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%)
Effect
Reported
Exon
DNA change (cDNA)
RNA change
Protein
Classification method
Clinical classification
DNA change (genomic) (hg19)
DNA change (hg38)
Published as
ISCN
DB-ID
Variant remarks
Reference
ClinVar ID
dbSNP ID
Origin
Segregation
Frequency
Re-site
VIP
Methylation
Owner
+/.
1
1
c.27_28del
r.(?)
p.(Gly10Glufs*15)
-
pathogenic
g.75262723_75262724del
g.74350488_74350489del
27_28delAG
-
GDAP1_000008
-
PubMed: Crimella 2010
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.77T>G
r.(?)
p.(Leu26Arg)
ACMG
likely pathogenic
g.75262773T>G
g.74350538T>G
c.T77G
-
GDAP1_000089
ACMG: PM1-PM2-PM3-PP1
PubMed: Chen C. 2020
-
-
Germline
-
-
-
-
-
Yvet den Hartog
+/.
3
1
c.92G>A
r.(?)
p.(Trp31*), p.(Trp31Ter)
-
pathogenic
g.75262788G>A
g.74350553G>A
GDAP1(NM_018972.2):c.92G>A (p.W31*)
-
GDAP1_000054
shared haplotype with family DUK1404, shared haplotype with family DUK1405, 1 more item
PubMed: Baxter 2002 , OMIM:var0001
-
rs121908112
CLASSIFICATION record, Germline
yes
-
-
-
-
Johan den Dunnen , VKGL-NL_AMC
+/.
1
1
c.101C>G
r.(?)
p.(Ser34Cys)
-
pathogenic
g.75262797C>G
g.74350562C>G
-
-
GDAP1_000004
-
PubMed: Crimella 2010
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
2
-
c.102=
r.(=)
p.(Ser34=)
-
benign
g.75262798G>C
g.74350563=
GDAP1(NM_018972.2):c.102G>C (p.S34=), GDAP1(NM_018972.4):c.102G>C (p.S34=)
-
GDAP1_000057
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen , VKGL-NL_AMC
?/.
1
-
c.109T>A
r.(?)
p.(Ser37Thr)
-
VUS
g.75262805T>A
g.74350570T>A
-
-
GDAP1_000061
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/.
1
-
c.117+28G>A
r.(=)
p.(=)
-
benign
g.75262841G>A
-
GDAP1(NM_018972.2):c.117+28G>A
-
GDAP1_000096
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+?/.
1
2
c.128dup
r.(?)
p.(Ile44AsnfsTer3)
-
likely pathogenic
g.75263519dup
g.74351284dup
c.128dupT
-
GDAP1_000081
-
PubMed: Ganapathy 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.140del
r.(?)
p.(Lys47Argfs*3)
ACMG
pathogenic (dominant)
g.75263531del
g.74351296del
140delA
-
GDAP1_000085
ACMG: PVS1-PM1-PM2, sister not available
PubMed: Ferese 2021
SCV001424519
-
Germline/De novo (untested)
-
-
-
-
-
Yvet den Hartog
+/.
1
-
c.154G>T
r.(?)
p.(Glu52*)
-
pathogenic
g.75263545G>T
g.74351310G>T
-
-
GDAP1_000070
-
-
-
-
Unknown
-
-
-
-
-
IMGAG
+/.
1
2
c.172_173delinsTTA
r.(?)
p.(Pro59Alafs*4)
-
pathogenic
g.75263563_75263564delinsTTA
g.74351328_74351329delinsTTA
172_173delCTinsTTA
-
GDAP1_000005
-
PubMed: Sevilla 2008
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
2
c.174_176delinsTGTG
r.(?)
p.(Pro59Valfs*4)
-
pathogenic
g.75263565_75263567delinsTGTG
g.74351330_74351332delinsTGTG
174_176delGCCinsTGTG
-
GDAP1_000006
-
PubMed: Auer-Grumbach 2008
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.208C>T
r.(?)
p.(Arg70Cys)
-
VUS
g.75263599C>T
g.74351364C>T
-
-
GDAP1_000071
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/.
