Global Variome shared LOVD
COL6A2 (collagen, type VI, alpha 2)
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Curator:
Johan den Dunnen
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Unique variants in the COL6A2 gene
This database is one of the gene variant databases from the
"Leiden Muscular Dystrophy pages" (LMDp)
.
The variants shown are described using the NM_001849.3 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.
571 entries on 6 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
1i
c.-27(-3053 _-2994)_-27(-900_-856)del
r.0
p.0
-
pathogenic
g.?
-
g.(46352739_46352798)_(46354892_46354936)del
-
COL6A2_000147
1 more item
PubMed: Bovolenta 2010
-
-
Germline
yes
-
-
-
-
Alessandra Ferlini
+/.
1
1_28
c.(?_-82)_(*297_?)del
r.0
p.0
-
pathogenic
g.?
-
-
-
COL6A2_000000
69 Kb deletion
L Medne ASHG 2010 A1669
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
5' UTR
c.-6G>A
r.spl
p.(=)
-
VUS
g.47531385G>A
g.46111471G>A
-
-
COL6A2_000273
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/.
1
_1_18i_
c.(?_-1)_(1521+1_?)del
r.0
p.0
-
pathogenic
g.(47500000_47531390)_(47541533_47552467)del
-
-
-
COL6A2_000144
69 kb deletion
PubMed: Foley 2011
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
+/.
3
_1_28_
c.(?_-1)_(*1_?)del
r.0
p.0
-
pathogenic
g.(46400000_46480000)_(48085000_qter)del, g.(46950000_47250000)_(48085000_qter)del,
1 more item
-
-
-
COL6A1_000161, COL6A1_000162, COL6A2_000145
1.09 Mb deletion PCBP3_PRMT2 (Incl. COL6A1, COL6A2), 47 kb deletion,
1 more item
PubMed: Foley 2011
-
-
Germline
?, yes
-
-
-
-
Johan den Dunnen
+/., +?/., ?/.
3
16, 3
c.?
r.(?)
p.(?), p.G2065S, p.V117A
-
likely pathogenic, pathogenic, VUS
g.?
-
350T>C (V117A), 6193G>A (G2065S)
-
COL6A2_000000, COL6A2_000008
no second variant, protein domain TH
PubMed: Camacho 2001
,
OMIM:var0003
,
PubMed: Nallamilli 2018
-
-
De novo, Germline
-
-
DdeI
-
-
Johan den Dunnen
,
Madhuri Hegde
+/.
1
-
c.13_25del
r.(?)
p.(Thr5Serfs*62)
-
pathogenic
g.47531403_47531415del
g.46111489_46111501del
c.11_23delGCACCTGCTCCGT
-
COL6A2_000452
-
PubMed: Yu 2017
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/., ?/.
4
2
c.22G>A
r.(?)
p.(Val8Met)
-
likely benign, VUS
g.47531412G>A
g.46111498G>A
COL6A2(NM_001849.3):c.22G>A (p.(Val8Met))
-
COL6A2_000274
no second variant, VKGL data sharing initiative Nederland
PubMed: Nallamilli 2018
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Madhuri Hegde
,
VKGL-NL_Leiden
+?/.
1
2
c.38del
r.(?)
p.(Gly13GlufsTer58)
-
likely pathogenic
g.47531428del
g.46111514del
-
-
COL6A2_000461
-
PubMed: Ganapathy 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.80C>T
r.(?)
p.(Ser27Leu)
-
VUS
g.47531470C>T
-
COL6A2(NM_001849.3):c.80C>T (p.(Ser27Leu))
-
COL6A2_000528
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
2
c.94G>A
r.(?)
p.(Glu32Lys)
-
VUS
g.47531484G>A
g.46111570G>A
-
-
COL6A2_000275
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
1
-
c.106_108del
r.(?)
p.(Asn36del)
-
VUS
g.47531496_47531498del
-
COL6A2(NM_001849.4):c.106_108del (p.(Asn36del))
-
COL6A2_000541
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
1
2
c.114_115+12del
r.spl
p.?
