G>A
r.(=), r.(?), r.262_417del, r.338_417del, r.spl, r.spl?
p.(=), p.(Glu139=), p.(Glu139=, Phe88_Glu139del), p.(Glu139=,?), p.?, p.Lys113ThrfsTer59,
1 more item
-
likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive), VUS
g.56293449C>T
g.58216088C>T
417G>A, c.417G>A, c.417G>A, p.Glu139=, E139E, MKS1 c.1208C>T, p.S403L,
2 more items
-
MKS1_000007, MKS1_000024
heterozygous, RNA exon 4 skipping, VKGL data sharing initiative Nederland
PubMed: Bachmann-Gagescu 2015
,
PubMed: Brooks 2018
,
PubMed: Consugar 2007
,
PubMed: Frank 2007
,
4 more items
-
rs386834048
CLASSIFICATION record, Germline, SUMMARY record, Unknown
?, yes
-
-
-
-
Johan den Dunnen
,
Anne Polvi
,
Hester Y. Kroes
,
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
,
VKGL-NL_VUmc
,
VKGL-NL_AMC
,
Isabel Filges
-?/.
1
-
c.417+10T>C
r.(=)
p.(=)
-
likely benign
g.56293439A>G
-
MKS1(NM_017777.3):c.417+10T>C
-
MKS1_000109
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
2
-
c.424C>T
r.(424c>u)
p.(Gln142*), p.(Gln142Ter)
-
pathogenic (recessive)
g.56292193G>A
g.58214832G>A
-
-
MKS1_000014
-
PubMed: Khaddour 2007
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Anne Polvi
+/., +?/.
4
6i
c.472C>T
r.(472c>u), r.(?), r.472c>u)
p.(Arg158*), p.(Arg158Ter)
ACMG
pathogenic, pathogenic (recessive)
g.56292145G>A
g.58214784G>A
MKS1 NM_017777: c.472C>T; p.(Arg158*)
-
MKS1_000015
heterozygous, confirmed on mRNA level
PubMed: Brunetti-Pierri 2021
,
PubMed: Khaddour 2007
-
-
Germline, SUMMARY record
yes
-
sbanfi
-
-
Johan den Dunnen
,
Anne Polvi
,
Sandro Banfi
?/.
2
5
c.485+12C>T
r.spl?
p.?
-
VUS
g.56292120G>A
-
c.485+12C>T
-
MKS1_000112
-
PubMed: Abu-Safieh-2012
, Abu-Safieh 2010
-
-
Germline
-
-
-
-
-
LOVD
?/.
1
-
c.491G>A
r.(?)
p.(Arg164His)
-
VUS
g.56292126C>T
-
MKS1(NM_017777.4):c.491G>A (p.(Arg164His))
-
MKS1_000145
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/., +?/.
3
5
c.493C>T
r.(?)
p.(Arg165Cys)
-
likely pathogenic, pathogenic
g.56292124G>A
g.58214763G>A
c.493C>T, c.493C>T; p.R165C, NM_017777.3:c.493C>T
-
MKS1_000100
-
PubMed: Bachmann-Gagescu 2015
,
PubMed: Brooks 2018
,
PubMed: Summers 2017
-
-
Germline
?
-
-
-
-
LOVD
+/., +?/.
2
6
c.496C>T
r.(496c>u), r.(?)
p.(Arg166Trp)
-
likely pathogenic (recessive), pathogenic (recessive)
g.56292121G>A
g.58214760G>A
p.Arg166Trp
-
MKS1_000021
-
PubMed: Tallila 2008
-
rs201845154
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Anne Polvi
?/.
1
5
c.511G>A
r.(?)
p.(Gly171Arg)
-
VUS
g.56292106C>T
-
c.511G>A
-
MKS1_000118
-
PubMed: Knopp 2015
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/.
2
5i
c.515+1G>A
r.spl
p.?
-
likely pathogenic (recessive), pathogenic (recessive)
g.56292101C>T
g.58214740C>T
IVS5+1G>A
-
MKS1_000017
-
PubMed: Frank 2007
-
-
Germline, SUMMARY record
-
-
-
-
-
Anne Polvi
-?/.