1
-
c.218C>G
r.(?)
p.(Ser73*)
-
pathogenic (recessive)
g.75263609C>G
g.74351374C>G
-
-
GDAP1_000090
-
PubMed: Jiang H 2021
-
-
Germline
-
-
-
-
-
Yvet den Hartog
+/.
2
2
c.233C>T
r.(?)
p.(Pro78Leu)
-
pathogenic
g.75263624C>T
g.74351389C>T
GDAP1(NM_018972.2):c.233C>T (p.P78L)
-
GDAP1_000007
VKGL data sharing initiative Nederland
PubMed: Bouhouche 2007
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen , VKGL-NL_AMC
+?/.
1
2
c.246C>G
r.(?)
p.(His82Gln)
-
likely pathogenic (recessive)
g.75263637C>G
g.74351402C>G
-
-
GDAP1_000086
-
PubMed: Xue H 2021
-
-
Unknown
-
-
-
-
-
Yvet den Hartog
-?/.
1
-
c.246C>T
r.(?)
p.(His82=)
-
likely benign
g.75263637C>T
g.74351402C>T
GDAP1(NM_018972.2):c.246C>T (p.H82=)
-
GDAP1_000058
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+?/.
1
-
c.288T>G
r.(?)
p.(Tyr96*)
-
likely pathogenic
g.75263679T>G
-
GDAP1(NM_018972.2):c.288T>G (p.Y96*)
-
GDAP1_000100
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
1
2
c.295C>T
r.(?)
p.(Gln99*)
-
pathogenic
g.75263686C>T
g.74351451C>T
-
-
GDAP1_000009
-
PubMed: Moroni 2009
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
2
-
c.310G>A
r.(?)
p.(Glu104Lys)
-
likely pathogenic
g.75263701G>A
g.74351466G>A
-
-
GDAP1_000077
proband appears homozygous, asymptomatic heterozygous parent
PubMed: Antoniadi 2015
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.310+6del
r.(=)
p.(=)
-
likely benign
g.75263707del
g.74351472del
GDAP1(NM_018972.2):c.310+6delG
-
GDAP1_000072
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/.
1
-
c.311-85T>C
r.(=)
p.(=)
-
benign
g.75272287T>C
-
GDAP1(NM_018972.2):c.311-85T>C
-
GDAP1_000097
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+?/.
3
-
c.311-23A>G
r.(=)
p.(=)
-
likely pathogenic (recessive)
g.75272349A>G
g.74360114A>G
-
-
GDAP1_000091
-
PubMed: Khani M 2020
-
-
Germline
-
-
-
-
-
Yvet den Hartog
+/.
1
-
c.311-1G>A
r.spl?
p.?
-
pathogenic
g.75272371G>A
g.74360136G>A
GDAP1(NM_018972.2):c.311-1G>A
-
GDAP1_000059
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
1
3
c.332C>A
r.(?)
p.(Pro111His)
-
pathogenic
g.75272393C>A
g.74360158C>A
-
-
GDAP1_000010
-
PubMed: Chung 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
3
c.341_344del
r.(?)
p.(Glu114Alafs*32)
-
pathogenic
g.75272402_75272405del
g.74360167_74360170del
341_344delAAAG
-
GDAP1_000011
-
PubMed: Ammar 2003
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
3
c.347T>C
r.(?)
p.(Met116Thr)
-
pathogenic
g.75272408T>C
g.74360173T>C
GDAP1(NM_018972.2):c.347T>C (p.M116T)
-
GDAP1_000012
VKGL data sharing initiative Nederland
PubMed: Kabzinska 2006
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen , VKGL-NL_AMC
+/.
2
3
c.347T>G
r.(?)
p.(Met116Arg)
-
pathogenic
g.75272408T>G
g.74360173T>G
GDAP1(NM_018972.2):c.347T>G (p.M116R)
-
GDAP1_000013
VKGL data sharing initiative Nederland
PubMed: Di Maria 2004
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen , VKGL-NL_AMC
+/.
2
3
c.349dup
r.(?)
p.(Tyr117Leufs*13), p.(Tyr117LeufsTer13)
-
pathogenic
g.75272410dup
g.74360175dup
GDAP1(NM_018972.2):c.349dupT (p.Y117Lfs*13)
-
GDAP1_000014, GDAP1_000060
VKGL data sharing initiative Nederland
PubMed: Senderek 2003
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen , VKGL-NL_AMC
+/., ?/.
