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
2
c.110dup
r.(?)
p.(Ile38Hisfs*27)
-
pathogenic
g.215976376dup
g.215111653dup
107insT
-
ABCA12_000026
-
PubMed: Thomas 2008
-
-
Germline
yes
-
-
-
-
Marianne Vos (LOVD-team)
-/.
1
-
c.164-11300A>G
r.(=)
p.(=)
-
benign
g.215940242T>C
g.215075519T>C
ABCA12(NM_173076.3):c.164-11300A>G
-
ABCA12_000066
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
?/.
1
-
c.172G>A
r.(?)
p.(Ala58Thr)
-
VUS
g.215928934C>T
-
ABCA12(NM_173076.3):c.172G>A (p.A58T)
-
ABCA12_000095
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+/.
1
-
c.178C>T
r.(?)
p.(Arg60Ter)
-
pathogenic
g.215928928G>A
g.215064205G>A
-
-
ABCA12_000083
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/.
1
-
c.317+9A>C
r.(=)
p.(=)
-
likely benign
g.215928780T>G
-
ABCA12(NM_173076.3):c.317+9A>C
-
ABCA12_000132
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
1
-
c.510G>A
r.(?)
p.(Leu170=)
-
likely benign
g.215914533C>T
-
ABCA12(NM_173076.2):c.510G>A (p.L170=)
-
ABCA12_000102
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
6
c.529del
r.(?)
p.(Ser177Glnfs*26)
-
pathogenic
g.215914515del
g.215049791del
-
-
ABCA12_000020
-
PubMed: Thomas 2006
-
-
Germline
yes
-
-
-
-
Marianne Vos (LOVD-team)
-?/.
1
-
c.535G>A
r.(?)
p.(Asp179Asn)
-
likely benign
g.215914508C>T
g.215049784C>T
ABCA12(NM_173076.2):c.535G>A (p.(Asp179Asn))
-
ABCA12_000085
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
1
6
c.596G>A
r.(?)
p.(Trp199*)
-
pathogenic
g.215914447C>T
g.215049723C>T
-
-
ABCA12_000019
-
PubMed: Thomas 2006
-
-
Germline
yes
-
-
-
-
Marianne Vos (LOVD-team)
+/.
1
6
c.682C>T
r.(?)
p.(Gln228*)
-
pathogenic
g.215914361G>A
g.215049637G>A
-
-
ABCA12_000018
-
PubMed: Thomas 2006
-
-
Germline
yes
-
-
-
-
Marianne Vos (LOVD-team)
-/.
1
-
c.709A>C
r.(?)
p.(Asn237His)
-
benign
g.215910724T>G
g.215046000T>G
-
-
ABCA12_000089
5 homozygous; Clinindb (India)
PubMed: Narang 2020 , Journal: Narang 2020
-
rs11890512
Germline
-
5/2794 individuals
-
-
-
Mohammed Faruq
+/.
3
7
c.859C>T
r.(?)
p.(Arg287*), p.(Arg287Ter)
-
pathogenic
g.215910574G>A
g.215045850G>A
-
-
ABCA12_000036
VKGL data sharing initiative Nederland
PubMed: Castiglia 2009
-
-
CLASSIFICATION record, Germline, Uniparental disomy, paternal allele
yes
-
-
-
-
Marianne Vos (LOVD-team) , VKGL-NL_Nijmegen
+/.
1
7i_8i
c.(872+1_873-1)_(985+1_986-1)del
r.(del)
p.?
-
pathogenic
g.(215896621_215901676)_(215901790215910560)del
-
del exon 8
-
ABCA12_000007
-
PubMed: Thomas 2006
-
-
Germline
yes
-
-
-
-
Marianne Vos (LOVD-team)
-/.
2
8
c.888G>A
r.(?)
p.(=), p.(Val296=)
-
benign
g.215901774C>T
g.215037050C>T
V296V
-
ABCA12_000008
VKGL data sharing initiative Nederland
PubMed: Kelsell 2005
-
-
CLASSIFICATION record, Germline
yes
-
-
-
-
Marianne Vos (LOVD-team) , VKGL-NL_Nijmegen
-/.
