Global Variome shared LOVD
ANO5 (anoctamin 5)
LOVD v.3.0 Build 30b [
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Curators:
Bernard Brais
,
Johan den Dunnen
, and
Marie-Josée Dicaire
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Unique variants in the ANO5 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_213599.2 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.
318 entries on 4 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_22_
c.-317_*3602{0}
r.0
p.0
-
pathogenic (recessive)
g.(?_22044899)_(22462949_?)del
-
minmal 22044899-22462949del
-
ANO5_000269
418 kb deletion involving ANO5
PubMed: Liewluck 2013
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
_1_7i
c.-317_(648+1_649-1)del
r.0?
p.0?
-
pathogenic (recessive)
g.(?_22214722)_(22249133_22257708)del
g.(?_22193176)_(22227587_22236162)del
del ex1-7
-
ANO5_000267
-
PubMed: Vazquez 2020
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
1
c.-162A>G
r.(?)
p.(=)
-
VUS
g.22214877A>G
g.22193331A>G
-
-
ANO5_000027
-
-
-
rs61879341
Germline
-
-
-
-
-
Johan den Dunnen
-/., ?/.
2
1
c.-136G>C
r.(?)
p.(=)
-
benign, VUS
g.22214903G>C
g.22193357G>C
-
-
ANO5_000026
VKGL data sharing initiative Nederland
-
-
rs12792259
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Nijmegen
-?/.
1
-
c.9C>G
r.(?)
p.(Asp3Glu)
-
likely benign
g.22215047C>G
-
ANO5(NM_213599.2):c.9C>G (p.D3E)
-
ANO5_000297
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
1
c.11C>G
r.(?)
p.(Pro4Arg)
-
VUS
g.22215049C>G
g.22193503C>G
-
-
ANO5_000131
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/.
1
1
c.13del
r.(?)
p.(Asp5Ilefs*17)
-
pathogenic
g.22215051del
g.22193505del
-
-
ANO5_000072
-
-
-
-
Germline
-
-
-
-
-
Tom Winder
+?/.
2
-
c.40G>A
r.(?)
p.(Gly14Arg)
ACMG
likely pathogenic (recessive)
g.22215078G>A
g.22193532G>A
-
-
ANO5_000330
ACMG PM2 PP3 (strong) PM3, ACMG PM2 PP3_S PM3
PubMed: Cavdarli 2023
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.41-15_41-12del
r.(=)
p.(=)
-
likely benign
g.22225335_22225338del
g.22203789_22203792del
ANO5(NM_213599.2):c.41-15_41-12delTCTT
-
ANO5_000250
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/., +?/., ?/.
5
1i
c.41-1G>A
r.spl
p.?
-
likely pathogenic, pathogenic, pathogenic (recessive), VUS
g.22225349G>A
g.22203803G>A
-
-
ANO5_000044
from website {DBsub-Emory}, no second variant
PubMed: Nallamilli 2018
,
PubMed: Ten Dam 2019
-
-
Germline
-
-
-
-
-
Ieke Ginjaar
,
Madhuri Hegde
,
Tom Winder
+?/.
1
-
c.69C>A
r.(?)
p.(Tyr23*)
-
likely pathogenic
g.22225378C>A
g.22203832C>A
-
-
ANO5_000279
combination of variants not reported
PubMed: Topf 2020
-
-
Germline
-
1/1001 cases
-
-
-
Johan den Dunnen
+/.
1
-
c.87G>A
r.spl
p.?
-
pathogenic (recessive)
g.22225396G>A
g.22203850G>A
-
-
ANO5_000271
-
PubMed: Ten Dam 2019
-
-
Germline
-
-
-
-
-
Ieke Ginjaar
+/.
2
3
c.108_109del
r.(?)
p.(Glu36Aspfs*7), p.(Glu36AspfsTer7)
-
pathogenic, pathogenic (recessive)
g.22232830_22232831del
g.22211284_22211285del
108_109delGA, c.103_104delAG
-
ANO5_000132
-
PubMed: Marti 2025
,
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Madhuri Hegde
-?/.
2
-
c.129A>G
r.(?)
p.(Glu43=)
-
likely benign
g.22232851A>G
g.22211305A>G
ANO5(NM_213599.2):c.129A>G (p.E43=), ANO5(NM_213599.3):c.129A>G (p.E43=)
-
ANO5_000081
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
?/.