8
3
c.358C>T
r.(?)
p.(Arg120Trp)
ACMG
pathogenic, VUS
g.75272419C>T
g.74360184C>T
c.C358T, GDAP1(NM_018972.2):c.358C>T (p.R120W)
-
GDAP1_000015
Daughter carried the same variant, but had no symptoms, VKGL data sharing initiative Nederland
PubMed: Ammar 2003 , PubMed: Antoniadi 2015 , PubMed: Chen C. 2020 , PubMed: Ganapathy 2019
-
rs104894078
CLASSIFICATION record, Germline, Unknown
-
-
-
-
-
Johan den Dunnen , Carmen Espinós , VKGL-NL_Nijmegen , VKGL-NL_AMC , Yvet den Hartog
+/.
2
3
c.359G>A
r.(?)
p.(Arg120Gln)
-
pathogenic
g.75272420G>A
g.74360185G>A
GDAP1(NM_018972.2):c.359G>A (p.R120Q)
-
GDAP1_000016
VKGL data sharing initiative Nederland
PubMed: Boerkoel 2003
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen , VKGL-NL_AMC
+/.
1
3
c.364C>A
r.(?)
p.(Gln122Lys)
-
pathogenic
g.75272425C>A
g.74360190C>A
-
-
GDAP1_000017
-
PubMed: Moroni 2009
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
3
3
c.368A>G
r.(?)
p.(His123Arg)
-
pathogenic, pathogenic (dominant)
g.75272429A>G
g.74360194A>G
-
-
GDAP1_000018
clinical severity variable
PubMed: Antoniadi 2015 , PubMed: Zimon 2011
-
rs397515442
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.371A>G
r.(?)
p.(Tyr124Cys)
-
VUS
g.75272432A>G
g.74360197A>G
-
-
GDAP1_000073
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
2
3
c.373C>T
r.(?)
p.(Arg125*)
-
pathogenic
g.75272434C>T
g.74360199C>T
-
-
GDAP1_000019
1 heterozygous, no homozygous; Clinindb (India)
PubMed: Moroni 2009 , PubMed: Narang 2020 , Journal: Narang 2020
-
rs745663149
Germline
-
1/2795 individuals
-
-
-
Johan den Dunnen , Mohammed Faruq
?/.
1
-
c.385G>A
r.(?)
p.(Asp129Asn)
-
VUS
g.75272446G>A
-
GDAP1(NM_018972.2):c.385G>A (p.D129N)
-
GDAP1_000103
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
2
3
c.389C>G
r.(?)
p.(Ser130Cys)
-
pathogenic
g.75272450C>G
g.74360215C>G
GDAP1(NM_018972.2):c.389C>G (p.S130C)
-
GDAP1_000020
VKGL data sharing initiative Nederland
PubMed: Kabzinska 2005
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen , VKGL-NL_AMC
+?/.
1
-
c.399G>A
r.(?)
p.(Met133Ile)
-
likely pathogenic
g.75272460G>A
g.74360225G>A
-
-
GDAP1_000078
-
PubMed: Antoniadi 2015
-
rs139808557
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
3
c.407A>C
r.(?)
p.(Tyr136Ser)
-
pathogenic (recessive)
g.75272468A>C
-
g.44104A>C
-
GDAP1_000095
-
-
-
-
Germline
yes
-
-
-
-
Sherifa Ahmed Hamed
+/.
1
3
c.439del
r.(?)
p.(Thr147Leufs*5)
-
pathogenic
g.75272500del
g.74360265del
-
-
GDAP1_000021
-
PubMed: Georgiou 2006
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.442G>A
r.(?)
p.(Val148Met)
-
VUS
g.75272503G>A
-
GDAP1(NM_018972.2):c.442G>A (p.(Val148Met))
-
GDAP1_000104
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
2
3
c.445G>T
r.(?)
p.(Asp149Tyr)
-
pathogenic
g.75272506G>T
g.74360271G>T
GDAP1(NM_018972.2):c.445G>T (p.D149Y)
-
GDAP1_000022
VKGL data sharing initiative Nederland
PubMed: Parman 2004
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen , VKGL-NL_AMC
+/.
