Global Variome shared LOVD
ABCA4 (ATP-binding cassette, sub-family A (ABC1), m...)
LOVD v.3.0 Build 30b [
Current LOVD status
]
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Curator:
Stéphanie Cornelis
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Unique variants in the ABCA4 gene
The
expert classification
of the variants can be found
here
.
To other Inherited Retinal Disease gene variant database:
ABCA4
ABCC6
ABHD12
ACBD5
ADAM9
ADAMTS18
ADIPOR1
AGBL5
AHI1
AIPL1
ALMS1
ARHGEF18
ARL2BP
ARL3
ARL6
ARMS2
ASRGL1
ATF6
ATP6
ATXN7
BBIP1
BBS1
BBS10
BBS12
BBS2
BBS4
BBS5
BBS7
BBS9
BEST1
C12orf65
C1QTNF5
C2
C21orf2
C2orf71
C3
C8orf37
CA4
CABP4
CACNA1F
CACNA2D4
CAPN5
CC2D2A
CCT2
CDH23
CDH3
CDHR1
CEP164
CEP250
CEP290
CEP78
CERKL
CFB
CFH
CHM
CIB2
CLN3
CLRN1
CLUAP1
CNGA1
CNGA3
CNGB1
CNGB3
CNNM4
COL11A1
COL2A1
COL9A1
CRB1
CRX
CSPP1
CTNNA1
CYP4V2
DFNB31
DHDDS
DHX38
DMD
DRAM2
DTHD1
EFEMP1
ELOVL4
EMC1
ERCC6
EXOC2
EXOSC2
EYS
FAM161A
FBLN5
FLVCR1
FSCN2
FZD4
GBN3
GDF6
GNAT1
GNAT2
GNPTG
GPR125
GPR179
GPR98
GRK1
GRM6
GUCA1A
GUCA1B
GUCY2D
HARS
HGSNAT
HK1
HMCN1
HMX1
HTRA1
IDH3B
IFT140
IFT172
IFT27
IFT81
IMPDH1
IMPG1
IMPG2
INPP5E
INVS
IQCB1
ITM2B
JAG1
KCNJ13
KCNV2
KIAA1549
KIF11
KIZ
KLHL7
KSS
LAMA1
LCA5
LHON
LRAT
LRIT3
LRP5
LZTFL1
MAK
MAPKAPK3
MERTK
MFN2
MFRP
MFSD8
MIR204
MKKS
MKS1
MTTP
MVK
MYO7A
NBAS
NDP
NEK2
NEUROD1
NMNAT1
NPHP1
NPHP3
NPHP4
NR2E3
NR2F1
NRL
NYX
OAT
OFD1
OPA1
OPA3
OPN1LW
OPN1MW
OPN1SW
OR2W3
OTX2
PANK2
PAX2
PCDH15
PCYT1A
PDE6A
PDE6B
PDE6C
PDE6G
PDE6H
PDZD7
PEX1
PEX2
PEX7
PGK1
PHYH
PITPNM3
PLA2G5
PLK4
PNPLA6
POC1B
PRCD
PRDM13
PROM1
PRPF3
PRPF31
PRPF4
PRPF6
PRPF8
PRPH2
RAB28
RAX2
RB1
RBP3
RBP4
RCBTB1
RD3
RDH11
RDH12
RDH5
REEP6
RGR
RGS9
RGS9BP
RHO
RIMS1
RLBP1
ROM1
RP1
RP1L1
RP2
RP9
RPE65
RPGR
RPGRIP1
RPGRIP1L
RS1
RTN4IP1
SAG
SDCCAG8
SEMA4A
SLC24A1
SLC25A46
SLC7A14
SNRNP200
SPATA7
SPP2
TEAD1
TH
TIMM8A
TIMP3
TL1
TLR3
TLR4
TMEM126A
TMEM237
TOPORS
TP
TREX1
TRIM32
TRNT1
TRPM1
TS2
TSPAN12
TTC8
TTLL5
TTPA
TUB
TUBGCP4
TULP1
UNC119
USH1C
USH1G
USH2A
VCAN
WDPCP
WDR19
WFS1
ZNF408
ZNF423
ZNF513
The variants shown are described using the NM_000350.2 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
All list views have search fields which can be used to search data. You can search for a complete word or you can search for a part of a search term. If you enclose two or more words in double quotes, LOVD will search for the combination of those words only exactly in the order you specify. Note that search terms are case-insensitive and that wildcards such as * are treated as normal text! For all options, like "and", "or", and "not" searches, or searching for prefixes or suffixes, see the table below.
