Global Variome shared LOVD
AMT (aminomethyltransferase)
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Unique variants in the AMT gene
The variants shown are described using the NM_000481.3 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'
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.
115 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.-3530C>T
r.(?)
p.(=)
-
VUS
g.49463413G>A
g.49425980G>A
NICN1(NM_032316.3):c.326C>T (p.(Ala109Val))
-
NICN1_000002
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
1
-
c.-2527A>G
r.(?)
p.(=)
-
likely benign
g.49462410T>C
g.49424977T>C
NICN1(NM_032316.3):c.572A>G (p.(His191Arg))
-
NICN1_000001
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
-
c.-76G>C
r.(?)
p.(=)
-
VUS
g.49459959C>G
-
AMT(NM_000481.4):c.-76G>C
-
AMT_000108
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+/.
1
1
c.-66A>G
r.(=)
p.(=)
-
pathogenic
g.49459949T>C
g.49422516T>C
1-66A>G
-
AMT_000036
-
-
-
-
Germline
yes
-
-
-
-
Johan Van Hove
+/.
1
1
c.-64A>G
r.(=)
p.(=)
-
pathogenic
g.49459947T>C
g.49422514T>C
1-64A>G
-
AMT_000035
-
-
-
-
Germline
yes
-
-
-
-
Johan Van Hove
+/.
2
1
c.-58C>T
r.(=)
p.(=)
-
pathogenic
g.49459941G>A
g.49422508G>A
1-58C>T
-
AMT_000034
-
-
-
-
Germline
yes
-
-
-
-
Johan Van Hove
+/., ?/?
3
1
c.-55C>T
r.(=), r.(?)
p.(=)
-
pathogenic, VUS
g.49459938G>A
g.49422505G>A
1-55C>T
-
AMT_000001
1 GCE patient (com-het)
PubMed: Pardal-Fernandez et al. 2009
-
-
Germline, SUMMARY record
yes
-
-
-
-
Anne Polvi
,
Johan Van Hove
+/.
7
1
c.2T>C
r.(?)
p.(Met1?)
-
pathogenic
g.49459882A>G
g.49422449A>G
-
-
AMT_000033
-
-
-
-
Germline
yes
-
-
-
-
Johan Van Hove
+/.
1
1
c.4C>T
r.(?)
p.(Gln2*)
-
pathogenic
g.49459880G>A
g.49422447G>A
-
-
AMT_000032
-
-
-
-
Germline
yes
-
-
-
-
Johan Van Hove
+/.
2
1
c.14dup
r.(?)
p.(Ser6Lysfs*22)
-
pathogenic
g.49459870dup
g.49422437dup
-
-
AMT_000031
-
-
-
-
Germline
yes
-
-
-
-
Johan Van Hove
+/.
8
1
c.15_18del
r.(?)
p.(Ser6Trpfs*89), p.(Ser6TrpfsTer89)
-
pathogenic
g.49459868_49459871del
g.49422435_49422438del
15_18del4, 15_18delAAGT
-
AMT_000028, AMT_000030
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record, Germline
yes
-
-
-
-
Johan Van Hove
,
VKGL-NL_Nijmegen
+/.
2
1
c.16del
r.(?)
p.(Ser6Valfs*90)
-
pathogenic
g.49459869del
g.49422436del
-
-
AMT_000029
-
-
-
-
Germline
yes
-
-
-
-
Johan Van Hove
+/+
1
1
c.59del
r.(?)
p.(Pro20Argfs*76)
-
pathogenic
g.49459830del
g.49422397del
183delC, c.54delC
-
AMT_000002
1 Japanese GCE family with 3 patients (P25; com-het)
PubMed: Kure et al.1998
,
PubMed: Kure et al. 2006
-
-
SUMMARY record
yes
-
-
-
-
Anne Polvi
+/+
1
1
c.61del
r.(?)