2
3
c.458C>T
r.(?)
p.(Pro153Leu)
-
pathogenic
g.75272519C>T
g.74360284C>T
GDAP1(NM_018972.2):c.458C>T (p.P153L)
-
GDAP1_000023
VKGL data sharing initiative Nederland
PubMed: Kabzinska 2007
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen , VKGL-NL_AMC
+/.
1
3
c.467C>G
r.(?)
p.(Ala156Gly)
-
pathogenic
g.75272528C>G
g.74360293C>G
-
-
GDAP1_000024
-
PubMed: Zimon 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., ?/.
3
3
c.469A>C
r.(?)
p.(Thr157Pro)
-
pathogenic, VUS
g.75272530A>C
g.74360295A>C
GDAP1(NM_018972.2):c.469A>C (p.T157P)
-
GDAP1_000025
VKGL data sharing initiative Nederland
PubMed: Claramunt 2005
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen , Carmen Espinós , VKGL-NL_AMC
?/.
1
-
c.477G>T
r.(?)
p.(Arg159Ser)
-
VUS
g.75272538G>T
g.74360303G>T
-
-
GDAP1_000062
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
2
3
c.482G>A
r.(?)
p.(Arg161His)
-
pathogenic
g.75272543G>A
g.74360308G>A
GDAP1(NM_018972.2):c.482G>A (p.R161H)
-
GDAP1_000026
VKGL data sharing initiative Nederland
PubMed: Baxter 2002 , OMIM:var0003
-
rs104894076
CLASSIFICATION record, Germline
yes
-
-
-
-
Johan den Dunnen , VKGL-NL_AMC
-/.
1
-
c.485-20C>G
r.(=)
p.(=)
-
benign
g.75274099C>G
g.74361864C>G
GDAP1(NM_018972.2):c.485-20C>G
-
GDAP1_000064
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
1
-
c.485-2A>G
r.spl?
p.?
-
pathogenic
g.75274117A>G
g.74361882A>G
GDAP1(NM_018972.2):c.485-2A>G
-
GDAP1_000065
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/., ?/.
7
4
c.487C>T
r.(?)
p.(Gln163*), p.(Gln163Ter)
ACMG
pathogenic, pathogenic (recessive), VUS
g.75274121C>T
g.74361886C>T
GDAP1(NM_018972.2):c.487C>T (p.Q163*)
-
GDAP1_000027
father/brother unaffected carriers, VKGL data sharing initiative Nederland
PubMed: Cuesta 2002 , OMIM:var0004 , PubMed: Fierro 2020
-
rs104894077
CLASSIFICATION record, Germline
yes
-
-
-
-
Johan den Dunnen , Carmen Espinós , VKGL-NL_Nijmegen , VKGL-NL_AMC , Yvet den Hartog
+?/.
1
-
c.501dup
r.(?)
p.(Glu168Argfs*3)
-
likely pathogenic
g.75274135dup
g.74361900dup
c.501dupA
-
GDAP1_000079
-
PubMed: Antoniadi 2015
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.506_512del
r.(?)
p.(Ser169*)
ACMG
pathogenic
g.75274140_75274146del
g.74361905_74361911del
c.505_511del
-
GDAP1_000092
ACMG: PVS1-PM1-PM2-PP1-PP3
PubMed: Chen C. 2020
-
-
Germline
-
-
-
-
-
Yvet den Hartog
-/.
2
-
c.507T>G
r.(?)
p.(Ser169=)
-
benign
g.75274141T>G
g.74361906T>G
GDAP1(NM_018972.2):c.507T>G (p.S169=), GDAP1(NM_018972.4):c.507T>G (p.S169=)
-
GDAP1_000066
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen , VKGL-NL_AMC
+/.
1
4
c.533A>G
r.(?)
p.(Asn178Ser)
-
pathogenic
g.75274167A>G
g.74361932A>G
-
-
GDAP1_000028
-
PubMed: Zhang 2004
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
4
c.558del
r.(?)
p.(Ile186Metfs*20)
-
pathogenic
g.75274192del
g.74361957del
-
-
GDAP1_000029
-
PubMed: Stojkovic 2004
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
4
c.571C>T
r.(?)
p.(Arg191*), p.(Arg191Ter)
-
pathogenic
g.75274205C>T
g.74361970C>T
GDAP1(NM_018972.2):c.571C>T (p.R191*)
-
GDAP1_000030
VKGL data sharing initiative Nederland
PubMed: Barankova 2007
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen , VKGL-NL_AMC
+/.
3
-
c.579+1G>A
r.spl?
p.?
-
pathogenic
g.75274214G>A
g.74361979G>A
GDAP1(NM_018972.2):c.579+1G>A
-
GDAP1_000067
1 heterozygous, no homozygous; Clinindb (India) , VKGL data sharing initiative Nederland
PubMed: Narang 2020 , Journal: Narang 2020
-
-
CLASSIFICATION record, Germline
-
1/2795 individuals
-
-
-
VKGL-NL_VUmc , VKGL-NL_AMC , Mohammed Faruq
+/., ?/.
5
5
c.581C>G
r.(?)
p.(Ser194*), p.(Ser194Ter)
-
pathogenic, VUS
g.75275175C>G
g.74362940C>G
GDAP1(NM_018972.2):c.581C>G (p.S194*)
-
GDAP1_000031
VKGL data sharing initiative Nederland
PubMed: Baxter 2002 , OMIM:var0002 , PubMed: Cuesta 2002 , OMIM:var0002 , PubMed: Nelis 2002 , OMIM:var0002
-
rs104894075
CLASSIFICATION record, Germline
yes
-
-
-
-
Johan den Dunnen , Carmen Espinós , VKGL-NL_AMC
+?/.
1
5
c.614T>G
r.(?)
p.(Leu205Trp)
-
likely pathogenic (maternal)
g.75275208T>G
g.74362973T>G
-
-
GDAP1_000087
Unaffected carrier mother
PubMed: Xue H 2021
-
-
Germline
-
-
-
-
-
Yvet den Hartog
+/.
1
5
c.652C>G
r.(?)
p.(Gln218Glu)
-
pathogenic
g.75275246C>G
g.74363011C>G
-
-
GDAP1_000032
-
PubMed: Chung 2008
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.653A>G
r.(?)
p.(Gln218Arg)
-
VUS
g.75275247A>G
g.74363012A>G
-
-
GDAP1_000076
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
5
c.656T>A
r.(?)
p.(Val219Asp)
-
pathogenic
g.75275250T>A
g.74363015T>A
-
-
GDAP1_000033
-
PubMed: Moroni 2009
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
5
c.656T>G
r.(?)
p.(Val219Gly)
-
pathogenic
g.75275250T>G
g.74363015T>G
-
-
GDAP1_000034
-
PubMed: Chung 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
3
-
c.667_671dup
r.(?)
p.(Gln224Hisfs*37)
-
pathogenic (recessive)
g.75275261_75275265dup
g.74363026_74363030dup
c.667_671dupTTGCA
-
GDAP1_000088
-
PubMed: Mai PT 2019
-
-
Germline
-
-
-
-
-
Yvet den Hartog
+/.
2
5
c.668T>A
r.(?)
p.(Leu223*), p.(Leu223Ter)
-
pathogenic
g.75275262T>A
g.74363027T>A
GDAP1(NM_018972.2):c.668T>A (p.L223*)
-
GDAP1_000035
VKGL data sharing initiative Nederland
PubMed: De Sandre-Giovannoli 2003
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen , VKGL-NL_AMC
+/.
1
5
c.678A>T
r.(?)
p.(Arg226Ser)
-
pathogenic
g.75275272A>T
g.74363037A>T
-
-
GDAP1_000036
-
PubMed: Crimella 2010
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
5
c.679A>G
r.(?)
p.(Asn227Asp)
-
pathogenic
g.75275273A>G
g.74363038A>G
-
-
GDAP1_000037
-
PubMed: Kabzinska 2010
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
5
c.685G>A
r.(?)
p.(Glu229Lys)
-
VUS
g.75275279G>A
g.74363044G>A
-
-
GDAP1_000082
-
-
-
-
Germline
-
-
-
-
-
LOVD
+/.