ACMG
pathogenic (recessive)
g.47531504_47531517del
g.46111590_46111603del
-
-
COL6A2_000522
ACMG PVS1, PM2_sup, PM3_sup, PP4
PubMed: Morel 2023
,
Journal: Morel 2023
#1324140, #476449
-
Germline
?
-
-
-
-
Victor Morel
?/.
1
2i
c.115+6del
r.(?)
p.(?)
-
VUS
g.47531511del
g.46111597del
115+6delA
-
COL6A2_000276
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
1
2i
c.115+10G>T
r.spl
p.(=)
-
VUS
g.47531515G>T
g.46111601G>T
-
-
COL6A2_000277
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
-?/., ?/.
2
2i
c.116-34G>A
r.(=), r.(?)
p.(=)
-
likely benign, VUS
g.47531859G>A
g.46111945G>A
-
-
COL6A2_000069
24 heterozygous, no homozygous;
Clinindb (India)
, from website {DBsub-Emory}
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs117154313
Germline
-
24/2793 individuals
-
-
-
Madhuri Hegde
,
Mohammed Faruq
-?/.
1
-
c.116-3C>T
r.(?)
p.(?)
-
likely benign
g.47531890C>T
-
-
-
COL6A2_000527
-
-
-
rs767597831
CLASSIFICATION record
-
-
-
-
-
MobiDetails
?/.
1
3
c.148G>A
r.(?)
p.(Val50Met)
-
VUS
g.47531925G>A
g.46112011G>A
-
-
COL6A2_000131
-
PubMed: Butterfield
-
-
Germline
?
-
-
-
-
Russell Butterfield
?/.
1
3
c.167G>A
r.(?)
p.(Ser56Asn)
-
VUS
g.47531944G>A
g.46112030G>A
-
-
COL6A2_000278
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
1
3
c.169G>A
r.(?)
p.(Val57Ile)
-
VUS
g.47531946G>A
g.46112032G>A
-
-
COL6A2_000279
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
2
3
c.176T>C
r.(?)
p.(Met59Thr)
-
VUS
g.47531953T>C
g.46112039T>C
-
-
COL6A2_000280
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
1
3
c.181T>C
r.(?)
p.(Ser61Pro)
-
VUS
g.47531958T>C
g.46112044T>C
-
-
COL6A2_000203
variant apparently homozygous
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
1
3
c.188C>T
r.(?)
p.(Thr63Met)
-
VUS
g.47531965C>T
g.46112051C>T
-
-
COL6A2_000281
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
-?/.
1
-
c.189G>A
r.(?)
p.(Thr63=)
-
likely benign
g.47531966G>A
-
COL6A2(NM_001849.3):c.189G>A (p.(Thr63=))
-
COL6A2_000497
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
2
-
c.202T>C
r.(?)
p.(Phe68Leu)
-
VUS
g.47531979T>C
g.46112065T>C
COL6A2(NM_058175.3):c.202T>C (p.F68L)
-
COL6A2_000204
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
,
VKGL-NL_AMC
+?/.
1
3
c.214C>T
r.(?)
p.(Gln72Ter)
-
likely pathogenic (recessive)
g.47531991C>T
g.46112077C>T
-
-
COL6A2_000475
-
PubMed: Fan 2018
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
3
c.224C>T
r.(?)
p.(Pro75Leu)
-
VUS
g.47532001C>T
g.46112087C>T
-
-
COL6A2_000282
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
1
-
c.228G>T
r.(?)
p.(Gln76His)
-
VUS
g.47532005G>T
-
COL6A2(NM_001849.3):c.228G>T (p.(Gln76His))
-
COL6A2_000513
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
3
c.229T>C
r.(?)
p.(Phe77Leu)
-
VUS
g.47532006T>C
g.46112092T>C
-
-
COL6A2_000283
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
1
3
c.284G>A
r.(?)
p.(Arg95His)
-
VUS
g.47532061G>A
g.46112147G>A
-
-
COL6A2_000284
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
2
-
c.289G>A
r.(?)
p.(Gly97Ser)
-
VUS
g.47532066G>A
g.46112152G>A
COL6A2(NM_001849.3):c.289G>A (p.(Gly97Ser))
-
COL6A2_000431
VKGL data sharing initiative Nederland
PubMed: Ayala-Ramirez 2025
ClinVar-476478
rs750027302
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Leiden
?/.
