Global Variome shared LOVD
POMGNT1 (protein O-linked mannose beta1,2-N-acetylg...)
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Curator:
Johan den Dunnen
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Unique variants in the POMGNT1 gene
This database is one of the gene variant databases from the
Leiden Muscular Dystrophy pages
The variants shown are described using the
NM_001243766.1
NM_017739.3
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.
233 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
?/.
1
-
c.-409G>A
r.(?)
p.(=)
-
VUS
g.46685735C>T
g.46220063C>T
LURAP1(NM_001013615.2):c.563C>T (p.(Ala188Val))
-
LURAP1_000002
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
1
-
c.-390T>C
r.(?)
p.(=)
-
likely benign
g.46685716A>G
g.46220044A>G
LURAP1(NM_001013615.2):c.544A>G (p.(Arg182Gly))
-
LURAP1_000012
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
1
-
c.-51G>A
r.(?)
p.(=)
-
likely benign
g.46685377C>T
-
POMGNT1(NM_001243766.2):c.-51G>A
-
LURAP1_000041
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/+?, +/.
2
1i_8i, 2i_8i
c.(-51+1_-50-1)_(751+1_752-1)del
r.?
p.0?, p.?
-
pathogenic
g.(46660074_46660224)_(46663544_46685376)del
-
deletion exons 2–8
-
POMGNT1_000134
1 Italian MDDGA3 (MEB) patient (com-het)
PubMed: Saredi 2012
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
-/.
2
2
c.-11G>A
r.(?)
p.(=)
-
benign
g.46663504C>T
g.46197832C>T
POMGNT1(NM_001243766.2):c.-11G>A
-
POMGNT1_000040
other pathogenic change in one patient, VKGL data sharing initiative Nederland
PubMed: Godfrey 2007
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_AMC
+?/.
1
1
c.0?
r.0?
p.0?
-
likely pathogenic
g.45862062_46510920dup
-
POMGNT1
-
MMACHC_000047
heterozygous, both parents wild type; t(2;14)(q37.3;q13) with duplication in chromosome 1p34.1
PubMed: Hanemaaijer 2009
-
-
De novo
yes
-
-
-
-
LOVD
+/.
2
17i_23_
c.1539+184_*38{0}
r.?
p.?
-
pathogenic, pathogenic (recessive)
g.46652588_46657586del
g.46186916_46191914del
1539+184_1983+2354del4972
-
POMGNT1_000113
5.0 Kb deletion exon 17-22
PubMed: Vuillaumier-Barrot 2011
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Anne Polvi
+/.
2
17i_22_, 17i_23_
c.1539+261_*38{0}
r.?
p.?
-
pathogenic, pathogenic (recessive)
g.46652537_46657509del
g.46186865_46191837del
1539+261_1983+2405del4972
-
POMGNT1_000062
5.0 Kb deletion exon 17-22
PubMed: Vuillaumier-Barrot 2011
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Anne Polvi
?/.
5
22
c.?
r.(?)
p.(Thr660Ile), p.?
-
VUS
g.?
-
1927T>G (F643V), 1933G>C (E645Q), 1979C>T (T660I)
-
NPHS2_000000, POMGNT1_000000
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/.
1
-
c.1A>T
r.(?)
p.(Met1?)
-
pathogenic
g.46663493T>A
g.46197821T>A
-
-
POMGNT1_000193
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
2
c.16C>T
r.(?)
p.(Pro6Ser)
-
VUS
g.46663478G>A
g.46197806G>A
-
-
POMGNT1_000241
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/+?, +/.
2
2
c.25dup
r.(25dup), r.(?)
p.(Leu9Profs*20)
-
pathogenic
g.46663473dup
g.46197801dup
25_26insC
-
POMGNT1_000052
1 German MDDGA3 (MEB) patient (com-het)
PubMed: Hehr 2007
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Rosário dos Santos
?/.
2
2
c.38T>C
r.(?)
p.(Phe13Ser)
-
VUS
g.46663456A>G
g.46197784A>G
POMGNT1(NM_001243766.1):c.38T>C (p.F13S)
-
POMGNT1_000183
no second variant, VKGL data sharing initiative Nederland
PubMed: Nallamilli 2018
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Madhuri Hegde
,
VKGL-NL_Rotterdam
?/.
