Global Variome shared LOVD
POMT1 (protein-O-mannosyltransferase 1)
LOVD v.3.0 Build 30b [
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Curator:
Johan den Dunnen
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Unique variants in the POMT1 gene
This database is one of the gene variant databases from the
Leiden Muscular Dystrophy pages
The variants shown are described using the NM_007171.3 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.
262 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.-32G>A
r.spl
p.?
-
pathogenic (recessive)
g.134378459G>A
g.131503072G>A
c.-32A>G
-
POMT1_000250
enzyme activity assessed in HeLa cells
PubMed: Song 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
1i
c.-30-2A>G
r.spl
p.?
-
likely pathogenic
g.134379574A>G
g.131504187A>G
c.-32A>G
-
POMT1_000142
-
-
-
-
Germline
-
-
-
-
-
Yanghaipo
-/.
3
2
c.-6T>G
r.(?)
p.(=)
-
benign
g.134379600T>G
g.131504213T>G
POMT1(NM_007171.3):c.-6T>G
-
POMT1_000045
VKGL data sharing initiative Nederland
from website {DBsub-Emory},
PubMed: Beltran-Valero de Bernabe 2002
-
-
CLASSIFICATION record, Germline, Unknown
-
-
-
-
-
Johan den Dunnen
,
Madhuri Hegde
,
VKGL-NL_Rotterdam
-/.
1
2
c.122+417GT(15_23)
r.(?)
p.(=)
-
benign
g.?
-
-
-
POMT1_000046
{GB181458}, marker D9S64
PubMed: Beltran-Valero de Bernabe 2002
,
PubMed: van Reeuwijk 2006
,
PubMed: Currier 2005
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
1
c.?
r.(?)
p.?
-
VUS
g.?
-
232G>C (E78Q)
-
PTCH1_000000
no second variant in POMT1
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+?/.
1
2
c.7G>T
r.(?)
p.(Gly3*)
-
likely pathogenic
g.134379612G>T
g.131504225G>T
-
-
POMT1_000091
-
-
-
-
Germline
-
-
-
-
-
Tom Winder
+?/.
1
-
c.35C>G
r.(?)
p.(Thr12Arg)
-
likely pathogenic
g.134379640C>G
-
POMT1(NM_001353193.2):c.35C>G (p.T12R)
-
UCK1_000038
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
-?/.
1
-
c.47A>G
r.(?)
p.(Asn16Ser)
-
likely benign
g.134379652A>G
g.131504265A>G
POMT1(NM_001077365.1):c.47A>G (p.(Asn16Ser))
-
UCK1_000002
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/., ?/.
3
2
c.78G>A
r.(?)
p.(=), p.(Gly26=)
-
likely benign, VUS
g.134379683G>A
g.131504296G>A
POMT1(NM_001077365.1):c.78G>A (p.(Gly26=))
-
POMT1_000096
VKGL data sharing initiative Nederland
from website {DBsub-Emory}
-
-
CLASSIFICATION record, Germline, Unknown
-
-
-
-
-
Madhuri Hegde
,
Tom Winder
,
VKGL-NL_Leiden
+?/.
2
2
c.81dup
r.(?)
p.(Leu28Thrfs*16)
-
likely pathogenic
g.134379686dup
g.131504299dup
-
-
POMT1_000080
-
-
-
-
Germline
-
-
-
-
-
Tom Winder
+/.
1
2
c.85A>C
r.(?)
p.(Ser29Arg)
-
pathogenic
g.134379690A>C
g.131504303A>C
-
-
POMT1_000048
-
PubMed: Willer 2012
-
-
Germline
-
-
-
-
-
Tom Winder
+/.
1
-
c.110C>T
r.(?)
p.(Pro37Leu)
-
pathogenic (recessive)
g.134379715C>T
g.131504328C>T
-
-
POMT1_000251
-
PubMed: Song 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
2
c.122+5G>A
r.[=, -30_122del]
p.[=, 0?]
-
pathogenic
g.134379732G>A
g.131504345G>A
-
-
POMT1_000095
-
PubMed: Bouchet 2007
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.123-6T>C
r.(=)
p.(=)
-
likely benign
g.134381495T>C
g.131506108T>C
POMT1(NM_001136113.2):c.123-6T>C
-
POMT1_000150
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-/., -?/.
