Global Variome shared LOVD
FKRP (fukutin related protein)
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Curator:
Johan den Dunnen
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Unique variants in the FKRP gene
This database is one of the gene variant databases from the
Leiden Muscular Dystrophy pages
The variants shown are described using the NM_024301.4 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'
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314 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
-
826C>A
r.(?)
p.(Leu276Ile)
-
pathogenic (recessive)
g.47259533C>A
g.46756276C>A
-
-
FKRP_000001
-
PubMed: Stehlikova 2014
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
16
1
c.-621C>G
r.(=)
p.(=)
-
benign
g.47248979C>G
g.46745722C>G
-
-
FKRP_000033
control chromosomes
PubMed: Walter
,
PubMed: Walter
,
PubMed: Frosk
-
-
Germline
-
41/84
BfaI-
-
-
Rolf Stucka
-?/., ?/.
2
_1
c.-304G>C
r.(=), r.(?)
p.(=)
-
likely benign, VUS
g.47249296G>C
g.46746039G>C
-
-
FKRP_000171
VKGL data sharing initiative Nederland
-
-
rs12984041
CLASSIFICATION record, Germline
-
-
-
-
-
Andreas Laner
,
VKGL-NL_Nijmegen
?/.
1
-
c.-302C>A
r.(?)
p.(=)
-
VUS
g.47249298C>A
g.46746041C>A
-
-
STRN4_000001
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/.
1
-
c.-253+9G>A
r.(=)
p.(=)
-
likely benign
g.47249356G>A
-
FKRP(NM_024301.5):c.-253+9G>A
-
FKRP_000316
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
1
-
c.-253+17_-253+26dup
r.(=)
p.(=)
-
likely benign
g.47249364_47249373dup
g.46746107_46746116dup
FKRP(NM_001039885.2):c.-305+4_-305+5insGGCCGGGCCG (p.(=))
-
FKRP_000241
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
1
-
c.-253+22_-253+26del
r.(=)
p.(=)
-
likely benign
g.47249369_47249373del
g.46746112_46746116del
-
-
FKRP_000242
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/., ?/.
3
1i
c.-253+22_-253+26dup
r.(=), r.(?)
p.(=)
-
likely benign, VUS
g.47249369_47249373dup
g.46746112_46746116dup
FKRP(NM_024301.5):c.-253+22_-253+26dupCCGGG, STRN4(NM_001039877.1):c.282+54_282+55insCGGCC (p.(=))
-
FKRP_000162
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Andreas Laner
,
VKGL-NL_Leiden
,
VKGL-NL_Utrecht
?/.
1
-
c.-253+298_-253+300dup
r.(=)
p.(=)
-
VUS
g.47249645_47249647dup
g.46746388_46746390dup
STRN4(NM_001039877.1):c.49_50insCCT (p.?)
-
FKRP_000173
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-/.
1
3
c.-190-84C>T
r.(?)
p.(=)
-
benign
g.47251688C>T
g.46748431C>T
-
-
FKRP_000049
-
rs1644332
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
1
3
c.-44C>T
r.(?)
p.(=)
-
benign
g.47251918C>T
g.46748661C>T
-
-
FKRP_000046
-
rs1693995
-
-
Germline
-
0.01
-
-
-
Johan den Dunnen
-?/.
1
-
c.-40+8C>G
r.(=)
p.(=)
-
likely benign
g.47251930C>G
g.46748673C>G
FKRP(NM_001039885.2):c.-84C>G (p.(=))
-
FKRP_000231
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-/.
4
4
c.-34C>T
r.(?), r.-34c>u
p.(=), p.=
-
benign
g.47258674C>T
g.46755417C>T
-
-
FKRP_000008
VKGL data sharing initiative Nederland
from website {DBsub-Emory},
PubMed: Brockington 2001
-
rs16980701
CLASSIFICATION record, Germline, Unknown
-
0.25
-
-
-
Johan den Dunnen
,
Madhuri Hegde
,
Andreas Laner
,
VKGL-NL_Nijmegen
+/., ?/.
11
1_4
c.?
r.?
p.?
-
pathogenic (recessive), VUS
g.?
-
-
-
FKRP_000000
unknown variant 2nd chromosome
-
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Ieke Ginjaar
+/.
6
4
c.1A>G
r.(?)
p.0?