3
2
c.46C>T
r.(?)
p.(Arg16Trp)
-
VUS
g.46663448G>A
g.46197776G>A
-
-
POMGNT1_000010
no second variant
PubMed: Nallamilli 2018
-
rs34058684
Germline
-
0.01
-
-
-
Johan den Dunnen
,
Madhuri Hegde
?/.
1
-
c.56G>A
r.(?)
p.(Arg19Gln)
-
VUS
g.46663438C>T
g.46197766C>T
667G>A;56G>A
-
POMGNT1_000259
-
PubMed: Song 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.73T>G
r.(?)
p.(Trp25Gly)
-
VUS
g.46663421A>C
-
POMGNT1(NM_017739.4):c.73T>G (p.(Trp25Gly))
-
LURAP1_000036
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
2
c.107G>A
r.(?)
p.(Arg36Gln)
-
VUS
g.46663387C>T
g.46197715C>T
-
-
POMGNT1_000240
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
1
2i
c.120+3A>G
r.spl?
p.?
-
VUS
g.46663371T>C
g.46197699T>C
-
-
POMGNT1_000239
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
-?/.
4
-
c.120+13C>T
r.(=)
p.(=)
-
likely benign
g.46663361G>A
g.46197689G>A
1 more item
-
POMGNT1_000182
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
,
VKGL-NL_AMC
-/.
1
-
c.121-278A>G
r.(=)
p.(=)
-
benign
g.46663034T>C
g.46197362T>C
POMGNT1(NM_001290129.2):c.-1A>G
-
LURAP1_000020
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/.
1
-
c.121-228A>G
r.(=)
p.(=)
-
likely benign
g.46662984T>C
-
POMGNT1(NM_001290129.2):c.50A>G (p.(His17Arg))
-
LURAP1_000040
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
3
c.172A>G
r.(?)
p.(Ile58Val)
-
VUS
g.46662705T>C
g.46197033T>C
-
-
POMGNT1_000238
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/., +?/.
9
3, 4
c.187C>T
r.(?)
p.(Arg63*), p.(Arg63Ter)
-
likely pathogenic, pathogenic, pathogenic (recessive)
g.46662690G>A
g.46197018G>A
281C>T (R63X), c.187C>T,
1 more item
-
POMGNT1_000009, POMGNT1_000076
VKGL data sharing initiative Nederland
PubMed: Mercuri 2009
,
PubMed: Panneman 2023
,
PubMed: Song 2021
,
PubMed: Taniguchi 2003
,
OMIM:var0009
,
1 more item
-
-
CLASSIFICATION record, Germline, Unknown
yes
1/309 unresolved RP cases
-
-
-
Johan den Dunnen
,
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
,
Daan Panneman
-/.
2
3i
c.235+33T>G
r.(?)
p.(=)
-
benign
g.46662609A>C
g.46196937A>C
-
-
POMGNT1_000041
found in several patients
from website {DBsub-Emory},
PubMed: Godfrey 2007
-
-
Germline, Unknown
-
-
-
-
-
Johan den Dunnen
,
Madhuri Hegde
-/.
1
-
c.236-16C>G
r.(=)
p.(=)
-
benign
g.46662537G>C
g.46196865G>C
POMGNT1(NM_017739.3):c.236-16C>G
-
POMGNT1_000180
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/., ?/.
2
3i
c.236-13T>C
r.(=), r.(?)
p.(=)
-
likely benign, VUS
g.46662534A>G
g.46196862A>G
POMGNT1(NM_017739.3):c.236-13T>C
-
POMGNT1_000065
VKGL data sharing initiative Nederland
from website {DBsub-Emory}
-
-
CLASSIFICATION record, Unknown
-
-
-
-
-
Madhuri Hegde
,
VKGL-NL_Rotterdam
?/.
1
-
c.244C>G
r.(?)
p.(Leu82Val)
ACMG
VUS
g.46662513G>C
g.46196841G>C
POMGNT1 c.244C>G, p.(Leu82Val)
-
POMGNT1_000274
heterozygous, probably non-causal incidental finding
PubMed: Zhu 2022
-
-
Germline/De novo (untested)
?
-
-
-
-
LOVD
?/.
1
4
c.244C>T
r.(?)
p.(=)
-
VUS
g.46662513G>A
g.46196841G>A
-
-
POMGNT1_000059
-
-
-
rs34212808
Germline
-
0.34
-
-
-
Johan den Dunnen
?/.