1
8i
c.985+85T>C
r.(=)
p.(=)
-
benign
g.215901592A>G
g.215036868A>G
-
-
ABCA12_000009
-
PubMed: Kelsell 2005
-
-
Germline
yes
-
-
-
-
Marianne Vos (LOVD-team)
+/.
1
9
c.1033A>C
r.(?)
p.(Thr345Pro)
-
pathogenic
g.215896573T>G
g.215031849T>G
-
-
ABCA12_000037
-
PubMed: Natsuga 2007
-
-
Germline
yes
-
-
-
-
Marianne Vos (LOVD-team)
+/.
1
9
c.1060C>T
r.(?)
p.(Gln354*)
-
pathogenic
g.215896546G>A
g.215031822G>A
-
-
ABCA12_000022
unknown variant 2nd chromosome
PubMed: Thomas 2006
-
-
Germline
yes
-
-
-
-
Marianne Vos (LOVD-team)
+/.
1
10
c.1131C>G
r.(?)
p.(Tyr377*)
-
pathogenic
g.215891593G>C
g.215026869G>C
-
-
ABCA12_000025
-
PubMed: Thomas 2006
-
-
Germline
yes
-
-
-
-
Marianne Vos (LOVD-team)
+/.
1
10
c.1160G>A
r.(?)
p.(Ser387Asn)
-
pathogenic
g.215891564C>T
g.215026840C>T
-
-
ABCA12_000039
-
PubMed: Akiyama 2006
-
-
De novo
yes
-
-
-
-
Marianne Vos (LOVD-team)
?/.
1
-
c.1235A>G
r.(?)
p.(Asn412Ser)
-
VUS
g.215890449T>C
-
ABCA12(NM_173076.2):c.1235A>G (p.N412S)
-
ABCA12_000097
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
12
c.1300C>T
r.(?)
p.(Arg434*)
-
pathogenic
g.215884508G>A
g.215019784G>A
-
-
ABCA12_000044
-
PubMed: Akiyama 2005
-
-
Germline
yes
-
-
-
-
Marianne Vos (LOVD-team)
-?/.
1
-
c.1607T>C
r.(?)
p.(Ile536Thr)
-
likely benign
g.215884110A>G
g.215019386A>G
ABCA12(NM_015657.3):c.653T>C (p.(Ile218Thr))
-
ABCA12_000088
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
-
c.1625T>C
r.(?)
p.(Val542Ala)
-
VUS
g.215884092A>G
g.215019368A>G
-
-
ABCA12_000087
6 heterozygous, no homozygous; Clinindb (India)
PubMed: Narang 2020 , Journal: Narang 2020
-
rs114651183
Germline
-
6/2794 individuals
-
-
-
Mohammed Faruq
+/.
1
15
c.1803G>A
r.(?)
p.(Trp601*)
-
pathogenic
g.215880367C>T
g.215015643C>T
-
-
ABCA12_000032
-
PubMed: Thomas 2008
-
-
Germline
yes
-
-
-
-
Marianne Vos (LOVD-team)
+?/.
1
-
c.1866del
r.(?)
p.(Cys622*)
ACMG
likely pathogenic
g.215880304del
g.215015580del
-
-
ABCA12_000058
-
PubMed: Trujillano 2017
-
-
Germline
-
-
-
-
-
Daniel Trujillano
?/.
1
-
c.1889C>A
r.(?)
p.(Ser630Tyr)
-
VUS
g.215880281G>T
-
ABCA12(NM_173076.2):c.1889C>A (p.S630Y)
-
ABCA12_000103
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/.
1
-
c.1923G>C
r.(?)
p.(Leu641=)
-
benign
g.215880247C>G
g.215015523C>G
ABCA12(NM_173076.3):c.1923G>C (p.L641=)
-
ABCA12_000065
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/.
1
16
c.2021_2022del
r.(?)
p.(Lys674Argfs*49)
-
pathogenic
g.215876796_215876797del
g.215012072_215012073del
2021_2022delAA
-
ABCA12_000043
-
PubMed: Akiyama 2005
-
-
Germline
yes
-
-
-
-
Marianne Vos (LOVD-team)
+/.
2
16
c.2025del
r.(?)
p.(Ile676Phefs*13)
-
pathogenic
g.215876791del
g.215012067del
-
-
ABCA12_000024
unknown variant 2nd chromosome
PubMed: Thomas 2006
-
-
Germline
yes
-
-
-
-
Marianne Vos (LOVD-team)
-/.
1
-
c.2124A>G
r.(?)
p.(Ala708=)
-
benign
g.215876371T>C
g.215011647T>C
-
-
ABCA12_000082
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
17
c.2140C>T
r.(?)
p.(Arg714*)
-
pathogenic
g.215876355G>A
g.215011631G>A
-
-
ABCA12_000016
-
PubMed: Thomas 2006
-
-
Germline
yes
-
-
-
-
Marianne Vos (LOVD-team)
+/.
1
17
c.2273dup
r.(?)
p.(Leu758Phefs*4)
-
pathogenic
g.215876227dup
g.215011503dup
2274insT
-
ABCA12_000023
unknown variant 2nd chromosome
PubMed: Thomas 2006
-
-
Germline
yes
-
-
-
-
Marianne Vos (LOVD-team)
-/., -?/.
2
-
c.2329T>A
r.(?)
p.(Ser777Thr)
-
benign, likely benign
g.215876166A>T
g.215011442A>T
ABCA12(NM_173076.3):c.2329T>A (p.S777T)
-
ABCA12_000064
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen , VKGL-NL_Nijmegen
?/.
2
-
c.2452A>G
r.(?)
p.(Thr818Ala)
-
VUS
g.215875075T>C
g.215010351T>C
ABCA12(NM_173076.2):c.2452A>G (p.T818A, p.(Thr818Ala))
-
ABCA12_000086
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden , VKGL-NL_Rotterdam
-?/.
1
-
c.2538G>A
r.(?)
p.(Ser846=)
-
likely benign
g.215872505C>T
g.215007781C>T
-
-
ABCA12_000081
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/.
1
-
c.3033A>G
r.(?)
p.(Pro1011=)
-
benign
g.215865575T>C
g.215000851T>C
-
-
ABCA12_000080
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
-
c.3180-6T>G
r.(=)
p.(=)
-
VUS
g.215862539A>C
g.214997815A>C
-
-
ABCA12_000079
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
22i_23i
c.(3179+1_3180-1)_(3294+1_3295-1)del
r.(del)
p.?
-
pathogenic
g.(215855756_215862418)_(215862534_15865428)del
-
del exon 23
-
ABCA12_000001
-
PubMed: Kelsell 2005
-
-
Germline
yes
-
-
-
-
Marianne Vos (LOVD-team)
+/.
2
23
c.3270T>G
r.(?)
p.(Tyr1090*)
-
pathogenic
g.215862443A>C
g.214997719A>C
-
-
ABCA12_000033
-
PubMed: Thomas 2008
-
-
Germline
yes
-
-
-
-
Marianne Vos (LOVD-team)
-?/.
1
-
c.3276A>G
r.(?)
p.(Lys1092=)
-
likely benign
g.215862437T>C
-
ABCA12(NM_173076.2):c.3276A>G (p.K1092=)
-
ABCA12_000101
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
2
23i
c.3295-2A>G
r.spl, r.[3295_3303del; 3295_3464del]
p.?, p.[Tyr1099_Lys1101del; Tyr1099Leufs*44]
-
pathogenic
g.215855757T>C
g.214991033T>C
IVS23-2A>G
-
ABCA12_000041
-
PubMed: Akiyama 2005
-
-
Germline
yes
-
-
-
-
Johan den Dunnen , Marianne Vos (LOVD-team)
+/.
1
24
c.3407G>A
r.(?)
p.(Gly1136Asp)
-
pathogenic
g.215855643C>T
g.214990919C>T
-
-
ABCA12_000045
-
PubMed: Akiyama 2008
-
-
Germline
yes
-
-
-
-
Marianne Vos (LOVD-team)
+/.
1
24
c.3535G>A
r.(?)
p.(Gly1179Arg)
-
pathogenic
g.215855515C>T
g.214990791C>T
-
-
ABCA12_000017
-
PubMed: Thomas 2006
-
-
Germline
yes
-
-
-
-
Marianne Vos (LOVD-team)
+/.
