Global Variome shared LOVD
LMNA (lamin A/C)
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Curator:
Johan den Dunnen
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Unique variants in the LMNA gene
This database is one of the gene variant databases from the
Leiden Muscular Dystrophy pages
The variants shown are described using the NM_170707.3 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.
738 entries on 8 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
1
c.-31735+?_357-?del
r.-31735_357del
p.0
-
pathogenic
g.156052975_156100408del
g.156083184_156130617del
-
-
LMNA_000209
not in 300 control chromosomes
PubMed: Van Tintelen 2007
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.-30584_-30583del
r.(?)
p.(=)
-
likely benign
g.156054126_156054127del
-
LMNA(NM_001282625.2):c.-319+1151_-319+1152del
-
LMNA_000800
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
1
-
c.-30583G>C
r.(?)
p.(=)
-
likely benign
g.156054127G>C
-
LMNA(NM_001282625.2):c.-319+1152G>C
-
LMNA_000801
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-/.
2
?, _1
c.-1242T>C
r.(=)
p.(=)
-
benign
g.156083468T>C
g.156113677T>C
-
-
LMNA_000236
-
PubMed: Halaschek-Wiener 2009
-
rs2485662
Germline
-
30/94 chromosomes
-
-
-
Johan den Dunnen
-/.
2
?, _1
c.-1214T>C
r.(=)
p.(=)
-
benign
g.156083496T>C
g.156113705T>C
-
-
LMNA_000235
-
PubMed: Halaschek-Wiener 2009
-
rs2485661
Germline
-
2/94 chromosomes
-
-
-
Johan den Dunnen
-/.
2
?, _1
c.-845dup
r.(=)
p.(=)
-
benign
g.156083865dup
g.156114074dup
-845dupC
-
LMNA_000237
-
PubMed: Halaschek-Wiener 2009
-
rs28365858
Germline
-
1/94 chromosomes
-
-
-
Johan den Dunnen
+?/.
1
_1_12_
c.(?_-249)_(*981_?)del
r.0
p.0
-
likely pathogenic
g.?
-
-
-
LMNA_000098
6 Mb deletion
PubMed: Eriksson
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., -/.
2
1
c.-128T>C
r.(?)
p.(=)
-
benign, pathogenic
g.156084582T>C
g.156114791T>C
-
-
LMNA_000159
not in 150 control chromosomes
PubMed: D Amico 2005
, Sara Benedetti 2005
-
-
De novo, Germline
-
-
-
-
-
Johan den Dunnen
-/.
1
-
c.-88G>T
r.(?)
p.(=)
-
benign
g.156084622G>T
g.156114831G>T
-
-
LMNA_000551
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
-
c.-59G>A
r.(?)
p.(=)
-
VUS
g.156084651G>A
g.156114860G>A
-
-
LMNA_000552
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
1
c.-3_12del
r.(?)
p.0?
-
pathogenic
g.156084707_156084721del
g.156114916_156114930del
-
-
LMNA_000151
-
PubMed: Walter 2005
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
5'UTR
c.-1C>A
r.(?)
p.(=)
-
VUS
g.156084709C>A
g.156114918C>A
-
-
LMNA_000502
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/.
1
-
c.?
r.?
p.?
-
pathogenic
g.?
-
c.1375C>T
-
ABCA4_000000
-
PubMed: Xie 2024
-
-
Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.1A>G
r.(?)
p.(Met1?)
-
likely pathogenic
g.156084710A>G
g.156114919A>G
LMNA(NM_170707.4):c.1A>G (p.M1?)
-
LMNA_000370
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+?/.
1
-
c.2T>C
r.(?)
p.(Met1?)
-
likely pathogenic
g.156084711T>C
-
-
-
LMNA_000806
-
-
-
-
Unknown
-
-
-
-
-
MobiDetails
+/.
1
-
c.3G>A
r.(?)
p.(Met1?)
-
pathogenic
g.156084712G>A
g.156114921G>A
-
-
LMNA_000703
ACMG PVS1 PS4-M PM1 PM2 PP3 PP5; no genotypes reported
PubMed: Nguyen 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/., ?/.
2
1
c.3G>T
r.(?)
p.0?