1
-
c.515+8C>T
r.(=)
p.(=)
-
likely benign
g.56292094G>A
g.58214733G>A
MKS1(NM_017777.3):c.515+8C>T
-
MKS1_000094
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/., ?/.
2
-
c.515+12C>T
r.(=), r.(?)
p.(=)
-
benign, VUS
g.56292090G>A
g.58214729G>A
MKS1(NM_017777.3):c.515+12C>T
-
MKS1_000052
VKGL data sharing initiative Nederland
PubMed: Khaddour 2007
-
-
CLASSIFICATION record, Germline
-
2/236
-
-
-
Johan den Dunnen
,
VKGL-NL_Rotterdam
-?/.
3
-
c.516-10T>C
r.(=)
p.(=)
-
likely benign
g.56291758A>G
g.58214397A>G
MKS1(NM_017777.3):c.516-10T>C, MKS1(NM_017777.4):c.516-10T>C
-
MKS1_000051
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
-/., ?/.
2
-
c.544G>A
r.(?)
p.(Val182Ile)
-
benign, VUS
g.56291720C>T
g.58214359C>T
MKS1(NM_017777.3):c.544G>A (p.V182I), MKS1(NM_017777.4):c.544G>A (p.V182I)
-
MKS1_000078
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
,
VKGL-NL_AMC
-?/.
3
-
c.644+8G>T
r.(=)
p.(=)
-
likely benign
g.56291612C>A
g.58214251C>A
MKS1(NM_017777.3):c.644+8G>T, MKS1(NM_017777.4):c.644+8G>T
-
MKS1_000090
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
,
VKGL-NL_AMC
-?/.
1
-
c.644+10G>A
r.(=)
p.(=)
-
likely benign
g.56291610C>T
-
MKS1(NM_017777.4):c.644+10G>A
-
MKS1_000140
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
1
-
c.729G>A
r.(?)
p.(Thr243=)
-
likely benign
g.56291146C>T
g.58213785C>T
MKS1(NM_017777.3):c.729G>A (p.T243=)
-
MKS1_000089
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.749+13A>G
r.(=)
p.(=)
-
likely benign
g.56291113T>C
g.58213752T>C
MKS1(NM_017777.3):c.749+13A>G
-
MKS1_000077
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.814G>A
r.(?)
p.(Ala272Thr)
-
likely benign
g.56290387C>T
-
MKS1(NM_001165927.1):c.784G>A (p.(Ala262Thr))
-
MKS1_000108
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
1
-
c.822G>A
r.(?)
p.(Pro274=)
-
likely benign
g.56290379C>T
g.58213018C>T
MKS1(NM_017777.4):c.822G>A (p.P274=)
-
MKS1_000075
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
1
8
c.829G>T
r.(?)
p.(Glu277*)
-
pathogenic
g.56290372C>A
g.58213011C>A
-
-
MKS1_000002
-
-
-
-
Unknown
-
-
-
-
-
Gerard C.P. Schaafsma
+?/., ?/.
9
8
c.857A>G
r.(?)
p.(Asp286Gly)
ACMG
likely pathogenic, VUS
g.56290344T>C
g.58212983T>C
D286G, MKS1 c.857A>G, p.(Asp286Gly),
1 more item
-
MKS1_000063
single heterozygous variant (recessive), VKGL data sharing initiative Nederland
PubMed: Jespersgaar 2019
,
PubMed: Leitch-2008
,
PubMed: Tiwari 2016
-
-
CLASSIFICATION record, Germline
?
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
,
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
,
VKGL-NL_AMC
-/., -?/.
5
8i
c.858+9A>G
r.(=), r.(?)
p.(=)
-
benign, likely benign
g.56290334T>C
g.58212973T>C
c.858+9A>G, MKS1(NM_017777.3):c.858+9A>G, MKS1(NM_017777.4):c.858+9A>G
-
MKS1_000050
VKGL data sharing initiative Nederland
PubMed: Khaddour 2007
,
PubMed: Knopp 2015
-
-
CLASSIFICATION record, Germline
-
79/232
-
-
-
Johan den Dunnen
,
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
?/.