1
-
c.251G>A
r.(?)
p.(Arg84His)
-
VUS
g.46662506C>T
-
POMGNT1(NM_001243766.1):c.251G>A (p.R84H)
-
LURAP1_000024
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.251G>T
r.(?)
p.(Arg84Leu)
-
VUS
g.46662506C>A
-
POMGNT1(NM_001243766.1):c.251G>T (p.(Arg84Leu))
-
LURAP1_000030
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
4
c.266G>A
r.(?)
p.(Arg89Gln)
-
VUS
g.46662491C>T
g.46196819C>T
-
-
POMGNT1_000237
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
1
4
c.268C>T
r.(?)
p.(Arg90Cys)
-
VUS
g.46662489G>A
g.46196817G>A
-
-
POMGNT1_000236
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
1
4
c.269G>A
r.(?)
p.(Arg90His)
-
VUS
g.46662488C>T
g.46196816C>T
-
-
POMGNT1_000235
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
1
-
c.275G>T
r.(?)
p.(Gly92Val)
-
VUS
g.46662482C>A
-
POMGNT1(NM_001243766.1):c.275G>T (p.G92V)
-
POMGNT1_000178
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
4
c.286C>T
r.(?)
p.(Arg96Trp)
-
VUS
g.46662471G>A
g.46196799G>A
-
-
POMGNT1_000234
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/.
3
-
c.296T>C
r.(?)
p.(Leu99Pro)
-
pathogenic, pathogenic (recessive)
g.46662461A>G
g.46196789A>G
-
-
POMGNT1_000156
-
PubMed: Fu 2017
,
PubMed: Song 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Xiaona Fu
-/., -?/.
4
-
c.301G>A
r.(?)
p.(Val101Ile)
-
benign, likely benign
g.46662456C>T
g.46196784C>T
POMGNT1(NM_001243766.1):c.301G>A (p.V101I), POMGNT1(NM_001243766.2):c.301G>A (p.V101I)
-
POMGNT1_000192
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
,
VKGL-NL_AMC
+/.
2
4
c.304G>T
r.(?)
p.(Glu102Ter)
ACMG
pathogenic (recessive)
g.46662453C>A
g.46196781C>A
-
-
POMGNT1_000272
ACMG PVS1, PM2, PP3, PP4
PubMed: Kritioti 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.333T>C
r.(?)
p.(Tyr111=)
-
likely benign
g.46662424A>G
-
POMGNT1(NM_001243766.1):c.333T>C (p.Y111=)
-
LURAP1_000027
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/+?, +/.
5
4
c.351del
r.(351del), r.(?)
p.(Thr118Argfs*26)
-
pathogenic, pathogenic (recessive)
g.46662406del, g.46662407del
g.46196735del
350delC, POMGNT1:c.351del
-
POMGNT1_000075, POMGNT1_000196
1 more item
PubMed: Colombo-2020
,
PubMed: Mercuri 2009
,
PubMed: Saredi 2012
-
rs386834028
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
-/.
2
4i
c.355-27C>T
r.(?)
p.(=)
-
benign
g.46661776G>A
g.46196104G>A
-
-
POMGNT1_000042
found in one patient
from website {DBsub-Emory},
PubMed: Godfrey 2007
-
-
Germline, Unknown
-
-
-
-
-
Johan den Dunnen
,
Madhuri Hegde
?/.
1
5
c.358C>G
r.(?)
p.(Leu120Val)
-
VUS
g.46661746G>C
g.46196074G>C
-
-
POMGNT1_000233
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+?/.
19
4
c.359T>G
r.(?)
p.(Leu120Arg)
-
likely pathogenic
g.46661745A>C
g.46196073A>C
POMGNT1 c.359T>G, p.Leu120Arg, POMGNT1:NM_001290129:exon4:c.359T>G:p.L120R
-
POMGNT1_000256
homozygous
PubMed: Chen 2020
,
PubMed: Wang 2016
-
-
Germline
?, yes
-
-
-
-
LOVD
+?/.