-
pathogenic, pathogenic (recessive)
g.47258708A>G
g.46755451A>G
Met1Val
-
FKRP_000034
homozygosity mapping, no second variant
PubMed: Mercuri
,
PubMed: Nallamilli 2018
,
PubMed: VanReeuwijk 2010
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Madhuri Hegde
+/.
1
4
c.3G>T
r.(?)
p.0?
-
pathogenic (recessive)
g.47258710G>T
g.46755453G>T
-
-
FKRP_000065
-
Yamamoto WMS2005 G.P.2.08
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/., ?/.
3
4
c.11C>G
r.(?)
p.(Thr4Ser)
-
likely benign, VUS
g.47258718C>G
g.46755461C>G
FKRP(NM_001039885.2):c.11C>G (p.T4S, p.(Thr4Ser))
-
FKRP_000175
no second variant, VKGL data sharing initiative Nederland
PubMed: Nallamilli 2018
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Madhuri Hegde
,
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
+/.
1
-
c.37G>T
r.(?)
p.(Ala13Ser)
-
pathogenic (recessive)
g.47258744G>T
g.46755487G>T
-
-
FKRP_000271
-
PubMed: Song 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
4
c.43A>C
r.(?)
p.(Thr15Pro)
-
VUS
g.47258750A>C
g.46755493A>C
-
-
FKRP_000194
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
1
4
c.54T>A
r.(?)
p.(=)
-
VUS
g.47258761T>A
g.46755504T>A
-
-
FKRP_000195
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
1
-
c.61C>T
r.(?)
p.(Leu21Phe)
-
VUS
g.47258768C>T
-
FKRP(NM_001039885.3):c.61C>T (p.L21F)
-
FKRP_000325
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/.
1
4
c.63C>A
r.(?)
p.(=)
-
benign
g.47258770C>A
g.46755513C>A
-
-
FKRP_000020
-
PubMed: Brockington 2001
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
1
4
c.63C>T
r.(?)
p.(=)
-
benign
g.47258770C>T
g.46755513C>T
-
-
FKRP_000094
-
-
-
-
Germline
-
-
-
-
-
Ieke Ginjaar
+?/.
1
-
c.74C>A
r.(?)
p.(Ser25*)
-
likely pathogenic (recessive)
g.47258781C>A
g.46755524C>A
-
-
FKRP_000248
-
PubMed: Punetha 2016
-
-
Germline/De novo (untested)
-
1/94 cases
-
-
-
Johan den Dunnen
+?/.
1
-
c.76_77del
r.(?)
p.(Trp26Alafs*6)
-
likely pathogenic (recessive)
g.47258783_47258784del
g.46755526_46755527del
-
-
FKRP_000249
-
PubMed: Punetha 2016
-
-
Germline/De novo (untested)
-
1/94 cases
-
-
-
Johan den Dunnen
-?/., ?/.
3
4
c.82C>G
r.(?)
p.(Gln28Glu)
-
likely benign, VUS
g.47258789C>G
g.46755532C>G
FKRP(NM_001039885.2):c.82C>G (p.(Gln28Glu))
-
FKRP_000176
no second variant, VKGL data sharing initiative Nederland
PubMed: Nallamilli 2018
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Madhuri Hegde
,
VKGL-NL_Leiden
,
VKGL-NL_Nijmegen
?/.
1
4
c.85C>T
r.(?)
p.(His29Tyr)
-
VUS
g.47258792C>T
g.46755535C>T
-
-
FKRP_000196
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
-/.
1
-
c.114G>T
r.(?)
p.(Gly38=)
-
benign
g.47258821G>T
g.46755564G>T
FKRP(NM_001039885.3):c.114G>T (p.G38=)
-
FKRP_000243
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/., -/., -?/-?
11
4
c.135C>T
r.(?), r.135c>u
p.(=), p.(Ala45=), p.=
-
benign, likely benign, pathogenic (recessive)
g.47258842C>T
g.46755585C>T
FKRP(NM_001039885.2):c.135C>T (p.A45=), FKRP(NM_001039885.3):c.135C>T (p.A45=)
-
FKRP_000021
in controls and LGMD patients, shared alleles from D19S412 to D19S540,
1 more item
from website {DBsub-Emory}, Yamamoto WMS2005 G.P.2.08,
PubMed: Brockington 2001
,
PubMed: de Paula 2003
,
1 more item
-
rs2287717
CLASSIFICATION record, Germline, Unknown
-
0.09
NgoMIV-
-
-
Johan den Dunnen
,
Madhuri Hegde
,
Vikki Stefans
,
Andreas Laner
,
VKGL-NL_Rotterdam
,
VKGL-NL_Nijmegen
,
VKGL-NL_AMC
+?/.
1
-
c.137del
r.(?)
p.(Gly46AlafsTer22)
-
likely pathogenic
g.47258844del
-
FKRP(NM_001039885.3):c.137delG (p.G46Afs*22)
-
FKRP_000232
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
?/.
1
4
c.143G>C
r.(?)
p.(Arg48Pro)
-
VUS
g.47258850G>C
g.46755593G>C
-
-
FKRP_000172
-
PubMed: Navarro-Cobos 2017
-
-
Germline
?
-
-
-
-
Miguel Angel Alcántara-Ortigoza
?/.
1
4
c.151G>A
r.(?)
p.(Val51Ile)
-
VUS
g.47258858G>A
g.46755601G>A
-
-
FKRP_000197
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/., ?/.
4
-
c.151G>T
r.(?)
p.(Val51Phe)
-
pathogenic (recessive), VUS
g.47258858G>T
g.46755601G>T
-
-
FKRP_000272
-
PubMed: Liang 2020
,
PubMed: Song 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
4
c.154_158dup
r.(?)
p.(Arg54Trpfs*14)
-
pathogenic (recessive)
g.47258861_47258865dup
g.46755604_46755608dup
-
-
FKRP_000035
-
PubMed: Mercuri
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
2
4
c.155T>A
r.(?)
p.(Leu52Gln)
-
VUS
g.47258862T>A
g.46755605T>A
-
-
FKRP_000141
-
-
-
-
Germline
-
-
-
-
-
Tom Winder
+/., +?/.
4
4
c.158_162dup
r.(?)
p.(Glu55Cysfs*15)
-
likely pathogenic (recessive), pathogenic (recessive)
g.47258865_47258869dup
g.46755608_46755612dup
158_162dupTGCGG
-
FKRP_000039
-
Sveen WMS2005 L.P.3.06
-
-
Germline
-
-
-
-
-
Marianne Schwartz
,
Akanchha Kesari
,
Tom Winder
+/.
1
-
c.160C>G
r.(?)
p.(Arg54Gly)
-
pathogenic (recessive)
g.47258867C>G
g.46755610C>G
-
-
FKRP_000273
-
PubMed: Song 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
4
4
c.160C>T
r.(?)
p.(Arg54Trp)
-
pathogenic, pathogenic (recessive)
g.47258867C>T
g.46755610C>T
-
-
FKRP_000084
VKGL data sharing initiative Nederland
PubMed: Harel 2004
,
OMIM:var0011
,
PubMed: Song 2021
-
rs28937905
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Nijmegen
+/.
1
-
c.161G>A
r.(?)
p.(Arg54Gln)
-
pathogenic (recessive)
g.47258868G>A
g.46755611G>A
-
-
FKRP_000274
-
PubMed: Song 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
3
4
c.162_165dup
r.(?)
p.(Phe56Glyfs*6)
-
pathogenic (recessive)
g.47258869_47258872dup
g.46755612_46755615dup
162_165dupGGAG
-
FKRP_000013
-
PubMed: Brockington
,
PubMed: Mercuri
,
PubMed: Torelli 2005
,
PubMed: Peat 2008
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Tom Winder
+/., +?/.
4
4
c.163G>C
r.(?)
p.(Glu55Gln)
-
likely pathogenic, pathogenic (recessive)
g.47258870G>C
g.46755613G>C
E55Q
-
FKRP_000091
-
Freixas ESHG2006 P0925,
PubMed: Gonzalez-Quereda 2020
,
PubMed: Wahbi 2008
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
2
4
c.169G>A
r.(?)
p.(Glu57Lys), p.Glu57Lys
ACMG
VUS
g.47258876G>A
g.46755619G>A
-
-
FKRP_000191
no second variant
PubMed: Nallamilli 2018
-
rs773024545
Germline
-
-
-
-
-
Madhuri Hegde
,
Andreas Laner
-/., ?/.
6
4
c.192C>T
r.(?)
p.(=), p.(Pro64=)
-
benign, VUS
g.47258899C>T
g.46755642C>T
FKRP(NM_001039885.2):c.192C>T (p.P64=), FKRP(NM_001039885.3):c.192C>T (p.P64=)
-
FKRP_000088
VKGL data sharing initiative Nederland
PubMed: de Paula 2003
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
Akanchha Kesari
,
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
-/.
1
4
c.201A>G
r.(?)
p.(=)
-
benign
g.47258908A>G
g.46755651A>G
-
-
FKRP_000079
-
PubMed: Boito 2005
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
4
c.206C>A
r.(?)
p.(Ser69Tyr)
-
likely benign
g.47258913C>A
g.46755656C>A
-
-
FKRP_000123
not in 212 control chromosomes
PubMed: Bouchet 2007
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., +?/.
4
4
c.206_208del
r.(?)
p.(Ser69del)
ACMG
likely pathogenic (recessive), pathogenic (recessive)
g.47258913_47258915del
g.46755656_46755658del
c.204_206delCTC, c.206_208delCCT
-
FKRP_000275
ACMG PM1, PM2, PM4, PP1
PubMed: Mao 2025
,
PubMed: Song 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.208T>A
r.(?)
p.(Phe70Ile)
-
pathogenic (recessive)
g.47258915T>A
g.46755658T>A
-
-
FKRP_000276
-
PubMed: Song 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
1
4
c.211C>G
r.(?)
p.(Leu71Val)
-
benign
g.47258918C>G
g.46755661C>G
-
-
FKRP_000066
-
Yamamoto WMS2005 G.P.2.08
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
4
c.214C>T
r.(?)
p.(Gln72*)
-
pathogenic (recessive)
g.47258921C>T
g.46755664C>T
-
-
FKRP_000138
-
-
-
-
Germline
-
-
-
-
-
Wolfram Kress
+/., -/., -?/.
9
4
c.235G>A
r.(?)
p.(Val79Met)
-
benign, likely benign, pathogenic (recessive)
g.47258942G>A
g.46755685G>A
FKRP(NM_001039885.2):c.235G>A (p.(Val79Met)), FKRP(NM_001039885.3):c.235G>A (p.V79M)
-
FKRP_000063
recombined allele from mother, VKGL data sharing initiative Nederland
from website {DBsub-Emory},
PubMed: de Paula 2003
,
PubMed: Vieira 2006
;
PubMed: Yamamoto 2008
,
2 more items
-
-
CLASSIFICATION record, Germline, Unknown
-
-
-
-
-
Johan den Dunnen
,
Madhuri Hegde
,
Tom Winder
,
VKGL-NL_Leiden
,
VKGL-NL_AMC
+/., ?/.
3
4
c.242_244del
r.(?)
p.(Val81del)
-
pathogenic (recessive), VUS
g.47258949_47258951del
g.46755692_46755694del
241_243delGTG, delTGG
-
FKRP_000110
no segregation analysis
PubMed: Wahbi 2008
,
PubMed: Westra 2019
-
-
Germline, Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
,
Tom Winder
+/., -/., -?/., ?/.
9
4
c.249C>T
r.(?)
p.(=), p.(Ala83=)
-
benign, likely benign, pathogenic (recessive), VUS
g.47258956C>T
g.46755699C>T
FKRP(NM_001039885.2):c.249C>T (p.A83=, p.(Ala83=)), FKRP(NM_001039885.3):c.249C>T (p.A83=)
-
FKRP_000022
not in 104 normal chromosomes, VKGL data sharing initiative Nederland
from website {DBsub-Emory}, Yamamoto WMS2005 G.P.2.08,
PubMed: Brockington 2001
,
PubMed: de Paula 2003
-
-
CLASSIFICATION record, Germline, Unknown
-
-
-
-
-
Johan den Dunnen
,
Madhuri Hegde
,
Andreas Laner
,
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
+/.
1
-
c.250G>T
r.(?)
p.(Asp84Tyr)
-
pathogenic (recessive)
g.47258957G>T
g.46755700G>T
-
-
FKRP_000277
-
PubMed: Song 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.253+2T>C
r.spl
p.?
ACMG
pathogenic
g.47258962T>C
-
-
-
FKRP_000187
1 more item
PubMed: Fichna 2018
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.263A>T
r.(?)
p.(Tyr88Phe)
-
likely pathogenic
g.47258970A>T
g.46755713A>T
-
-
FKRP_000303
-
PubMed: Liang 2020
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., ?/.