1
3
c.130G>A
r.(?)
p.(Glu44Lys)
-
VUS
g.22232852G>A
g.22211306G>A
-
-
ANO5_000133
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
-?/.
1
-
c.138+9C>T
r.(=)
p.(=)
-
likely benign
g.22232869C>T
-
ANO5(NM_213599.2):c.138+9C>T
-
ANO5_000265
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.138+10G>A
r.(=)
p.(=)
-
likely benign
g.22232870G>A
-
ANO5(NM_213599.3):c.138+10G>A
-
ANO5_000320
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
-/., ?/.
2
3i
c.138+169G>A
r.(=)
p.(=)
-
benign, VUS
g.22233029G>A
g.22211483G>A
ANO5(NM_213599.2):c.138+169G>A
-
ANO5_000009
VKGL data sharing initiative Nederland
-
-
rs4922980
CLASSIFICATION record, Germline
-
0.31-0.88
-
-
-
Johan den Dunnen
,
VKGL-NL_Utrecht
?/.
1
3i
c.138+206T>C
r.(=)
p.(=)
-
VUS
g.22233066T>C
g.22211520T>C
-
-
ANO5_000010
-
-
-
rs4922981
Germline
-
0.19-0.90
-
-
-
Johan den Dunnen
-/.
1
-
c.139-164del
r.(=)
p.(=)
-
benign
g.22239628del
-
ANO5(NM_213599.2):c.139-164delT
-
ANO5_000321
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/.
2
3i
c.139-1del
r.spl
p.?
-
pathogenic
g.22239791del
g.22218245del
139-1delG
-
ANO5_000134
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
2
4
c.139C>T
r.(?)
p.(Pro47Ser)
-
VUS
g.22239792C>T
g.22218246C>T
-
-
ANO5_000135
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/.
4
4
c.148C>T
r.(?)
p.(Arg50*)
-
pathogenic, pathogenic (recessive)
g.22239801C>T
g.22218255C>T
-
-
ANO5_000052
-
PubMed: Ten Dam 2019
,
PubMed: Wahbi 2012
,
PubMed: Yu 2017
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Ieke Ginjaar
,
Tom Winder
?/.
1
4
c.149G>A
r.(?)
p.(Arg50Gln)
-
VUS
g.22239802G>A
g.22218256G>A
-
-
ANO5_000136
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/., ?/.
10
4
c.155A>G
r.(?)
p.(Asn52Ser)
-
pathogenic, pathogenic (recessive), VUS
g.22239808A>G
g.22218262A>G
N52K
-
ANO5_000048
conflicting interpretations of pathogenicity; 6 heterozygous, no homozygous;
Clinindb (India)
PubMed: Marti 2025
,
PubMed: Nallamilli 2018
,
PubMed: Narang 2020
,
Journal: Narang 2020
,
3 more items
-
rs143777403
Germline
-
6/2794 individuals
-
-
-
Johan den Dunnen
,
Ieke Ginjaar
,
Madhuri Hegde
,
Mohammed Faruq
+/.
2
4
c.169C>T
r.(?)
p.(Arg57Trp)
-
pathogenic (recessive)
g.22239822C>T
g.22218276C>T
g.48361T>C
-
ANO5_000272
-
PubMed: Saat 2021
,
PubMed: Ten Dam 2019
-
-
Germline
-
-
-
-
-
Ieke Ginjaar
,
Ibrahim Sahin
?/.
2
4
c.170G>A
r.(?)
p.(Arg57Gln)
-
VUS
g.22239823G>A
g.22218277G>A
ANO5(NM_213599.3):c.170G>A (p.R57Q)
-
ANO5_000137
no second variant, VKGL data sharing initiative Nederland
PubMed: Nallamilli 2018
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Madhuri Hegde
,
VKGL-NL_Groningen
+/., +?/., ?/.
27
4
c.172C>T
r.(?)
p.(Arg58Trp)
ACMG
likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive), VUS
g.22239825C>T
g.22218279C>T
ANO5(NM_213599.3):c.172C>T (p.(Arg58Trp)), NM_001142649:c.169C>T
-
ANO5_000039
ACMG PP1-S, PM2, PM3, PP1-M, PP1, PP2, PP3, PP4, combination of variants not reported,
6 more items
PubMed: Marti 2025
,
PubMed: Monies 2016
,
PubMed: Nallamilli 2018
,
PubMed: Punetha 2016
,
5 more items
-
-
CLASSIFICATION record, Germline
-
3/1001 cases
-
-
-
Johan den Dunnen
,
Madhuri Hegde
,
Tom Winder
,
Jaya Punetha
,
VKGL-NL_Leiden
,
VKGL-NL_Nijmegen
+/., +?/.