-
likely pathogenic, VUS
g.156084712G>T
g.156114921G>T
M1?, Met1Ile
-
LMNA_000252
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
,
Tom Winder
+?/.
1
-
c.4C>A
r.(?)
p.(Glu2Lys)
ACMG
likely pathogenic (dominant)
g.156084713C>A
hg19
-
-
LMNA_000764
-
PubMed: Forouzandeh 2016
-
-
Germline
yes
-
-
-
-
Matheus Wilke
-/.
1
1
c.5A>G
r.(?)
p.(Glu2Gly)
-
benign
g.156084714A>G
g.156114923A>G
-
-
LMNA_000136
-
-
-
rs11549669
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.5A>T
r.(?)
p.(Glu2Val)
-
VUS
g.156084714A>T
g.156114923A>T
-
-
LMNA_000553
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/.
1
-
c.11C>A
r.(?)
p.(Pro4Gln)
-
likely pathogenic
g.156084720C>A
g.156114929C>A
LMNA(NM_005572.3):c.11C>A (p.(Pro4Gln))
-
LMNA_000554
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
2
1
c.16C>T
r.(?)
p.(Gln6*)
-
pathogenic
g.156084725C>T
g.156114934C>T
-
-
LMNA_000001
-
PubMed: Bonne
,
PubMed: Becane
,
OMIM:var0001
,
PubMed: Walsh 2017
-
-
Germline
-
1/740 cases
+BfaI
-
-
Johan den Dunnen
?/.
2
-
c.20G>A
r.(?)
p.(Arg7Gln)
-
VUS
g.156084729G>A
g.156114938G>A
-
-
LMNA_000752
-
PubMed: Cavdarli 2023
-
rs751916168
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
1
c.29C>T
r.(?)
p.(Thr10Ile)
-
pathogenic
g.156084738C>T
g.156114947C>T
-
-
LMNA_000108
-
from website {DBsub-Emory},
PubMed: Csoka
-
-
Germline, Unknown
-
-
-
-
-
Johan den Dunnen
,
Madhuri Hegde
+/.
1
1
c.31del
r.(?)
p.(Arg11Alafs*85)
-
pathogenic
g.156084740del
g.156114949del
delC31 (Thr10fs*85)
-
LMNA_000328
-
PubMed: Pasotti 2008
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
-/.
1
-
c.33C>T
r.(?)
p.(Arg11=)
-
benign
g.156084742C>T
g.156114951C>T
LMNA(NM_170707.4):c.33C>T (p.R11=)
-
LMNA_000372
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
1
-
c.37G>A
r.(?)
p.(Gly13Arg)
-
VUS
g.156084746G>A
-
LMNA(NM_170707.2):c.37G>A (p.G13R)
-
LMNA_000716
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/.
1
-
c.42G>A
r.(?)
p.(Ala14=)
-
benign
g.156084751G>A
g.156114960G>A
LMNA(NM_170707.4):c.42G>A (p.A14=)
-
LMNA_000373
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+?/., -/-?, -/., -?/.
10
1
c.51C>T
r.(=), r.(?), r.51c>u
p.(=), p.(Ser17=)
-
benign, likely benign, likely pathogenic
g.156084760C>T
g.156114969C>T
Ser17Ser,
1 more item
-
LMNA_000135
no homozygous individuals, unclassified variant, VKGL data sharing initiative Nederland
from website {DBsub-Emory},
PubMed: Gaudy-Marqueste 2009
,
PubMed: Sebillon
-
rs11549668
CLASSIFICATION record, Germline, Unknown
-
3/254 chromosomes
-
-
-
Johan den Dunnen
,
Madhuri Hegde
,
Birgit Neitzel
,
Andreas Laner
,
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
,
VKGL-NL_AMC
+?/.
1
-
c.64T>G
r.(?)
p.(Ser22Ala)
-
likely pathogenic
g.156084773T>G
g.156114982T>G
-
-
LMNA_000639
2 heterozygous, no homozygous;
Clinindb (India)
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs794728599
Germline
-
2/2760 individuals
-
-
-
Mohammed Faruq
+/.