p.(Ala21Profs*75)
-
pathogenic
g.49459824del
g.49422391del
c.60delG
-
AMT_000003
1 Caucasian GCE family (P30; com-het)
PubMed: Kure et al. 2006
-
-
SUMMARY record
yes
-
-
-
-
Anne Polvi
+/+
1
1
c.63del
r.(?)
p.(Leu22Cysfs*74)
-
pathogenic
g.49459822del
g.49422389del
c.61delC
-
AMT_000004
No C in position 61, nex C:s in positions c.62_63; 1 Oriental GCE family (P91; com-het)
PubMed: Kure et al. 2006
-
-
SUMMARY record
yes
-
-
-
-
Anne Polvi
?/.
1
-
c.101G>A
r.(?)
p.(Arg34His)
-
VUS
g.49459694C>T
g.49422261C>T
AMT(NM_000481.3):c.101G>A (p.R34H)
-
AMT_000106
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
2
c.125A>C
r.(?)
p.(His42Pro)
-
pathogenic
g.49459670T>G
g.49422237T>G
-
-
AMT_000113
-
-
-
rs121964983
Germline
-
-
-
-
-
Zhizi Zhou
+/+
1
2
c.125A>G
r.(125a>g)
p.(His42Arg)
-
pathogenic
g.49459670T>C
g.49422237T>C
-
-
AMT_000005
1 Israeli-Arab GCE family (hom)
PubMed: Kure et al. 1998
,
PubMed: Kure et al. 2006
-
rs121964983
SUMMARY record
yes
-
-
-
-
Anne Polvi
+/+, +/.
5
2
c.139G>A
r.(139g>a), r.(?)
p.(Gly47Arg)
-
pathogenic
g.49459656C>T
g.49422223C>T
-
-
AMT_000006
1 GCE family (com-het)
PubMed: Nanao et al. 1994
-
rs121964982
Germline, SUMMARY record
yes
-
-
-
-
Anne Polvi
,
Johan Van Hove
+/+
1
2
c.139G>T
r.(139g>u)
p.(Gly47Trp)
-
pathogenic
g.49459656C>A
g.49422223C>A
-
-
AMT_000008
1 Caucasian GCE family (P34; het)
PubMed: Kure et al. 2006
-
-
SUMMARY record
yes
-
-
-
-
Anne Polvi
+/+
1
2
c.148del
r.(?)
p.(Val50Trpfs*46)
-
pathogenic
g.49459648del
g.49422215del
c.147delG
-
AMT_000009
1 Oriental GCE family (P115; com-het)
PubMed: Kure et al. 2006
-
-
SUMMARY record
yes
-
-
-
-
Anne Polvi
+/.
2
2
c.178_181del
r.(?)
p.(Gln60Thrfs*35)
-
pathogenic
g.49459614_49459617del
g.49422181_49422184del
178_181delCAGT
-
AMT_000074
-
-
-
-
Germline
yes
-
-
-
-
Johan Van Hove
?/.
1
-
c.203C>T
r.(?)
p.(Ser68Leu)
-
VUS
g.49459592G>A
g.49422159G>A
AMT(NM_000481.4):c.203C>T (p.S68L)
-
AMT_000097
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+/+, +/.
3
2
c.212A>C
r.(212a>c), r.(?)
p.(His71Pro)
-
pathogenic
g.49459583T>G
g.49422150T>G
-
-
AMT_000010
1 Caucasian GCE family (P75; com-het)
PubMed: Kure et al. 2006
-
rs149105054
Germline, SUMMARY record
yes
-
-
-
-
Anne Polvi
,
Johan Van Hove
+/+, +/.
9
2
c.217C>T
r.(217c>u), r.(?)
p.(Arg73Cys)
-
pathogenic
g.49459578G>A
g.49422145G>A
-
-
AMT_000011
1 Caucasian GCE family (P75; com-het)
PubMed: Kure et al. 2006
-
-
Germline, SUMMARY record
yes
-
-
-
-
Anne Polvi
,
Johan Van Hove
+/+, +/.