2
-
c.123-6_123-5dup
r.spl?
p.?
-
benign, likely benign
g.134381495_134381496dup
g.131506108_131506109dup
POMT1(NM_001077365.1):c.123-6_123-5dup (p.?), POMT1(NM_001136113.2):c.123-6_123-5dupTT
-
UCK1_000003
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_AMC
-/., ?/.
6
2, 2i
c.123-5dup
r.spl?
p.(=), p.?
-
benign, VUS
g.134381496dup
g.131506109dup
122-5dupT,
1 more item
-
POMT1_000052, POMT1_000149
other pathogenic change in several patients, VKGL data sharing initiative Nederland
from website {DBsub-Emory},
PubMed: Godfrey 2007
-
rs10122068
CLASSIFICATION record, Germline, Unknown
-
-
-
-
-
Johan den Dunnen
,
Madhuri Hegde
,
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
,
VKGL-NL_AMC
-?/.
1
-
c.123-4dup
r.spl?
p.?
-
likely benign
g.134381497dup
g.131506110dup
POMT1(NM_001136113.2):c.123-4dupC
-
UCK1_000004
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-/.
1
3
c.129C>T
r.(?)
p.(=)
-
benign
g.134381507C>T
g.131506120C>T
-
-
POMT1_000065
other pathogenic change in one patient
PubMed: Godfrey 2007
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., +?/., ?/.
3
3
c.130G>A
r.(?)
p.(Glu44Lys)
-
likely pathogenic, pathogenic (recessive), VUS
g.134381508G>A
g.131506121G>A
-
-
POMT1_000127
enzyme activity assessed in HeLa cells
PubMed: Song 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Tom Winder
,
Yanghaipo
+/., +?/.
2
3
c.132A>C
r.(?)
p.(Glu44Asp)
-
likely pathogenic, pathogenic
g.134381510A>C
g.131506123A>C
-
-
POMT1_000139
no second variant
PubMed: Nallamilli 2018
,
PubMed: O'Grady 2016
-
-
Germline
yes
-
-
-
-
Madhuri Hegde
,
Sandra Cooper
-?/.
1
-
c.141T>C
r.(?)
p.(Tyr47=)
-
likely benign
g.134381519T>C
g.131506132T>C
POMT1(NM_001136113.1):c.141T>C (p.Y47=)
-
POMT1_000152
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
6
3
c.[145_146ins290; 145_160dup]
r.123_229del
p.Phe42Leufs*20
-
pathogenic
g.?
-
145insAlu
-
POMT1_000102
antisense insertion AluYa5 repeat, probable founder mutation (incl. D9S64 113 allele)
PubMed: Bouchet 2007
,
PubMed: Bouchet 2007
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
3
c.151A>T
r.(?)
p.(Ile51Phe)
-
VUS
g.134381529A>T
g.131506142A>T
-
-
POMT1_000199
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
1
-
c.165G>C
r.(?)
p.(Met55Ile)
-
VUS
g.134381543G>C
-
POMT1(NM_001353198.1):c.3G>C (p.M1?)
-
UCK1_000029
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
-
c.176T>G
r.(?)
p.(Phe59Cys)
-
pathogenic (recessive)
g.134381554T>G
g.131506167T>G
-
-
POMT1_000252
enzyme activity assessed in HeLa cells
PubMed: Song 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., +?/., -/.
3
3
c.180_182del
r.(?)
p.(Phe60del)
-
benign, likely pathogenic, pathogenic (recessive)
g.134381558_134381560del
g.131506171_131506173del
180-182delCCT, c.180-182delCCT
-
POMT1_000101
-
PubMed: Bouchet 2007
,
PubMed: Song 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Yanghaipo
+/., +?/.
2
-
c.184del
r.(?)
p.(Asp62Metfs*18)
-
likely pathogenic, pathogenic
g.134381562del
-
POMT1(NM_001077365.2):c.184del (p.(Asp62Metfs*18)), POMT1(NM_007171.4):c.184delG (p.D62Mfs*18)
-
UCK1_000030
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_VUmc
+/.