2
-
c.868C>T
r.(?)
p.(Arg290Trp)
-
VUS
g.56289786G>A
g.58212425G>A
MKS1(NM_017777.3):c.868C>T (p.R290W), MKS1(NM_017777.4):c.868C>T (p.R290W)
-
MKS1_000049
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
,
VKGL-NL_AMC
?/.
2
9
c.869G>A
r.(?)
p.(Arg290Gln)
-
VUS
g.56289785C>T
g.58212424C>T
G869A, MKS1(NM_017777.4):c.869G>A (p.(Arg290Gln))
-
MKS1_000102
VKGL data sharing initiative Nederland
PubMed: Katagiri 2014
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
VKGL-NL_Leiden
-?/.
1
-
c.874A>G
r.(?)
p.(Lys292Glu)
-
likely benign
g.56289780T>C
-
MKS1(NM_017777.3):c.874A>G (p.K292E)
-
MKS1_000130
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+?/.
1
-
c.915+4A>T
r.spl?
p.?
-
likely pathogenic
g.56289735T>A
-
MKS1(NM_017777.4):c.915+4A>T
-
MKS1_000139
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
-/.
2
-
c.915+19_915+21del
r.(=), r.(?)
p.(=)
-
benign
g.56289719_56289721del
g.58212358_58212360del
915+19_915+21delTGC, MKS1(NM_017777.3):c.915+19_915+21delTGC
-
MKS1_000048
VKGL data sharing initiative Nederland
PubMed: Khaddour 2007
-
-
CLASSIFICATION record, Germline
-
6/182
-
-
-
Johan den Dunnen
,
VKGL-NL_Rotterdam
-/.
1
-
c.916-119G>A
r.(=)
p.(=)
-
benign
g.56288502C>T
g.58211141C>T
MKS1(NM_017777.3):c.916-119G>A
-
MKS1_000047
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/., +?/.
4
10
c.950G>A
r.(?)
p.(Gly317Gln), p.(Gly317Glu)
-
likely pathogenic, pathogenic
g.56288349C>T
g.58210988C>T
c.950G>A; p.G317Q, MKS1 c.950G>A, p.G317E, NM_017777.3:c.950G>A
-
MKS1_000023
heterozygous
PubMed: Bachmann-Gagescu 2015
,
PubMed: Brooks 2018
,
PubMed: Slaats 2016
-
-
Germline, Unknown
?, yes
-
-
-
-
Hester Y. Kroes
+/., +?/.
3
11, 11i
c.958G>A
r.(?), r.spl?, r.[958g>a, spl?]
p.?, p.Val320Ile, p.[Val320Ile, ?]
-
likely pathogenic (recessive), pathogenic, pathogenic (recessive)
g.56288341C>T
g.58210980C>T
MKS1 c.958G>A (p.Val320Ile)
-
MKS1_000016
double homozygous, variant last nucleotide exon 11, affects splicing
PubMed: Khaddour 2007
,
PubMed: Tallapaka 2019
-
-
Germline, SUMMARY record
yes
-
-
-
-
Johan den Dunnen
,
Anne Polvi
?/.
1
-
c.1010A>G
r.(?)
p.(Glu337Gly)
-
VUS
g.56288034T>C
g.58210673T>C
MKS1(NM_001165927.1):c.980A>G (p.(Glu327Gly))
-
MKS1_000046
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
1
-
c.1014G>A
r.(?)
p.(Leu338=)
-
likely benign
g.56288030C>T
-
MKS1(NM_017777.4):c.1014G>A (p.L338=)
-
MKS1_000119
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/., +?/.
2
11i
c.1024+1G>A
r.(959_1024del)
p.(Val320_His342delinsAsp)
-
likely pathogenic (recessive), pathogenic (recessive)
g.56288019C>T
g.58210658C>T
IVS11+1G>A
-
MKS1_000008
-
PubMed: Consugar 2007
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Anne Polvi
+/.
3
13
c.1048C>T
r.(1048c>u), r.(?)
p.(Gln350*), p.(Gln350Ter)
-
pathogenic (recessive)
g.56285921G>A
g.58208560G>A
1048C>G (Q350X)
-
MKS1_000010
-
PubMed: Khaddour 2007
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Anne Polvi
+/.
1
-
c.1058del
r.(?)
p.(Gly353GlufsTer2)
ACMG
pathogenic
g.56285912del
g.58208551del
MKS1 c.1058delG , p.(Gly353GlufsTer2)
-
MKS1_000128
heterozygous
PubMed: Luo 2020
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
1
-
c.1086_1088del
r.(?)
p.(Leu363del)
-
likely pathogenic
g.56285881_56285883del
g.58208520_58208522del
MKS1 c.1085_1088delCCT, p.S362del
-
MKS1_000125
1 more item
PubMed: Romani 2014
-
-
Germline/De novo (untested)
?
-
-
-
-
LOVD
-/.
1
-
c.1096-32C>T
r.(?)
p.(=)
-
benign
g.56285567G>A
g.58208206G>A
-
-
MKS1_000134
-
PubMed: Khaddour 2007
-
-
Germline
-
1/164
-
-
-
Johan den Dunnen
+?/.
1
13
c.1112_1114del
r.(?)
p.(Phe371del)
-
likely pathogenic
g.56285517_56285519del
-
F371del
-
MKS1_000111
-
PubMed: Leitch-2008
-
-
Germline
-
0/96 ethnically matched controls
-
-
-
LOVD
+/., +?/.
18
13
c.1115_1117del
r.(?)
p.(Ser372del)
-
likely pathogenic, pathogenic
g.56285516_56285518del
g.58208155_58208157del
1115_1117delCCT, c.1115_1117del; p.S372del, MKS1 c.1115_1117delCCT, p.(Ser372del),
2 more items
-
MKS1_000022
heterozygous, homozygous, hypomorphic allele with variable expressivity; homozygous
PubMed: Bachmann-Gagescu 2015
,
PubMed: Brooks 2018
,
PubMed: Irfanullah 2016
,
PubMed: Slaats 2016
-
-
Germline, Unknown
?, yes
-
-
-
-
Hester Y. Kroes
,
Irfan Ullah
+/.
1
-
c.1122_1126dup
r.(?)
p.(Thr376Asnfs*56)
-
pathogenic
g.56285505_56285509dup
g.58208144_58208148dup
MKS1(NM_017777.3):c.1122_1126dupATTCA (p.T376Nfs*56)
-
MKS1_000093
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
3
-
c.1126dup
r.(?)
p.(Thr376Asnfs*3)
-
pathogenic (recessive)
g.56285505dup
g.58208144dup
1126dupA, 1126duplA, NM_017777.3:c.1126dupA:p.(Thr376Asnfs*3)
-
MKS1_000065
-
PubMed: Maddirevula 2018
,
PubMed: Shaheen 2013
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/., -?/.
2
-
c.1128G>A
r.(?)
p.(Thr376=)
-
benign, likely benign
g.56285503C>T
g.58208142C>T
MKS1(NM_017777.3):c.1128G>A (p.T376=), MKS1(NM_017777.4):c.1128G>A (p.T376=)
-
MKS1_000045
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
-?/.
1
-
c.1148A>G
r.(?)
p.(His383Arg)
-
likely benign
g.56285483T>C
g.58208122T>C
MKS1(NM_017777.3):c.1148A>G (p.H383R)
-
MKS1_000043
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
-
c.1181G>A
r.(?)
p.(Trp394Ter)
-
pathogenic
g.56285347C>T
g.58207986C>T
MKS1(NM_017777.3):c.1181G>A (p.W394*)
-
MKS1_000073
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.1196G>C
r.(?)
p.(Cys399Ser)
-
VUS
g.56285332C>G
g.58207971C>G
-
-
MKS1_000062
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/.
1
-
c.1196G>T
r.(?)
p.(Cys399Phe)
-
benign
g.56285332C>A
g.58207971C>A
MKS1(NM_017777.4):c.1196G>T (p.C399F)
-
MKS1_000072
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/., +?/.
5
14
c.1208C>T
r.(?)
p.(Ser403Leu)
-
likely pathogenic, pathogenic
g.56285320G>A
g.58207959G>A
c.1208C>T, c.1208C>T; p.S403L, MKS1 c.1208C>T, p.S403L, NM_017777.3:c.1208C>T
-
MKS1_000028
heterozygous
PubMed: Bachmann-Gagescu 2015
,
PubMed: Brooks 2018
,
PubMed: Slaats 2016
,
PubMed: Summers 2017
-
-
Germline
?, yes
-
-
-
-
Hester Y. Kroes
?/.
1
-
c.1232G>A
r.(?)
p.(Arg411His)
-
VUS
g.56285296C>T
-
MKS1(NM_017777.4):c.1232G>A (p.(Arg411His))
-
MKS1_000144
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+?/.
1
-
c.1261C>T
r.(?)
p.(Pro421Ser)
-
likely pathogenic
g.56285267G>A
g.58207906G>A
MKS1 c.625C>T, p.P218S
-
MKS1_000124
heterozygous
PubMed: Slaats 2016
-
-
Germline
yes
-
-
-
-
LOVD
?/.
1
-
c.1268C>T
r.(?)
p.(Thr423Ile)
-
VUS
g.56285260G>A
g.58207899G>A
-
-
MKS1_000098
-
PubMed: Comander 2017
-
-
Germline
-
-
-
-
-
LOVD
-/.
3
-
c.1273+11G>A
r.(=), r.(?)
p.(=)
-
benign
g.56285244C>T
g.58207883C>T
MKS1(NM_017777.3):c.1273+11G>A
-
MKS1_000042
VKGL data sharing initiative Nederland
PubMed: Khaddour 2007
-
-
CLASSIFICATION record, Germline
-
2/196
-
-
-
Johan den Dunnen
,
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
-/.
1
-
c.1273+39C>T
r.(=)
p.(=)
-
benign
g.56285216G>A
g.58207855G>A
MKS1(NM_017777.3):c.1273+39C>T
-
MKS1_000041
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
?/.
1
-
c.1279C>T
r.(?)
p.(His427Tyr)
-
VUS
g.56284574G>A
-
MKS1(NM_017777.3):c.1279C>T (p.H427Y)
-
MKS1_000107
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.1286T>A
r.(?)
p.(Leu429Gln)
-
VUS
g.56284567A>T
g.58207206A>T
MKS1(NM_017777.4):c.1286T>A (p.L429Q)
-
MKS1_000084
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-?/.
2
-
c.1287G>A
r.(?)
p.(Leu429=)
-
likely benign
g.56284566C>T
g.58207205C>T
MKS1(NM_017777.3):c.1287G>A (p.L429=)
-
MKS1_000092
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
+?/., -?/., ?/.
5
15
c.1349T>C
r.(?)
p.(Ile450Thr)
ACMG
likely benign, likely pathogenic, VUS
g.56284504A>G
g.58207143A>G
I450T, MKS1(NM_017777.3):c.1349T>C (p.I450T), MKS1(NM_017777.4):c.1349T>C (p.I450T),
1 more item
-
MKS1_000040
VKGL data sharing initiative Nederland
PubMed: Leitch-2008
,
PubMed: Sharon 2019
-
-
CLASSIFICATION record, Germline
-
0/96 ethnically matched controls, 1/2420 IRD families
-
-
-
Global Variome, with Curator vacancy
,
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
,
VKGL-NL_AMC
?/.
1
-
c.1370A>G
r.(?)
p.(Glu457Gly)
-
VUS
g.56284483T>C
g.58207122T>C
MKS1 c.1370A>G, p.Glu457Gly
-
MKS1_000117
heterozygous
PubMed: Zampaglione 2020
-
-
Unknown
?
-
-
-
-
LOVD
?/.
1
-
c.1372G>T
r.(?)
p.(Asp458Tyr)
-
VUS
g.56284481C>A
-
MKS1(NM_017777.4):c.1372G>T (p.(Asp458Tyr))
-
MKS1_000143
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
1
15
c.1382A>G
r.(?)
p.(Tyr461Cys)
-
pathogenic
g.56284471T>C
-
c.1382A>G
-
MKS1_000116
-
PubMed: Xing-2014
-
-
Germline
-
-
-
-
-
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