1
-
c.292G>A
r.(?)
p.(Gly98Ser)
-
VUS
g.47532069G>A
-
COL6A2(NM_058175.3):c.292G>A (p.G98S)
-
COL6A2_000498
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
+/.
1
3
c.310C>T
r.(?)
p.(Gln104Ter)
-
pathogenic
g.47532087C>T
g.46112173C>T
-
-
COL6A2_000462
-
PubMed: Ganapathy 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.313G>A
r.(?)
p.(Val105Met)
-
VUS
g.47532090G>A
-
COL6A2(NM_001849.3):c.313G>A (p.(Val105Met))
-
COL6A2_000529
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/., -/., -?/., ?/.
7
3
c.316G>A
r.(?)
p.(Glu106Lys), p.Glu106Lys
-
benign, likely benign, pathogenic, VUS
g.47532093G>A
g.46112179G>A
1 more item
-
COL6A2_000070
from website {DBsub-Emory}, VKGL data sharing initiative Nederland
PubMed: O'Grady 2016
-
-
CLASSIFICATION record, Germline
yes
-
-
-
-
Madhuri Hegde
,
Alessandra Ferlini
,
Shireen Lamandé
,
VKGL-NL_Leiden
,
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
,
VKGL-NL_AMC
?/.
1
3
c.332C>T
r.(?)
p.(Pro111Leu)
-
VUS
g.47532109C>T
g.46112195C>T
-
-
COL6A2_000285
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/.
1
3
c.348dup
r.(?)
p.(Ser117Leufs*159)
-
pathogenic
g.47532125dup
g.46112211dup
-
-
COL6A2_000164
-
-
-
-
Germline
?
-
-
-
-
Alessandra Ferlini
?/.
1
3
c.386G>A
r.(?)
p.(Arg129His)
-
VUS
g.47532163G>A
g.46112249G>A
-
-
COL6A2_000286
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+?/.
2
-
c.388C>T
r.(?)
p.(Arg130Cys)
-
likely pathogenic
g.47532165C>T
g.46112251C>T
-
-
COL6A2_000202
combination of variants not reported,
1 more item
MYO-SEQ project, UK,
PubMed: Topf 2020
-
-
Germline
?
1/1001 cases
-
-
-
Johan den Dunnen
,
Alison Blain
?/.
1
3
c.410C>T
r.(?)
p.(Ala137Val)
-
VUS
g.47532187C>T
g.46112273C>T
-
-
COL6A2_000287
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
1
-
c.421A>G
r.(?)
p.(Met141Val)
-
VUS
g.47532198A>G
-
COL6A2(NM_001849.4):c.421A>G (p.(Met141Val))
-
COL6A2_000542
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
2
3
c.436C>T
r.(?)
p.(Arg146Trp)
-
VUS
g.47532213C>T
g.46112299C>T
-
-
COL6A2_000288
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
1
3
c.446G>A
r.(?)
p.(Arg149His)
-
VUS
g.47532223G>A
g.46112309G>A
-
-
COL6A2_000289
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
-?/.
1
-
c.460G>A
r.(?)
p.(Val154Ile)
-
likely benign
g.47532237G>A
-
COL6A2(NM_001849.3):c.460G>A (p.(Val154Ile))
-
COL6A2_000530
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
2
3
c.472G>A
r.(?)
p.(Val158Met)
-
VUS
g.47532249G>A
g.46112335G>A
COL6A2(NM_058175.2):c.472G>A (p.V158M)
-
COL6A2_000290
no second variant, VKGL data sharing initiative Nederland
PubMed: Nallamilli 2018
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Madhuri Hegde
,
VKGL-NL_Rotterdam
-/.