2
-
c.173G>A
r.(?)
p.(Arg58Gln)
ACMG
likely pathogenic (recessive), pathogenic (recessive)
g.22239826G>A
g.22218280G>A
-
-
ANO5_000228
ACMG PM3, PP1, PP2, PP3
PubMed: Marinakis 2021
,
PubMed: Ten Dam 2019
-
-
Germline
-
-
-
-
-
Ieke Ginjaar
,
Jan Traeger-Synodinos
+?/.
1
-
c.180+2T>C
r.spl
p.?
-
likely pathogenic (recessive)
g.22239835T>C
g.22218289T>C
-
-
ANO5_000249
-
PubMed: Izumi 2015
, Izumi 2020 (submitted)
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
1
-
c.180+21T>C
r.(=)
p.(=)
-
benign
g.22239854T>C
g.22218308T>C
ANO5(NM_213599.3):c.180+21T>C
-
ANO5_000082
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
?/.
1
4i
c.180+223G>A
r.(=)
p.(=)
-
VUS
g.22240056G>A
g.22218510G>A
-
-
ANO5_000008
-
-
-
rs4620717
Germline
-
0.02-0.47
-
-
-
Johan den Dunnen
+/.
2
4i_5i
c.(180+1_181-1)_(294+1_295-1)del
r.?
p.?
ACMG
pathogenic (recessive)
g.(22239834_22242642)_(22242757_22247529)del
g.(22218288_22221096)_(22218288_22221096)del
del ex5, NM_001142649.1 del ex5
-
ANO5_000318
ACMG PVS1, PM2, PP3
PubMed: Marinakis 2021
-
-
Germline
-
-
-
-
-
Jan Traeger-Synodinos
-?/., ?/.
3
5
c.185A>G
r.(?)
p.(Gln62Arg)
-
likely benign, VUS
g.22242647A>G
g.22221101A>G
ANO5(NM_001142649.1):c.182A>G (p.(Gln61Arg)), ANO5(NM_213599.2):c.185A>G (p.Q62R)
-
ANO5_000138
no second variant, VKGL data sharing initiative Nederland
PubMed: Nallamilli 2018
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Madhuri Hegde
,
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
+/., +?/., ?/.
283
5
c.191dup
r.(?), r.191dup
p.(Asn64Lysfs*15), p.(Asn64LysfsTer15), p.Asn64Lysfs*15
ACMG
likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive), VUS
g.22242653dup
g.22221107dup
191dupA, 192dupA, 193dupA, c.184_185insA, c.191dup,
1 more item
-
ANO5_000004, ANO5_000040, ANO5_000084
ACMG grading: PVS1, PS3, ACMG PM2, PM3, PP3, PP4_mod, ACMG PVS1, PP5, PP3, from website {DBsub-Emory},
10 more items
Bolduc ASHG2011 A114, Penttila WMS2010 P1.26,
PubMed: Penttila 2012
,
PubMed: Bolduc 2010
,
OMIM:var0004
,
24 more items
-
rs137854521
,
rs575136178
CLASSIFICATION record, Germline, Germline/De novo (untested), Unknown
yes
1/100, 18/1001 cases, 2/210
-
-
-
Johan den Dunnen
,
Ieke Ginjaar
,
Madhuri Hegde
,
Tom Winder
,
Andreas Laner
,
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
Lidia Gonzalez-Quereda
,
VKGL-NL_Groningen
,
VKGL-NL_Utrecht
,
VKGL-NL_VUmc
,
VKGL-NL_AMC
?/.
1
-
c.191dupA
r.(?)
p.(Asn64Lysfs*15)
-
likely pathogenic
g.22242653dupA
-
-
-
ANO5_000317
-
https://pubmed.ncbi.nlm.nih.gov/35741838/
-
-
Unknown
-
-
-
-
-
Gisela Gaina
?/.
1
5
c.217T>C
r.(?)
p.(Phe73Leu)
-
VUS
g.22242679T>C
g.22221133T>C
-
-
ANO5_000139
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/., +?/.