7
3
c.193G>A
r.(?)
p.(Gly65Arg)
-
pathogenic
g.134381571G>A
g.131506184G>A
G193A
-
POMT1_000016
not in 400 control chromosomes
PubMed: DAmico 2006
,
PubMed: Messina 2008
,
PubMed: Mercuri 2009
,
OMIM:var0010
,
PubMed: Godfrey 2007
,
2 more items
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Rosário dos Santos
,
Tom Winder
+/., +?/., ?/.
7
3
c.197C>T
r.(?)
p.(Pro66Leu)
-
likely pathogenic, likely pathogenic (recessive), pathogenic (recessive), VUS
g.134381575C>T
g.131506188C>T
1 more item
-
POMT1_000153
combination of variants not reported, no second variant, VKGL data sharing initiative Nederland
PubMed: Johnson 2018
,
PubMed: Nallamilli 2018
,
PubMed: Song 2021
,
PubMed: Topf 2020
-
-
CLASSIFICATION record, Germline
-
1/1001 cases
-
-
-
Johan den Dunnen
,
Madhuri Hegde
,
VKGL-NL_Leiden
,
VKGL-NL_Groningen
,
VKGL-NL_VUmc
?/.
1
3
c.202T>C
r.(?)
p.(Phe68Leu)
-
VUS
g.134381580T>C
g.131506193T>C
-
-
POMT1_000200
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
1
3
c.208C>A
r.(?)
p.(His70Asn)
-
VUS
g.134381586C>A
g.131506199C>A
-
-
POMT1_000201
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/.
1
3
c.226G>A
r.(?)
p.(Gly76Arg)
-
pathogenic
g.134381604G>A
g.131506217G>A
-
-
POMT1_000002
not in 210 control chromosomes (Nederland, Turkey)
1 more item
-
rs28941782
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.230G>A
r.(?)
p.(Gly77Asp)
ACMG
VUS
g.134381790G>A
g.131506403G>A
-
-
POMT1_000242
-
-
-
-
Germline
-
-
-
-
-
Andreas Laner
?/.
1
-
c.246C>T
r.(?)
p.(Phe82=)
-
VUS
g.134381806C>T
g.131506419C>T
POMT1(NM_001353196.1):c.139C>T (p.R47*)
-
UCK1_000020
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
4
c.247G>A
r.(?)
p.(Asp83Asn)
-
VUS
g.134381807G>A
g.131506420G>A
-
-
POMT1_000202
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
1
-
c.251G>A
r.(?)
p.(Gly84Asp)
-
VUS
g.134381811G>A
g.131506424G>A
-
-
POMT1_000189
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
2
-
c.260dup
r.(?)
p.(Leu87Phefs*11), p.(Leu87PhefsTer11)
-
pathogenic (recessive)
g.134381820dup
g.131506433dup
c.260dupT
-
POMT1_000245
-
PubMed: Song 2021
,
PubMed: Yu 2017
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., +?/.
13
4i
c.280+1G>T
r.230_280del, r.spl, r.spl?
p.?, p.Gly77_Ala93del
ACMG
likely pathogenic, pathogenic
g.134381841G>T
g.131506454G>T
NM_001077365.1:c.280+1G>T
-
POMT1_000090
ACMG PVS1, PM2, PP1, ACMG PVS1, PS4, PM2, PP1
PubMed: Anazi 2017
,
PubMed: Judas 2009
,
PubMed: Lommel 2010
,
PubMed: Manzini 2008
,
1 more item
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Rosário dos Santos
,
Tom Winder
-?/.
1
4i
c.280+271G>A
r.(=)
p.(=)
-
likely benign
g.134382111G>A
g.131506724G>A
-
-
POMT1_000140
-
PubMed: Almomani 2011
-
rs10448341
Germline
-
-
-
-
-
Gerard C.P. Schaafsma
-/.
1
5
c.281-82C>T
r.(?)
p.(=)
-
benign
g.134382673C>T
g.131507286C>T
-
-
POMT1_000053
-
-
-
rs6597501
Germline
-
-
-HhI
-
-
Johan den Dunnen
+/.