5
2
c.230C>T
r.(230c>u), r.(?)
p.(Ser77Leu)
-
pathogenic
g.49459565G>A
g.49422132G>A
-
-
AMT_000012
1 Caucasian GCE family (P21; hom)
PubMed: Kure et al. 2006
-
-
Germline, SUMMARY record
yes
-
-
-
-
Anne Polvi
,
Johan Van Hove
+/.
4
2
c.248A>G
r.(?)
p.(His83Arg)
-
pathogenic
g.49459547T>C
g.49422114T>C
-
-
AMT_000073
-
-
-
-
Germline
yes
-
-
-
-
Johan Van Hove
-?/.
1
-
c.258+242A>G
r.(=)
p.(=)
-
likely benign
g.49459295T>C
g.49421862T>C
AMT(NM_000481.3):c.258+242A>G
-
AMT_000096
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-?/.
1
-
c.259-109T>G
r.(=)
p.(=)
-
likely benign
g.49459114A>C
g.49421681A>C
AMT(NM_000481.3):c.259-109T>G
-
AMT_000095
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/+, +/.
3
2i
c.259-1G>C
r.spl?
p.(Thr87_Gln113del), p.?
-
pathogenic
g.49459006C>G
g.49421573C>G
259-1>C, IVS2-1G>C
-
AMT_000013
1 GCE family (com-het)
PubMed: Pardal-Fernandez et al. 2009
-
-
Germline, SUMMARY record
yes
-
-
-
-
Anne Polvi
,
Johan Van Hove
+/., +?/.
10
3
c.280C>T
r.(?), r.280c>u
p.(Arg94Trp), p.Arg94Trp
-
likely pathogenic, pathogenic
g.49458984G>A
g.49421551G>A
-
-
AMT_000072
-
-
-
-
Germline
yes
-
-
-
-
Johan Van Hove
,
Pilar Rodriguez Pombo
?/.
1
-
c.281G>A
r.(?)
p.(Arg94Gln)
-
VUS
g.49458983C>T
-
AMT(NM_000481.4):c.281G>A (p.(Arg94Gln))
-
AMT_000115
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
2
3
c.293T>G
r.(?)
p.(Met98Arg)
-
pathogenic
g.49458971A>C
g.49421538A>C
-
-
AMT_000071
-
-
-
-
Germline
yes
-
-
-
-
Johan Van Hove
+/., +?/., ?/.
7
3
c.317T>C
r.(?)
p.(Ile106Thr)
-
likely pathogenic, pathogenic, VUS
g.49458947A>G
g.49421514A>G
AMT(NM_000481.3):c.317T>C (p.I106T)
-
AMT_000070
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record, Germline
yes
-
-
-
-
Johan Van Hove
,
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
-?/.
1
-
c.339+3G>A
r.spl?
p.?
-
likely benign
g.49458922C>T
-
AMT(NM_000481.3):c.339+3G>A
-
AMT_000109
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
2
3i
c.340-1G>A
r.spl
p.(Gly114_GlyLeun157del), p.?
-
pathogenic
g.49457776C>T
g.49420343C>T
IVS3-1G>A
-
AMT_000069
-
-
-
-
Germline
yes
-
-
-
-
Johan Van Hove
+/.
4
4
c.350C>T
r.(?)
p.(Ser117Leu)
-
pathogenic
g.49457765G>A
g.49420332G>A
-
-
AMT_000068
-
-
-
-
Germline
yes
-
-
-
-
Johan Van Hove
-?/.
2
-
c.354G>A
r.(?)
p.(Leu118=)
-
likely benign
g.49457761C>T
g.49420328C>T
AMT(NM_000481.3):c.354G>A (p.L118=)
-
AMT_000105
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
+/.