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.
2868 entries on 29 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
17i_50
c.(2653+1_2654-1)_(*1_?)del
r.?
p.?
ACMG
VUS
g.(?_94458792)_(94514514_94517188)del
g.(?_93993236)_(94048958_94051632)del
-
-
ABCA4_002890
ACMG PVS1_S, PM2_sup; severity category not assesed
Journal: Cornelis 2023
-
-
SUMMARY record
-
-
-
-
-
Stéphanie Cornelis
?/.
1
22i_50_
c.(3328+1_3523-1)_*400{0}
r.?
p.?
-
VUS
g.(?_94458393)_(94505684_94508316)del
g.(?_93992837)_(94040128_94042760)del
-
-
ABCA4_002595
-
PubMed: Cornelis 2024
,
Journal: Cornelis 2024
-
-
Unknown
-
-
-
-
-
Frans Cremers
+?/.
1
48i_50_
c.(6729+1_6730-1)_(*1_?)del
r.?
p.?
ACMG
likely pathogenic
g.(?_94458792)_(94461752_94463416)del
g.(?_93993236)_(93996196_93997860)del
-
-
NPHS2_000000
ACMG PVS1_S, PM2_sup, PM3_sup; severity category severe
Journal: Cornelis 2023
-
-
SUMMARY record
-
-
-
-
-
Stéphanie Cornelis
?/.
1
49i_50_
c.(6816+1_6817-1)_(*1_?)del
r.?
p.?
ACMG
VUS
g.(?_94458792)_(94458799_94461664)del
g.(?_93993236)_(93993243_93996108)del
-
-
ABCA4_002889
ACMG PVS1_S, PM2_sup; severity category not assesed
Journal: Cornelis 2023
-
-
SUMMARY record
-
-
-
-
-
Stéphanie Cornelis
?/.
1
44
c.*10C>A
r.(?)
p.?
ACMG
VUS
g.94458783G>T
g.93993227G>T
-
-
ABCA4_000874
ACMG PM3, PP3_m; severity category mild/moderate
Journal: Cornelis 2023
-
-
SUMMARY record
-
-
-
-
-
Stéphanie Cornelis
?/.
1
5
c.*55G>A
r.(?)
p.?
ACMG
VUS
g.94458738C>T
g.93993182C>T
-
-
ABCA4_001553
ACMG PM2_sup, PM3_sup, PP3_m; severity category benign
Journal: Cornelis 2023
-
-
SUMMARY record
-
-
-
-
-
Stéphanie Cornelis
?/.
1
_1_1i
c.-104_(66+1_67-1){0}
r.0?
p.0?
-
VUS
g.(94578623_94586535)_(94586602_?)del
g.(94113067_94120979)_(94121046_?)del
-
-
ABCA4_002758
-
PubMed: Cornelis 2024
,
Journal: Cornelis 2024
-
-
Germline
yes
-
-
-
-
Frans Cremers
+?/.
1
1_40i
c.-104_5714+250{0}
r.0?
p.0?
ACMG
likely pathogenic
g.94476106_94586955del
g.94010550_94121399del
c.-354_5714+250del
-
ABCA4_002291
ACMG PVS1, PM2_sup; severity category not assesed
Journal: Cornelis 2023
-
-
SUMMARY record
-
-
-
-
-
Stéphanie Cornelis
+?/.
1
24_50
c.3525_*400{0}
r.?
p.?
ACMG
likely pathogenic
g.94402743_94505682del
g.93937187_94040126del
c.3524_*56050del
-
ABCA4_002888
ACMG PVS1, PM2_sup; severity category not assesed
Journal: Cornelis 2023
-
-
SUMMARY record
-
-
-
-
-
Stéphanie Cornelis
+/., +?/.
2
39i, 39i_50_
c.5585-164_*400{0}
r.(?), r.?
p.(?), p.?
ACMG
likely pathogenic, pathogenic (recessive)
g.94457537_94476649del, g.94457539_94476651del
g.93991981_94011093del, g.93991983_94011095del
c.5585-164_*1256del, [5585-166_*1254del]
-
ABCA4_001552
ACMG PVS1, PM2_sup; severity category not assesed, no segregation analysis done
Journal: Cornelis 2023
,
PubMed: Bauwens 2019
-
-
SUMMARY record, Unknown
-
-
-
-
-
Stéphanie Cornelis
./.