3
5
c.385C>T
r.(?)
p.(Arg129Trp)
ACMG
likely pathogenic, likely pathogenic (dominant)
g.46661719G>A
g.46196047G>A
POMGNT1 c.385C > T (p.Arg129Trp)
-
POMGNT1_000271
ACMG PM1, PM2, PM3, PP2, PP3, PP4, heterozygous
PubMed: Borisovna 2019
,
PubMed: Kritioti 2021
-
rs375431575
Germline
yes
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.386G>A
r.(?)
p.(Arg129Gln)
ACMG
likely pathogenic (recessive)
g.46661718C>T
-
-
-
POMGNT1_000254
ACMG PM1, PM2, PP2, PP3, PP4
PubMed: Mohammadi 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.387G>A
r.(?)
p.(Arg129=)
-
likely benign
g.46661717C>T
g.46196045C>T
POMGNT1(NM_001243766.2):c.387G>A (p.R129=)
-
LURAP1_000009
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
1
5
c.418A>G
r.(?)
p.(Thr140Ala)
-
VUS
g.46661686T>C
g.46196014T>C
-
-
POMGNT1_000232
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
1
-
c.419C>T
r.(?)
p.(Thr140Met)
-
VUS
g.46661685G>A
g.46196013G>A
POMGNT1(NM_017739.3):c.419C>T (p.T140M)
-
LURAP1_000017
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
5i_6
c.421-29_452del
r.spl
p.?
-
pathogenic
g.46661571_46661631del
g.46195899_46195959del
421-29_452del61
-
POMGNT1_000049
-
PubMed: Bouchet 2007
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
-?/., ?/.
2
5i
c.421-7C>A
r.(=), r.spl
p.(=)
-
likely benign, VUS
g.46661603G>T
g.46195931G>T
POMGNT1(NM_001243766.1):c.421-7C>A
-
POMGNT1_000231
VKGL data sharing initiative Nederland
PubMed: Nallamilli 2018
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Madhuri Hegde
,
VKGL-NL_Rotterdam
+/.
1
5i_9i
c.(420+1_421-1)_(879+1_880-1)del
r.?
p.?
-
pathogenic (recessive)
g.(46659598_46659945)_(46661597_46661683)del
g.(46193926_46194273)_(46195925_46196011)del
del ex6-9
-
POMGNT1_000262
enzyme activity, protein expression and subcellular localization assessed in HeLa cells
PubMed: Song 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.445del
r.(?)
p.(Phe149Leufs*19)
-
likely pathogenic
g.46661572del
g.46195900del
POMGNT1 Phe149 frameshift 167 stop
-
POMGNT1_000015
probably c.447del (reference Taniguchi et al., 2007); heterozygous
PubMed: Voglmeir 2011
-
-
Unknown
?
-
-
-
-
LOVD
+/+?, +/.
2
6
c.447del
r.(447del), r.(?)
p.(Phe149Leufs*19)
-
pathogenic
g.46661572del
g.46195900del
447delT, 541delT
-
POMGNT1_000015
1 Japan MDDGA3 (MEB) patient (com-het)
PubMed: Taniguchi 2003
,
PubMed: Manya 2003
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
+/.
2
6
c.447dup
r.(?)
p.(Asp150*)
-
pathogenic
g.46661572dup
g.46195900dup
447dupT, 447dupT A(sp150fsX)
-
POMGNT1_000028
not in 188 control chromosomes
PubMed: Clement
,
PubMed: Longman 2004
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
6
c.452C>T
r.(?)
p.(Thr151Met)
-
VUS
g.46661565G>A
g.46195893G>A
-
-
POMGNT1_000230
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
-/.
1
6
c.458C>G
r.(?)
p.(Ser153*)
-
benign
g.46661559G>C
g.46195887G>C
-
-
POMGNT1_000046
-
PubMed: Manya 2008
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.461C>A
r.(?)
p.(Pro154His)
-
VUS
g.46661556G>T
g.46195884G>T
-
-
LURAP1_000019
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
6
c.461C>T
r.(?)
p.(Pro154Leu)
-
VUS
g.46661556G>A
g.46195884G>A
-
-
POMGNT1_000229
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/.
1
-
c.466G>A
r.(?)
p.(Glu156Lys)
-
pathogenic (recessive)
g.46661551C>T
g.46195879C>T
-
-
POMGNT1_000251
1 more item
PubMed: Xu 2016
,
PubMed: Xu 2016
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
6
c.477dup
r.(?)
p.(Met160Hisfs*21)
-
pathogenic
g.46661541dup
g.46195869dup
477dupC
-
POMGNT1_000228
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
1
-
c.499G>A
r.(?)
p.(Val167Ile)
-
VUS
g.46661518C>T
g.46195846C>T
-
-
POMGNT1_000249
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
-
Germline
-
1/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
?/.
1
6
c.503C>T
r.(?)
p.(Ala168Val)
-
VUS
g.46661514G>A
g.46195842G>A
-
-
POMGNT1_000227
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/.
1
-
c.511C>T
r.(?)
p.(Arg171Ter)
-
pathogenic (recessive)
g.46661506G>A
g.46195834G>A
-
-
POMGNT1_000270
-
PubMed: Song 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.512G>T
r.(?)
p.(Arg171Leu)
-
VUS
g.46661505C>A
g.46195833C>A
-
-
POMGNT1_000191
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/., +?/+?
2
6
c.526A>C
r.(526a>c), r.(?)
p.(Thr176Pro)
-
likely pathogenic, pathogenic
g.46661491T>G
g.46195819T>G
Thr176Pro
-
POMGNT1_000043
1 MDDGA3 (MEB) patient (hom) with unknown ethnicity
PubMed: Godfrey 2007
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.528dup
r.(?)
p.(Val177CysfsTer4)
-
pathogenic (recessive)
g.46661489dup
g.46195817dup
c.528dupT
-
POMGNT1_000269
-
PubMed: Song 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.534+15C>T
r.(=)
p.(=)
-
likely benign
g.46661468G>A
-
POMGNT1(NM_017739.3):c.534+15C>T
-
LURAP1_000029
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
?/.
1
-
c.550C>T
r.(?)
p.(His184Tyr)
-
VUS
g.46660618G>A
g.46194946G>A
-
-
LURAP1_000018
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
7
c.561C>G
r.(?)
p.(Asp187Glu)
-
VUS
g.46660607G>C
g.46194935G>C
-
-
POMGNT1_000226
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/., +?/+?
3
7
c.575T>C
r.(575u>c), r.(?)
p.(Leu192Pro)
-
likely pathogenic, pathogenic, pathogenic (recessive)
g.46660593A>G
g.46194921A>G
-
-
POMGNT1_000150
1 Chinese MDDGA3 (MEB) patient (com-het)
PubMed: Jiao 2013
,
PubMed: Song 2021
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
+/+?, +/.
3
7
c.593del
r.(593del), r.(?)
p.(Ser198Thrfs*43)
-
pathogenic
g.46660575del
g.46194903del
593del premTerm240, 593delG
-
POMGNT1_000051
1 American and 1 German MDDGA3 (MEB) patient (both com-het)
PubMed: Hehr 2007
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Rosário dos Santos
+/., +?/.
3
7
c.594C>G
r.(?)
p.(Ser198Arg)
-
likely pathogenic, likely pathogenic (recessive), pathogenic (recessive)
g.46660574G>C
g.46194902G>C
POMGNT1 S198R
-
POMGNT1_000137
protein concentrations of the expressed mutant rPOMGnT1 protein diminished; heterozygous
PubMed: Biancheri 2006
,
PubMed: Mercuri 2009
,
PubMed: Voglmeir 2011
-
-
Germline, SUMMARY record, Unknown
-, ?
-
-
-
-
Johan den Dunnen
,
Anne Polvi
+?/.
1
7
c.617G>A
r.(?)
p.(Trp206Ter)
-
likely pathogenic
g.46660551C>T
g.46194879C>T
-
-
POMGNT1_000255
-
PubMed: Ganapathy 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.622G>A
r.(?)
p.(Asp208Asn)
-
likely pathogenic
g.46660546C>T
g.46194874C>T
-
-
POMGNT1_000253
-
PubMed: Stone 2017
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/.
3
7
c.630G>T
r.(?)
p.(Trp210Cys)
-
likely pathogenic, pathogenic
g.46660538C>A
g.46194866C>A
POMGNT1(NM_001243766.1):c.630G>T (p.W210C), POMGNT1(NM_001243766.2):c.630G>T (p.W210C)
-
POMGNT1_000050
not in 200 control chromosomes, VKGL data sharing initiative Nederland
PubMed: Bouchet 2007
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
+/., +?/.
7
7
c.636C>T
r.(=), r.(?), r.535_652del, r.spl
p.(=), p.(Phe212=), p.Asp179Valfs*23
-
likely pathogenic, likely pathogenic (recessive), pathogenic
g.46660532G>A
g.46194860G>A
POMGNT1(NM_001243766.2):c.636C>T (p.F212=)
-
POMGNT1_000030
strong NMD for variant in muscle, VKGL data sharing initiative Nederland
from website {DBsub-Emory},
PubMed: Bouchet 2007
,
PubMed: Cummings 2017
,
PubMed: Mercuri 2009
,
1 more item
-
-
CLASSIFICATION record, Germline, SUMMARY record, Unknown
-
-
-
-
-
Johan den Dunnen
,
Anne Polvi
,
Madhuri Hegde
,
VKGL-NL_Groningen
+/.
1
-
c.637G>A
r.(?)
p.(Val213Met)
-
pathogenic (recessive)
g.46660531C>T
g.46194859C>T
-
-
POMGNT1_000268
-
PubMed: Song 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/+?, +/.
4
7
c.643C>T
r.(?), r.535_652del
p.(Arg215Ter), p.Asp179Valfs*23
ACMG
pathogenic, pathogenic (recessive)
g.46660525G>A
g.46194853G>A
in-frame skip exon 7
-
POMGNT1_000139
1 Italian MDDGA3 (MEB) patient (com-het) and 1 MDDGA3 (MEB) patient (hom) with unknown ethnicity,
1 more item
PubMed: Cavdarli 2023
,
PubMed: Saredi 2012
-
rs386834034
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
+/., +?/.
3
7i
c.652+1G>A
r.spl, r.spl?
p.?
-
likely pathogenic, likely pathogenic (recessive), pathogenic
g.46660515C>T
g.46194843C>T
POMGNT1 mutation in intron 7
-
POMGNT1_000044
probably c.652+1G>A (reference Godfrey et al.. 2007); heterozygous
PubMed: Godfrey 2007
,
PubMed: Voglmeir 2011
-
-
Germline, SUMMARY record, Unknown
-, ?
-
-
-
-
Johan den Dunnen
,
Anne Polvi
-?/.
1
-
c.653-9T>C
r.(=)
p.(=)
-
likely benign
g.46660332A>G
g.46194660A>G
POMGNT1(NM_017739.3):c.653-9T>C
-
POMGNT1_000177
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
8
c.664G>A
r.(?)
p.(Gly222Arg)
-
VUS
g.46660312C>T
g.46194640C>T
-
-
POMGNT1_000225
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/+?, +/., +?/.
6
8
c.667G>A
r.(667g>a), r.(?)
p.(Glu223Lys)
-
likely pathogenic, pathogenic, pathogenic (recessive)
g.46660309C>T
g.46194637C>T
667G>A;56G>A, 761G>A, POMGNT1 E223K
-
POMGNT1_000016
1 Japanese and 1 Belgian MDDGA3 (MEB) patient (both com-het),
1 more item
PubMed: Song 2021
,
PubMed: Taniguchi 2003
,
PubMed: Manya 2003
,
PubMed: Voglmeir 2011
-
-
Germline, SUMMARY record, Unknown
-, ?
-
-
-
-
Johan den Dunnen
-/.
6
8
c.681A>G
r.(?)
p.(=), p.(?), p.(Lys227=)
-
benign
g.46660295T>C
g.46194623T>C
POMGNT1(NM_001243766.2):c.681A>G (p.K227=), POMGNT1(NM_017739.3):c.681A>G (p.K227=)
-
POMGNT1_000045
other pathogenic change in several patients, VKGL data sharing initiative Nederland
from website {DBsub-Emory},
PubMed: Godfrey 2007
-
rs2292487
CLASSIFICATION record, Germline, Unknown
-
-
-
-
-
Johan den Dunnen
,
Madhuri Hegde
,
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
,
VKGL-NL_AMC
+/., +?/.
2
8
c.703G>C
r.(?)
p.(Gly235Arg)
-
likely pathogenic (recessive), pathogenic
g.46660273C>G
g.46194601C>G
-
-
POMGNT1_000154
-
PubMed: Yis 2014
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Anne Polvi
?/.
1
8
c.706G>A
r.(?)
p.(Asp236Asn)
-
VUS
g.46660270C>T
g.46194598C>T
-
-
POMGNT1_000224
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
1
9
c.758A>T
r.(?)
p.(Glu253Val)
-
VUS
g.46660067T>A
g.46194395T>A
-
-
POMGNT1_000223
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+?/.
1
-
c.776_777del
r.(?)
p.(Thr259Argfs*14)
-
likely pathogenic
g.46660051_46660052del
-
POMGNT1(NM_017739.4):c.776_777del (p.(Thr259Argfs*14))
-
LURAP1_000039
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
2
9
c.788G>A
r.(?)
p.(Arg263His)
-
VUS
g.46660037C>T
g.46194365C>T
-
-
POMGNT1_000222
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
1
-
c.790C>T
r.(?)
p.(Arg264Cys)
-
VUS
g.46660035G>A
g.46194363G>A
-
-
POMGNT1_000248
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
rs369306680
Germline
-
1/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
+/., +?/., -?/., ?/.
10
9
c.794G>A
r.(?), r.794g>a
p.(Arg265His), p.Arg265His
-
likely benign, likely pathogenic, likely pathogenic (recessive), pathogenic, VUS
g.46660031C>T
g.46194359C>T
935G>A, 935G>A (R265H), POMGNT1 R265H
-
POMGNT1_000019
conflicting interpretations of pathogenicity; 1 heterozygous, no homozygous;
Clinindb (India)
,
4 more items
PubMed: Bouchet 2007
,
PubMed: Narang 2020
,
Journal: Narang 2020
,
PubMed: Shenoy 2010
,
3 more items
-
rs386834010
Germline, SUMMARY record, Unknown
-, ?, yes
1/2795 individuals
-
-
-
Johan den Dunnen
,
Anne Polvi
,
Tom Winder
,
Mohammed Faruq
+/.
5
-
c.794G>C
r.(?)
p.(Arg265Pro)
-
pathogenic, pathogenic (recessive)
g.46660031C>G
g.46194359C>G
-
-
POMGNT1_000157
-
PubMed: Fu 2017
,
PubMed: Song 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Xiaona Fu
+/.
1
-
c.796C>T
r.(?)
p.(Arg266Trp)
-
pathogenic (recessive)
g.46660029G>A
g.46194357G>A
-
-
POMGNT1_000267
-
PubMed: Song 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
9
c.797G>A
r.(?)
p.(Arg266Gln)
-
VUS
g.46660028C>T
g.46194356C>T
-
-
POMGNT1_000221
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
-/.
1
9
c.805_807del
r.(?)
p.(Cys269del)
-
benign
g.46660019_46660021del
g.46194347_46194349del
-
-
POMGNT1_000047
-
PubMed: Manya 2008
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., +?/.
3
9
c.806G>A
r.(?)
p.(Cys269Tyr)
-
likely pathogenic, likely pathogenic (recessive), pathogenic
g.46660019C>T
g.46194347C>T
900G>A, POMGNT1 C269Y
-
POMGNT1_000048
protein concentrations of the expressed mutant rPOMGnT1 protein diminished; heterozygous
PubMed: Taniguchi 2003
,
PubMed: Manya 2003
,
PubMed: Matsumoto 2005
,
PubMed: Voglmeir 2011
-
-
Germline, SUMMARY record, Unknown
-, ?
-
-
-
-
Johan den Dunnen
,
Anne Polvi
?/.
1
-
c.811A>G
r.(?)
p.(Lys271Glu)
-
VUS
g.46660014T>C
-
POMGNT1(NM_017739.4):c.811A>G (p.(Lys271Glu))
-
LURAP1_000038
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
-
c.812A>G
r.(?)
p.(Lys271Arg)
-
VUS
g.46660013T>C
g.46194341T>C
-
-
POMGNT1_000247
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
-
Germline
-
1/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
-?/.
1
-
c.816T>C
r.(?)
p.(Val272=)
-
likely benign
g.46660009A>G
g.46194337A>G
POMGNT1(NM_017739.3):c.816T>C (p.V272=)
-
LURAP1_000016
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
4
9
c.839G>A
r.(?)
p.(Ser280Asn)
-
VUS
g.46659986C>T
g.46194314C>T
POMGNT1(NM_001243766.1):c.839G>A (p.S280N)
-
POMGNT1_000220
no second variant, VKGL data sharing initiative Nederland
PubMed: Nallamilli 2018
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Madhuri Hegde
,
VKGL-NL_Rotterdam
+/., ?/.
3
9
c.860T>G
r.(?)
p.(Ile287Ser)
-
pathogenic (recessive), VUS
g.46659965A>C
g.46194293A>C
c.860T>G
-
POMGNT1_000250
2 more items
PubMed: Panneman 2023
,
PubMed: Xu 2016
,
PubMed: Xu 2016
-
-
Germline, Unknown
yes
1/309 unresolved RP cases
-
-
-
Johan den Dunnen
,
Daan Panneman
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