3
4
c.265C>G
r.(?)
p.(Pro89Ala)
-
pathogenic (recessive), VUS
g.47258972C>G
g.46755715C>G
-
-
FKRP_000115
-
PubMed: de Paula 2003
,
PubMed: Vieira 2006
;
PubMed: Yamamoto 2008
,
PubMed: Song 2021
,
1 more item
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.266C>A
r.(?)
p.(Pro89Gln)
-
VUS
g.47258973C>A
g.46755716C>A
-
-
FKRP_000313
no segregation analysis
PubMed: Westra 2019
-
-
Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
+/.
1
4
c.266C>G
r.(?)
p.(Pro89Arg)
-
pathogenic (recessive)
g.47258973C>G
g.46755716C>G
-
-
FKRP_000057
not in 100 control chromosomes
PubMed: Matsumoto 2005
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
4
c.266C>T
r.(?)
p.(Pro89Leu)
-
pathogenic (recessive)
g.47258973C>T
g.46755716C>T
P89L
-
FKRP_000040
-
PubMed: Quijano-Roy 2006
,
PubMed: Wahbi 2008
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.271_322del
r.(?)
p.(Leu91TrpfsTer21)
-
pathogenic (recessive)
g.47258978_47259029del
g.46755721_46755772del
c.264_315del
-
FKRP_000278
-
PubMed: Song 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.272del
r.(?)
p.(Leu91ArgfsTer38)
-
pathogenic (recessive)
g.47258979del
g.46755722del
c.272delT
-
FKRP_000279
-
PubMed: Song 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
4
c.275C>G
r.(?)
p.(Ala92Gly)
-
pathogenic (recessive)
g.47258982C>G
g.46755725C>G
-
-
FKRP_000067
not in 104 normal chromosomes
Yamamoto WMS2005 G.P.2.08
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., +?/.
3
4
c.278T>C
r.(?)
p.(Leu93Pro)
ACMG
likely pathogenic, pathogenic (recessive)
g.47258985T>C
g.46755728T>C
L93P
-
FKRP_000055
ACMG: 4 (PM2, PM3, PP2, PP3); additional variants in CAPN3, DMD, NEB, SYNE1 x2, CCDC78, TTN x4
PubMed: Fichna 2018
,
PubMed: Wahbi 2008
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Lab Müller-Reible
+/.
1
4
c.280C>T
r.(?)
p.(Pro94Ser)
-
pathogenic (recessive)
g.47258987C>T
g.46755730C>T
Pro104Ser
-
FKRP_000121
not in 320 control chromosomes
PubMed: Guglieri 2007
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
4
c.283C>T
r.(?)
p.(Arg95Cys)
-
VUS
g.47258990C>T
g.46755733C>T
-
-
FKRP_000113
father carrier
PubMed: Yis 2010
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.283del
r.(?)
p.(Arg95AlafsTer34)
-
likely pathogenic
g.47258990del
g.46755733del
-
-
FKRP_000304
-
PubMed: Gonzalez-Quereda 2020
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
2
-
c.292A>T
r.(?)
p.(Asn98Tyr)
-
VUS
g.47258999A>T
g.46755742A>T
-
-
FKRP_000311
-
PubMed: Cavdarli 2023
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.295G>T
r.(?)
p.(Val99Leu)
-
VUS
g.47259002G>T
-
FKRP(NM_024301.5):c.295G>T (p.(Val99Leu))
-
FKRP_000330
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
1
-
c.306G>A
r.(?)
p.(Ala102=)
-
likely benign
g.47259013G>A
g.46755756G>A
FKRP(NM_001039885.2):c.306G>A (p.A102=)
-
FKRP_000177
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
2
4
c.313C>T
r.(?)
p.(Gln105*)
-
pathogenic, pathogenic (recessive)
g.47259020C>T
g.46755763C>T
-
-
FKRP_000064
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
,
Tom Winder
-?/., ?/.
3
-
c.315G>T
r.(?)
p.(Gln105His)
-
likely benign, VUS
g.47259022G>T
-
1 more item
-
FKRP_000256
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
?/.
2
4
c.319G>T
r.(?)
p.(Ala107Ser)
-
VUS
g.47259026G>T
g.46755769G>T
-
-
FKRP_000198
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/.
6
4
c.328C>T
r.(?)
p.(Arg110Trp)
-
pathogenic (recessive)
g.47259035C>T
g.46755778C>T
-
-
FKRP_000125
-
PubMed: Kang 2007
,
PubMed: Song 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Rosário dos Santos
+/.
4
4
c.329G>C
r.(?)
p.(Arg110Pro)
-
pathogenic (recessive)
g.47259036G>C
g.46755779G>C
-
-
FKRP_000103
-
PubMed: Quijano-Roy 2006
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
1
-
c.336C>G
r.(?)
p.(Ala112=)
-
benign
g.47259043C>G
g.46755786C>G
FKRP(NM_001039885.3):c.336C>G (p.A112=)
-
FKRP_000233
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/., +?/., -/., -?/., ?/.
40
4
c.(341C>G), c.341C>G
r.(?)
p.(Ala114Gly)
-
benign, likely benign, likely pathogenic, pathogenic (recessive), VUS
g.47259048C>G
g.46755791C>G
1 more item
-
FKRP_000016
unknown variant 2nd chromosome, VKGL data sharing initiative Nederland
from website {DBsub-Emory}, Ginjaar WMS2005, Luce 2021, submitted, Nevo ESHG2008 P01.202,
11 more items
-
-
CLASSIFICATION record, Germline, Germline/De novo (untested), Unknown
-
1/100, 1/94 cases, 2/104, 2/212, 4/170
-
-
-
Gerard C.P. Schaafsma
,
Johan den Dunnen
,
Ieke Ginjaar
,
Rosário dos Santos
,
Lab Müller-Reible
,
Madhuri Hegde
,
Akanchha Kesari
,
Florencia Giliberto
,
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
,
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
,
VKGL-NL_AMC
?/.
2
4
c.344C>T
r.(?)
p.(Ser115Leu)
-
VUS
g.47259051C>T
g.46755794C>T
-
-
FKRP_000122
no second variant, not in 100 control chromosomes
Cittadella ESHG2008 P01.204,
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Madhuri Hegde
+/.
3
-
c.350C>G
r.(?)
p.(Pro117Arg)
-
pathogenic (recessive)
g.47259057C>G
g.46755800C>G
-
-
FKRP_000280
-
PubMed: Song 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
1
-
c.360C>T
r.(?)
p.(Tyr120=)
-
benign
g.47259067C>T
g.46755810C>T
FKRP(NM_001039885.3):c.360C>T (p.Y120=)
-
FKRP_000234
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
1
4
c.362T>A
r.(?)
p.(Val121Glu)
-
pathogenic (recessive)
g.47259069T>A
g.46755812T>A
-
-
FKRP_000028
-
PubMed: Walter
-
-
Germline
-
-
-
-
-
Rolf Stucka
+/., ?/.
4
4
c.374T>C
r.(?)
p.(Phe125Ser)
-
pathogenic (recessive), VUS
g.47259081T>C
g.46755824T>C
-
-
FKRP_000058
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Lab Müller-Reible
,
Madhuri Hegde
,
Tom Winder
?/.
2
4
c.385G>C
r.(?)
p.(Val129Leu)
-
VUS
g.47259092G>C
g.46755835G>C
-
-
FKRP_000199
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/.
2
4
c.387_390dup
r.(?)
p.(?), p.(Asp131Thrfs*2)
-
pathogenic (recessive)
g.47259094_47259097dup
g.46755837_46755840dup
386_389dup, 390dupACCG
-
FKRP_000003
-
PubMed: Brockington
,
PubMed: Mercuri
,
OMIM:var0005
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Lab Müller-Reible
?/.
1
4
c.395G>A
r.(?)
p.(Gly132Glu)
-
VUS
g.47259102G>A
g.46755845G>A
-
-
FKRP_000200
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/.
2
4
c.400C>T
r.(?)
p.(Arg134Trp)
-
pathogenic (recessive)
g.47259107C>T
g.46755850C>T
-
-
FKRP_000085
not in 200 control chromosomes
PubMed: de Paula 2003
;
PubMed: Yamamoto 2008
,
OMIM:var0014
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., ?/.
2
4
c.401G>C
r.(?)
p.(Arg134Pro)
-
pathogenic (recessive), VUS
g.47259108G>C
g.46755851G>C
-
-
FKRP_000201
-
PubMed: Nallamilli 2018
,
PubMed: Song 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Madhuri Hegde
-/.
1
-
c.414T>A
r.(?)
p.(Pro138=)
-
benign
g.47259121T>A
g.46755864T>A
FKRP(NM_001039885.3):c.414T>A (p.P138=)
-
FKRP_000244
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/., +?/., -/., -?/., ?/.
27
4
c.427C>A
r.(?)
p.(Arg143Ser)
-
benign, likely benign, likely pathogenic, pathogenic (recessive), VUS
g.47259134C>A
g.46755877C>A
1 more item
-
FKRP_000007
not in 200 control chromosomes, rare variant: dbSNP (rs148206382) 10/2000 and ESP 37/7538,
3 more items
Freixas ESHG2006 P0925, from website {DBsub-Emory}, Politano et al., Neuromuscul.Disord. 12: 733,
7 more items
-
rs148206382
CLASSIFICATION record, Germline, Unknown
?
1/120 chromosomes NMD cases
-
-
-
Johan den Dunnen
,
Ieke Ginjaar
,
Madhuri Hegde
,
Akanchha Kesari
,
Tom Winder
,
VKGL-NL_Leiden
,
Miguel Angel Alcántara-Ortigoza
,
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
,
VKGL-NL_AMC
+/.
1
4
c.427_438del
r.(?)
p.(Arg143_Glu146del)
-
pathogenic (recessive)
g.47259134_47259145del
g.46755877_46755888del
426_437del
-
FKRP_000006
-
PubMed: Brockington
,
PubMed: Mercuri
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
4
c.430A>G
r.(?)
p.(Met144Val)
-
pathogenic (recessive)
g.47259137A>G
g.46755880A>G
-
-
FKRP_000111
-
-
-
-
Germline
-
-
-
-
-
Tom Winder
+/.
1
4
c.437A>C
r.(?)
p.(Glu146Ala)
-
pathogenic (recessive)
g.47259144A>C
g.46755887A>C
-
-
FKRP_000129
-
PubMed: Manzini 2008
-
-
Germline
-
-
-
-
-
Rosário dos Santos
+/.
1
-
c.443T>C
r.(?)
p.(Leu148Pro)
-
pathogenic (recessive)
g.47259150T>C
g.46755893T>C
-
-
FKRP_000281
-
PubMed: Song 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.446G>A
r.(?)
p.(Arg149His)
-
VUS
g.47259153G>A
g.46755896G>A
FKRP(NM_001039885.2):c.446G>A (p.R149H)
-
FKRP_000178
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
-
c.447_451del
r.(?)
p.(Ala150LysfsTer107)
-
pathogenic (recessive)
g.47259154_47259158del
g.46755897_46755901del
c.446_450delGCGCA
-
FKRP_000282
-
PubMed: Song 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/., ?/.
9
4
c.456C>G
r.(?)
p.(Ser152Arg)
-
likely benign, VUS
g.47259163C>G
g.46755906C>G
1 more item
-
FKRP_000095
no second variant, VKGL data sharing initiative Nederland
PubMed: Nallamilli 2018
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Ieke Ginjaar
,
Madhuri Hegde
,
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
,
VKGL-NL_AMC
?/.
2
4
c.466G>A
r.(?)
p.(Val156Met)
-
VUS
g.47259173G>A
g.46755916G>A
FKRP(NM_001039885.3):c.466G>A (p.V156M)
-
FKRP_000202
VKGL data sharing initiative Nederland
PubMed: Nallamilli 2018
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Madhuri Hegde
,
VKGL-NL_AMC
+/.
2
4
c.469G>C
r.(?)
p.(Ala157Pro)
-
pathogenic (recessive)
g.47259176G>C
g.46755919G>C
-
-
FKRP_000083
-
Sveen WMS2005 L.P.3.06
-
-
Germline
-
-
-
-
-
Marianne Schwartz
,
Tom Winder
-/.
1
-
c.474C>G
r.(?)
p.(Ala158=)
-
benign
g.47259181C>G
-
FKRP(NM_001039885.3):c.474C>G (p.A158=)
-
FKRP_000307
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
5
4
c.478G>T
r.(?)
p.(Val160Phe)
-
pathogenic (recessive)
g.47259185G>T
g.46755928G>T
-
-
FKRP_000078
-
Luce 2021, submitted,
PubMed: Boito 2005
,
PubMed: de Paula 2003
;
PubMed: Yamamoto 2008
,
1 more item
-
-
Germline, Unknown
-
-
-
-
-
Johan den Dunnen
,
Florencia Giliberto
?/.
1
-
c.493C>T
r.(?)
p.(Pro165Ser)
-
VUS
g.47259200C>T
-
-
-
FKRP_000267
-
PubMed: Chakravorty 2020
-
-
Germline
-
-
-
-
-
Johan den Dunnen
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