1
5
c.692C>T
r.(?)
p.(Pro231Leu)
-
pathogenic
g.75275286C>T
g.74363051C>T
-
-
GDAP1_000038
-
PubMed: Xin 2008
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.693A>T
r.(?)
p.(Pro231=)
-
likely benign
g.75275287A>T
-
GDAP1(NM_018972.2):c.693A>T (p.P231=)
-
GDAP1_000101
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
1
-
c.694+1G>A
r.spl?
p.?
ACMG
pathogenic (recessive)
g.75275289G>A
g.74363054G>A
-
-
GDAP1_000093
ACMG: PVS1-PM2-PP1
PubMed: Chen C. 2020
-
-
Germline
-
-
-
-
-
Yvet den Hartog
+/.
1
-
c.694+1G>C
r.spl
p.?
ACMG
likely pathogenic
g.75275289G>C
g.74363054G>C
-
-
GDAP1_000069
ACMG pvs1, pm2
PubMed: Dohrn 2017 , Journal: Dohrn 2017
-
-
Germline
-
1/612 cases
-
-
-
Johan den Dunnen
-/.
1
-
c.694+24C>T
r.(=)
p.(=)
-
benign
g.75275312C>T
-
GDAP1(NM_018972.2):c.694+24C>T
-
GDAP1_000098
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/.
1
3
c.712T>G
r.(?)
p.(Trp238Gly)
-
pathogenic (recessive)
g.75276237T>G
-
-
-
GDAP1_000094
-
-
-
-
Unknown
-
-
-
-
-
Sherifa Ahmed Hamed
+?/.
1
-
c.714G>C
r.(?)
p.(Trp238Cys)
-
likely pathogenic
g.75276239G>C
g.74364004G>C
-
-
GDAP1_000080
-
PubMed: Antoniadi 2015
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., +?/.
3
6
c.715C>T
r.(?)
p.(Leu239Phe)
-
likely pathogenic, pathogenic
g.75276240C>T
g.74364005C>T
GDAP1(NM_018972.2):c.715C>T (p.L239F), GDAP1(NM_018972.4):c.715C>T (p.(Leu239Phe))
-
GDAP1_000039
VKGL data sharing initiative Nederland
PubMed: Ammar 2003
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen , VKGL-NL_Leiden , VKGL-NL_AMC
+/.
1
6
c.719G>A
r.(?)
p.(Cys240Tyr)
-
pathogenic
g.75276244G>A
g.74364009G>A
-
-
GDAP1_000040
-
PubMed: Cassereau 2009
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
6
c.740C>T
r.(?)
p.(Ala247Val)
-
pathogenic
g.75276265C>T
g.74364030C>T
-
-
GDAP1_000041
-
PubMed: Abe 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
6
c.767A>G
r.(?)
p.(His256Arg)
-
pathogenic, pathogenic (recessive)
g.75276292A>G
g.74364057A>G
-
-
GDAP1_000042
-
PubMed: Lin 2011 , PubMed: Zhang 2004
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
3
6
c.769C>T
r.(?)
p.(Arg257*), p.(Arg257Ter)
-
pathogenic
g.75276294C>T
g.74364059C>T
GDAP1(NM_018972.4):c.769C>T (p.(Arg257Ter))
-
GDAP1_000043
VKGL data sharing initiative Nederland
PubMed: Ganapathy 2019 , PubMed: Shagina 2010
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen , VKGL-NL_Leiden
+/.
2
6
c.786del
r.(?)
p.(Phe263Leufs*22)
-
pathogenic
g.75276311del
g.74364076del
-
-
GDAP1_000044
-
PubMed: Nelis 2002
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
+?/.
1
6
c.802_803del
r.(?)
p.(Trp268GlyfsTer22)
-
likely pathogenic
g.75276327_75276328del
g.74364092_74364093del
c.802_803delTG
-
GDAP1_000083
-
PubMed: Ganapathy 2019
-
rs765346218
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
6
c.811G>A
r.(?)
p.(Gly271Arg)
-
pathogenic
g.75276336G>A
g.74364101G>A
GDAP1(NM_018972.2):c.811G>A (p.G271R)
-
GDAP1_000045
VKGL data sharing initiative Nederland
PubMed: Ammar 2003
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen , VKGL-NL_AMC
+/.