1
1
c.65C>T
r.(?)
p.(Ser22Leu)
-
pathogenic
g.156084774C>T
g.156114983C>T
-
-
LMNA_000153
-
PubMed: Pethig 2005
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
1
c.65_66delinsT
r.(?)
p.(Ser22Phefs*74)
-
pathogenic
g.156084774_156084775delinsT
g.156114983_156114984delinsT
-
-
LMNA_000503
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+?/.
2
1
c.71C>G
r.(?)
p.(Thr24Ser)
-
likely pathogenic
g.156084780C>G
g.156114989C>G
-
-
LMNA_000354
-
-
-
-
Germline
yes
-
-
-
-
Zohreh Fattahi
?/.
2
-
c.71C>T
r.(?)
p.(Thr24Ile)
-
VUS
g.156084780C>T
g.156114989C>T
LMNA(NM_170707.4):c.71C>T (p.T24I)
-
LMNA_000374
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
+/., ?/.
2
1
c.73C>G
r.(?)
p.(Arg25Gly)
-
pathogenic, VUS
g.156084782C>G
g.156114991C>G
-
-
LMNA_000092
VKGL data sharing initiative Nederland
PubMed: Vytopil 2003
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Nijmegen
+?/., ?/.
2
1
c.73C>T
r.(?)
p.(Arg25Cys)
-
likely pathogenic, VUS
g.156084782C>T
g.156114991C>T
-
-
LMNA_000288
not in 600 control chromosomes, VKGL data sharing initiative Nederland
PubMed: van Tintelen 2007
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Nijmegen
+/.
1
1
c.74C>G
-
p.Arg25Pro
-
NA
g.156084783C>G
-
-
-
LMNA_000349
1 more item
PubMed: Scherner 2011
-
-
In vitro (cloned)
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.74del
r.(?)
p.(Arg25ProfsTer71)
-
VUS
g.156084783del
g.156114992del
-
-
LMNA_000555
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/.
1
-
c.74G>A
r.(?)
p.(Arg25His)
-
likely pathogenic
g.156084783G>A
-
LMNA(NM_170707.2):c.74G>A (p.R25H)
-
LMNA_000705
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
1
c.74G>C
r.(?)
p.(Arg25Pro)
-
pathogenic
g.156084783G>C
g.156114992G>C
-
-
LMNA_000064
-
PubMed: Brown
-
-
Germline
-
-
-SfaNI
-
-
Johan den Dunnen
-?/.
1
-
c.75C>G
r.(?)
p.(Arg25=)
-
likely benign
g.156084784C>G
g.156114993C>G
-
-
LMNA_000556
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
1
c.77T>G
r.(?)
p.(Ile26Ser)
-
VUS
g.156084786T>G
g.156114995T>G
-
-
LMNA_000213
-
-
-
-
Germline
-
-
-
-
-
Lab Müller-Reible
-?/.
1
-
c.78C>T
r.(?)
p.(Ile26=)
-
likely benign
g.156084787C>T
g.156114996C>T
LMNA(NM_170707.2):c.78C>T (p.I26=)
-
LMNA_000557
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/.
1
1
c.80C>T
r.(?)
p.(Thr27Ile)
-
pathogenic
g.156084789C>T
g.156114998C>T
-
-
LMNA_000132
not in 266 control chromosomes
-
-
-
Germline
-
-
HphI
-
-
Rosário dos Santos
+/.
1
1
c.82C>G
r.(?)
p.(Arg28Trp)
-
pathogenic
g.156084791C>G
g.156115000C>G
Arg28Trp
-
LMNA_000324
-
PubMed: Pasotti 2008
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
+/.
3
1
c.82C>T
r.(?)
p.(Arg28Trp)
-
pathogenic
g.156084791C>T
g.156115000C>T
g.498C>T
-
LMNA_000052
not in 400 control chromosomes
PubMed: Decaudain 2007
,
PubMed: Garg 2002
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Lab Müller-Reible
+/., ?/.
4
1
c.83G>A
r.(?)
p.(Arg28Gln)
-
pathogenic, VUS
g.156084792G>A
g.156115001G>A
R28Q
-
LMNA_000261
no second variant, not in 300 control chromosomes; probably de novo, parents not available
PubMed: Komaki 2011
,
PubMed: Nallamilli 2018
-
-
Germline, Unknown
-
-
-
-
-
Johan den Dunnen
,
Madhuri Hegde
,
Tom Winder
+/.
2
1
c.91G>A
r.(?)
p.(Glu31Lys)
ACMG
pathogenic (dominant)
g.156084800G>A
g.156115009G>A
-
-
LMNA_000646
-
PubMed: Fan 2020
,
PubMed: Fan 2021
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+/.
2
1
c.91_93del
r.(?)
p.(Glu31del)
ACMG
pathogenic (dominant)
g.156084800_156084802del
g.156115009_156115011del
91_93delGAG
-
LMNA_000647
-
PubMed: Fan 2020
,
PubMed: Fan 2021
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+/.
2
1
c.94A>G
r.(?)
p.(Lys32Glu)
ACMG
pathogenic (dominant)
g.156084803A>G
g.156115012A>G
-
-
LMNA_000648
-
PubMed: Fan 2020
,
PubMed: Fan 2021
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+/.
23
1
c.94_96del
r.(?)
p.(Lys32del)
ACMG
pathogenic, pathogenic (dominant)
g.156084803_156084805del
g.156115012_156115014del
92_94delAGA, 94_96delAAG, 94_96delAGA, c.94_96delAAG, K32del
-
LMNA_000059
ACMG PM1, PM2, PM4, PP5, ACMG PM2, PM6, PP2, PP3, de novo, in patient,
2 more items
Lacene, Neuromuscul.Disord. 12: 722,
PubMed: D Amico 2005
, Sara Benedetti 2005,
PubMed: Fan 2020
,
8 more items
-
rs60872029
CLASSIFICATION record, De novo, Germline, Germline/De novo (untested), Unknown
-
-
-
-
-
Johan den Dunnen
,
Madhuri Hegde
,
Sandra Cooper
,
VKGL-NL_Nijmegen
,
Florence van Tienen
+/.
2
1
c.95A>C
r.(?)
p.(Lys32Thr)
ACMG
pathogenic (dominant)
g.156084804A>C
g.156115013A>C
-
-
LMNA_000649
-
PubMed: Fan 2020
,
PubMed: Fan 2021
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+/.
2
1
c.96G>T
r.(?)
p.(Lys32Asn)
ACMG
pathogenic (dominant)
g.156084805G>T
g.156115014G>T
-
-
LMNA_000650
-
PubMed: Fan 2020
,
PubMed: Fan 2021
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+/.
1
1
c.98A>G
r.(?)
p.Glu33Gly
-
pathogenic
g.156084807A>G
g.156115016A>G
-
-
LMNA_000215
-
PubMed: Lo 2008
-
-
Germline
-
?
?
-
-
Maria Chiotis
+?/.
1
1
c.99G>C
r.(?)
p.(Glu33Asp)
-
likely pathogenic
g.156084808G>C
g.156115017G>C
-
-
LMNA_000504
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/.
2
1
c.99G>T
r.(?)
p.(Glu33Asp)
-
pathogenic
g.156084808G>T
g.156115017G>T
LMNA(NM_170707.4):c.99G>T (p.E33D)
-
LMNA_000104
VKGL data sharing initiative Nederland
PubMed: Goizet
,
PubMed: Vital 2005
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_AMC
+/., ?/.
3
1
c.99_101del
r.(?)
p.(Glu33del)
ACMG
pathogenic (dominant), VUS
g.156084808_156084810del
g.156115017_156115019del
95_97delAGG, c.98_100delAGG
-
LMNA_000651
-
PubMed: Fan 2020
,
PubMed: Fan 2021
,
PubMed: Ganapathy 2019
-
-
De novo, Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
1
c.100G>A
r.(?)
p.(Asp34Asn)
-
VUS
g.156084809G>A
g.156115018G>A
-
-
LMNA_000505
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/.
1
1
c.103C>G
r.(?)
p.(Leu35Val)
-
pathogenic
g.156084812C>G
g.156115021C>G
-
-
LMNA_000093
not in 200 control chromosomes; de novo, in patient
PubMed: Vytopil 2003
,
PubMed: Sanna
-
-
De novo
-
-
-
-
-
Johan den Dunnen
?/.
1
1
c.111G>A
r.(?)
p.(=)
-
VUS
g.156084820G>A
g.156115029G>A
-
-
LMNA_000506
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/.
1
1
c.115A>G
r.(?)
p.(Asn39Asp)
-
pathogenic
g.156084824A>G
g.156115033A>G
N39D
-
LMNA_000272
not in 300 control chromosomes; probably de novo, parents not available
PubMed: Komaki 2011
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
+/., +?/., ?/.
19
1
c.116A>G
r.(?)
p.(Asn39Ser)
ACMG
likely pathogenic, likely pathogenic (dominant), pathogenic, pathogenic (dominant), VUS
g.156084825A>G
g.156115034A>G
116A>AG
-
LMNA_000268
ACMG PM1 PM2 PP3 PP5  , ACMG ​PM1 PM2 PP3 PP5  , combination of variants not reported,
2 more items
PubMed: Cavdarli 2023
,
PubMed: Chakravorty 2020
,
PubMed: Fan 2020
,
PubMed: Fan 2021
,
PubMed: Topf 2020
,
4 more items
-
rs57983345
CLASSIFICATION record, De novo, Germline, Germline/De novo (untested), Unknown
-
1/1001 cases
-
-
-
Johan den Dunnen
,
Madhuri Hegde
,
Tom Winder
,
Wolfram Kress
,
Sandra Cooper
,
VKGL-NL_Nijmegen
+/., +?/.
3
1
c.117T>G
r.(?)
p.(Asn39Lys)
ACMG
likely pathogenic, pathogenic (dominant)
g.156084826T>G
g.156115035T>G
-
-
LMNA_000652
-
PubMed: Fan 2020
,
PubMed: Fan 2021
,
PubMed: Gonzalez-Quereda 2020
-
-
De novo, Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
1
c.121C>A
r.(?)
p.(Arg41Ser)
-
pathogenic
g.156084830C>A
g.156115039C>A
R41S
-
LMNA_000273
not in 300 control chromosomes; probably de novo, parents not available
PubMed: Komaki 2011
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
+/.
2
1
c.122G>C
r.(?)
p.(Arg41Pro)
ACMG
pathogenic (dominant)
g.156084831G>C
g.156115040G>C
-
-
LMNA_000653
-
PubMed: Fan 2020
,
PubMed: Fan 2021
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+/., ?/.
2
1
c.127G>A
r.(?)
p.(Ala43Thr)
-
pathogenic, VUS
g.156084836G>A
g.156115045G>A
-
-
LMNA_000066
VKGL data sharing initiative Nederland
PubMed: Brown
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Nijmegen
+/.
1
1
c.130G>T
r.(?)
p.(Val44Phe)
-
pathogenic
g.156084839G>T
g.156115048G>T
-
-
LMNA_000060
-
Lacene, Neuromuscul.Disord. 12: 722
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.132C>T
r.(?)
p.(Val44=)
-
likely benign
g.156084841C>T
g.156115050C>T
-
-
LMNA_000558
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/.
1
1
c.133T>A
r.(?)
p.(Tyr45Asn)
-
likely pathogenic
g.156084842T>A
g.156115051T>A
-
-
LMNA_000294
-
-
-
-
Unknown
-
-
-
-
-
Tom Winder
+/., ?/.
3
1
c.134A>G
r.(?)
p.(Tyr45Cys)
-
pathogenic, VUS
g.156084843A>G
g.156115052A>G
-
-
LMNA_000036
-
PubMed: Bonne
,
PubMed: Nallamilli 2018
,
PubMed: Scherner 2011
-
-
Germline, Unknown
-
-
-
-
-
Johan den Dunnen
,
Madhuri Hegde
+/., ?/.
3
1
c.139G>C
r.(?)
p.(Asp47His)
-
pathogenic, VUS
g.156084848G>C
g.156115057G>C
-
-
LMNA_000179
no segregation analysis, VKGL data sharing initiative Nederland
Tverskaya ESHG2006 P0740,
PubMed: Westra 2019
-
-
CLASSIFICATION record, Germline, Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
,
Svetlana Tverskaya
,
VKGL-NL_Nijmegen
+/.
2
1
c.143G>C
r.(?)
p.(Arg48Pro)
ACMG
pathogenic (dominant)
g.156084852G>C
g.156115061G>C
-
-
LMNA_000654
-
PubMed: Fan 2020
,
PubMed: Fan 2021
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+/., +?/.