1
5
c.291del
r.(?)
p.(Ser97Argfs*26)
-
pathogenic
g.134382765del
g.131507378del
-
-
POMT1_000116
-
PubMed: Vajsar, 2008
-
-
Germline
-
-
-
-
-
Rosário dos Santos
+/.
3
5
c.313C>T
r.(?)
p.(Arg105Cys)
-
pathogenic, pathogenic (recessive)
g.134382787C>T
g.131507400C>T
-
-
POMT1_000025
-
PubMed: Song 2021
,
PubMed: van Reeuwijk 2006
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Yanghaipo
+/.
1
5
c.314G>A
r.(?)
p.(Arg105His)
-
pathogenic
g.134382788G>A
g.131507401G>A
-
-
POMT1_000026
-
PubMed: van Reeuwijk 2006
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
1
5
c.318G>A
r.(?)
p.(=)
-
benign
g.134382792G>A
g.131507405G>A
-
-
POMT1_000066
found in one patient
PubMed: Godfrey 2007
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
2
-
c.330C>G
r.(?)
p.(Leu110=)
-
likely benign
g.134382804C>G
g.131507417C>G
POMT1(NM_001353198.1):c.168C>G (p.L56=), POMT1(NM_007171.3):c.330C>G (p.(Leu110=))
-
UCK1_000005
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
+/.
1
5
c.357del
r.(?)
p.(Tyr120Thrfs*3)
ACMG
pathogenic
g.134382831del
g.131507444del
357delC
-
POMT1_000248
-
-
-
-
Germline
yes
-
-
-
-
Young Jun Ko
+/.
1
-
c.361C>T
r.(?)
p.(Gln121*)
-
pathogenic
g.134382835C>T
-
-
-
UCK1_000031
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/.
1
5
c.385del
r.(?)
p.(Ser129Leufs*11)
-
likely pathogenic
g.134382859del
g.131507472del
-
-
POMT1_000082
-
-
-
-
Germline
-
-
-
-
-
Tom Winder
+/.
1
5
c.418_420del
r.(?)
p.(Met140del)
-
pathogenic
g.134382892_134382894del
g.131507505_131507507del
418_420delATG
-
POMT1_000020
-
PubMed: van Reeuwijk 2006
,
PubMed: Messina 2008
,
PubMed: Mercuri 2009
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
5
c.424A>T
r.(?)
p.(Ile142Phe)
-
VUS
g.134382898A>T
g.131507511A>T
-
-
POMT1_000203
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/., +?/.
4
5
c.427G>A
r.(?), r.(spl?), r.281_427del, r.spl?
p.(Glu143Lys), p.(Glu143Lys?), p.(Tyr95_Glu143del), p.Tyr95_Glu143del
-
likely pathogenic, pathogenic
g.134382901G>A
g.131507514G>A
-
-
POMT1_000023
not in 216 control chromosomes, two genetic etiologies
PubMed: Bouchet 2007
,
PubMed: Park 2017
,
PubMed: van Reeuwijk 2006
-
-
Germline
-
1/209 cases
-
-
-
Johan den Dunnen
+/.
1
5
c.427G>T
r.(spl?)
p.(Glu143*)
-
pathogenic
g.134382901G>T
g.131507514G>T
-
-
POMT1_000067
-
PubMed: Godfrey 2007
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
3
5
c.427+1_427+2insA
r.281_427del, r.spl?
p.?, p.Tyr95_143Lysdel, p.Tyr95_Lys143del
-
pathogenic
g.134382902_134382903insA
g.131507515_131507516insA
427+2insA
-
POMT1_000112
VKGL data sharing initiative Nederland
PubMed: Bouchet 2007
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Nijmegen
-/.
6
5i, 6
c.428-21T>C
r.(=), r.(?)
p.(=)
-
benign
g.134384277T>C
g.131508890T>C
POMT1(NM_001136113.2):c.428-21T>C, POMT1(NM_007171.3):c.428-21T>C
-
POMT1_000054
VKGL data sharing initiative Nederland
from website {DBsub-Emory},
PubMed: Godfrey 2007
-
rs11243404
CLASSIFICATION record, Germline, Unknown
-
-
-
-
-
Johan den Dunnen
,
Madhuri Hegde
,
Judith Pagan
,
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
+/.
1
-
c.428-2_428-1del
r.spl
p.?
-
pathogenic (recessive)
g.134384299_134384300del
g.131508912_131508913del
c.428-2_428-1delAG
-
POMT1_000253
-
PubMed: Song 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.438C>T
r.(?)
p.(Leu146=)
-
likely benign
g.134384308C>T
-
POMT1(NM_001077365.1):c.438C>T (p.(Leu146=))
-
UCK1_000036
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+?/.
1
6
c.485del
r.(?)
p.(Phe162Serfs*10)
-
likely pathogenic
g.134384355del
g.131508968del
-
-
POMT1_000093
-
-
-
-
Germline
-
-
-
-
-
Tom Winder
+/.
1
6
c.512T>G
r.(?)
p.(Leu171Arg)
-
pathogenic
g.134384382T>G
g.131508995T>G
-
-
POMT1_000122
not in 102 control chromosomes; POMT2, FKRP normal
PubMed: Lommel 2010
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
+/.
2
6
c.517_523delinsG
r.(?)
p.(Phe173_Asn175delinsAsp)
-
pathogenic
g.134384387_134384393delinsG
g.131509000_131509006delinsG
-
-
POMT1_000069
-
PubMed: Godfrey 2007
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Tom Winder
+/.
1
6
c.539+1G>T
r.spl?
p.?
-
pathogenic
g.134384410G>T
g.131509023G>T
-
-
POMT1_000113
-
PubMed: Bouchet 2007
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.558G>T
r.(?)
p.(Trp186Cys)
-
likely pathogenic
g.134385148G>T
g.131509761G>T
-
-
POMT1_000244
1 heterozygous, no homozygous;
Clinindb (India)
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs772370177
Germline
-
1/2795 individuals
-
-
-
Mohammed Faruq
?/.
2
7
c.586G>A
r.(?)
p.(Ala196Thr)
-
VUS
g.134385176G>A
g.131509789G>A
-
-
POMT1_000204
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/.
15
7
c.598G>C
r.(?)
p.(Ala200Pro)
-
pathogenic
g.134385188G>C
g.131509801G>C
-
-
POMT1_000001
not in 212 control chromosomes
PubMed: Balci 2005
,
PubMed: Dincer 2003
,
OMIM:var0005
,
PubMed: Godfrey 2007
-
-
Germline
-
-
-HpyCH4V
-
-
Johan den Dunnen
?/.
1
7
c.599C>G
r.(?)
p.(Ala200Gly)
-
VUS
g.134385189C>G
g.131509802C>G
-
-
POMT1_000205
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/.
2
7
c.605+1G>A
r.540_605del, r.spl
p.?, p.Phe181_Gly202del
-
pathogenic, pathogenic (recessive)
g.134385196G>A
g.131509809G>A
-
-
POMT1_000114
-
PubMed: Bouchet 2007
,
PubMed: Song 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.606-15G>C
r.(=)
p.(=)
-
likely benign
g.134385275G>C
g.131509888G>C
POMT1(NM_007171.3):c.606-15G>C
-
POMT1_000154
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
8
c.619G>T
r.(?)
p.(Gly207Cys)
-
VUS
g.134385303G>T
g.131509916G>T
-
-
POMT1_000206
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/.
1
8
c.620G>T
r.(?)
p.(Gly207Val)
-
pathogenic
g.134385304G>T
g.131509917G>T
-
-
POMT1_000027
-
PubMed: van Reeuwijk 2006
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
2
8
c.634G>A
r.(?)
p.(Val212Met)
-
VUS
g.134385318G>A
g.131509931G>A
-
-
POMT1_000207
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
1
8
c.641T>C
r.(?)
p.(Val214Ala)
-
VUS
g.134385325T>C
g.131509938T>C
-
-
POMT1_000208
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
1
-
c.647G>C
r.(?)
p.(Gly216Ala)
-
VUS
g.134385331G>C
-
POMT1(NM_001077365.2):c.647G>C (p.(Gly216Ala))
-
UCK1_000041
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
2
-
c.677T>C
r.(?)
p.(Leu226Pro)
-
VUS
g.134385361T>C
g.131509974T>C
-
-
POMT1_000261
-
PubMed: Cavdarli 2023
-
rs1554774207
Germline, Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.692T>A
r.(?)
p.(Leu231*)
-
likely pathogenic
g.134385376T>A
g.131509989T>A
-
-
POMT1_000263
carrier
PubMed: Tavakoli 2023
-
-
Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
?/.
1
8
c.698A>G
r.(?)
p.(Asn233Ser)
-
VUS
g.134385382A>G
g.131509995A>G
-
-
POMT1_000209
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
1
8
c.718T>C
r.(?)
p.(Cys240Arg)
-
VUS
g.134385402T>C
g.131510015T>C
-
-
POMT1_000210
no second variant in POMT1
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
-/.
1
8
c.718T>G
r.(?)
p.(Cys240Gly)
-
benign
g.134385402T>G
g.131510015T>G
-
-
POMT1_000030
-
PubMed: van Reeuwijk 2006
-
rs4997217
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.732G>A
r.(?)
p.(Pro244=)
-
likely benign
g.134385416G>A
g.131510029G>A
POMT1(NM_007171.3):c.732G>A (p.P244=)
-
UCK1_000007
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.737G>A
r.(?)
p.(Cys246Tyr)
-
likely benign
g.134385421G>A
-
POMT1(NM_001077365.1):c.699+38G>A (p.(=))
-
UCK1_000034
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-/.
1
8
c.739A>G
r.(?)
p.(Met247Val)
-
benign
g.134385423A>G
g.131510036A>G
.724A>G
-
POMT1_000031
-
PubMed: van Reeuwijk 2006
-
rs4995933
Germline
-
-
-
-
-
Johan den Dunnen
-/., -?/.
9
8
c.751C>T
r.(?)
p.(Arg251Trp)
-
benign, likely benign
g.134385435C>T
g.131510048C>T
751_752delinsTG, p.Gln251Trp?,
1 more item
-
POMT1_000032
VKGL data sharing initiative Nederland
from website {DBsub-Emory},
PubMed: Beltran-Valero de Bernabe 2002
,
PubMed: Currier 2005
,
2 more items
-
rs3887873
CLASSIFICATION record, Germline, Unknown
-
0.17, 2/14
-
-
-
Johan den Dunnen
,
Madhuri Hegde
,
VKGL-NL_Rotterdam
,
VKGL-NL_Nijmegen
,
VKGL-NL_AMC
-/.
1
8
c.751_752delinsTA
r.(?)
p.(Arg251*)
-
benign
g.134385435_134385436delinsTA
-
-
-
POMT1_000128
1 more item
from website {DBsub-Emory}
-
-
Unknown
-
-
-
-
-
Madhuri Hegde
-/.
2
-
c.752=
r.(=)
p.(Arg251=)
-
benign
g.134385436A>G
g.131510049A>G
POMT1(NM_007171.4):c.752A>G (p.Q251R)
-
POMT1_000155
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
,
VKGL-NL_AMC
-/.
7
8
c.752G>A
r.(?)
p.(Arg251Gln)
-
benign
g.134385436G>A
-
752A>G, 931G>A, p.(Gln251Trp) erroneous
-
POMT1_000033
1 more item
from website {DBsub-Emory},
PubMed: Beltran-Valero de Bernabe 2002
,
PubMed: Bouchet 2007
,
3 more items
-
rs2296949
Germline, Unknown
-
0.17
-
-
-
Johan den Dunnen
,
Madhuri Hegde
+?/.
1
-
c.765+1G>A
r.spl
p.?
-
likely pathogenic
g.134385450G>A
g.131510063G>A
-
-
POMT1_000247
combination of variants not reported
PubMed: Topf 2020
-
-
Germline
-
2/1001 cases
-
-
-
Johan den Dunnen
+/.
2
-
c.765+2T>C
r.spl
p.?
-
pathogenic
g.134385451T>C
g.131510064T>C
-
-
POMT1_000262
possible di-genic inheritance
PubMed: Cavdarli 2023
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
3
8i, 9
c.766-48A>G
r.(?)
p.(=)
-
benign
g.134385599A>G
g.131510212A>G
-
-
POMT1_000055
-
from website {DBsub-Emory},
PubMed: Bouchet 2007
,
PubMed: Godfrey 2007
-
rs2018621
Germline, Unknown
-
-
-
-
-
Johan den Dunnen
,
Madhuri Hegde
-/.
1
9
c.766-43T>G
r.(?)
p.(=)
-
benign
g.134385604T>G
g.131510217T>G
-
-
POMT1_000071
other pathogenic change in several patients
PubMed: Godfrey 2007
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
8i
c.766-28A>G
r.(?)
p.(=)
-
VUS
g.134385619A>G
g.131510232A>G
-
-
POMT1_000129
-
from website {DBsub-Emory}
-
-
Unknown
-
-
-
-
-
Madhuri Hegde
+/.
1
-
c.780T>A
r.(?)
p.(Cys260Ter)
-
pathogenic
g.134385661T>A
g.131510274T>A
-
-
POMT1_000191
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
2
9
c.790G>A
r.(?)
p.(Ala264Thr)
-
VUS
g.134385671G>A
g.131510284G>A
-
-
POMT1_000211
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
1
9
c.791C>T
r.(?)
p.(Ala264Val)
-
VUS
g.134385672C>T
g.131510285C>T
-
-
POMT1_000212
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
1
-
c.794G>C
r.(?)
p.(Arg265Pro)
ACMG
VUS
g.134385675G>C
g.131510288G>C
-
-
POMT1_000243
-
-
-
-
Germline
-
-
-
-
-
Andreas Laner
+/.
2
9
c.818C>T
r.(?)
p.(Pro273Leu)
-
pathogenic
g.134385699C>T
g.131510312C>T
997C>T
-
POMT1_000049
reported DNA/protein descriptions do not match
PubMed: Willer 2012
-
-
Germline, Unknown
-
-
-
-
-
Johan den Dunnen
,
Tom Winder
?/.
1
9
c.823G>A
r.(?)
p.(Val275Ile)
-
VUS
g.134385704G>A
g.131510317G>A
-
-
POMT1_000213
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/.
1
9
c.827T>G
r.(?)
p.(Leu276Arg)
-
pathogenic
g.134385708T>G
g.131510321T>G
1006T>G
-
POMT1_000124
reported DNA/protein descriptions do not match
PubMed: Willer 2012
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
?/.
1
9
c.842_844del
r.(?)
p.(Phe281del)
-
VUS
g.134385723_134385725del
g.131510336_131510338del
-
-
POMT1_000092
-
-
-
-
Germline
-
-
-
-
-
Tom Winder
-?/.
1
-
c.846C>T
r.(?)
p.(Tyr282=)
-
likely benign
g.134385727C>T
-
POMT1(NM_001353198.1):c.684C>T (p.Y228=)
-
UCK1_000032
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/., ?/.
6
9
c.855G>C
r.(?)
p.(Leu285Phe)
-
benign, VUS
g.134385736G>C
g.131510349G>C
POMT1(NM_001353198.1):c.693G>C (p.L231F)
-
POMT1_000034
found in several patients, found once in patient with pathogenic change elsewhere, no second variant,
1 more item
PubMed: Godfrey 2007
,
PubMed: Nallamilli 2018
,
PubMed: van Reeuwijk 2006
-
-
CLASSIFICATION record, Germline
-
0.01
-
-
-
Johan den Dunnen
,
Madhuri Hegde
,
VKGL-NL_Rotterdam
,
VKGL-NL_Nijmegen
?/.
1
-
c.869G>A
r.(?)
p.(Arg290His)
-
VUS
g.134385750G>A
-
POMT1(NM_001353198.1):c.707G>A (p.R236H)
-
UCK1_000037
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
9
c.905T>G
r.(?)
p.(Phe302Cys)
-
VUS
g.134385786T>G
g.131510399T>G
-
-
POMT1_000073
-
PubMed: Godfrey 2007
-
-
Germline
-
-
-
-
-
Johan den Dunnen
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