1
6
c.817C>G
r.(?)
p.(Arg273Gly)
-
pathogenic
g.75276342C>G
g.74364107C>G
-
-
GDAP1_000046
-
PubMed: Kabzinska 2010
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
6
c.821C>T
r.(?)
p.(Pro274Leu)
-
pathogenic
g.75276346C>T
g.74364111C>T
-
-
GDAP1_000047
-
PubMed: Zimon 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
6
c.836A>G
r.(?)
p.(Tyr279Cys)
-
pathogenic
g.75276361A>G
g.74364126A>G
-
-
GDAP1_000048
-
PubMed: Rougeot 2008
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., +?/.
6
6
c.844C>T
r.(?)
p.(Arg282Cys)
-
likely pathogenic, pathogenic, pathogenic (recessive)
g.75276369C>T
g.74364134C>T
GDAP1(NM_018972.2):c.844C>T (p.R282C)
-
GDAP1_000049
VKGL data sharing initiative Nederland
PubMed: Antoniadi 2015 , PubMed: Dohrn 2017 , Journal: Dohrn 2017 , PubMed: Nelis 2002 , OMIM:var0006
-
rs28937906
CLASSIFICATION record, Germline
yes
1/612 cases
-
-
-
Johan den Dunnen , Carmen Espinós , VKGL-NL_AMC
+/., +?/.
3
6
c.845G>A
r.(?)
p.(Arg282His)
-
likely pathogenic, pathogenic, pathogenic (recessive)
g.75276370G>A
g.74364135G>A
GDAP1(NM_018972.2):c.845G>A (p.R282H)
-
GDAP1_000050
VKGL data sharing initiative Nederland
PubMed: Abe 2011 , PubMed: Lin 2011
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen , VKGL-NL_AMC
+/.
1
6
c.862dup
r.(?)
p.(Thr288Asnfs*3)
-
pathogenic
g.75276387dup
g.74364152dup
863insA
-
GDAP1_000051
-
PubMed: Cuesta 2002 , OMIM:var0005
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.864dup
r.(?)
p.(Phe289IlefsTer2)
-
pathogenic
g.75276389dup
g.74364154dup
GDAP1(NM_018972.2):c.864dupA (p.F289Ifs*2)
-
GDAP1_000068
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
1
6
c.866T>C
r.(?)
p.(Phe289Ser)
-
VUS
g.75276391T>C
g.74364156T>C
-
-
GDAP1_000084
-
-
-
-
Germline
-
-
-
-
-
LOVD
+/.
1
6
c.891C>G
r.(?)
p.(Asn297Lys)
-
pathogenic
g.75276416C>G
g.74364181C>G
-
-
GDAP1_000052
-
PubMed: Moroni 2009
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.917C>T
r.(?)
p.(Pro306Leu)
-
VUS
g.75276442C>T
g.74364207C>T
-
-
GDAP1_000063
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
2
6
c.929G>A
r.(?)
p.(Arg310Gln)
-
pathogenic
g.75276454G>A
g.74364219G>A
GDAP1(NM_018972.2):c.929G>A (p.R310Q)
-
GDAP1_000053
VKGL data sharing initiative Nederland
PubMed: Azzedine 2003
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen , VKGL-NL_AMC
?/.
1
-
c.965C>T
r.(?)
p.(Thr322Met)
-
VUS
g.75276490C>T
g.74364255C>T
GDAP1(NM_018972.2):c.965C>T (p.T322M)
-
GDAP1_000075
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
1
6
c.980G>A
r.(?)
p.(Gly327Asp)
-
pathogenic
g.75276505G>A
g.74364270G>A
-
-
GDAP1_000055
-
PubMed: Kabzinska 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.1006G>T
r.(?)
p.(Ala336Ser)
-
VUS
g.75276531G>T
-
GDAP1(NM_018972.4):c.1006G>T (p.A336S)
-
GDAP1_000102
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-/.
1
6
c.1057C>T
r.(?)
p.(Pro353Ser)
-
benign
g.75276582C>T
g.74364347C>T
-
-
GDAP1_000056
-
-
-
-
Germline
-
-
-
-
-
Andreas Laner
-/.
1
-
c.*2942T>G
r.(=)
p.(=)
-
benign
g.75279544T>G
-
GDAP1(NM_001362931.2):c.694+4256T>G
-
GDAP1_000099
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht