Global Variome shared LOVD
GNPTAB (N-acetylglucosamine-1-phosphate transferase...)
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Curator:
Renata Voltolini Velho
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Unique variants in the GNPTAB gene
The variants shown are described using the NM_024312.4 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%)
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Operator
Column type
Example
Matches
Text
Arg
all entries containing 'Arg'
space
Text
Arg Ser
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Arg|Ser
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!
Text
!fs
all entries not containing 'fs'
^
Text
^p.(Arg
all entries beginning with 'p.(Arg'
$
Text
Ser)$
all entries ending with 'Ser)'
=""
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all entries with this field empty
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="p.0"
all entries exactly matching 'p.0'
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!=""
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!=""
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!="p.0"
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combination
Text
*|Ter !fs
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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
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>
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'
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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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334 entries on 4 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.-41_-39del
r.(?)
p.(=)
-
benign, likely benign
g.102224513_102224515del
g.101830735_101830737del
GNPTAB(NM_024312.4):c.-41_-39delGGC, GNPTAB(NM_024312.5):c.-41_-39delGGC
-
GNPTAB_000055, GNPTAB_000056
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
+/+?
1
1
c.1A>G
r.(?)
p.(Met1?)
-
pathogenic
g.102224453T>C
g.101830675T>C
-
-
GNPTAB_000293
-
-
-
-
Germline
-
-
-
-
-
Renata Voltolini Velho
+/+
1
1
c.10A>C
r.(?)
p.(Lys4Gln)
-
pathogenic
g.102224444T>G
g.101830666T>G
-
-
GNPTAB_000060
-
Journal: Kudo et al. 2006
-
-
Germline
-
-
-
-
-
Renata Voltolini Velho
-/-
1
1
c.18G>A
r.(?)
p.(Leu6=)
-
benign
g.102224436C>T
g.101830658C>T
GNPTAB(NM_024312.4):c.18G>A (p.L6=)
-
GNPTAB_000054
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/+
1
1
c.22A>G
r.(?)
p.(Arg8Gly)
-
pathogenic
g.102224432T>C
g.101830654T>C
-
-
GNPTAB_000061
-
Journal: Tüysüz 2016
-
-
Germline
-
-
-
-
-
Renata Voltolini Velho
+/+
1
1
c.44C>A
r.(?)
p.(Ser15Tyr)
-
pathogenic
g.102224410G>T
g.101830632G>T
-
-
GNPTAB_000062
-
Journal: Cathey 2010
-
-
Germline
-
-
-
-
-
Renata Voltolini Velho
+?/+?, +?/.
2
1
c.70T>G
r.(?)
p.(Phe24Val)
-
likely pathogenic
g.102224384A>C
g.101830606A>C
-
-
GNPTAB_000009
Found in cis with the c.1000C>T.
-
-
rs141329633
Germline, Unknown
?
2/1013 patients
-
-
-
Muhammad Raza
,
Renata Voltolini Velho
+/+
1
1
c.77G>A
r.(?)
p.(Gly26Asp)
-
pathogenic
g.102224377C>T
g.101830599C>T
-
-
GNPTAB_000249
-
-
-
-
Germline/De novo (untested)
-
-
-
-
-
Renata Voltolini Velho
+/+
1
1
c.80T>A
r.(?)
p.(Val27Asp)
-
pathogenic
g.102224374A>T
g.101830596A>T
-
-
GNPTAB_000250
-
-
-
-
Germline/De novo (untested)
-
-
-
-
-
Renata Voltolini Velho
+/+
1
1
c.83T>A
r.(?)
p.(Val28Asp)
-
pathogenic
g.102224371A>T
g.101830593A>T
-
-
GNPTAB_000286
-
-
-
-
Germline
-
-
-
-
-
Renata Voltolini Velho
+/+
1
1
c.88_89del
r.(?)
p.(Thr30Hisfs*24)
-
pathogenic
g.102224366_102224367del
g.101830588_101830589del
-
-
GNPTAB_000063
-
Journal: Wang 2018
-
-
Germline/De novo (untested)
-
-
-
-
-
Renata Voltolini Velho
+/+
1
1
c.99del
r.(?)
p.(Ala34Profs*49)
-
pathogenic
g.102224356del
g.101830578del
-
-
GNPTAB_000064
-
Journal: Liu 2016
-
-
Germline/De novo (untested)
-
-
-
-
-
Renata Voltolini Velho
+/+
1
1
c.100G>C
r.(?)
p.(Ala34Pro)
-
pathogenic
g.102224354C>G
g.101830576C>G
-
-
GNPTAB_000065
-
Journal: Wang 2018
-
-
Germline/De novo (untested)
-
-
-
-
-
Renata Voltolini Velho
+/+
1
1
c.107A>C
r.(?)
p.(Gln36Pro)
-
pathogenic
g.102224347T>G
g.101830569T>G
-
-
GNPTAB_000251
-
-
-
-
Germline/De novo (untested)
-
-
-
-
-
Renata Voltolini Velho
?/.
1
-
c.112G>C
r.(?)
p.(Gly38Arg)
-
VUS
g.102224342C>G
-
GNPTAB(NM_024312.4):c.112G>C (p.(Gly38Arg))
-
GNPTAB_000334
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-/-?
1
1i
c.117+27C>G
r.(=)
p.(=)
-
benign
g.102224310G>C
g.101830532G>C
GNPTAB(NM_024312.4):c.117+27C>G
-
GNPTAB_000053
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/+
1
1i
c.118-2A>G
r.118_203del
p.Val40Alafs*8
-
pathogenic
g.102190542T>C
g.101796764T>C
-
-
GNPTAB_000067
-
Journal: Bargal 2006
-
-
Germline
-
-
-
-
-
Renata Voltolini Velho
+/+
1
1i
c.118-1G>A
r.spl?
p.?
-
pathogenic
g.102190541C>T
g.101796763C>T
-
-
GNPTAB_000066
-
Journal: Liu 2016
-
-
Germline/De novo (untested)
-
-
-
-
-
Renata Voltolini Velho
+/+
1
2
c.118_223dup
r.118_203dup
p.(Leu69Trpfs*43)
-
pathogenic
g.102183816_102190540dup
g.101790038_101796762dup
Duplication ex 2
-
GNPTAB_000075
-
Journal: Otomo 2009
-
-
Germline/De novo (untested)
-
-
-
-
-
Renata Voltolini Velho
+/+
1
2
c.120_2715+2del
r.118_2715del
p.(Val40_Asp905del)
-
pathogenic
g.102157980_102190540del
g.101764202_101796762del
-
-
GNPTAB_000068
delEx2-13
-
-
-
Germline
-
-
-
-
-
Renata Voltolini Velho
+/+
1
2
c.121del
r.(?)
p.(Val41Phefs*42)
-
pathogenic
g.102190538del
g.101796760del
-
-
GNPTAB_000069
-
Journal: Encarnação 2009
-
-
Germline
-
-
-
-
-
Renata Voltolini Velho
+/+
1
2
c.132G>A
r.(?)
p.(Trp44*)
-
pathogenic
g.102190526C>T
g.101796748C>T
-
-
GNPTAB_000070
-
Journal: Wang 2018
-
-
Germline/De novo (untested)
-
-
-
-
-
Renata Voltolini Velho
+/+, +/.
2
2
c.136C>T
r.(?)
p.(Arg46*), p.(Arg46Ter)
-
pathogenic, pathogenic (recessive)
g.102190522G>A
g.101796744G>A
-
-
GNPTAB_000071
-
Journal: Cathey 2010
,
PubMed: Cobos 2015
-
-
Germline, Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
,
Renata Voltolini Velho
+?/., -/., -?/.
3
2
c.137G>A
r.(?)
p.(Arg46Gln)
-
benign, likely benign, likely pathogenic
g.102190521C>T
g.101796743C>T
GNPTAB(NM_024312.4):c.137G>A (p.R46Q, p.(Arg46Gln))
-
GNPTAB_000008
VKGL data sharing initiative Nederland
-
-
rs117566084
CLASSIFICATION record, Germline
yes
11/1013 patients
-
-
-
Muhammad Raza
,
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
+/+
1
2
c.163dup
r.163dup
p.Ser55Phefs*3
-
pathogenic
g.102190496dup
g.101796718dup
-
-
GNPTAB_000072
-
Journal: Zarghooni & Dittakavi 2009
-
-
Germline/De novo (untested)
-
-
-
-
-
Renata Voltolini Velho
+/+
1
2
c.168T>A
r.(?)
p.(Tyr56*)
-
pathogenic
g.102190490A>T
g.101796712A>T
-
-
GNPTAB_000073
-
Journal: Cathey 2010
-
-
Germline
-
-
-
-
-
Renata Voltolini Velho
+/+
1
2
c.171del
r.(?)
p.(Asp58Thrfs*25)
-
pathogenic
g.102190487del
g.101796709del
-
-
GNPTAB_000074
-
Journal: Cathey 2010
-
-
Germline
-
-
-
-
-
Renata Voltolini Velho
+/+
1
2
c.196C>T
r.(?)
p.(Gln66*)
-
pathogenic
g.102190462G>A
g.101796684G>A
-
-
GNPTAB_000076
-
Journal: Oussoren 2018
-
-
Germline/De novo (untested)
-
-
-
-
-
Renata Voltolini Velho
+/+
1
2
c.227A>G
r.(?)
p.(Asp76Gly)
-
pathogenic
g.102183812T>C
g.101790034T>C
-
-
GNPTAB_000077
-
Journal: Ludwig 2017
-
-
Germline/De novo (untested)
-
-
-
-
-
Renata Voltolini Velho
+/+, +?/+
3
3
c.232_234del
r.(?)
p.(Val78del)
-
pathogenic
g.102183808_102183810del
g.101790030_101790032del
GNPTAB(NM_024312.4):c.232_234delGTT (p.V78del)
-
GNPTAB_000006
VKGL data sharing initiative Nederland
Journal: Cathey 2010
-
rs281864952
CLASSIFICATION record, Germline
yes
1/1013 patients
-
-
-
Muhammad Raza
,
VKGL-NL_Rotterdam
,
Renata Voltolini Velho
+/+
1
3
c.242G>T
r.242g>u
p.Trp81Leu
-
pathogenic
g.102183797C>A
g.101790019C>A
-
-
GNPTAB_000078
-
Journal: Encarnacao 2009
-
-
Germline
-
-
-
-
-
Renata Voltolini Velho
+/+
1
3
c.244del
r.(?)
p.(Val82*)
-
pathogenic
g.102183797del
g.101790019del
-
-
GNPTAB_000288
-
-
-
-
Germline
-
-
-
-
-
Renata Voltolini Velho
+/+
1
3
c.251dup
r.(?)
p.(Thr85Hisfs*4)
-
pathogenic
g.102183789dup
g.101790011dup
-
-
GNPTAB_000252
-
-
-
-
Germline/De novo (untested)
-
-
-
-
-
Renata Voltolini Velho
+/+
1
3
c.310C>T
r.(?)
p.(Gln104*)
-
pathogenic
g.102183729G>A
g.101789951G>A
-
-
GNPTAB_000079
-
Journal: Paik 2005
-
-
Germline/De novo (untested)
-
-
-
-
-
Renata Voltolini Velho
+/+
1
3i
c.323+1G>A
r.204_323del
p.Leu69_Arg108del
-
pathogenic
g.102183715C>T
g.101789937C>T
-
-
GNPTAB_000285
-
-
-
-
Germline
-
-
-
-
-
Renata Voltolini Velho
+/.
1
-
c.324-2A>G
r.spl?
p.?
-
pathogenic
g.102182369T>C
-
GNPTAB(NM_024312.4):c.324-2A>G
-
GNPTAB_000327
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/+
1
4
c.325G>T
r.(?)
p.(Glu109*)
-
pathogenic
g.102182366C>A
g.101788588C>A
-
-
GNPTAB_000253
-
-
-
-
Germline/De novo (untested)
-
-
-
-
-
Renata Voltolini Velho
+?/.
1
4
c.337A>G
r.(?)
p.(Lys113Glu)
-
likely pathogenic
g.102182354T>C
g.101788576T>C
-
-
GNPTAB_000007
-
-
-
rs140656599
Germline
yes
3/1013 patients
-
-
-
Muhammad Raza
+/+
1
4
c.344_345del
r.(?)
p.(Thr115Asnfs*5)
-
pathogenic
g.102182349_102182350del
g.101788571_101788572del
-
-
GNPTAB_000080
-
Journal: Cathey 2010
-
-
Germline/De novo (untested)
-
-
-
-
-
Renata Voltolini Velho
?/.
1
-
c.355A>G
r.(?)
p.(Thr119Ala)
-
VUS
g.102182336T>C
g.101788558T>C
-
-
GNPTAB_000320
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/.
1
4i
c.365+96_365+97del
r.(=)
p.(=)
-
benign
g.102182232_102182233del
g.101788454_101788455del
GNPTAB(NM_024312.4):c.365+96_365+97delGT
-
GNPTAB_000052
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/+
1
4i
c.366-1G>C
r.spl?
p.?
-
pathogenic
g.102179996C>G
g.101786218C>G
-
-
GNPTAB_000081
-
Journal: Oussoren 2018
-
-
Germline/De novo (untested)
-
-
-
-
-
Renata Voltolini Velho
+/+
1
5
c.376_379del
r.(?)
p.(Leu126Serfs*4)
-
pathogenic
g.102179984_102179987del
g.101786206_101786209del
-
-
GNPTAB_000254
-
-
-
-
Germline/De novo (untested)
-
-
-
-
-
Renata Voltolini Velho
+/+
1
5
c.377T>A
r.(?)
p.(Leu126*)
-
pathogenic
g.102179984A>T
g.101786206A>T
-
-
GNPTAB_000255
-
-
-
-
Germline/De novo (untested)
-
-
-
-
-
Renata Voltolini Velho
?/.
1
5
c.382T>C
r.(?)
p.(Cys128Arg)
-
VUS
g.102179979A>G
g.101786201A>G
GNPTAB(NM_024312.4):c.382T>C (p.(Cys128Arg))
-
GNPTAB_000051
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/+
1
5
c.441del
r.411del
p.Asn148Thrfs*4
-
pathogenic
g.102179921del
g.101786143del
-
-
GNPTAB_000082
-
Journal: Encarnacao 2009
-
-
Germline
-
-
-
-
-
Renata Voltolini Velho
+/+
1
5
c.471_472del
r.(?)
p.(Tyr158Serfs*8)
-
pathogenic
g.102179890_102179891del
g.101786112_101786113del
-
-
GNPTAB_000083
-
Journal: Yang 2017
-
-
Germline/De novo (untested)
-
-
-
-
-
Renata Voltolini Velho
?/.
1
5
c.500T>A
r.(?)
p.(Ile167Asn)
-
VUS
g.102179861A>T
g.101786083A>T
-
-
GNPTAB_000010
-
-
-
-
Germline
no
-
-
-
-
Muhammad Raza
?/.
1
-
c.512C>T
r.(?)
p.(Ala171Val)
-
VUS
g.102179849G>A
-
GNPTAB(NM_024312.4):c.512C>T (p.(Ala171Val))
-
GNPTAB_000337
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/+, ?/.
2
5
c.516dup
r.(?)
p.(Pro173Thrfs*14)
-
pathogenic, VUS
g.102179848dup
g.101786070dup
GNPTAB(NM_024312.4):c.516dupA (p.(Pro173fs))
-
GNPTAB_000294
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
VKGL-NL_Leiden
,
Renata Voltolini Velho
+/+
1
5
c.517_518insA
r.(?)
p.(Pro173Hisfs*14)
-
pathogenic
g.102179843_102179844insT
g.101786065_101786066insT
-
-
GNPTAB_000084
-
Journal: Cathey 2010
-
-
Germline/De novo (untested)
-
-
-
-
-
Renata Voltolini Velho
+/+
1
5
c.523_524delinsG
r.(?)
p.(Asn175Alafs*38)
-
pathogenic
g.102179837_102179838delinsC
g.101786059_101786060delinsC
-
-
GNPTAB_000085
-
Journal: Liu 2016
-
-
Germline/De novo (untested)
-
-
-
-
-
Renata Voltolini Velho
+/+
1
5
c.545T>A
r.545u>a
p.Val182Asp
-
pathogenic
g.102179816A>T
g.101786038A>T
-
-
GNPTAB_000086
-
Journal: Zarghooni & Dittakavi 2009
-
-
Germline/De novo (untested)
-
-
-
-
-
Renata Voltolini Velho
+/+
1
5
c.555_556ins[U14569.1:g.1_288]
r.555_556ins[[U14569.1:g.1_288inv];540_555]
p.Glu123*
-
pathogenic
g.102179805_102179806ins[U14569.1:g.1_288]
-
Alu insertion mutation
-
GNPTAB_000284
-
Journal: Tappino 2008
-
-
Germline/De novo (untested)
-
-
-
-
-
Renata Voltolini Velho
+?/+?
1
5
c.560G>C
r.(?)
p.(Ser187Thr)
-
likely pathogenic
g.102179801C>G
g.101786023C>G
-
-
GNPTAB_000011
-
-
-
-
Germline
yes
1/1013 patients
-
-
-
Muhammad Raza
+/+, +?/.
2
5
c.569A>T
r.(?), r.568_571del
p.(Asp190Val), p.Asp190Leufs*31
ACMG
likely pathogenic (recessive), pathogenic
g.102179792T>A
g.101786014T>A
-
-
GNPTAB_000087
ACMG PM2, PP2, PP3, PP4, PP5, Splicing defect or p.Asp190Val
Journal: Kudo 2006
,
PubMed: Marinakis 2021
-
-
Germline, Germline/De novo (untested)
-
-
-
-
-
Jan Traeger-Synodinos
,
Renata Voltolini Velho
+/+?
1
5
c.571G>A
r.(?)
p.(Val191Ile)
-
pathogenic
g.102179790C>T
g.101786012C>T
-
-
GNPTAB_000291
-
-
-
-
Germline
-
Not detected in 100 control chromosomes
-
-
-
Renata Voltolini Velho
+/+
1
5i
c.571+3A>C
r.366_571del
p.Glu123*
-
pathogenic
g.102179787T>G
g.101786009T>G
-
-
GNPTAB_000088
Exon 5 splicing affected. Exon 5 skipped.
Journal: Zarghooni & Dittakavi 2009
-
-
Germline/De novo (untested)
-
-
-
-
-
Renata Voltolini Velho
-?/.
1
-
c.572-7G>A
r.(=)
p.(=)
-
likely benign
g.102174406C>T
-
GNPTAB(NM_024312.5):c.572-7G>A
-
GNPTAB_000344
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
6
c.580G>A
r.(?)
p.(Ala194Thr)
-
VUS
g.102174391C>T
g.101780613C>T
-
-
GNPTAB_000012
-
-
-
-
Germline
no
1/677 controls
-
-
-
Muhammad Raza
-?/.
1
-
c.609C>G
r.(?)
p.(Ser203Arg)
-
likely benign
g.102174362G>C
-
GNPTAB(NM_024312.4):c.609C>G (p.(Ser203Arg))
-
GNPTAB_000326
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/+
1
6
c.613C>T
r.(?)
p.(Gln205*)
-
pathogenic
g.102174358G>A
g.101780580G>A
-
-
GNPTAB_000256
-
-
-
-
Germline/De novo (untested)
-
-
-
-
-
Renata Voltolini Velho
+/+
1
6
c.614A>C
r.614a>c
p.Gln205Pro
-
pathogenic
g.102174357T>G
g.101780579T>G
-
-
GNPTAB_000089
-
Journal: Zarghooni & Dittakavi 2009
-
-
Germline/De novo (untested)
-
-
-
-
-
Renata Voltolini Velho
+/+, +/.
2
6
c.616_619del
r.(?)
p.(Thr206Tyrfs*6), p.(Thr206TyrfsTer6)
-
pathogenic, pathogenic (recessive)
g.102174359_102174362del
g.101780581_101780584del
616_619delACAG
-
GNPTAB_000090
-
Journal: Kudo 2006
,
PubMed: Cobos 2015
-
-
Germline, Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
,
Renata Voltolini Velho
+/+
1
6
c.625_629del
r.(?)
p.(Arg209Leufs*6)
-
pathogenic
g.102174344_102174348del
g.101780566_101780570del
-
-
GNPTAB_000091
-
Journal: Cathey 2010
-
-
Germline/De novo (untested)
-
-
-
-
-
Renata Voltolini Velho
+/+
1
6i
c.637-6T>G
r.(=)
p.(Thr213Phefs*11)
-
pathogenic
g.102174070A>C
g.101780292A>C
-
-
GNPTAB_000093
Affect splicing exon 7, cDNA analysis reveal p.Thr213Phefs*11.
Journal: Yang 2017
-
-
Germline
-
-
-
-
-
Renata Voltolini Velho
+/+
1
6i
c.637-1G>A
r.spl?
p.?
-
pathogenic
g.102174065C>T
g.101780287C>T
-
-
GNPTAB_000092
-
Journal: Cathey 2010
-
-
Germline
-
-
-
-
-
Renata Voltolini Velho
+/+
1
7
c.648_651del
r.(?)
p.(Glu217Serfs*4)
-
pathogenic
g.102174052_102174055del
g.101780274_101780277del
-
-
GNPTAB_000094
-
Journal: Cathey 2010
-
-
Germline
-
-
-
-
-
Renata Voltolini Velho
?/.
1
-
c.707C>A
r.(?)
p.(Thr236Lys)
-
VUS
g.102173994G>T
-
GNPTAB(NM_024312.5):c.707C>A (p.(Thr236Lys))
-
GNPTAB_000343
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/+
1
7
c.749dup
r.749dup
p.Asn250Lysfs*5
-
pathogenic
g.102173955dup
g.101780177dup
-
-
GNPTAB_000095
-
Journal: Tappino 2009
-
-
Germline/De novo (untested)
-
-
-
-
-
Renata Voltolini Velho
+/+
1
7
c.750_751insA
r.(?)
p.(Leu251Thrfs*4)
-
pathogenic
g.102173950_102173951insT
g.101780172_101780173insT
-
-
GNPTAB_000096
-
Journal: Cathey 2010
-
-
Germline
-
-
-
-
-
Renata Voltolini Velho
+/+
1
7
c.755_759del
r.755_759del
p.Ser252*
-
pathogenic
g.102173943_102173947del
g.101780165_101780169del
-
-
GNPTAB_000097
-
Journal: Tappino 2009
-
-
Germline/De novo (untested)
-
-
-
-
-
Renata Voltolini Velho
+/+, +/.
2
7
c.771G>A
r.(?), r.637_771del
p.(=), p.Thr213_Leu257del
-
pathogenic
g.102173930C>T
g.101780152C>T
GNPTAB(NM_024312.5):c.771G>A (p.(Leu257=))
-
GNPTAB_000098
Splicing defect, exon 7 skipped., VKGL data sharing initiative Nederland
Journal: Steet 2005
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
VKGL-NL_Leiden
,
Renata Voltolini Velho
-?/.
1
-
c.771+7A>C
r.(=)
p.(=)
-
likely benign
g.102173923T>G
-
GNPTAB(NM_024312.4):c.771+7A>C (p.(=))
-
GNPTAB_000325
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/+
1
8
c.831del
r.(?)
p.(Gln278Lysfs*3)
-
pathogenic
g.102164879del
g.101771101del
-
-
GNPTAB_000099
-
Journal: Ludwig 2017
-
-
Germline
-
-
-
-
-
Renata Voltolini Velho
+/+
1
8
c.832C>T
r.(?)
p.(Gln278*)
-
pathogenic
g.102164875G>A
g.101771097G>A
-
-
GNPTAB_000100
-
Journal: Kudo 2006
-
-
Germline
-
-
-
-
-
Renata Voltolini Velho
+/+, +/.
2
8
c.835G>T
r.(?)
p.(Glu279*), p.(Glu279Ter)
-
pathogenic, pathogenic (recessive)
g.102164872C>A
g.101771094C>A
-
-
GNPTAB_000101
-
Journal: Cobos 2014
,
PubMed: Cobos 2015
-
-
Germline, Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
,
Renata Voltolini Velho
-?/.
1
-
c.842A>G
r.(?)
p.(Asn281Ser)
-
likely benign
g.102164865T>C
g.101771087T>C
GNPTAB(NM_024312.5):c.842A>G (p.N281S)
-
GNPTAB_000316
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/.
1
8
c.849_850del
r.(?)
p.(Gln283Hisfs*2)
-
pathogenic
g.102164858_102164859del
g.101771080_101771081del
-
-
GNPTAB_000003
-
-
-
-
Germline
-
-
-
-
-
Gerard C.P. Schaafsma
+/+
1
8
c.850del
r.(?)
p.(Thr284Leufs*5)
-
pathogenic
g.102164859del
g.101771081del
-
-
GNPTAB_000102
-
Journal: Kudo 2006
-
-
Germline
-
-
-
-
-
Renata Voltolini Velho
+/+
1
8
c.857dup
r.857dup
p.Asn287Glufs*5
-
pathogenic
g.102164851dup
g.101771073dup
-
-
GNPTAB_000103
-
Journal: Tappino 2009
-
-
Germline/De novo (untested)
-
-
-
-
-
Renata Voltolini Velho
-?/.
1
-
c.866C>G
r.(?)
p.(Thr289Ser)
-
likely benign
g.102164841G>C
g.101771063G>C
GNPTAB(NM_024312.4):c.866C>G (p.(Thr289Ser))
-
GNPTAB_000319
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/+
1
8
c.879dup
r.(?)
p.(Glu294Argfs*20)
-
pathogenic
g.102164831dup
g.101771053dup
-
-
GNPTAB_000295
-
-
-
-
Germline
-
-
-
-
-
Renata Voltolini Velho
?/.
1
8
c.886A>G
r.(?)
p.(Thr296Ala)
-
VUS
g.102164821T>C
g.101771043T>C
-
-
GNPTAB_000013
-
-
-
-
Germline
no
1/677 controls
-
-
-
Muhammad Raza
+/+
1
8
c.890dup
r.(?)
p.(Ser298Lysfs*16)
-
pathogenic
g.102164817dup
g.101771039dup
-
-
GNPTAB_000104
-
Journal: Wang et al. 2018
-
-
Germline/De novo (untested)
-
-
-
-
-
Renata Voltolini Velho
+/+
1
8
c.914dup
r.(?)
p.(Asp305Glufs*9)
-
pathogenic
g.102164793dup
g.101771015dup
-
-
GNPTAB_000105
-
Journal: Otomo et al. 2009
-
-
Germline/De novo (untested)
-
-
-
-
-
Renata Voltolini Velho
-?/.
1
-
c.933+5G>A
r.spl?
p.?
-
likely benign
g.102164769C>T
g.101770991C>T
GNPTAB(NM_024312.4):c.933+5G>A (p.?)
-
GNPTAB_000315
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/+
1
9
c.934_1013del
r.(?)
p.(Ser312Alafs*23)
-
pathogenic
g.102164286_102164365del
g.101770508_101770587del
delEx9
-
GNPTAB_000118
-
Journal: Wang 2018
-
-
Germline/De novo (untested)
-
-
-
-
-
Renata Voltolini Velho
+/+
1
9
c.940C>T
r.940c>u
p.Gln314*
-
pathogenic
g.102164357G>A
g.101770579G>A
-
-
GNPTAB_000106
-
Journal: Tappino 2009
-
-
Germline/De novo (untested)
-
-
-
-
-
Renata Voltolini Velho
?/.
1
-
c.950A>T
r.(?)
p.(Asp317Val)
-
VUS
g.102164347T>A
-
GNPTAB(NM_024312.5):c.950A>T (p.(Asp317Val))
-
GNPTAB_000342
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+?/.
1
9
c.961A>G
r.(?)
p.(Ser321Gly)
-
likely pathogenic
g.102164336T>C
g.101770558T>C
-
-
GNPTAB_000014
-
-
-
rs137853824
Germline
yes
1/1013 patients
-
-
-
Muhammad Raza
+/+
1
9
c.991del
r.(?)
p.(Tyr331Thrfs*28)
-
pathogenic
g.102164306del
g.101770528del
-
-
GNPTAB_000257
-
-
-
-
Germline/De novo (untested)
-
-
-
-
-
Renata Voltolini Velho
+/+
1
9
c.992A>G
r.(?)
p.(Tyr331Cys)
-
pathogenic
g.102164305T>C
g.101770527T>C
-
-
GNPTAB_000107
-
Journal: Yang 2017
-
-
Germline/De novo (untested)
-
-
-
-
-
Renata Voltolini Velho
+/+
1
9
c.1000C>T
r.(?)
p.(Arg334*)
-
pathogenic
g.102164297G>A
g.101770519G>A
-
-
GNPTAB_000108
-
Journal: Cathey 2010
-
-
Germline
-
-
-
-
-
Renata Voltolini Velho
+/+, +/.
2
9
c.1001G>A
r.(?)
p.(Arg334Gln)
-
pathogenic, pathogenic (recessive)
g.102164296C>T
g.101770518C>T
ref?:c.1001G>A
-
GNPTAB_000110
-
Journal: Cathey 2010
,
PubMed: Soden 2014
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Renata Voltolini Velho
+/+
1
9
c.1001G>C
r.(?)
p.(Arg334Pro)
-
pathogenic
g.102164296C>G
g.101770518C>G
-
-
GNPTAB_000111
-
Journal: Wang 2018
-
-
Germline/De novo (untested)
-
-
-
-
-
Renata Voltolini Velho
+/+
1
9
c.1001G>T
r.(?)
p.(Arg334Leu)
-
pathogenic
g.102164296C>A
g.101770518C>A
-
-
GNPTAB_000109
-
Journal: Otomo 2009
-
-
Germline/De novo (untested)
-
-
-
-
-
Renata Voltolini Velho
+/+
1
9
c.1016dup
r.(?)
p.(His339Glnfs*23)
-
pathogenic
g.102164281dup
g.101770503dup
-
-
GNPTAB_000258
-
-
-
-
Germline/De novo (untested)
-
-
-
-
-
Renata Voltolini Velho
+/+, +/.
2
9
c.1017_1020dup
r.(?)
p.(Pro341Cysfs*22), p.(Pro341CysfsTer22)
-
pathogenic, pathogenic (recessive)
g.102164280_102164283dup
g.101770502_101770505dup
ref?:c.1017_1020dupTGCA
-
GNPTAB_000112
-
Journal: Soden 2014
,
PubMed: Soden 2014
-
-
Germline, Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
,
Renata Voltolini Velho
+/+
1
9
c.1021_1023del
r.(?)
p.(Pro341del)
-
pathogenic
g.102164276_102164278del
g.101770498_101770500del
-
-
GNPTAB_000113
-
Journal: Mistri 2018
-
-
Germline/De novo (untested)
-
-
-
-
-
Renata Voltolini Velho
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