1
-
c.3539G>C
r.(?)
p.(Ser1180Thr)
-
pathogenic (dominant)
g.215855511C>G
-
G3539C
-
ABCA12_000098
-
PubMed: Cao 2020
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.3666C>A
r.(?)
p.(Tyr1222*)
ACMG
likely pathogenic
g.215854304G>T
g.214989580G>T
-
-
ABCA12_000059
-
PubMed: Trujillano 2017
-
-
Germline
-
-
-
-
-
Daniel Trujillano
+/.
1
25
c.3673C>T
r.(?)
p.(Arg1225*)
-
pathogenic
g.215854297G>A
g.214989573G>A
-
-
ABCA12_000034
-
PubMed: Thomas 2008
-
-
Germline
yes
-
-
-
-
Marianne Vos (LOVD-team)
+/.
1
26
c.3704G>C
r.(?)
p.(Trp1235Ser)
-
pathogenic
g.215854178C>G
g.214989454C>G
Trp1235Ser
-
ABCA12_000050
-
PubMed: Sakai 2009
-
-
Germline
?
-
-
-
-
Marianne Vos (LOVD-team)
-/.
1
-
c.3726G>A
r.(?)
p.(Pro1242=)
-
benign
g.215854156C>T
g.214989432C>T
-
-
ABCA12_000078
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
2
26
c.3746C>A
r.(?)
p.(Ser1249*)
-
pathogenic
g.215854136G>T
g.214989412G>T
Ser1249Term
-
ABCA12_000014
-
PubMed: Akiyama 2007 , PubMed: Thomas 2006
-
-
Germline
yes
-
-
-
-
Marianne Vos (LOVD-team)
+/.
1
26i
c.3829+1G>A
r.(?)
p.?
-
pathogenic
g.215854052C>T
g.214989328C>T
-
-
ABCA12_000027
-
PubMed: Thomas 2008
-
-
Germline
yes
-
-
-
-
Marianne Vos (LOVD-team)
+/.
1
27
c.3882G>A
r.(?)
p.(Trp1294*)
-
pathogenic
g.215560710C>T
g.214695986C>T
4102G>A (W1293*)
-
ABCA12_000049
-
PubMed: Rajpar 2006
-
-
Germline
yes
-
-
-
-
Marianne Vos (LOVD-team)
+/.
1
27
c.3891G>A
r.(?)
p.(Arg1297*)
-
pathogenic
g.215852456C>T
g.214987732C>T
-
-
ABCA12_000015
1 more item
PubMed: Thomas 2006
-
-
Germline
yes
-
-
-
-
Marianne Vos (LOVD-team)
?/.
1
-
c.3895G>A
r.(?)
p.(Gly1299Arg)
-
VUS
g.215852452C>T
-
ABCA12(NM_173076.3):c.3895G>A (p.(Gly1299Arg))
-
ABCA12_000131
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
1
27i_53i
c.(3976+1_3977-1)_(*1_?)del
r.(del)
p.?
-
pathogenic
g.(?_215797357)_(215851453_215852370)del
-
del exon 28-53
-
ABCA12_000004
-
PubMed: Kelsell 2005
-
-
Germline
yes
-
-
-
-
Marianne Vos (LOVD-team)
-/.
1
-
c.4126T>C
r.(?)
p.(Leu1376=)
-
benign
g.215851303A>G
g.214986579A>G
-
-
ABCA12_000076
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/., +?/.
7
28
c.4139A>G
r.(?)
p.(Asn1380Ser)
-
likely pathogenic, pathogenic
g.215851290T>C
g.214986566T>C
ABCA12(NM_173076.2):c.4139A>G (p.N1380S)
-
ABCA12_000055
VKGL data sharing initiative Nederland
PubMed: Lefevre 2003
-
-
CLASSIFICATION record, Germline
yes
-
-
-
-
Marianne Vos (LOVD-team) , VKGL-NL_Rotterdam , VKGL-NL_Nijmegen
+/.
1
28
c.4142G>A
r.(?)
p.(Gly1381Glu)
-
pathogenic
g.215851287C>T
g.214986563C>T
-
-
ABCA12_000053
-
PubMed: Lefevre 2003
-
-
Germline
yes
-
-
-
-
Marianne Vos (LOVD-team)
+/.
2
28
c.4158_4160del
r.(?)
p.(Thr1387del)
-
pathogenic
g.215851274_215851276del
g.214986550_214986552del
4158_4160delTAC
-
ABCA12_000040
-
PubMed: Akiyama 2005 , PubMed: Akiyama 2006
-
-
Germline
yes
-
-
-
-
Marianne Vos (LOVD-team)
-/.
1
-
c.4163+20T>C
r.(=)
p.(=)
-
benign
g.215851246A>G
g.214986522A>G
ABCA12(NM_173076.3):c.4163+20T>C
-
ABCA12_000063
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+?/.
1
-
c.4178G>A
r.(?)
p.(Gly1393Glu)
-
likely pathogenic
g.215848575C>T
-
ABCA12(NM_173076.3):c.4178G>A (p.(Gly1393Glu))
-
ABCA12_000130
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-/.
1
-
c.4225A>G
r.(?)
p.(Ile1409Val)
-
benign
g.215848528T>C
g.214983804T>C
-
-
ABCA12_000074
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
29
c.4262del
r.(?)
p.(Gly1421Glufs*39)
-
pathogenic
g.215848493del
g.214983769del
-
-
ABCA12_000029
-
PubMed: Thomas 2008
-
-
Germline
yes
-
-
-
-
Marianne Vos (LOVD-team)
+/.
1
30
c.4481T>C
r.(?)
p.(Ile1494Thr)
-
pathogenic
g.215847009A>G
g.214982285A>G
-
-
ABCA12_000038
no variant found 2nd chromosome
PubMed: Natsuga 2007
-
-
Germline
yes
-
-
-
-
Marianne Vos (LOVD-team)
+?/.
1
-
c.4538C>G
r.(?)
p.(Ser1513Cys)
-
likely pathogenic
g.215846952G>C
g.214982228G>C
-
-
ABCA12_000073
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
2
30
c.4541G>A
r.(?)
p.(Arg1514His)
-
pathogenic
g.215846949C>T
g.214982225C>T
Arg1514His
-
ABCA12_000052
no variant found 2nd chromosome
PubMed: Lefevre 2003 , PubMed: Sakai 2009
-
-
Germline
?, yes
-
-
-
-
Marianne Vos (LOVD-team)
?/.
1
-
c.4579+4T>G
r.spl?
p.?
-
VUS
g.215846907A>C
-
ABCA12(NM_173076.3):c.4579+4T>G
-
ABCA12_000122
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
-
c.4593T>G
r.(?)
p.(Ile1531Met)
-
VUS
g.215845354A>C
-
ABCA12(NM_173076.3):c.4593T>G (p.I1531M)
-
ABCA12_000094
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+/.
1
31
c.4615G>A
r.(?)
p.(Glu1539Lys)
-
pathogenic
g.215845332C>T
g.214980608C>T
-
-
ABCA12_000057
-
PubMed: Lefevre 2003
-
-
Germline
yes
-
-
-
-
Marianne Vos (LOVD-team)
?/.
1
-
c.4618G>T
r.(?)
p.(Ala1540Ser)
-
VUS
g.215845329C>A
-
ABCA12(NM_173076.2):c.4618G>T (p.(Ala1540Ser))
-
ABCA12_000117
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
-
c.4640T>C
r.(?)
p.(Ile1547Thr)
-
VUS
g.215845307A>G
-
ABCA12(NM_173076.3):c.4640T>C (p.(Ile1547Thr))
-
ABCA12_000115
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
1
-
c.4707C>T
r.(?)
p.(Gly1569=)
-
likely benign
g.215845240G>A
-
ABCA12(NM_173076.2):c.4707C>T (p.G1569=)
-
ABCA12_000096
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/.
1
-
c.4941C>T
r.(?)
p.(Ile1647=)
-
benign
g.215843564G>A
g.214978840G>A
ABCA12(NM_173076.2):c.4941C>T (p.I1647=)
-
ABCA12_000062
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
2
32
c.4951G>A
r.(?)
p.(Glu1651Ser), p.(Gly1651Ser)
-
pathogenic
g.215843554C>T
g.214978830C>T
4851G>A
-
ABCA12_000056
-
PubMed: Lefevre 2003
-
-
Germline
yes
-
-
-
-
Marianne Vos (LOVD-team)
+/.
1
33
c.5005C>T
r.(?)
p.(Gln1669*)
-
pathogenic
g.215843163G>A
g.214978439G>A
-
-
ABCA12_000046
-
PubMed: Akiyama 2008
-
-
Germline
yes
-
-
-
-
Marianne Vos (LOVD-team)
+/.
1
33
c.5012del
r.(?)
p.(Asn1671Ilefs*4)
-
pathogenic
g.215843162del
g.214978438del
5229delA
-
ABCA12_000005
-
PubMed: Kelsell 2005
-
-
Germline
yes
-
-
-
-
Marianne Vos (LOVD-team)
+/.
1
33_33i
c.5126_5128+1del
r.spl
p.?
-
pathogenic
g.215843040_215843043del
g.214978316_214978319del
5125_5128delGATG
-
ABCA12_000028
no variant found 2nd chromosome
PubMed: Thomas 2008
-
-
Germline
yes
-
-
-
-
Marianne Vos (LOVD-team)
?/.
1
-
c.5204A>G
r.(?)
p.(Lys1735Arg)
-
VUS
g.215840686T>C
-
ABCA12(NM_173076.2):c.5204A>G (p.K1735R)
-
ABCA12_000093
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.5219C>T
r.(?)
p.(Thr1740Ile)
-
VUS
g.215840671G>A
-
ABCA12(NM_173076.3):c.5219C>T (p.T1740I)
-
ABCA12_000111
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+/.
2
34
c.5231G>A
r.(?)
p.(Trp1744*)
-
pathogenic
g.215840659C>T
g.214975935C>T
-
-
ABCA12_000012
-
PubMed: Kelsell 2005 , PubMed: Thomas 2006
-
-
Germline
yes
-
-
-
-
Marianne Vos (LOVD-team)
?/.
1
-
c.5345C>G
r.(?)
p.(Ser1782Cys)
-
VUS
g.215840545G>C
-
ABCA12(NM_173076.3):c.5345C>G (p.(Ser1782Cys))
-
ABCA12_000129
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
1
34i
c.5381+3_5381+4del
r.(spl?)
p.?
-
pathogenic
g.215840505_215840506del
g.214975781_214975782del
5381+3delAA
-
ABCA12_000035
-
PubMed: Thomas 2008
-
-
Germline
yes
-
-
-
-
Marianne Vos (LOVD-team)
+/.
1
35
c.5393C>T
r.(?)
p.(Pro1798Leu)
-
pathogenic
g.215839577G>A
g.214974853G>A
Pro1798Leu
-
ABCA12_000047
unknown variant found 2nd chromosome
PubMed: Sakai 2009
-
-
Germline
?
-
-
-
-
Marianne Vos (LOVD-team)
-/.
1
-
c.5617G>A
r.(?)
p.(Val1873Ile)
-
benign
g.215835070C>T
g.214970346C>T
ABCA12(NM_173076.2):c.5617G>A (p.V1873I)
-
ABCA12_000061
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
37
c.5641C>T
r.(?)
p.(Arg1881*)
-
pathogenic
g.215835046G>A
g.214970322G>A
-
-
ABCA12_000021
-
PubMed: Thomas 2006
-
-
Germline
yes
-
-
-
-
Marianne Vos (LOVD-team)
+/.
1
37
c.5690G>C
r.(spl?)
p.(Arg1897Thr)
-
pathogenic
g.215834997C>G
g.214970273C>G
-
-
ABCA12_000031
no variant found 2nd chromosome
PubMed: Thomas 2008
-
-
Germline
yes
-
-
-
-
Marianne Vos (LOVD-team)
+/.
2
39
c.5848C>T
r.(?)
p.(Arg1950*)
-
pathogenic
g.215831608G>A
g.214966884G>A
Arg1950X
-
ABCA12_000042
-
PubMed: Akiyama 2005 , PubMed: Sakai 2009
-
-
Germline
?, yes
-
-
-
-
Marianne Vos (LOVD-team)
+/.
1
40
c.5939C>A
r.(?)
p.(Thr1980Lys)
-
pathogenic
g.215823748G>T
g.214959024G>T
Thr1980Lys
-
ABCA12_000048
unknown variant found 2nd chromosome
PubMed: Sakai 2009
-
-
Germline
?
-
-
-
-
Marianne Vos (LOVD-team)
+?/., ?/.
2
-
c.6091T>G
r.(?)
p.(Trp2031Gly)
-
likely pathogenic, VUS
g.215823027A>C
g.214958303A>C
ABCA12(NM_173076.3):c.6091T>G (p.W2031G)
-
ABCA12_000060
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht , VKGL-NL_Nijmegen
+/.
1
42
c.6161_6162del
r.(?)
p.(Ala2054Aspfs*10)
-
pathogenic
g.(215821458_215797357)_(215851453215821459)del
g.214956734_214956735del
6378delGC
-
ABCA12_000003
-
PubMed: Kelsell 2005
-
-
Germline
yes
-
-
-
-
Marianne Vos (LOVD-team)
+/.
1
42i
c.6233+1G>T
r.(spl?)
p.?
-
pathogenic
g.215821386C>A
g.214956662C>A
-
-
ABCA12_000011
-
PubMed: Kelsell 2005
-
-
Germline
yes
-
-
-
-
Marianne Vos (LOVD-team)
+/.
1
-
c.6234-1G>C
r.6234_6393del
p.Tyr2079Leufs*2
-
pathogenic (recessive)
g.215820086C>G
g.214955362C>G
-
-
ABCA12_000106
transcripts cycloheximide sensitive
PubMed: Bournazos 2022
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
2
-
c.6306C>T
r.(?)
p.(Tyr2102=)
-
benign
g.215820013G>A
g.214955289G>A
ABCA12(NM_173076.3):c.6306C>T (p.Y2102=)
-
ABCA12_000072
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht , VKGL-NL_Nijmegen
?/.
1
-
c.6361T>C
r.(?)
p.(Tyr2121His)
-
VUS
g.215819958A>G
-
ABCA12(NM_173076.3):c.6361T>C (p.(Tyr2121His))
-
ABCA12_000128
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
1
-
c.6386A>G
r.(?)
p.(Asn2129Ser)
-
likely benign
g.215819933T>C
-
ABCA12(NM_173076.2):c.6386A>G (p.N2129S)
-
ABCA12_000092
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
44
c.6443_6445del
r.(?)
p.(Pro2141del)
-
pathogenic
g.215818781_215818783del
g.214954057_214954059del
-
-
ABCA12_000030
-
PubMed: Thomas 2008
-
-
Germline
yes
-
-
-
-
Marianne Vos (LOVD-team)
+/.
1
44
c.6481C>T
r.(?)
p.(Gln2161*)
-
pathogenic
g.215818744G>A
g.214954020G>A
-
-
ABCA12_000013
-
PubMed: Thomas 2006
-
-
Germline
yes
-
-
-
-
Marianne Vos (LOVD-team)
?/.
1
-
c.6529C>T
r.(?)
p.(Pro2177Ser)
-
VUS
g.215818696G>A
-
ABCA12(NM_173076.3):c.6529C>T (p.(Pro2177Ser))
-
ABCA12_000127
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
-
c.6530C>A
r.(?)
p.(Pro2177Gln)
-
VUS
g.215818695G>T
-
ABCA12(NM_173076.3):c.6530C>A (p.(Pro2177Gln))
-
ABCA12_000126
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
2
44
c.6610C>T
r.(?)
p.(Arg2204*), p.(Arg2204Ter)
-
pathogenic
g.215818615G>A
g.214953891G>A
R2203*
-
ABCA12_000010
VKGL data sharing initiative Nederland
PubMed: Kelsell 2005
-
-
CLASSIFICATION record, Germline
yes
-
-
-
-
Marianne Vos (LOVD-team) , VKGL-NL_Nijmegen
?/.
1
-
c.6962+5G>A
r.spl?
p.?
-
VUS
g.215813759C>T
-
ABCA12(NM_173076.3):c.6962+5G>A
-
ABCA12_000135
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden