Global Variome shared LOVD
TMEM67 (transmembrane protein 67)
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Unique variants in the TMEM67 gene
The variants shown are described using the NM_153704.5 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.
253 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
8
c.?
r.(?)
p.(Gln467*), p.(Gly195ilefs*13), p.?
-
likely pathogenic, pathogenic
g.94792827_94792830delAATG, g.?
-
c.755T/C, MKS3:p.Gln467*, MKS3:p.Gly195ilefs*13
-
RP1_000000, TMEM67_000173
-
PubMed: Knopp 2015
,
PubMed: Otto 2011
-
-
Germline
-
-
-
-
-
LOVD
?/.
1
-
c.17G>A
r.(?)
p.(Gly6Glu)
-
VUS
g.94767159G>A
-
TMEM67(NM_153704.6):c.17G>A (p.(Gly6Glu))
-
TMEM67_000248
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/., -?/.
3
-
c.25G>A
r.(?)
p.(Val9Met)
-
likely benign, pathogenic (recessive)
g.94767167G>A
g.93754939G>A
TMEM67(NM_153704.5):c.25G>A (p.V9M, p.(Val9Met))
-
TMEM67_000051
VKGL data sharing initiative Nederland
PubMed: Consugar 2015
-
-
CLASSIFICATION record, Germline
yes
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
?/.
1
-
c.29C>T
r.(?)
p.(Ala10Val)
-
VUS
g.94767171C>T
g.93754943C>T
-
-
TMEM67_000070
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
-
c.37G>A
r.(?)
p.(Val13Ile)
-
VUS
g.94767179G>A
g.93754951G>A
TMEM67(NM_153704.6):c.37G>A (p.V13I)
-
TMEM67_000091
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+?/.
1
1
c.41G>A
r.(?)
p.(Trp14*)
-
likely pathogenic
g.94767183G>A
g.93754955G>A
-
-
TMEM67_000046
-
PubMed: Kroes 2016
-
-
Unknown
?
-
-
-
-
Sanne Savelberg
-?/.
1
-
c.48C>G
r.(?)
p.(Leu16=)
-
likely benign
g.94767190C>G
-
TMEM67(NM_153704.6):c.48C>G (p.L16=)
-
TMEM67_000222
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/.
1
-
c.66G>A
r.(?)
p.(Val22=)
-
likely benign
g.94767208G>A
g.93754980G>A
TMEM67(NM_153704.6):c.66G>A (p.V22=)
-
TMEM67_000052
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-?/.
1
-
c.114C>T
r.(?)
p.(Thr38=)
-
likely benign
g.94767256C>T
g.93755028C>T
TMEM67(NM_153704.5):c.114C>T (p.T38=)
-
TMEM67_000129
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/., -?/.
2
-
c.120T>C
r.(?)
p.(Ser40=)
-
benign, likely benign
g.94767262T>C
g.93755034T>C
TMEM67(NM_153704.5):c.120T>C (p.S40=), TMEM67(NM_153704.6):c.120T>C (p.S40=)
-
TMEM67_000053
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
?/.
1
-
c.123C>A
r.(?)
p.(Phe41Leu)
-
VUS
g.94767265C>A
-
TMEM67(NM_153704.5):c.123C>A (p.F41L)
-
TMEM67_000191
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
2
-
c.124C>G
r.(?)
p.(Pro42Ala)
-
VUS
g.94767266C>G
g.93755038C>G
TMEM67(NM_153704.6):c.124C>G (p.P42A, p.(Pro42Ala))
-
TMEM67_000054
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_AMC
?/.
1
-
c.125_130dup
r.(?)
p.(Pro42_Phe43dup)
-
VUS
g.94767267_94767272dup
-
TMEM67(NM_153704.6):c.125_130dup (p.(Pro42_Phe43dup))
-
TMEM67_000254
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+?/.
1
-
c.154_156del
r.(?)
p.(Asn52del)
-
VUS
g.94767296_94767298del
g.93755068_93755070del
149_151del
-
TMEM67_000251
-
PubMed: Boissel 2017
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., +?/.
2
1b
c.161A>G
r.(161a>g), r.(?)
p.(Tyr54Cys)
-
likely pathogenic (recessive), pathogenic (recessive)
g.94767303A>G
g.93755075A>G
-
-
TMEM67_000002
-
PubMed: Tallila 2008
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Anne Polvi
-/.
1
-
c.184T>C
r.(?)
p.(Cys62Arg)
-
benign
g.94767326T>C
g.93755098T>C
C62C
-
TMEM67_000224
-
PubMed: Khaddour 2007
-
-
Germline
-
2/200
-
-
-
Johan den Dunnen
-?/.
1
-
c.186T>C
r.(?)
p.(Cys62=)
-
likely benign
g.94767328T>C
g.93755100T>C
TMEM67(NM_153704.5):c.186T>C (p.C62=)
-
TMEM67_000055
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.194G>T
r.(?)
p.(Cys65Phe)
-
VUS
g.94767336G>T
g.93755108G>T
TMEM67(NM_153704.5):c.194G>T (p.C65F)
-
TMEM67_000056
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/.
1
-
c.224-4_224-3del
r.spl?
p.?
-
benign
g.94768002_94768003del
g.93755774_93755775del
TMEM67(NM_153704.5):c.224-4_224-3delTT
-
TMEM67_000057
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-?/.
1
-
c.224-3del
r.spl?
p.?
-
likely benign
g.94768003del
-
TMEM67(NM_153704.5):c.224-3del (p.?)
-
TMEM67_000249
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/., +?/.
2
1i
c.224-2A>T
r.(?), r.spl?
p.(?), p.?
-
likely pathogenic, pathogenic
g.94768004A>T
g.93755776A>T
c.224-2A>T
-
TMEM67_000172
-
PubMed: Brooks 2018
,
PubMed: Summers 2017
-
-
Germline
?
-
-
-
-
LOVD
+/.
2
1i
c.224-2del
r.224_312del
p.Gly75Glufs*15, p.Gly75GlufsTer15
-
pathogenic (recessive)
g.94768004del
g.93755776del
IVS1-2delA
-
TMEM67_000003
RNA skips exon 2
PubMed: Consugar 2007
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Anne Polvi
+/., +?/.
2
-
c.233G>A
r.(?)
p.(Cys78Tyr)
-
likely pathogenic (recessive), pathogenic
g.94768015G>A
g.93755787G>A
TMEM67 c.233G>A, p.(Cys78Tyr)
-
TMEM67_000217
heterozygous
PubMed: Barabino 2020
,
PubMed: Boissel 2017
-
-
Germline, In vitro (cloned)
?
-
-
-
-
Johan den Dunnen
+/., +?/.
2
-
c.244C>T
r.(?)
p.(Pro82Ser)
ACMG
likely pathogenic, pathogenic
g.94768026C>T
g.93755798C>T
NM_153704.5:c.244C>T
-
TMEM67_000150
-
Kim 2023 submitted,
PubMed: Bachmann-Gagescu 2015
ClinVar-217715
-
Germline, Germline/De novo (untested)
-
-
-
-
-
Hyeongmin Kim
+/., +?/.
9
2
c.245C>G
r.(?)
p.(Pro82Arg)
-
likely pathogenic, pathogenic
g.94768027C>G
g.93755799C>G
c.245C>G, c.245C>G; p.P82R, NM_153704.5:c.245C>G
-
TMEM67_000151
-
PubMed: Bachmann-Gagescu 2015
,
PubMed: Brooks 2018
,
PubMed: Summers 2017
-
-
Germline
?
-
-
-
-
LOVD
+?/.
1
2
c.270T>G
r.(?)
p.(Asn90Lys)
-
likely pathogenic (recessive)
g.94768052T>G
g.93755824T>G
-
-
TMEM67_000225
-
PubMed: Iannicelli 2010
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., ?/.
3
-
c.274G>A
r.(?)
p.(Gly92Arg)
-
pathogenic, VUS
g.94768056G>A
g.93755828G>A
NPHP11 c. 274G>A, p.Gly92Arg, NPHP11 c.274G>A, p.Gly92Arg p.