1
-
c.483C>T
r.(?)
p.(Thr161=)
-
benign
g.47532260C>T
g.46112346C>T
-
-
COL6A2_000240
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
-
c.487G>A
r.(?)
p.(Gly163Ser)
-
VUS
g.47532264G>A
-
COL6A2(NM_058175.3):c.487G>A (p.G163S)
-
COL6A2_000540
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
?/.
1
3
c.493G>A
r.(?)
p.(Val165Ile)
-
VUS
g.47532270G>A
g.46112356G>A
-
-
COL6A2_000291
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
1
-
c.499G>A
r.(?)
p.(Gly167Ser)
-
VUS
g.47532276G>A
g.46112362G>A
COL6A2(NM_058175.3):c.499G>A (p.G167S)
-
COL6A2_000206
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
1
-
c.503G>T
r.(?)
p.(Ser168Ile)
-
VUS
g.47532280G>T
g.46112366G>T
-
-
COL6A2_000449
-
PubMed: Punetha 2016
-
-
Germline/De novo (untested)
-
1/94 cases
-
-
-
Johan den Dunnen
-?/.
1
3
c.507C>T
r.(?)
p.(=)
-
likely benign
g.47532284C>T
g.46112370C>T
-
-
COL6A2_000138
-
PubMed: Butterfield
-
-
Germline
?
-
-
-
-
Russell Butterfield
-?/., ?/.
4
3
c.510C>T
r.(?)
p.(=), p.(Cys170=)
-
likely benign, VUS
g.47532287C>T
g.46112373C>T
COL6A2(NM_001849.3):c.510C>T (p.(Cys170=)), COL6A2(NM_058175.2):c.510C>T (p.C170=)
-
COL6A2_000071
from website {DBsub-Emory}, VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Madhuri Hegde
,
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
VKGL-NL_Nijmegen
+/., -?/., ?/.
20
3
c.511G>A
r.(?)
p.(Gly171Arg)
-
likely benign, pathogenic, VUS
g.47532288G>A
g.46112374G>A
1 more item
-
COL6A2_000159
inherited from unaffected father, no second variant, VKGL data sharing initiative Nederland
PubMed: Morales-Rosado 2018
,
PubMed: Nallamilli 2018
-
-
CLASSIFICATION record, Germline
yes
-
-
-
-
Johan den Dunnen
,
Madhuri Hegde
,
Alessandra Ferlini
,
VKGL-NL_Leiden
,
VKGL-NL_Groningen
,
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
,
VKGL-NL_AMC
-?/.
1
3
c.532G>A
r.(?)
p.(Glu178Lys)
-
likely benign
g.47532309G>A
g.46112395G>A
-
-
COL6A2_000128
-
-
-
-
Germline
-
-
-
-
-
Tom Winder
?/.
1
-
c.542G>A
r.(?)
p.(Arg181His)
-
VUS
g.47532319G>A
-
COL6A2(NM_058175.2):c.542G>A (p.R181H)
-
COL6A2_000458
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
3
c.545A>G
r.(?)
p.(Glu182Gly)
-
VUS
g.47532322A>G
g.46112408A>G
-
-
COL6A2_000292
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
4
3
c.568G>A
r.(?)
p.(Val190Met)
-
VUS
g.47532345G>A
g.46112431G>A
COL6A2(NM_001849.4):c.568G>A (p.V190M)
-
COL6A2_000293
no second variant, VKGL data sharing initiative Nederland
PubMed: Nallamilli 2018
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Madhuri Hegde
,
VKGL-NL_Groningen
?/.
1
3
c.581A>G
r.(?)
p.(Gln194Arg)
-
VUS
g.47532358A>G
g.46112444A>G
-
-
COL6A2_000294
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/.
1
-
c.595C>T
r.(?)
p.(Gln199*)
-
pathogenic
g.47532372C>T
-
-
-
COL6A2_000514
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
3
c.596A>G
r.(?)
p.(Gln199Arg)
-
VUS
g.47532373A>G
g.46112459A>G
-
-
COL6A2_000295
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
1
3
c.605G>A
r.(?)
p.(Arg202Gln)
-
VUS
g.47532382G>A
g.46112468G>A
-
-
COL6A2_000296
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
-?/.
1
-
c.624G>T
r.(?)
p.(Pro208=)
-
likely benign
g.47532401G>T
-
COL6A2(NM_058175.2):c.624G>T (p.P208=)
-
COL6A2_000499
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
3
c.627C>T
r.(?)
p.(=)
-
VUS
g.47532404C>T
g.46112490C>T
-
-
COL6A2_000297
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
-?/., ?/.
4
3
c.628G>A
r.(?)
p.(Glu210Lys)
-
likely benign, VUS
g.47532405G>A
g.46112491G>A
COL6A2(NM_001849.3):c.628G>A (p.(Glu210Lys)), g.14395G>A
-
COL6A2_000298
no second variant, VKGL data sharing initiative Nederland
PubMed: Nallamilli 2018
,
PubMed: Saat 2021
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Madhuri Hegde
,
VKGL-NL_Leiden
,
Ibrahim Sahin
?/.
1
3
c.637C>T
r.(?)
p.(Arg213Cys)
-
VUS
g.47532414C>T
g.46112500C>T
-
-
COL6A2_000299
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
2
3
c.638G>A
r.(?)
p.(Arg213His)
-
VUS
g.47532415G>A
g.46112501G>A
COL6A2(NM_001849.4):c.638G>A (p.R213H)
-
COL6A2_000300
no second variant, VKGL data sharing initiative Nederland
PubMed: Nallamilli 2018
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Madhuri Hegde
,
VKGL-NL_Groningen
+?/.
1
3
c.641_645del
r.(?)
p.(Asn214Ilefs*60)
-
likely pathogenic
g.47532418_47532422del
g.46112504_46112508del
-
-
COL6A2_000052
-
-
-
-
Germline
-
-
-
-
-
Tom Winder
-?/., ?/.
2
3
c.643G>A
r.(?)
p.(Asp215Asn)
-
likely benign, VUS
g.47532420G>A
g.46112506G>A
COL6A2(NM_058175.2):c.643G>A (p.D215N)
-
COL6A2_000301
VKGL data sharing initiative Nederland
PubMed: Nallamilli 2018
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Madhuri Hegde
,
VKGL-NL_Rotterdam
-/.
5
3
c.663C>T
r.(?), r.663c>u
p.(=), p.(Pro221=), p.=
-
benign
g.47532440C>T
g.46112526C>T
COL6A2(NM_058175.3):c.663C>T (p.P221=), P221P
-
COL6A2_000072
from website {DBsub-Emory}, VKGL data sharing initiative Nederland
PubMed: Gualandi 2012
-
-
CLASSIFICATION record, Germline
?
-
-
-
-
Madhuri Hegde
,
Alessandra Ferlini
,
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
,
VKGL-NL_AMC
?/.
1
-
c.664G>A
r.(?)
p.(Asp222Asn)
-
VUS
g.47532441G>A
-
COL6A2(NM_058175.2):c.664G>A (p.D222N)
-
COL6A2_000455
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/., -?/.
17
3
c.679G>A
r.(?), r.679a>g
p.(Asp227Asn), p.Asp227Asn
-
benign, likely benign
g.47532456G>A
g.46112542G>A
679A>G,
1 more item
-
COL6A2_000031
64 heterozygous, no homozygous;
Clinindb (India)
, from website {DBsub-Emory}, N1,
1 more item
PubMed: Baker 2007
,
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs35881321
CLASSIFICATION record, Germline
-
64/2795 individuals
-
-
-
Johan den Dunnen
,
Madhuri Hegde
,
VKGL-NL_Leiden
,
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
,
VKGL-NL_AMC
,
Mohammed Faruq
+/.