5
1
c.148C>A
r.(?), r.0
p.(Arg50Ser)
ACMG
likely pathogenic (dominant), pathogenic
g.156084857C>A
g.156115066C>A
-
-
LMNA_000065
-
PubMed: Brown
-
rs59931416
Germline
yes
-
+AluI
-
-
Johan den Dunnen
,
Tom Winder
,
Andreas Laner
,
MarÃa Eugenia Foncuberta
+/.
1
1
c.148C>T
r.(?)
p.(Arg50Cys)
-
pathogenic
g.156084857C>T
g.156115066C>T
-
-
LMNA_000309
-
-
-
-
Germline
-
-
-
-
-
Tom Winder
?/.
1
-
c.149G>A
r.(?)
p.(Arg50His)
-
VUS
g.156084858G>A
-
-
-
LMNA_000796
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
2
1
c.149G>C
r.(?)
p.(Arg50Pro)
-
pathogenic
g.156084858G>C
g.156115067G>C
-
-
LMNA_000037
de novo, in patient
PubMed: Bonne
,
PubMed: Quijano-Roy 2008
-
-
De novo, Germline
-
-
-
-
-
Johan den Dunnen
?/.
2
1
c.149G>T
r.(?)
p.(Arg50Leu)
-
VUS
g.156084858G>T
g.156115067G>T
-
-
LMNA_000507
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
-?/.
1
-
c.150C>T
r.(?)
p.(Arg50=)
-
likely benign
g.156084859C>T
g.156115068C>T
LMNA(NM_170707.2):c.150C>T (p.R50=)
-
LMNA_000378
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-/.
1
-
c.153G>T
r.(?)
p.(Ser51=)
-
benign
g.156084862G>T
g.156115071G>T
LMNA(NM_170707.4):c.153G>T (p.S51=)
-
LMNA_000379
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+?/.
1
-
c.154C>G
r.(?)
p.(Leu52Val)
-
likely pathogenic
g.156084863C>G
g.156115072C>G
-
-
LMNA_000677
-
PubMed: Walsh 2017
-
-
Germline
-
1/740 cases
-
-
-
Johan den Dunnen
+/.
1
1
c.155T>C
r.(?)
p.(Leu52Pro)
-
pathogenic
g.156084864T>C
g.156115073T>C
-
-
LMNA_000211
-
Rudenskaya ESHG2007 P0780
-
-
Germline
-
-
-
-
-
Svetlana Tverskaya
+?/.
1
-
c.157G>A
r.(?)
p.(Glu53Lys)
-
likely pathogenic
g.156084866G>A
-
-
-
LMNA_000748
-
-
-
-
Unknown
-
-
-
-
-
MobiDetails
+/.
1
1
c.158A>T
r.(?)
p.(Glu53Val)
-
pathogenic
g.156084867A>T
g.156115076A>T
-
-
LMNA_000190
-
Song ASHG2006 P485
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
5
1
c.161C>T
r.(?)
p.(Thr54Met)
-
VUS
g.156084870C>T
g.156115079C>T
LMNA(NM_001282625.1):c.161C>T (p.(Thr54Met)), LMNA(NM_170707.4):c.161C>T (p.T54M)
-
LMNA_000380
no segregation analysis, VKGL data sharing initiative Nederland
van Tienen, submitted,
PubMed: Westra 2019
-
-
CLASSIFICATION record, Germline/De novo (untested)
?
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Leiden
,
VKGL-NL_Nijmegen
,
VKGL-NL_AMC
,
Florence van Tienen
+/.
2
1
c.162_163del
r.(?)
p.(Asn56Argfs*11), p.(Asn56ArgfsTer11)
-
pathogenic, pathogenic (dominant)
g.156084871_156084872del
g.156115080_156115081del
162_163delGG
-
LMNA_000508
ACMG PVS1, PM2, PS4, PP5
PubMed: Nallamilli 2018
,
PubMed: Radziwonik-Fraczyk 2024
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Madhuri Hegde
+/.
1
1
c.169G>C
r.169g>c
p.Ala57Pro
-
pathogenic
g.156084878G>C
g.156115087G>C
-
-
LMNA_000087
-
PubMed: Chen
,
OMIM:var0030
-
rs28928903
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.169G>T
r.(?)
p.(Ala57Ser)
-
VUS
g.156084878G>T
-
LMNA(NM_170707.4):c.169G>T (p.A57S)
-
LMNA_000739
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+?/., ?/.
3
1
c.173G>T
r.(?)
p.(Gly58Val)
-
likely pathogenic, VUS
g.156084882G>T
g.156115091G>T
LMNA(NM_170707.3):c.173G>T (p.(Gly58Val))
-
LMNA_000492
VKGL data sharing initiative Nederland
van Tienen, submitted
-
-
CLASSIFICATION record, Germline
?
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Nijmegen
,
Florence van Tienen
?/.
1
-
c.175C>A
r.(?)
p.(Leu59Met)
-
VUS
g.156084884C>A
g.156115093C>A
-
-
LMNA_000678
-
PubMed: Walsh 2017
-
-
Germline
-
1/304 cases
-
-
-
Johan den Dunnen
+/.
1
1
c.176T>G
r.(?)
p.(Leu59Arg)
-
pathogenic
g.156084885T>G
g.156115094T>G
-
-
LMNA_000208
not in 232 control chromosomes; de novo, in patient
PubMed: Nguyen 2006
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+/.
11
1
c.178C>G
r.(?)
p.(Arg60Gly)
-
pathogenic
g.156084887C>G
g.156115096C>G
-
-
LMNA_000005
-
PubMed: Fatkin 1999
,
PubMed: Carboni 2009
,
OMIM:var0005
,
PubMed: van der Kooi
,
OMIM:var0005
-
rs28928900
Germline
-
-
+Sau96I
-
-
Johan den Dunnen
+/.
1
1
c.184C>G
r.(?)
p.(Arg62Gly)
-
pathogenic
g.156084893C>G
g.156115102C>G
-
-
LMNA_000053
-
PubMed: Garg 2002
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.184C>T
r.(?)
p.(Arg62Cys)
-
likely pathogenic
g.156084893C>T
-
LMNA(NM_170707.4):c.184C>T (p.R62C)
-
LMNA_000697
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
2
1
c.188T>A
r.(?)
p.(Ile63Asn)
-
pathogenic
g.156084897T>A
g.156115106T>A
I63N
-
LMNA_000071
de novo, in patient
PubMed: Boriani 2003
,
PubMed: Vytopil
-
-
De novo, Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
1
c.188T>G
r.(?)
p.(Ile63Ser)
-
pathogenic
g.156084897T>G
g.156115106T>G
-
-
LMNA_000038
-
PubMed: Bonne
,
PubMed: Vytopil
,
PubMed: Muchir
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
2
1
c.194A>G
r.(?)
p.(Glu65Gly)
-
likely pathogenic
g.156084903A>G
g.156115112A>G
-
-
LMNA_000100
not in 130 control chromosomes
PubMed: Vytopil 2003
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
1, 11
c.203_208del
r.(?)
p.(Glu68_Val69del)
-
pathogenic
g.156084912_156084917del
g.156115121_156115126del
del212_8
-
LMNA_000171
-
PubMed: Arbustini 2005
,
PubMed: Pasotti 2008
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
?/.
1
1
c.208G>A
r.(?)
p.(Val70Ile)
-
VUS
g.156084917G>A
g.156115126G>A
-
-
LMNA_000488
-
van Tienen, submitted
-
-
Unknown
?
-
-
-
-
Florence van Tienen
+?/., ?/.
3
1
c.215G>A
r.(?)
p.(Arg72His)
-
likely pathogenic, VUS
g.156084924G>A
g.156115133G>A
LMNA(NM_170707.2):c.215G>A (p.R72H)
-
LMNA_000559
variant definitively linked to disease, VKGL data sharing initiative Nederland
Fusco 2042, submitted
-
rs727504340
CLASSIFICATION record, Germline, Unknown
-
-
beatrice.alessandrini
-
-
VKGL-NL_Rotterdam
,
Carmela Fusco
,
Beatrice Alessandrini
?/.
1
-
c.218A>C
r.(?)
p.(Glu73Ala)
-
VUS
g.156084927A>C
-
LMNA(NM_170707.4):c.218A>C (p.E73A)
-
LMNA_000766
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
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