-
TMEM67_000132
heterozygous
PubMed: Kang 2016
,
PubMed: Riazuddin 2017
-
-
Germline, Unknown
?
-
-
-
-
Johan den Dunnen
+?/.
2
-
c.297G>T
r.(?)
p.(Lys99Asn)
-
likely pathogenic
g.94768079G>T
g.93755851G>T
c.297G>T, NM_153704.5:c.297G>T
-
TMEM67_000152
-
PubMed: Bachmann-Gagescu 2015
,
PubMed: Brooks 2018
-
-
Germline
?
-
-
-
-
LOVD
?/.
1
-
c.299G>A
r.(?)
p.(Cys100Tyr)
-
VUS
g.94768081G>A
-
TMEM67(NM_153704.5):c.299G>A (p.C100Y)
-
TMEM67_000134
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
2
2
c.300C>A
r.(?)
p.(Cys100*), p.(Cys100Ter)
-
pathogenic, pathogenic (recessive)
g.94768082C>A
g.93755854C>A
NM_153704.5:c.300C>A
-
TMEM67_000153
-
PubMed: Bachmann-Gagescu 2015
,
PubMed: Iannicelli 2010
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.312+2T>G
r.spl?
p.?
-
likely pathogenic
g.94768096T>G
g.93755868T>G
TMEM67(NM_153704.6):c.312+2T>G
-
TMEM67_000130
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
1
-
c.312+5G>A
r.224_312del
p.?
-
pathogenic
g.94768099G>A
g.93755871G>A
-
-
TMEM67_000141
-
PubMed: Suzuki 2016
-
-
Germline
-
-
-
-
-
LOVD
?/.
1
-
c.312+10A>G
r.(=)
p.(=)
-
VUS
g.94768104A>G
-
TMEM67(NM_153704.6):c.312+10A>G
-
TMEM67_000255
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
1
-
c.312+18T>A
r.(=)
p.(=)
-
likely benign
g.94768112T>A
-
TMEM67(NM_153704.5):c.312+18T>A
-
TMEM67_000213
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/.
1
2i
c.313-3T>G
r.313_316del
p.Lys105Valfs*16
-
likely pathogenic (recessive)
g.94770708T>G
g.93758480T>G
-
-
TMEM67_000125
-
PubMed: Bui 2020
,
Journal: Bui 2020
-
-
Germline
yes
-
-
-
-
Minh Tuan Huynh
?/.
1
-
c.316G>A
r.(?)
p.(Gly106Ser)
-
VUS
g.94770714G>A
g.93758486G>A
-
-
TMEM67_000071
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/., +?/.
3
3
c.329A>G
r.(?), r.328a>g
p.(Asp110Gly), p.Asp110Gly
-
likely pathogenic, pathogenic, pathogenic (recessive)
g.94770727A>G
g.93758499A>G
c.329A>G
-
TMEM67_000128
-
PubMed: Enokizono 2017
,
PubMed: Suzuki 2016
,
PubMed: Tsurusaki 2013
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/., -?/.
2
-
c.369C>T
r.(?)
p.(Ala123=)
-
benign, likely benign
g.94770767C>T
g.93758539C>T
TMEM67(NM_153704.5):c.369C>T (p.A123=), TMEM67(NM_153704.6):c.369C>T (p.A123=)
-
TMEM67_000058
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
?/.
2
3
c.370G>A
r.(?)
p.(Glu124Lys)
-
VUS
g.94770768G>A
g.93758540G>A
-
-
TMEM67_000226
-
PubMed: Iannicelli 2010
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
3a
c.383_384del
r.(383_384del), r.(?)
p.(His128Leufs*13)
-
pathogenic (recessive)
g.94770781_94770782del
g.93758553_93758554del
383-384delAC
-
TMEM67_000004
-
PubMed: Smith 2006
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Anne Polvi
+/.
1
-
c.385del
r.(?)
p.(Cys129Valfs*7)
-
pathogenic
g.94770783del
-
-
-
TMEM67_000218
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
2
3, 3a
c.387T>A
r.(387u>a), r.(?)
p.(Cys129*), p.(Cys129Ter)
-
pathogenic (recessive)
g.94770785T>A
g.93758557T>A
-
-
TMEM67_000037
-
PubMed: Iannicelli 2010
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Anne Polvi
+/.
1
-
c.389C>G
r.(?)
p.(Pro130Arg)
-
pathogenic
g.94770787C>G
g.93758559C>G
NM_153704.5:c.389C>G
-
TMEM67_000154
-
PubMed: Bachmann-Gagescu 2015
-
-
Germline
-
-
-
-
-
LOVD
+/.
1
-
c.395G>C
r.(?)
p.(Gly132Ala)
-
pathogenic
g.94770793G>C
g.93758565G>C
-
-
TMEM67_000142
-
PubMed: Suzuki 2016
-
-
Germline
-
-
-
-
-
LOVD
-?/.
1
-
c.406+1301G>A
r.(=)
p.(=)
-
likely benign
g.94772105G>A
g.93759877G>A
TMEM67(NM_001142301.1):c.47G>A (p.R16Q)
-
TMEM67_000095
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.406+1301G>T
r.(=)
p.(=)
-
likely benign
g.94772105G>T
-
TMEM67(NM_001142301.1):c.47G>T (p.R16L)
-
TMEM67_000214
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/.
1
-
c.406+1332C>T
r.(=)
p.(=)
-
benign
g.94772136C>T
g.93759908C>T
-
-
TMEM67_000072
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/.
1
-
c.406+1345dup
r.(=)
p.(=)
-
likely benign
g.94772149dup
g.93759921dup
TMEM67(NM_001142301.1):c.91dup (p.(Tyr31Leufs*4))
-
TMEM67_000096
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-/., -?/.
3
-
c.406+1361C>T
r.(=)
p.(=)
-
benign, likely benign
g.94772165C>T
g.93759937C>T
TMEM67(NM_001142301.1):c.107C>T (p.(Pro36Leu), p.P36L)
-
TMEM67_000059
87 heterozygous, no homozygous;
Clinindb (India)
, VKGL data sharing initiative Nederland
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs73324992
CLASSIFICATION record, Germline
-
87/2795 individuals
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Utrecht
,
Mohammed Faruq
-?/.
1
-
c.406+1375G>T
r.(=)
p.(=)
-
likely benign
g.94772179G>T
-
TMEM67(NM_001142301.1):c.121G>T (p.(Ala41Ser))
-
TMEM67_000219
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-/., -?/.
2
-
c.406+1379G>A
r.(=)
p.(=)
-
benign, likely benign
g.94772183G>A
g.93759955G>A
TMEM67(NM_001142301.1):c.125G>A (p.(Cys42Tyr), p.C42Y)
-
TMEM67_000060
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Utrecht
?/.
2
-
c.406+1421A>G
r.(=)
p.(=)
-
VUS
g.94772225A>G
g.93759997A>G
TMEM67(NM_001142301.1):c.163+4A>G, TMEM67(NM_153704.6):c.406+1421A>G
-
TMEM67_000078
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_AMC
-/.
1
-
c.407-894_407-893del
r.(=)
p.(=)
-
benign
g.94775176_94775177del
g.93762948_93762949del
TMEM67(NM_153704.5):c.407-894_407-893delAG
-
TMEM67_000079
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/.
3
4
c.415_416del
r.(?)
p.(Asp139Hisfs*2)
-
pathogenic
g.94776078_94776079del
g.93763850_93763851del
c.415_416del (p.Asp139Hisfs*2)
-
TMEM67_000047
-
PubMed: Watson 2016
,
PubMed: Watson 2016
,
Journal: Watson 2016
-
-
Germline
-
-
-
-
-
Christopher Watson
-/.
1
-
c.429A>T
r.(?)
p.(Thr143=)
-
benign
g.94776092A>T
g.93763864A>T
TMEM67(NM_153704.6):c.429A>T (p.T143=)
-
TMEM67_000061
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
1
-
c.442G>T
r.(?)
p.(Ala148Ser)
-
pathogenic
g.94776105G>T
g.93763877G>T
-
-
TMEM67_000143
-
PubMed: Suzuki 2016
-
-
Germline
-
-
-
-
-
LOVD
+/.
1
-
c.475T>C
r.(?)
p.(Ser159Pro)
-
pathogenic
g.94776138T>C
g.93763910T>C
-
-
TMEM67_000144
-
PubMed: Suzuki 2016
-
-
Germline
-
-
-
-
-
LOVD
-/.
1
-
c.506+18G>T
r.(?)
p.(=)
-
benign
g.94776187G>T
g.93763959G>T
-
-
TMEM67_000227
-
PubMed: Khaddour 2007
-
-
Germline
-
1/224
-
-
-
Johan den Dunnen
-/.
1
-
c.507-19T>C
r.(?)
p.(=)
-
benign
g.94777615T>C
g.93765387T>C
-
-
TMEM67_000228
-
PubMed: Khaddour 2007
-
-
Germline
-
1/210
-
-
-
Johan den Dunnen
?/.
1
-
c.511G>A
r.(?)
p.(Val171Ile)
-
VUS
g.94777638G>A
g.93765410G>A
TMEM67(NM_153704.5):c.511G>A (p.V171I)
-
TMEM67_000097
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
3
5
c.514C>T
r.(?)
p.(Arg172*), p.(Arg172Ter)
-
pathogenic
g.94777641C>T
g.93765413C>T
c.514C>T (p.Arg172*), TMEM67(NM_153704.5):c.514C>T (p.R172*)
-
TMEM67_000049
VKGL data sharing initiative Nederland
PubMed: Watson 2016
,
PubMed: Watson 2016
,
Journal: Watson 2016
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Christopher Watson
,
VKGL-NL_Utrecht
+/.
2
-
c.515G>A
r.(?)
p.(Arg172Gln)
-
pathogenic
g.94777642G>A
g.93765414G>A
NM_153704.5:c.515G>A, TMEM67(NM_153704.6):c.515G>A (p.(Arg172Gln))
-
TMEM67_000098
VKGL data sharing initiative Nederland
PubMed: Bachmann-Gagescu 2015
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
-
c.515G>C
r.(?)
p.(Arg172Pro)
-
VUS
g.94777642G>C
-
TMEM67(NM_153704.6):c.515G>C (p.R172P)
-
TMEM67_000229
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-?/.
1
-
c.561A>G
r.(?)
p.(Ser187=)
-
likely benign
g.94777688A>G
g.93765460A>G
TMEM67(NM_153704.5):c.561A>G (p.S187=)
-
TMEM67_000099
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.576+10A>T
r.(=)
p.(=)
-
likely benign
g.94777713A>T
g.93765485A>T
TMEM67(NM_153704.5):c.576+10A>T
-
TMEM67_000100
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.577-11T>C
r.(=)
p.(=)
-
likely benign
g.94777789T>C
g.93765561T>C
TMEM67(NM_153704.6):c.577-11T>C
-
TMEM67_000101
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
4
6
c.579del
r.(?)
p.(Gly195Aspfs*27), p.(Gly195AspfsTer27)
-
pathogenic, pathogenic (recessive)
g.94777802del
g.93765574del
579delA, TMEM67(NM_153704.5):c.579delA (p.G195Dfs*27)
-
TMEM67_000005, TMEM67_000080
VKGL data sharing initiative Nederland
PubMed: Consugar 2007
,
PubMed: Iannicelli 2010
-
-
CLASSIFICATION record, Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Anne Polvi
,
VKGL-NL_Rotterdam
+/., +?/.
13
6, 7
c.579_580del
r.(?)
p.(Gly195Ilefs*13), p.(Gly195IlefsTer13)
-
likely pathogenic, pathogenic, pathogenic (recessive)
g.94777802_94777803del
g.93765574_93765575del
579_580delAG, c.579_580del, c.579_580del (p.Gly195Ilefs*13), c.579_580del; p.G195Ifs*13,
3 more items
-
TMEM67_000038, TMEM67_000048
VKGL data sharing initiative Nederland
PubMed: Bachmann-Gagescu 2015
,
PubMed: Brooks 2018
,
PubMed: Enokizono 2017
,
PubMed: Iannicelli 2010
,
3 more items
-
-
CLASSIFICATION record, Germline, SUMMARY record
?