1
4
c.395_400del
r.(?)
p.(Val132_Asn134delinsAsp)
-
pathogenic
g.49457715_49457720del
g.49420282_49420287del
395_400del6
-
AMT_000067
-
-
-
-
Germline
yes
-
-
-
-
Johan Van Hove
+/+, +/.
3
4
c.434A>T
r.(434a>u), r.(?)
p.(Asn145Ile)
-
pathogenic
g.49457681T>A
g.49420248T>A
-
-
AMT_000014
2 Caucasian/Native American sibs (com-het) with GCE
PubMed: Toone et al. 2001
-
-
Germline, SUMMARY record
yes
-
-
-
-
Anne Polvi
,
Johan Van Hove
+/.
2
4
c.451_466del
r.(?)
p.(Lys151Cysfs*25)
-
pathogenic
g.49457649_49457664del
g.49420216_49420231del
451_466del16
-
AMT_000065
-
-
-
-
Germline
yes
-
-
-
-
Johan Van Hove
+/+, +/., ?/.
8
4
c.452_466del
r.(452_466del), r.(?)
p.(Lys151_Leu155del)
-
pathogenic, VUS
g.49457650_49457664del
g.49420217_49420231del
452_466del15, AMT(NM_000481.3):c.452_466delAAGATTTGGCCCTCA (p.K151_L155del)
-
AMT_000015
1 Caucasian GCE family (P61; com-het), VKGL data sharing initiative Nederland
PubMed: Kure et al. 2006
-
-
CLASSIFICATION record, Germline, SUMMARY record
yes
-
-
-
-
Anne Polvi
,
Johan Van Hove
,
VKGL-NL_Rotterdam
+/.
2
4
c.453_466del
r.(?)
p.(Lys151Asnfs*22)
-
pathogenic
g.49457650_49457663del
g.49420217_49420230del
452_465del14
-
AMT_000066
-
-
-
-
Germline
yes
-
-
-
-
Johan Van Hove
-?/.
1
-
c.463C>T
r.(?)
p.(Leu155Phe)
-
likely benign
g.49457652G>A
g.49420219G>A
AMT(NM_000481.3):c.463C>T (p.L155F)
-
AMT_000104
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/+, +/.
6
4i
c.471+2T>C
r.spl?
p.?
-
pathogenic
g.49457642A>G
g.49420209A>G
IVS4+2T>C
-
AMT_000016
1 Caucasian GCE family (P31; com-het)
PubMed: Kure et al. 2006
-
-
Germline, SUMMARY record
yes
-
-
-
-
Anne Polvi
,
Johan Van Hove
-/.
1
-
c.471+12_471+13del
r.(=)
p.(=)
-
benign
g.49457633_49457634del
g.49420200_49420201del
AMT(NM_000481.3):c.471+12_471+13delCT
-
AMT_000094
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/.
3
5
c.496C>T
r.(?)
p.(Gln166*)
-
pathogenic
g.49457197G>A
g.49419764G>A
-
-
AMT_000064
-
-
-
-
Germline
yes
-
-
-
-
Johan Van Hove
+/.
1
5
c.515T>C
r.(?)
p.(Leu172Pro)
-
pathogenic
g.49457178A>G
g.49419745A>G
-
-
AMT_000063
-
-
-
-
Germline
yes
-
-
-
-
Johan Van Hove
+/.
2
5
c.534_535dup
r.(?)
p.(Leu179Profs*3)
-
pathogenic
g.49457159_49457160dup
g.49419726_49419727dup
534_535insCC
-
AMT_000062
-
-
-
-
Germline
yes
-
-
-
-
Johan Van Hove
+/+
1
5
c.535del
r.(?)
p.(Leu179Cysfs*2)
-
pathogenic
g.49457160del
g.49419727del
-
-
AMT_000017
1 Oriental GCE family (P91; com-het)
PubMed: Kure et al. 2006
-
-
SUMMARY record
yes
-
-
-
-
Anne Polvi
+/.