1
3
c.688_689dup
r.(?)
p.(Ile231Profs*9)
-
pathogenic
g.47532465_47532466dup
g.46112551_46112552dup
-
-
COL6A2_000001
protein domain N1
PubMed: Lampe 2005
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.697C>T
r.(?)
p.(Arg233Cys)
-
VUS
g.47532474C>T
g.46112560C>T
-
-
chr21_000921
-
PubMed: Ayala-Ramirez 2025
ClinVar-542969
rs1379543505
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.709dup
r.(?)
p.(Val237Glyfs*39)
-
VUS
g.47532486dup
-
COL6A2(NM_001849.3):c.709dupG (p.(Val237fs))
-
COL6A2_000531
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-/., -?/.
6
3i
c.714+9C>T
r.(=), r.(?)
p.(=)
-
benign, likely benign
g.47532500C>T
g.46112586C>T
COL6A2(NM_001849.3):c.714+9C>T (p.(=)), COL6A2(NM_058175.3):c.714+9C>T
-
COL6A2_000073
175 heterozygous;
Clinindb (India)
, 2 homozygous;
Clinindb (India)
, from website {DBsub-Emory},
1 more item
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs78822624
CLASSIFICATION record, Germline
-
175/2794 individuals, 2/2794 individuals
-
-
-
Madhuri Hegde
,
VKGL-NL_Leiden
,
VKGL-NL_Nijmegen
,
VKGL-NL_AMC
,
Mohammed Faruq
-?/.
1
-
c.714+10G>A
r.(=)
p.(=)
-
likely benign
g.47532501G>A
g.46112587G>A
COL6A2(NM_001849.3):c.714+10G>A (p.(=))
-
COL6A2_000432
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-/.
1
3i
c.714+29G>A
r.(?)
p.(=)
-
benign
g.47532520G>A
g.46112606G>A
-
-
COL6A2_000074
from website {DBsub-Emory}
-
-
-
Germline
-
-
-
-
-
Madhuri Hegde
-/.
1
3i
c.714+45C>T
r.(?)
p.(=)
-
benign
g.47532536C>T
g.46112622C>T
-
-
COL6A2_000075
from website {DBsub-Emory}
-
-
-
Germline
-
-
-
-
-
Madhuri Hegde
-/.
1
-
c.714+53C>T
r.(=)
p.(=)
-
benign
g.47532544C>T
-
COL6A2(NM_058175.2):c.714+53C>T
-
COL6A2_000506
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
?/.
2
4
c.730G>A
r.(?)
p.(Gly244Arg)
-
VUS
g.47532733G>A
g.46112819G>A
-
-
COL6A2_000302
1 heterozygous, no homozygous;
Clinindb (India)
PubMed: Nallamilli 2018
,
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs199806576
Germline
-
1/2795 individuals
-
-
-
Madhuri Hegde
,
Mohammed Faruq
+?/.
1
4i_5i
c.(735+1_802-1)?
r.736_801del
p.(del)
-
likely pathogenic
g.(47532739_47535785)?
g.(46112825_46115871)?
736_801del66
-
COL6A2_000185
-
PubMed: O'Grady 2016
-
-
De novo
-
-
-
-
-
Shireen Lamandé
+/., ?/.
2
4i_5
c.736-7_739del
r.spl
p.?
-
pathogenic, VUS
g.47533915_47533925del
g.46114001_46114011del
736-7_739del11
-
COL6A2_000303
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/.
1
4i
c.736-1G>A
r.spl
p.(Cys246_Lys267del)
-
pathogenic (dominant)
g.47533921G>A
g.46114007G>A
-
-
COL6A2_000476
-
PubMed: Zhang 2014
,
PubMed: Fan 2018
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+/.
3
-
c.736-1G>C
r.[736_801del,735_736ins[736-129_736-2;c]]
p.?
-
pathogenic (dominant)
g.47533921G>C
g.46114007G>C
-
-
COL6A2_000537
-
PubMed: Xie 2024
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
?/.
1
-
c.759A>G
r.(?)
p.(Glu253=)
-
VUS
g.47533945A>G
g.46114031A>G
COL6A2(NM_058175.3):c.759A>G (p.E253=)
-
COL6A2_000208
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/.