3
5
c.220C>T
r.(?)
p.(Arg74*)
-
likely pathogenic, pathogenic, pathogenic (recessive)
g.22242682C>T
g.22221136C>T
-
-
ANO5_000066
combination of variants not reported
PubMed: Magri 2015
,
PubMed: Magrib 2012
,
PubMed: Topf 2020
-
-
Germline
-
2/1001 cases
-
-
-
Johan den Dunnen
?/.
2
5
c.223G>C
r.(?)
p.(Asp75His)
-
VUS
g.22242685G>C
g.22221139G>C
-
-
ANO5_000140
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/., +?/.
15
5
c.242A>G
r.(?)
p.(Asp81Gly)
ACMG
likely pathogenic, pathogenic
g.22242704A>G
g.22221158A>G
D81G
-
ANO5_000056
ACMG grading: PS3, PS4, PM3, PP3; additional variants in BAG3, FLNC, CHRNE, CACNA1S, TTN x2,
2 more items
Fichna ESHG2016 P10.20,
PubMed: Fichna 2018
,
PubMed: Nallamilli 2018
,
PubMed: Penttila 2012
,
1 more item
-
-
Germline
yes
1/82 cases LGMD
-
-
-
Johan den Dunnen
,
Madhuri Hegde
-?/.
2
-
c.259G>A
r.(?)
p.(Val87Ile)
-
likely benign
g.22242721G>A
g.22221175G>A
ANO5(NM_001142649.1):c.256G>A (p.(Val86Ile)), ANO5(NM_213599.2):c.259G>A (p.V87I)
-
ANO5_000236
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Utrecht
?/.
1
-
c.265G>T
r.(?)
p.(Asp89Tyr)
-
VUS
g.22242727G>T
g.22221181G>T
ANO5(NM_213599.3):c.265G>T (p.D89Y)
-
ANO5_000085
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-/.
6
5
c.267T>C
r.(?)
p.(=), p.(Asp89=)
-
benign
g.22242729T>C
g.22221183T>C
D89D,
1 more item
-
ANO5_000007
from website {DBsub-Emory}, VKGL data sharing initiative Nederland
-
-
rs4312063
CLASSIFICATION record, Germline
-
-
-
-
-
Madhuri Hegde
,
VKGL-NL_Groningen
,
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
,
VKGL-NL_VUmc
,
VKGL-NL_AMC
+/.
1
-
c.274A>T
r.(?)
p.(Lys92*)
-
pathogenic (recessive)
g.22242736A>T
g.22221190A>T
-
-
ANO5_000273
-
PubMed: Ten Dam 2019
-
-
Germline
-
-
-
-
-
Ieke Ginjaar
?/.
1
5
c.279C>T
r.(?)
p.(=)
-
VUS
g.22242741C>T
g.22221195C>T
-
-
ANO5_000141
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
-?/.
1
5
c.283G>A
r.(?)
p.(Glu95Lys)
-
likely benign
g.22242745G>A
g.22221199G>A
Glu95Lys
-
ANO5_000070
-
-
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/., ?/.
2
5
c.294G>A
r.(?)
p.(=), p.(Ala98=)
-
likely benign, VUS
g.22242756G>A
g.22221210G>A
ANO5(NM_213599.3):c.294G>A (p.A98=)
-
ANO5_000086
no second variant, VKGL data sharing initiative Nederland
PubMed: Nallamilli 2018
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Madhuri Hegde
,
VKGL-NL_Groningen
?/.
1
5i
c.295-325A>G
r.(=)
p.(=)
-
VUS
g.22247205A>G
g.22225659A>G
-
-
ANO5_000011
-
-
-
rs4509753
Germline
-
0.35-0.94
-
-
-
Johan den Dunnen
-/., -?/.
3
-
c.295-8dup
r.(=)
p.(=)
-
benign, likely benign
g.22247522dup
g.22225976dup
ANO5(NM_213599.3):c.295-8dup, ANO5(NM_213599.3):c.295-8dupT
-
ANO5_000088
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Groningen
,
VKGL-NL_AMC
+/.
1
5i
c.295-1G>A
r.spl
p.?
-
pathogenic
g.22247529G>A
g.22225983G>A
-
-
ANO5_000065
-
PubMed: Schessl 2012
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
6
c.304_308del
r.(?)
p.(Lys102Valfs*2)
-
pathogenic
g.22247539_22247543del
g.22225993_22225997del
304_308delAAAGA
-
ANO5_000142
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
1
-
c.317C>A
r.(?)
p.(Thr106Asn)
-
VUS
g.22247552C>A
g.22226006C>A
ANO5(NM_213599.3):c.317C>A (p.T106N)
-
ANO5_000089
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+/.
2
6
c.323T>G
r.(?)
p.(Leu108Arg)
-
pathogenic
g.22247558T>G
g.22226012T>G
-
-
ANO5_000050
-
PubMed: Sarkozy 2012
,
PubMed: Wahbi 2012
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
6
c.331A>C
r.(?)
p.(Thr111Pro)
-
VUS
g.22247566A>C
g.22226020A>C
-
-
ANO5_000143
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+?/.
1
6
c.352del
r.(?)
p.(Glu118Lysfs*23)
-
pathogenic (recessive)
g.22247587del
g.22226041del
-
-
ANO5_000075
-
PubMed: Punetha 2016
-
-
Unknown
-
-
-
-
-
Jaya Punetha
+?/.
1
6i
c.363+2T>C
r.(spl?)
p.?
-
likely pathogenic
g.22247600T>C
g.22226054T>C
-
-
ANO5_000074
-
-
-
-
Germline
-
-
-
-
-
Tom Winder
?/.
2
6i
c.363+4A>G
r.(spl?), r.spl?
p.(?), p.?
-
VUS
g.22247602A>G
g.22226056A>G
-
-
ANO5_000028
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
,
Tom Winder
?/.
1
-
c.363+269_363+270del
r.(?)
p.(=)
ACMG
VUS
g.22247867_22247868del
g.22226321_22226322del
-
-
ANO5_000340
-
-
-
-
Germline
-
-
-
-
-
Camille Verebi
-/., ?/.
3
6i
c.364-8del
r.(=)
p.(=)
-
benign, VUS
g.22248840del
g.22227294del
ANO5(NM_213599.3):c.364-8delT
-
ANO5_000045, ANO5_000090, ANO5_000091
from website {DBsub-Emory}, VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Madhuri Hegde
,
VKGL-NL_Groningen
,
VKGL-NL_AMC
+?/.
1
-
c.364-2A>G
r.spl
p.?
-
likely pathogenic
g.22248846A>G
g.22227300A>G
-
-
ANO5_000280
combination of variants not reported
PubMed: Topf 2020
-
-
Germline
-
2/1001 cases
-
-
-
Johan den Dunnen
+/.
1
-
c.364-1G>A
r.spl?
p.?
-
pathogenic
g.22248847G>A
g.22227301G>A
-
-
ANO5_000237
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
2
-
c.368C>T
r.(?)
p.(Ser123Leu)
-
pathogenic (recessive)
g.22248852C>T
g.22227306C>T
-
-
ANO5_000229
-
PubMed: Ten Dam 2019
-
-
Germline
-
-
-
-
-
Ieke Ginjaar
+/., ?/.
2
-
c.377G>T
r.(?)
p.(Gly126Val)
-
pathogenic (recessive), VUS
g.22248861G>T
g.22227315G>T
-
-
ANO5_000125
VKGL data sharing initiative Nederland
PubMed: Ten Dam 2019
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Ieke Ginjaar
,
VKGL-NL_Nijmegen
?/.
1
7
c.385T>C
r.(?)
p.(Tyr129His)
-
VUS
g.22248869T>C
g.22227323T>C
-
-
ANO5_000144
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+?/.
1
-
c.395A>T
r.(?)
p.(Lys132Met)
-
likely pathogenic
g.22248879A>T
g.22227333A>T
-
-
ANO5_000281
combination of variants not reported
PubMed: Topf 2020
-
-
Germline
-
1/1001 cases
-
-
-
Johan den Dunnen
+/.
3
7
c.397A>T
r.(?)
p.(Ile133Phe)
-
pathogenic, pathogenic (recessive)
g.22248881A>T
g.22227335A>T
-
-
ANO5_000067
-
PubMed: Magri 2015
,
PubMed: Magrib 2012
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
-
c.412G>T
r.(?)
p.(Glu138Ter)
-
pathogenic
g.22248896G>T
g.22227350G>T
-
-
ANO5_000331
possible di-genic inheritance
PubMed: Cavdarli 2023
-
rs1554924356
Germline, Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
?/.
1
7
c.415G>T
r.(?)
p.(Val139Leu)
-
VUS
g.22248899G>T
g.22227353G>T
-
-
ANO5_000145
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
1
7
c.424A>T
r.(?)
p.(Thr142Ser)
-
VUS
g.22248908A>T
g.22227362A>T
-
-
ANO5_000146
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
-?/.
1
-
c.429T>C
r.(?)
p.(Tyr143=)
-
likely benign
g.22248913T>C
g.22227367T>C
ANO5(NM_213599.2):c.429T>C (p.Y143=)
-
ANO5_000239
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.443G>A
r.(?)
p.(Gly148Glu)
-
likely benign
g.22248927G>A
-
ANO5(NM_213599.2):c.443G>A (p.(Gly148Glu))
-
ANO5_000333
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
-
c.463G>A
r.(?)
p.(Glu155Lys)
-
VUS
g.22248947G>A
g.22227401G>A
ANO5(NM_213599.3):c.463G>A (p.E155K)
-
ANO5_000257
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-?/.
1
-
c.468T>C
r.(?)
p.(Ser156=)
-
likely benign
g.22248952T>C
-
ANO5(NM_213599.2):c.468T>C (p.S156=)
-
ANO5_000309
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.478C>T
r.(?)
p.(Arg160Cys)
-
likely benign
g.22248962C>T
-
ANO5(NM_213599.2):c.478C>T (p.R160C)
-
ANO5_000298
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
7
c.479G>A
r.(?)
p.(Arg160His)
-
VUS
g.22248963G>A
g.22227417G>A
-
-
ANO5_000147
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
1
7
c.482C>T
r.(?)
p.(Pro161Leu)
-
VUS
g.22248966C>T
g.22227420C>T
-
-
ANO5_000148
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
-?/., ?/.
2
7
c.553G>C
r.(?)
p.(Glu185Gln)
-
likely benign, VUS
g.22249037G>C
g.22227491G>C
ANO5(NM_213599.3):c.553G>C (p.E185Q)
-
ANO5_000092
no second variant, VKGL data sharing initiative Nederland
PubMed: Nallamilli 2018
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Madhuri Hegde
,
VKGL-NL_Groningen
?/.
1
7
c.579A>T
r.(?)
p.(Arg193Ser)
-
VUS
g.22249063A>T
g.22227517A>T
-
-
ANO5_000149
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
-?/.
1
-
c.581A>G
r.(?)
p.(His194Arg)
-
likely benign
g.22249065A>G
g.22227519A>G
ANO5(NM_213599.2):c.581A>G (p.H194R)
-
ANO5_000251
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/.
1
-
c.583C>T
r.(?)
p.(Arg195Trp)
-
likely pathogenic
g.22249067C>T
-
-
-
ANO5_000296
-
-
-
rs149040903
CLASSIFICATION record
-
-
-
-
-
MobiDetails
-/., -?/.
3
-
c.604G>A
r.(?)
p.(Glu202Lys)
-
benign, likely benign
g.22249088G>A
g.22227542G>A
1 more item
-
ANO5_000093
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Utrecht
,
VKGL-NL_VUmc
-/., -?/.
2
-
c.616A>G
r.(?)
p.(Thr206Ala)
-
benign, likely benign
g.22249100A>G
g.22227554A>G
ANO5(NM_001142649.1):c.613A>G (p.(Thr205Ala)), ANO5(NM_213599.3):c.616A>G (p.T206A)
-
ANO5_000094
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Groningen
?/.
1
-
c.619T>G
r.(?)
p.(Phe207Val)
-
VUS
g.22249103T>G
-
ANO5(NM_213599.3):c.619T>G (p.F207V)
-
ANO5_000299
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
?/.
1
7i
c.648+5G>C
r.spl?
p.?
-
VUS
g.22249137G>C
g.22227591G>C
-
-
ANO5_000150
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+?/.
1
7i
c.648+6T>A
r.(spl?)
p.(?)
-
likely pathogenic
g.22249138T>A
g.22227592T>A
-
-
ANO5_000041
-
-
-
-
Germline
-
-
-
-
-
Tom Winder
?/.
1
7i
c.649-166A>G
r.(=)
p.(=)
-
VUS
g.22257543A>G
g.22235997A>G
-
-
ANO5_000013
-
-
-
rs4534588
Germline
-
0-0.53
-
-
-
Johan den Dunnen
?/.
1
7i
c.649-114T>G
r.(=)
p.(=)
-
VUS
g.22257595T>G
g.22236049T>G
-
-
ANO5_000012
-
-
-
rs11820954
Germline
-
0-0.55
-
-
-
Johan den Dunnen
+?/.
1
-
c.649-2A>G
r.spl
p.?
-
likely pathogenic
g.22257707A>G
g.22236161A>G
-
-
ANO5_000282
combination of variants not reported
PubMed: Topf 2020
-
-
Germline
-
1/1001 cases
-
-
-
Johan den Dunnen
?/.
1
8
c.653A>G
r.(?)
p.(Tyr218Cys)
-
VUS
g.22257713A>G
g.22236167A>G
-
-
ANO5_000151
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
1
8
c.656A>G
r.(?)
p.(Tyr219Cys)
-
VUS
g.22257716A>G
g.22236170A>G
-
-
ANO5_000152
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
1
8
c.664T>C
r.(?)
p.(Ser222Pro)
-
VUS
g.22257724T>C
g.22236178T>C
-
-
ANO5_000153
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
2
-
c.674C>T
r.(?)
p.(Pro225Leu)
-
VUS
g.22257734C>T
g.22236188C>T
ANO5(NM_213599.2):c.674C>T (p.(Pro225Leu)), ANO5(NM_213599.3):c.674C>T (p.P225L)
-
ANO5_000095
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Groningen
-?/.
2
-
c.680G>C
r.(?)
p.(Gly227Ala)
-
likely benign
g.22257740G>C
g.22236194G>C
ANO5(NM_001142649.1):c.677G>C (p.(Gly226Ala)), ANO5(NM_213599.2):c.680G>C (p.G227A)
-
ANO5_000240
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Utrecht
?/.
3
8
c.689A>G
r.(?)
p.(Asp230Gly)
-
VUS
g.22257749A>G
g.22236203A>G
-
-
ANO5_000154
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/., +?/., ?/.
49
8
c.692G>T
r.(?)
p.(Gly231Val)
ACMG
likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive), VUS
g.22257752G>T
g.22236206G>T
NM_00114264:689G>T (Gly230Val),
1 more item
-
ANO5_000005
ACMG PM2 PP3 PP5 (strong), ACMG PM2 PP3 PP5_S, ACMG PP3, PP2, PP5, PM3, no second variant,
4 more items
PubMed: Bolduc 2010
,
OMIM:var0005
,
PubMed: Bruels 2022
,
PubMed: Cavdarli 2023
,
PubMed: Liewluck 2013
,
12 more items
-
rs137854523
CLASSIFICATION record, Germline, Germline/De novo (untested)
-
2/1001 cases
-
-
-
Johan den Dunnen
,
Ieke Ginjaar
,
Madhuri Hegde
,
Tom Winder
,
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
Jan Traeger-Synodinos
,
VKGL-NL_Groningen
,
VKGL-NL_VUmc
,
Camille Verebi
+?/.
1
-
c.709A>G
r.(?)
p.(Ile237Val)
ACMG
likely pathogenic (recessive)
g.22257769A>G
g.22236223A>G
-
-
ANO5_000319
ACMG PM2, PM3, PP2, PP3
PubMed: Marinakis 2021
-
-
Germline
-
-
-
-
-
Jan Traeger-Synodinos
-?/.
1
-
c.720G>T
r.(?)
p.(Leu240=)
-
likely benign
g.22257780G>T
g.22236234G>T
ANO5(NM_213599.3):c.720G>T (p.L240=)
-
ANO5_000096
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-?/.
1
-
c.721C>T
r.(?)
p.(Leu241=)
-
likely benign
g.22257781C>T
-
ANO5(NM_213599.3):c.721C>T (p.L241=)
-
ANO5_000329
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
+?/., ?/.
4
8
c.749A>G
r.(?)
p.(Tyr250Cys)
-
likely pathogenic, VUS
g.22257809A>G
g.22236263A>G
-
-
ANO5_000155
combination of variants not reported
PubMed: Nallamilli 2018
,
PubMed: Topf 2020
-
-
Germline
-
1/1001 cases
-
-
-
Johan den Dunnen
,
Madhuri Hegde
+/.
2
8i
c.762+1G>A
r.spl
p.?
-
pathogenic
g.22257823G>A
g.22236277G>A
-
-
ANO5_000156
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
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