2
5
c.545T>C
r.(?)
p.(Leu182Pro)
-
pathogenic
g.49457148A>G
g.49419715A>G
-
-
AMT_000061
-
-
-
-
Germline
yes
-
-
-
-
Johan Van Hove
+/., +?/.
2
5
c.548A>G
r.(?)
p.(Gln183Arg)
-
pathogenic
g.49457145T>C
g.49419712T>C
-
-
AMT_000060
-
-
-
-
Germline
yes
-
-
-
-
Johan Van Hove
,
Zhizi Zhou
-?/.
1
-
c.550+10C>G
r.(=)
p.(=)
-
likely benign
g.49457133G>C
-
AMT(NM_000481.4):c.550+10C>G
-
AMT_000114
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
2
6
c.551_552delinsAA
r.(?)
p.(Gly184Glu)
-
pathogenic
g.49456837_49456838delinsTT
g.49419404_49419405delinsTT
-
-
AMT_000059
-
-
-
-
Germline
yes
-
-
-
-
Johan Van Hove
+/+, +/.
2
6
c.574C>T
r.(574c>u), r.(?)
p.(Gln192*)
-
pathogenic
g.49456815G>A
g.49419382G>A
-
-
AMT_000018
1 GCE patient (het)
PubMed: Toone et al. 2000
-
rs121964986
Germline, SUMMARY record
yes
-
-
-
-
Anne Polvi
,
Johan Van Hove
?/.
2
-
c.593A>T
r.(?)
p.(Asp198Val)
-
VUS
g.49456796T>A
g.49419363T>A
AMT(NM_000481.3):c.593A>T (p.D198V, p.(Asp198Val))
-
AMT_000093
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
-/., -?/.
4
-
c.631G>A
r.(?)
p.(Glu211Lys)
-
benign, likely benign
g.49456758C>T
g.49419325C>T
AMT(NM_000481.3):c.631G>A (p.(Glu211Lys), p.E211K), AMT(NM_000481.4):c.631G>A (p.E211K)
-
AMT_000092
73 heterozygous, no homozygous;
Clinindb (India)
, VKGL data sharing initiative Nederland
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs116192290
CLASSIFICATION record, Germline
-
73/2795 individuals
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Groningen
,
VKGL-NL_Utrecht
,
Mohammed Faruq
+/., +?/+?, -?/., ?/.
5
6
c.635T>C
r.(635u>c), r.(?)
p.(Val212Ala)
-
likely benign, likely pathogenic, pathogenic, VUS
g.49456754A>G
g.49419321A>G
AMT(NM_000481.3):c.635T>C (p.V212A), AMT(NM_000481.4):c.635T>C (p.V212A)
-
AMT_000019
1 GCE patient, VKGL data sharing initiative Nederland
PubMed: Toone et al. 2003
-
rs201141125
CLASSIFICATION record, Germline, SUMMARY record
yes
-
-
-
-
Anne Polvi
,
Johan Van Hove
,
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
+?/.
1
-
c.645_646del
r.(?)
p.(Ser216TrpfsTer60)
-
likely pathogenic
g.49456745_49456746del
g.49419312_49419313del
AMT(NM_000481.3):c.645_646del (p.(Ser216TrpfsTer60))
-
AMT_000103
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+?/.
1
-
c.655C>T
r.(?)
p.(Arg219Cys)
ACMG
likely pathogenic
g.49456734G>A
-
-
-
AMT_000111
-
-
-
rs150079386
Unknown
-
-
-
-
-
Cordula Haas
+/.
12
6
c.664C>T
r.(?)
p.(Arg222Cys)
-
pathogenic
g.49456725G>A
g.49419292G>A
-
-
AMT_000058
-
-
-
-
Germline
yes
-
-
-
-
Johan Van Hove
+/.