1
5
c.777C>G
r.777c>g
p.=
-
benign
g.47533963C>G
g.46114049C>G
G777C
-
COL6A2_000047
-
PubMed: Giusti 2005
-
-
Germline
-
-
-
-
-
Anne Lampe
+/.
1
5
c.780_781insCCCCCC
r.(?)
p.(Pro260_Lys261insProPro)
-
pathogenic
g.47533966_47533967insCCCCCC
g.46114052_46114053insCCCCCC
-
-
COL6A2_000550
-
Fortunato 2025, submitted
-
-
Germline
-
-
-
-
-
Marcella Neri
+/.
1
5
c.785G>A
r.(?)
p.(Gly262Asp)
-
pathogenic
g.47533971G>A
g.46114057G>A
-
-
COL6A2_000184
-
PubMed: O'Grady 2016
-
-
De novo
-
-
-
-
-
Shireen Lamandé
?/.
1
5
c.790C>T
r.(?)
p.(Arg264Cys)
-
VUS
g.47533976C>T
g.46114062C>T
-
-
COL6A2_000304
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
12
5
c.791G>A
r.(?)
p.(Arg264His)
-
VUS
g.47533977G>A
g.46114063G>A
1 more item
-
COL6A2_000305
no second variant, VKGL data sharing initiative Nederland
PubMed: Nallamilli 2018
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Madhuri Hegde
,
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
VKGL-NL_VUmc
?/.
1
5
c.793G>A
r.(?)
p.(Gly265Arg)
-
VUS
g.47533979G>A
g.46114065G>A
-
-
COL6A2_000306
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/.
1
5_5i
c.796_801+6del
r.spl
p.?
-
pathogenic
g.47533982_47533993del
g.46114068_46114079del
-
-
COL6A2_000307
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+?/.
2
5i
c.801+1del
r.spl
p.?
-
likely pathogenic
g.47533988del
g.46114074del
47533986AG>A
-
COL6A2_000192
combination of variants not reported,
1 more item
MYO-SEQ project, UK,
PubMed: Topf 2020
-
-
Germline
?
1/1001 cases
-
-
-
Johan den Dunnen
,
Alison Blain
+/.
1
5i
c.801+2T>C
r.spl?
p.?
-
pathogenic
g.47533989T>C
g.46114075T>C
-
-
COL6A2_000181
-
PubMed: O'Grady 2016
-
-
De novo
-
-
-
-
-
Shireen Lamandé
+/.
2
5i
c.801+3A>C
r.736_801del, r.spl?
p.?, p.Cys246_Lys267del
-
pathogenic
g.47533990A>C
g.46114076A>C
-
-
COL6A2_000155
RNA expression 2/7
PubMed: Martoni 2009
-
-
De novo, Germline
?, yes
-
-
-
-
Alessandra Ferlini
+/.
1
-
c.801+3_801+4dup
r.spl?
p.?
-
pathogenic (dominant)
g.47533990_47533991dup
g.46114076_46114077dup
-
-
COL6A2_000241
-
PubMed: Westra 2019
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.801+4del
r.spl?
p.?
-
pathogenic (dominant)
g.47533991del
g.46114077del
-
-
COL6A2_000242
-
PubMed: Westra 2019
-
-
De novo
-
-
-
-
-
Johan den Dunnen
?/.
1
5i
c.801+45C>A
r.(?)
p.(=)
-
VUS
g.47534032C>A
g.46114118C>A
-
-
COL6A2_000076
from website {DBsub-Emory}
-
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/.
2
5i
c.801+631_882del
r.(?)
p.(Gly268_Ile294del), p.[=, Gly268_Ile294del]
-
pathogenic
g.47534618_47535949del
g.46114704_46116035del
HGVS c.[=/801+631_882del]
-
COL6A2_000000
1332 bp deletion; protein domain TH
PubMed: Baker 2005
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
5i
c.802-2A>G
r.spl
p.?
-
pathogenic, pathogenic (dominant)
g.47535784A>G
g.46115870A>G
-
-
COL6A2_000308
no second variant
Fortunato 2025, submitted,
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
,
Marcella Neri
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