-
-
-
-
Johan den Dunnen
,
Anne Polvi
,
Christopher Watson
,
VKGL-NL_Leiden
,
VKGL-NL_VUmc
+/.
1
-
c.579_580delAG
r.(?)
p.(Gly195Ilefs*13)
-
pathogenic
g.94777802_94777803del
g.93765574_93765575del
NPHP11 c.579_580delAG, Gly195Ilefs*13
-
TMEM67_000038
heterozygous
PubMed: Kang 2016
-
-
Unknown
?
-
-
-
-
LOVD
?/.
1
-
c.584G>T
r.(?)
p.(Gly195Val)
-
VUS
g.94777807G>T
g.93765579G>T
-
-
TMEM67_000102
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/., +?/.
23
6, 7
c.622A>T
r.(622a>u), r.(?)
p.(Arg208*), p.(Arg208Ter)
-
likely pathogenic, pathogenic, pathogenic (recessive)
g.94777845A>T
g.93765617A>T
c.622A/T, c.622A>T, c.622A>T (p.Arg208*), c.622A>T; p.R208*, NM_153704.5:c.622A>T, R208X,
1 more item
-
TMEM67_000006
VKGL data sharing initiative Nederland
PubMed: Bachmann-Gagescu 2015
,
PubMed: Brancati 2018
,
Journal: Brancati 2018
,
PubMed: Brooks 2018
,
7 more items
-
rs137853108
CLASSIFICATION record, Germline, SUMMARY record, Unknown
?, yes
-
-
-
-
Johan den Dunnen
,
Anne Polvi
,
Christopher Watson
,
Sanne Savelberg
,
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
,
VKGL-NL_VUmc
,
VKGL-NL_AMC
,
Francesco Brancati
+?/.
1
-
c.628T>C
r.(?)
p.(Ser210Pro)
-
likely pathogenic
g.94777851T>C
-
TMEM67(NM_153704.6):c.628T>C (p.S210P)
-
TMEM67_000103
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
+/.
2
7
c.648del
r.(?)
p.(Val217Leufs*5)
-
pathogenic (recessive)
g.94777871del
g.93765643del
647delA
-
TMEM67_000007
-
PubMed: Smith 2006
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Anne Polvi
+/., +?/.
5
7i
c.651+2T>G
r.spl, r.spl?
p.?
-
likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive)
g.94777876T>G
g.93765648T>G
651+2G>T, TMEM67(NM_153704.6):c.651+2T>G
-
TMEM67_000008
VKGL data sharing initiative Nederland
PubMed: Khaddour 2007
-
-
CLASSIFICATION record, Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Anne Polvi
,
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
,
VKGL-NL_AMC
-?/.
1
-
c.652-16A>G
r.(=)
p.(=)
-
likely benign
g.94784801A>G
-
TMEM67(NM_153704.6):c.652-16A>G
-
TMEM67_000192
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
1
-
c.652G>T
r.(?)
p.(Gly218Cys)
-
VUS
g.94784817G>T
-
-
-
TMEM67_000206
-
-
-
-
Germline
-
-
-
-
-
Alejandro Brea-Fernández
-/., -?/., ?/.
5
7
c.653G>C
r.(?)
p.(Gly218Ala)
-
benign, likely benign, VUS
g.94784818G>C
g.93772590G>C
MKS3: G218A,
1 more item
-
TMEM67_000073
VKGL data sharing initiative Nederland
PubMed: Leitch-2008
-
-
CLASSIFICATION record, Germline
-
0/96 ethnically matched controls
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
+/.
4
7, 8
c.675G>A
r.(675g>a), r.(?)
p.(Trp225*), p.(Trp225Ter)
-
pathogenic, pathogenic (recessive)
g.94784840G>A
g.93772612G>A
NM_153704.5:c.675G>A
-
TMEM67_000039
-
PubMed: Bachmann-Gagescu 2015
,
PubMed: Iannicelli 2010
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Anne Polvi
+?/.
1
7
c.685_687delGAA
r.?
p.(Glu229del)
-
likely pathogenic
g.94784850_94784852delGAA
-
MKS6/JBTS9:c.685_687delGAA
-
TMEM67_000194
-
PubMed: Knopp 2015
-
-
Germline
-
-
-
-
-
LOVD
-?/., ?/.
2
-
c.717A>G
r.(?)
p.(Val239=)
-
likely benign, VUS
g.94792823A>G
g.93780595A>G
TMEM67(NM_001142301.1):c.474A>G (p.(=)), TMEM67(NM_153704.5):c.717A>G (p.V239=)
-
TMEM67_000104
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Utrecht
+/.
1
-
c.722C>G
r.(?)
p.(Ala241Gly)
-
pathogenic
g.94792828C>G
g.93780600C>G
-
-
TMEM67_000145
-
PubMed: Suzuki 2016
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/.
6
8
c.725A>G
r.(?)
p.(Asn242Ser)
ACMG
likely pathogenic, likely pathogenic (recessive), pathogenic
g.94792831A>G
g.93780603A>G
NM_153704.5:c.725A>G, TMEM67/MKS3/ c.725A>G, p.Asn242Ser
-
TMEM67_000042
heterozygous
PubMed: Bachmann-Gagescu 2015
,
PubMed: Bui 2020
,
Journal: Bui 2020
,
PubMed: Hu 2019
,
PubMed: Kang 2016
,
1 more item
212652
-
Germline, Unknown
?, yes
-
-
-
-
Johan den Dunnen
,
Sanne Savelberg
,
Minh Tuan Huynh
+/.
1
-
c.730A>G
r.(?)
p.(Thr244Ala)
-
pathogenic
g.94792836A>G
g.93780608A>G
NM_153704.5:c.730A>G
-
TMEM67_000155
-
PubMed: Bachmann-Gagescu 2015
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/.
2
9
c.734C>T
r.(734c>u), r.(?)
p.(Ser245Phe)
-
likely pathogenic (recessive), pathogenic (recessive)
g.94792840C>T
g.93780612C>T
-
-
TMEM67_000009
-
PubMed: Tallila 2008
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Anne Polvi
+/.
1
-
c.739C>G
r.(?)
p.(Gln247Glu)
-
pathogenic (recessive)
g.94792845C>G
g.93780617C>G
NM_153704.5:c.739C>G:p.(Gln247Glu)
-
TMEM67_000135
-
PubMed: Maddirevula 2018
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/.
2
8
c.748G>A
r.(?)
p.(Gly250Arg)
-
likely pathogenic, pathogenic
g.94792854G>A
g.93780626G>A
c.748G>A, c.748G>A, p.G250R
-
TMEM67_000174
-
PubMed: Brooks 2018
,
PubMed: Summers 2017
-
-
Germline
?
-
-
-
-
LOVD
+/., +?/.
25
8
c.755T>C
r.(755u>c), r.(?)
p.(Met252Thr)
-
likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive)
g.94792861T>C
g.93780633T>C
c.755T/C, c.755T>C, c.755T>C; p.M252T, M252T, NM_153704.5:c.755T>C,
1 more item
-
TMEM67_000010
VKGL data sharing initiative Nederland
PubMed: Bachmann-Gagescu 2015
,
PubMed: Brooks 2018
,
PubMed: Consugar 2007
,
PubMed: Iannicelli 2010
,
5 more items
-
-
CLASSIFICATION record, Germline, SUMMARY record
?
-
-
-
-
Johan den Dunnen
,
Anne Polvi
,
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
+/., +?/.
3
8
c.769A>G
r.(?)
p.(Met257Val)
-
likely pathogenic, pathogenic
g.94792875A>G
g.93780647A>G
c.769A>G, c.769A>G; p.M257V, NM_153704.5:c.769A>G
-
TMEM67_000156
single heterozygous variant, no variant found on second allele
PubMed: Bachmann-Gagescu 2015
,
PubMed: Brooks 2018
,
PubMed: Summers 2017
-
-
Germline
?
-
-
-
-
LOVD
-?/.
1
8
c.777C>T
r.?
p.(His259His)
-
likely benign
g.94792883C>T
-
MKS6/JBTS9:c.777C>T
-
TMEM67_000195
-
PubMed: Knopp 2015
-
-
Germline
-
-
-
-
-
LOVD
-/., -?/.
8
8
c.781G>A
r.(?)
p.(Asp261Asn)
-
benign, likely benign
g.94792887G>A
g.93780659G>A
MKS3/JBTS6:c.781G>A,
1 more item
-
TMEM67_000081
137 heterozygous, no homozygous;
Clinindb (India)
, VKGL data sharing initiative Nederland
PubMed: Khaddour 2007
,
PubMed: Knopp 2015
,
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs35793208
CLASSIFICATION record, Germline
-
137/2793 individuals, 6/252
-
-
-
Johan den Dunnen
,
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
,
VKGL-NL_Utrecht
,
VKGL-NL_AMC
,
Mohammed Faruq
+?/., ?/.
2
8
c.797A>C
r.(?)
p.(Asp266Ala)
-
likely pathogenic, VUS
g.94792903A>C
g.93780675A>C
TMEM67(NM_001142301.1):c.554A>C (p.(Asp185Ala))
-
TMEM67_000045
VKGL data sharing initiative Nederland
PubMed: Kroes 2016
-
-
CLASSIFICATION record, Unknown
?
-
-
-
-
Sanne Savelberg
,
VKGL-NL_Leiden
?/.
1
-
c.857C>G
r.(?)
p.(Ser286Cys)
-
VUS
g.94792963C>G
g.93780735C>G
TMEM67(NM_153704.5):c.857C>G (p.S286C)
-
TMEM67_000082
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.859A>G
r.(?)
p.(Ile287Val)
-
likely benign
g.94792965A>G
-
TMEM67(NM_153704.5):c.859A>G (p.I287V)
-
TMEM67_000220
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-/., -?/.
2
-
c.869+9A>G
r.(=)
p.(=)
-
benign, likely benign
g.94792984A>G
g.93780756A>G
TMEM67(NM_153704.5):c.869+9A>G, TMEM67(NM_153704.6):c.869+9A>G
-
TMEM67_000105
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
-/.
1
-
c.870-29A>G
r.(?)
p.(=)
-
benign
g.94793073A>G
g.93780845A>G
-
-
TMEM67_000230
-
PubMed: Khaddour 2007
-
-
Germline
-
2/256
-
-
-
Johan den Dunnen
+/., +?/.
3
9i
c.870-2A>G
r.spl
p.?
-
likely pathogenic (recessive), pathogenic (recessive)
g.94793100A>G
g.93780872A>G
INV8-2A>G
-
TMEM67_000011
-
PubMed: Khaddour 2007
,
PubMed: Smith 2006
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Anne Polvi
+/., +?/.
2
10
c.888G>T
r.(888g>u), r.(?)
p.(Trp296Cys)
-
likely pathogenic (recessive), pathogenic (recessive)
g.94793120G>T
g.93780892G>T
-
-
TMEM67_000013
-
PubMed: Tallila 2008
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Anne Polvi
?/.
1
9
c.903C>G
r.(?)
p.(Asp301Glu)
-
VUS
g.94793135C>G
g.93780907C>G
-
-
TMEM67_000231
-
PubMed: Iannicelli 2010
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., +?/.
4
9
c.950C>G
r.(?)
p.(Thr317Arg)
-
likely pathogenic, pathogenic
g.94793182C>G
g.93780954C>G
c.950C>G, c.950C>G; p.T317R
-
TMEM67_000175
-
PubMed: Brooks 2018
,
PubMed: Summers 2017
-
-
Germline
?
-
-
-
-
LOVD
+?/., ?/.
4
9
c.958A>T
r.(?)
p.(Ser320Cys)
-
likely pathogenic, VUS
g.94793190A>T
g.93780962A>T
MKS3: S320C,
1 more item
-
TMEM67_000062
VKGL data sharing initiative Nederland
PubMed: Leitch-2008
-
-
CLASSIFICATION record, Germline
-
0/96 ethnically matched controls
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
VKGL-NL_Nijmegen
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