3
6
c.665G>A
r.(?)
p.(Arg222His)
-
pathogenic
g.49456724C>T
g.49419291C>T
-
-
AMT_000056
-
-
-
-
Germline
yes
-
-
-
-
Johan Van Hove
+/.
1
6
c.665G>C
r.(?)
p.(Arg222Pro)
-
pathogenic
g.49456724C>G
g.49419291C>G
-
-
AMT_000057
-
-
-
-
Germline
yes
-
-
-
-
Johan Van Hove
+/+, +/.
2
6
c.674A>G
r.(674a>g), r.(?)
p.(Tyr225Cys)
-
pathogenic
g.49456715T>C
g.49419282T>C
-
-
AMT_000020
1 GCE patient
PubMed: Toone et al. 2003
-
-
Germline, SUMMARY record
yes
-
-
-
-
Anne Polvi
,
Johan Van Hove
-?/.
1
-
c.681A>G
r.(?)
p.(Gly227=)
-
likely benign
g.49456708T>C
g.49419275T>C
AMT(NM_000481.3):c.681A>G (p.G227=)
-
AMT_000102
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
6
c.685G>C
r.(?)
p.(Asp229His)
-
pathogenic
g.49456704C>G
g.49419271C>G
-
-
AMT_000055
-
-
-
-
Germline
yes
-
-
-
-
Johan Van Hove
+/.
1
6_6i
c.695_696+33delinsGGCTGTACAGA
r.spl?
p.(Asp229Glyfs*10)
-
pathogenic
g.49456660_49456694delinsTCTGTACAGCC
g.49419227_49419261delinsTCTGTACAGCC
695_696+33del35insGGCTGTACAGA
-
AMT_000053
-
-
-
-
Germline
yes
-
-
-
-
Johan Van Hove
+/.
1
6i
c.696+5G>C
r.spl?
p.?
-
pathogenic
g.49456688C>G
g.49419255C>G
IVS6+5G>C
-
AMT_000054
-
-
-
-
Germline
yes
-
-
-
-
Johan Van Hove
-?/.
1
-
c.697-3C>T
r.spl?
p.?
-
likely benign
g.49456587G>A
g.49419154G>A
AMT(NM_000481.3):c.697-3C>T
-
AMT_000101
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
7
c.731dup
r.(?)
p.(Thr245Asnfs*32)
-
pathogenic
g.49456550dup
g.49419117dup
-
-
AMT_000052
-
-
-
-
Germline
yes
-
-
-
-
Johan Van Hove
+/.
1
7
c.781A>G
r.(?)
p.(Arg261Gly)
-
pathogenic
g.49456500T>C
g.49419067T>C
-
-
AMT_000112
-
-
-
-
Germline
-
-
-
-
-
Zhizi Zhou
+/.
3
7
c.793C>A
r.(?)
p.(Arg265Ser)
-
pathogenic
g.49456488G>T
g.49419055G>T
-
-
AMT_000050
-
-
-
-
Germline
yes
-
-
-
-
Johan Van Hove
+/.
4
7
c.793C>T
r.(?)
p.(Arg265Cys)
-
pathogenic
g.49456488G>A
g.49419055G>A
-
-
AMT_000051
-
-
-
-
Germline
yes
-
-
-
-
Johan Van Hove
+/.
6
7
c.794G>A
r.(?)
p.(Arg265His)
-
pathogenic
g.49456487C>T
g.49419054C>T
AMT(NM_000481.3):c.794G>A (p.R265H)
-
AMT_000049
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record, Germline
yes
-
-
-
-
Johan Van Hove
,
VKGL-NL_Utrecht
+/+, +/.
3
7
c.806G>A
r.(806g>a), r.(?)
p.(Gly269Asp)
ACMG
pathogenic, pathogenic (recessive)
g.49456475C>T
g.49419042C>T
-
-
AMT_000007
1 GCE family (hom)
PubMed: Hu 2019
,
PubMed: Nanao et al. 1994
-
rs121964981
Germline, SUMMARY record
yes
-
-
-
-
Johan den Dunnen
,
Anne Polvi
,
Johan Van Hove
+/.
1
7
c.808_811inv
r.(?)
p.(Leu270_Cys271delinsArgGly)
-
pathogenic
g.49456470_49456473inv
g.49419037_49419040inv
808_811del4insAGAG
-
AMT_000048
-
-
-
-
Germline
yes
-
-
-
-
Johan Van Hove
-?/.
1
-
c.822G>C
r.(?)
p.(Gly274=)
-
likely benign
g.49456459C>G
g.49419026C>G
AMT(NM_000481.3):c.822G>C (p.G274=)
-
AMT_000100
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.825T>A
r.(?)
p.(Asn275Lys)
-
VUS
g.49456456A>T
g.49419023A>T
AMT(NM_000481.3):c.825T>A (p.N275K)
-
AMT_000091
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/+, +/.
5
7
c.826G>C
r.(826g>c), r.(?)
p.(Asp276His)
-
pathogenic
g.49456455C>G
g.49419022C>G
-
-
AMT_000021
1 GCE family
PubMed: Kure et al.1998
,
PubMed: Kure et al. 2006
-
rs121964984
Germline, SUMMARY record
yes
-
-
-
-
Anne Polvi
,
Johan Van Hove
+/.
1
7
c.845C>T
r.(?)
p.(Thr282Ile)
-
pathogenic
g.49456436G>A
g.49419003G>A
-
-
AMT_000047
-
-
-
-
Germline
yes
-
-
-
-
Johan Van Hove
+/.
1
7i
c.877+5G>A
r.spl?
p.?
-
pathogenic
g.49456399C>T
g.49418966C>T
IVS7+5G>A
-
AMT_000046
-
-
-
-
Germline
yes
-
-
-
-
Johan Van Hove
+/+, +/.
2
7i
c.878-1G>A
r.spl?
p.(Lys294fs), p.?
-
pathogenic
g.49455407C>T
g.49417974C>T
IVS7-1G>A
-
AMT_000022
5 GCE patients
PubMed: Toone et al. 2001
,
PubMed: Toone et al. 2001
-
rs181134220
Germline, SUMMARY record
yes
-
-
-
-
Anne Polvi
,
Johan Van Hove
+/.
3
8
c.879G>A
r.(=)
p.(=)
-
pathogenic
g.49455405C>T
g.49417972C>T
IVS7-1G>A
-
AMT_000045
-
-
-
-
Germline
yes
-
-
-
-
Johan Van Hove
+/.
4
8
c.886C>T
r.(?)
p.(Arg296Cys)
-
pathogenic
g.49455398G>A
g.49417965G>A
-
-
AMT_000044
-
-
-
-
Germline
yes
-
-
-
-
Johan Van Hove
+/+, +/.
8
8
c.887G>A
r.(887g>a), r.(?)
p.(Arg296His)
-
pathogenic
g.49455397C>T
g.49417964C>T
-
-
AMT_000023
1 GCE patient
PubMed: Toone et al. 2003
-
-
Germline, SUMMARY record
yes
-
-
-
-
Anne Polvi
,
Johan Van Hove
+/.
1
8
c.887G>C
r.(?)
p.(Arg296Pro)
-
pathogenic
g.49455397C>G
g.49417964C>G
-
-
AMT_000043
-
-
-
-
Germline
yes
-
-
-
-
Johan Van Hove
+/.
2
-
c.889C>T
r.(?)
p.(Arg297Ter)
-
pathogenic
g.49455395G>A
g.49417962G>A
AMT(NM_000481.3):c.889C>T (p.R297*)
-
AMT_000090
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
-?/.
1
-
c.891A>G
r.(?)
p.(Arg297=)
-
likely benign
g.49455393T>C
g.49417960T>C
AMT(NM_000481.3):c.891A>G (p.R297=)
-
AMT_000099
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.898A>G
r.(?)
p.(Met300Val)
-
likely benign
g.49455386T>C
-
AMT(NM_000481.3):c.898A>G (p.M300V)
-
AMT_000110
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/.
1
8
c.921G>A
r.(?)
p.(Val307=)
-
pathogenic
g.49455363C>T
g.49417930C>T
-
-
AMT_000042
1 more item
-
-
-
Germline
yes
-
-
-
-
Johan Van Hove
?/.
1
-
c.923T>C
r.(?)
p.(Ile308Thr)
-
VUS
g.49455361A>G
g.49417928A>G
AMT(NM_000481.3):c.923T>C (p.(Ile308Thr))
-
AMT_000089
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
1
-
c.953G>A
r.(?)
p.(Arg318Gln)
-
likely benign
g.49455331C>T
g.49417898C>T
AMT(NM_000481.4):c.953G>A (p.R318Q)
-
AMT_000088
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-/.
2
-
c.954G>A
r.(?)
p.(Arg318=)
-
benign
g.49455330C>T
g.49417897C>T
AMT(NM_000481.3):c.954G>A (p.R318=), AMT(NM_000481.4):c.954G>A (p.R318=)
-
AMT_000087
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
,
VKGL-NL_Utrecht
+/.
2
8
c.958C>G
r.(?)
p.(Arg320Gly)
-
pathogenic
g.49455326G>C
g.49417893G>C
-
-
AMT_000040
-
-
-
-
Germline
yes
-
-
-
-
Johan Van Hove
+/.
2
8
c.958C>T
r.(?)
p.(Arg320Cys)
-
pathogenic
g.49455326G>A
g.49417893G>A
-
-
AMT_000041
-
-
-
-
Germline
yes
-
-
-
-
Johan Van Hove
+/+, +/.
40
8
c.959G>A
r.(959g>a), r.(?)
p.(Arg320His)
-
pathogenic
g.49455325C>T
g.49417892C>T
AMT(NM_000481.3):c.959G>A (p.(Arg320His), p.R320H)
-
AMT_000024
9 GCE patients, VKGL data sharing initiative Nederland
PubMed: Nanao et al. 1994
,
PubMed: Toone et al. 2000
,
PubMed: Toone et al. 2001
-
rs121964985
CLASSIFICATION record, Germline, SUMMARY record
yes
-
-
-
-
Anne Polvi
,
Johan Van Hove
,
VKGL-NL_Leiden
,
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
+/+
1
8
c.970_972del
r.(?)
p.(Met324del)
-
pathogenic
g.49455314_49455316del
g.49417881_49417883del
c.970_972delATG: 320Met Deletion
-
AMT_000025
1 Oriental GCE family (P115; com-het)
PubMed: Kure et al. 2006
-
-
SUMMARY record
yes
-
-
-
-
Anne Polvi
+/+
1
8
c.982_983delinsT
r.(982_983delgcinsu)
p.(Ala328Serfs*10)
-
pathogenic
g.49455301_49455302delinsA
g.49417868_49417869delinsA
c.982_972GC>T
-
AMT_000026
1 Caucasian GCE family (P61; com-het)
PubMed: Kure et al. 2006
-
-
SUMMARY record
yes
-
-
-
-
Anne Polvi
+/.
3
8
c.987del
r.(?)
p.(Met330Cysfs*8)
-
pathogenic
g.49455301del
g.49417868del
-
-
AMT_000039
-
-
-
-
Germline
yes
-
-
-
-
Johan Van Hove
+/.
4
8
c.992G>A
r.(?)
p.(Arg331Gln)
-
pathogenic
g.49455292C>T
g.49417859C>T
-
-
AMT_000038
-
-
-
-
Germline
yes
-
-
-
-
Johan Van Hove
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