1
-
c.-4263834_*647310del
r.?
p.?
-
pathogenic
g.93811483_98850435del
-
-
-
DPYD_000017
decreased gene dosage
PubMed: DDDS 2015
,
Journal: DDDS 2015
-
-
De novo
-
-
-
-
-
Johan den Dunnen
-?/., ?/.
2
1
c.-92C>T
r.(?)
p.(?)
ACMG
likely benign, VUS
g.94586693G>A
g.94121137G>A
c.-92C>T, Heterozygous
-
ABCA4_002258
ACMG PM2_sup, BP4_m; severity category not assesed
Journal: Cornelis 2023
,
PubMed: Goetz 2020
-
-
SUMMARY record, Unknown
-
-
-
-
-
Stéphanie Cornelis
-?/., ?/.
2
1
c.-14G>A
r.(?)
p.(?)
ACMG
likely benign, VUS
g.94586615C>T
g.94121059C>T
c.2588G>C p.Gly863Ala
-
ABCA4_002257
ACMG PM2_sup, BP4_m; severity category not assesed
Journal: Cornelis 2023
,
PubMed: Georgiou 2019
-
-
SUMMARY record, Unknown
-
-
-
-
-
Stéphanie Cornelis
+?/.
1
_1_24
c.(?_-1)_3525del
r.0?
p.0?
ACMG
likely pathogenic
g.94505681_(94586602_?)del
g.94040125_(94121046_?)del
-
-
ABCA4_002897
ACMG PVS1, PM2_sup; severity category not assesed
Journal: Cornelis 2023
-
-
SUMMARY record
-
-
-
-
-
Stéphanie Cornelis
+?/.
1
_1_50_
c.-104_*400{0}
r.0
p.0
-
likely pathogenic
g.(?_94458792)_(94586602_?)del
g.(?_93993236)_(94121046_?)del
c.(?_1)_(6822_?)del
-
ABCA4_000000
deletion encompassing region flanked by extragenic markers D1S435 and D1S2793
PubMed: Valverde 2006
-
-
Germline
-
-
-
-
-
Stéphanie Cornelis
+/.
1
14i_17i
c.(2160+1_2161-1)_(2653+1_2654-1)
r.(?)
p.(?)
-
pathogenic (recessive)
g.(94514514_94517188)_(94522379_94526092)del
g.(94048958_94051632)_(94056823_94060536)del
del ex15-17
-
ABCA4_002591
-
PubMed: Goetz 2020
-
-
Unknown
-
-
-
-
-
Stéphanie Cornelis
+/.
2
17i_50_
c.(2653+1_2654-1)_*400{0}
r.?
p.(Gly885Valfs*71), p.?
ACMG
pathogenic, pathogenic (recessive)
g.(?_94458393)_(94514514_94517188)del
g.(?_93992837)_(94048958_94051632)del
c.(2653+1_2654-1)_(*1_?)del
-
ABCA4_002592
-
PubMed: Khan 2020
,
PubMed: Tracewska 2019
-
-
Germline, Unknown
yes
0 (in-house database, ~5000 samples)
-
-
-
Anna Tracewska
+?/.
1
23i_50_
c.(3522+1_3523-1)_*400{0}
r.?
p.?
-
likely pathogenic
g.(?_94402743)_(94505684_94506764)del
g.(?_93937187)_(94040128_94041208)del
chr1:g.94402743_94505682del
-
ABCA4_002761
heterozygous
PubMed: Turro 2020
-
-
Germline/De novo (untested)
?
-
-
-
-
LOVD
+/.
1
49i_50_
c.(6817-1_6818+1)_*400{0}
r.?
p.?
-
pathogenic (recessive)
g.(?_94458393)_(94458796_94458799)del
g.(?_93992837)_93993240_93993243)del
c.(6816+1_6817-1)_(*1_?)del#
-
ABCA4_002590
-
PubMed: Jespersgaard 2019
-
-
Unknown
-
-
-
-
-
Stéphanie Cornelis
+?/.
1
-
c.0?
r.0?
p.(?)
-
likely pathogenic
g.94476106_94586955del
g.94010550_94121399del
ABCA4 chr1:94476106_94586955del
-
ABCA4_002291
range 110244-110849 bp in various techniques, heterozygous
PubMed: Zampaglione 2020
-
-
Unknown
?
-
-
-
-
LOVD
+/., +?/., -?/., ?/.
15
13, 23, 23i_50_, 36, 38, ?
c.?
r.(?), r.?
p.(?), p.(W439*), p.?
ACMG
likely benign, likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive),
1 more item
g.94452244_94471761del, g.94528142C>T, g.94586187_94587411del, g.?
-
11-basepair deletion in exon 38, 3896G>T intronic, 5226delC, c.1928G>A, c.3385C>T, D654N,
7 more items
-
ABCA4_000000, NPHS2_000000
ACMG PM2, PVS1, no variant 2nd chromosome
PubMed: Birch 2001
,
PubMed: Carss 2017
,
PubMed: Cideciyan 2009
,
PubMed: Downs 2007
,
PubMed: Oh 2004
,
8 more items
-
rs114572202
Germline, Unknown
?
-
-
-
-
Johan den Dunnen
,
Julia Lopez
,
Stéphanie Cornelis
+/., +?/.
41
1
c.1A>G
r.(1a>g), r.(?), r.?
p.(Met1?), p.(Met1Val), p.0?, p.?
ACMG
likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive)
g.94586601T>C
g.94121045T>C
1A>G, c.1A>G, c.1A>G (p.?), c.1A>G p.(M1?), c.1A>G p.(Met1?), c.1A>G p.Met1? het,
16 more items
-
ABCA4_000264
compound heterozygous, Homozygous, no segregation analysis done, no variant 2nd chromosome,
2 more items
Bianco 2023, submitted, Prevention Genetics, Sharon, submitted,
PubMed: Burke 2010
,
PubMed: Cai 2018
,
23 more items
-
-
CLASSIFICATION record, Germline, Germline/De novo (untested), SUMMARY record, Unknown
-, ?, yes
1/2420 IRD families
-
-
-
Global Variome, with Curator vacancy
,
Dror Sharon
,
Frans Cremers
,
Stéphanie Cornelis
,
VKGL-NL_Nijmegen
,
Rebekkah Hitti-Malin
,
Lorenzo Bianco
,
Zelia Corradi
+/., ?/.
2
1
c.1_3delinsGTC
r.(?)
p.(Met1?), p.0?
ACMG
pathogenic, VUS
g.94586599_94586601delinsGAC
g.94121043_94121045delinsGAC
ATG>GTC
-
ABCA4_000262
-
PubMed: Briggs 2001
,
PubMed: Cornelis 2017
,
Journal: Cornelis 2017
,
Journal: Cornelis 2023
-
-
Germline, SUMMARY record
-
-
-
-
-
Stéphanie Cornelis
+?/.
1
_1_50_
c.-104_*400{0}
r.0
p.0
ACMG
likely pathogenic (recessive)
g.(?_94458792)_(94586602_?)del
g.(?_93993236)_(94121046_?)del
del gene, c.(?_-1)_(*1_?)del
-
ABCA4_000862
deletion entire ABCA4 gene
PubMed: Cornelis 2017
,
Journal: Cornelis 2017
,
Journal: Cornelis 2023
-
-
SUMMARY record
-
-
-
-
-
Stéphanie Cornelis
+/., +?/.
5
1
c.2T>C
r.(2u>c), r.(?)
p.(=,?), p.(Met1?), p.0?
ACMG
likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive)
g.94586600A>G
g.94121044A>G
c.2T<C, Codon 1 ATG-ACG Met-Thr, M1T
-
ABCA4_000263
case unsolved, no variant 2nd chromosome
PubMed: Cornelis 2017
,
Journal: Cornelis 2017
,
Journal: Cornelis 2023
,
PubMed: Maia-Lopes 2008
,
3 more items
-
-
Germline, SUMMARY record, Unknown
-
2, 121346, 0, 0.00001648
-
-
-
Johan den Dunnen
,
Stéphanie Cornelis
+/., +?/.
3
1
c.3G>A
r.(3g>a), r.(?)
p.(Met1?), p.(Met1Ile), p.0?
ACMG
likely pathogenic, likely pathogenic (recessive), pathogenic (recessive)
g.94586599C>T
g.94121043C>T
c.3G>A
-
ABCA4_000261
no segregation analysis done,
2 more items
PubMed: Cornelis 2017
,
Journal: Cornelis 2017
,
Journal: Cornelis 2023
,
PubMed: Riveiro-Alvarez 2013
,
1 more item
-
-
Germline, SUMMARY record, Unknown
?
3, 121340, 0, 0.00002472
-
-
-
Stéphanie Cornelis
+/., ?/.
2
1
c.3G>T
r.(?)
p.(?), p.(Met1?)
ACMG
pathogenic (recessive), VUS
g.94586599C>A
g.94121043C>A
c.3G>T, Heterozygous
-
ABCA4_002256
ACMG PVS1_M, PM2_sup, PM5; severity category uncertain
Journal: Cornelis 2023
,
PubMed: Goetz 2020
-
-
SUMMARY record, Unknown
-
-
-
-
-
Stéphanie Cornelis
+/., +?/.
3
1
c.16C>T
r.(?)
p.(Gln6*), p.(Gln6Ter)
ACMG
likely pathogenic, pathogenic, pathogenic (recessive)
g.94586586G>A
g.94121030G>A
c.16C>T, p.Gln6Ter
-
ABCA4_002255
ACMG PVS1, PM2_sup, PP3_m; severity category severe
Journal: Cornelis 2023
,
PubMed: Fujinami 2019
,
PubMed: Hitti-Malin 2024
,
Journal: Hitti-Malin 2024
-
-
Germline, SUMMARY record, Unknown
-
-
-
-
-
Stéphanie Cornelis
,
Rebekkah Hitti-Malin
+?/.
1
-
c.18_19del
r.(?)
p.(Gln6HisfsTer47)
ACMG
likely pathogenic (recessive)
g.94586584_94586585del
g.94121028_94121029del
-
-
ABCA4_002370
-
-
-
-
Germline
-
-
-
-
-
Nancy Xilotl de Jesús
+?/., ?/.
2
1
c.20T>A
r.(?)
p.(Ile7Lys)
ACMG
likely pathogenic (recessive), VUS
g.94586582A>T
g.94121026A>T
c.20T>A, p.Ile7Lys Heterozygous
-
ABCA4_002254
ACMG PM2_sup; severity category uncertain
Journal: Cornelis 2023
,
PubMed: Goetz 2020
-
-
SUMMARY record, Unknown
-
-
-
-
-
Stéphanie Cornelis
+/.
5
1
c.21dup
r.(?)
p.(Gln8Hisfs*33), p.(Gln8ThrfsTer46)
ACMG
pathogenic, pathogenic (recessive)
g.94586581dup
g.94121025dup
c.20insA p.Q8fs, c.21_22insA,p.Gln8ThrfsTer46, p.[Gln8fs];c.[5461-10T>C]
-
ABCA4_002253
ACMG PVS1, PS4; severity category severe
Journal: Cornelis 2023
,
PubMed: Cornelis 2024
,
Journal: Cornelis 2024
,
PubMed: Fakin 2016
,
2 more items
-
-
Germline/De novo (untested), SUMMARY record, Unknown
yes
-
-
-
-
Stéphanie Cornelis
+/., +?/.
5
1
c.22C>T
r.(22c>u), r.(?)
p.(Gln8*), p.(Gln8Ter)
ACMG
likely pathogenic, pathogenic, pathogenic (recessive)
g.94586580G>A
g.94121024G>A
c.22C>T, c.22C>T p.(Gln8*)
-
ABCA4_000260
not statistically tested, classification unknown
PubMed: Cornelis 2017
,
Journal: Cornelis 2017
,
Journal: Cornelis 2023
,
PubMed: Dan 2019
,
2 more items
-
-
Germline, SUMMARY record, Unknown
?, yes
-
-
-
-
Stéphanie Cornelis
,
Handong Dan
,
Lu Tian
+/.
3
1
c.29dup
r.(?)
p.(Leu10Phefs*44), p.(Leu10PhefsTer44)
ACMG
pathogenic, pathogenic (recessive)
g.94586576dup
g.94121020dup
c.29_30insT p.Leu10PhefsTer44
-
ABCA4_002252
ACMG PVS1, PM2_sup, PP3_m; severity category not assesed, no variant 2nd chromosome
Journal: Cornelis 2023
,
PubMed: Cornelis 2024
,
Journal: Cornelis 2024
,
PubMed: Tanna 2019
-
-
Germline/De novo (untested), SUMMARY record, Unknown
-
-
-
-
-
Stéphanie Cornelis
+/., +?/., ?/.
15
1
c.32T>C
r.(32u>c), r.(?)
p.(Leu11Pro)
ACMG
likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive), VUS
g.94586570A>G
g.94121014A>G
32T>C, c.32T>C, c.32T>C p. Leu11Pro, c.32T>C p.(Leu11Pro), c.32T>C p.Leu11Pro het,
4 more items
-
ABCA4_000154
-
Prevention Genetics,
PubMed: Cideciyan 2009
,
PubMed: Cornelis 2024
,
Journal: Cornelis 2024
,
9 more items
-
-
Germline, SUMMARY record, Unknown
-, yes
1, 121404, 0, 0.000008237, ExAC 1, 121404, 0, 0.000008237
-
-
-
Frans Cremers
,
Stéphanie Cornelis
-?/., ?/.
2
1
c.33C>T
r.(?)
p.(Leu11=)
ACMG
likely benign, VUS
g.94586569G>A
g.94121013G>A
c.33C>T p.Leu11Leu Het
-
ABCA4_002251
ACMG BP4_m, BP7; severity category not assesed, no variant 2nd chromosome
Prevention Genetics,
Journal: Cornelis 2023
-
-
SUMMARY record, Unknown
-
-
-
-
-
Stéphanie Cornelis
+/.
2
1
c.36G>A
r.(?)
p.(Trp12*), p.(Trp12Ter)
ACMG
pathogenic, pathogenic (recessive)
g.94586566C>T
g.94121010C>T
c.36G>A p.W12*
-
ABCA4_002250
ACMG PVS1, PM2_sup, PM3_sup, PP3_m; severity category severe
Journal: Cornelis 2023
,
PubMed: Smaragda 2018
-
-
SUMMARY record, Unknown
-
-
-
-
-
Stéphanie Cornelis
+?/., ?/.
2
1
c.37A>G
r.(?)
p.(Lys13Glu), p.(Lys13Gly)
ACMG
likely pathogenic (recessive), VUS
g.94586565T>C
g.94121009T>C
c.37A>G, p.Lys13Glu Heterozygous
-
ABCA4_002249
ACMG PM2_sup, PP3_m; severity category not assesed
Journal: Cornelis 2023
,
PubMed: Goetz 2020
-
-
SUMMARY record, Unknown
-
-
-
-
-
Stéphanie Cornelis
?/.
3
1
c.38_46del
r.(38_46del), r.(?)
p.(Lys13_Trp15del)
ACMG
VUS
g.94586561_94586569del
g.94121005_94121013del
Deletion Lys13-Trp15
-
ABCA4_000258
-
PubMed: Briggs 2001
,
PubMed: Cornelis 2017
,
Journal: Cornelis 2017
,
Journal: Cornelis 2023
-
-
Germline, SUMMARY record
-
-
-
-
-
Stéphanie Cornelis
+/., +?/., ?/.
5
1
c.42C>A
r.(?)
p.(Asn14Lys)
ACMG
likely pathogenic, likely pathogenic (recessive), pathogenic (recessive), VUS
g.94586560G>T
g.94121004G>T
c.42C>A, p.N14K
-
ABCA4_001330
ACMG PS4, PM3, PP3; severity category mild/moderate, no segregation analysis done
Journal: Cornelis 2023
,
PubMed: Hu 2019
,
PubMed: Jiang 2016
,
PubMed: Tian 2024
-
-
Germline, SUMMARY record, Unknown
-
-
-
-
-
Stéphanie Cornelis
,
Fangyuan Hu
,
Lu Tian
+/.
4
1
c.42_48delinsAG
r.(?)
p.(Asn14Lysfs*38), p.(Asn14LysfsTer38)
ACMG
pathogenic, pathogenic (recessive)
g.94586554_94586560delinsCT
g.94120998_94121004delinsCT
c.42_48delinsAG p.Asn14Lysfs*38 het, c.42_48delinsAG, p.Asn14Lysfs*38 Heterozygous
-
ABCA4_002248
ACMG PVS1, PS4, PP3_m; severity category severe
Prevention Genetics,
Journal: Cornelis 2023
,
PubMed: Cornelis 2024
,
Journal: Cornelis 2024
,
1 more item
-
-
Germline/De novo (untested), SUMMARY record, Unknown
-
-
-
-
-
Stéphanie Cornelis
+/.
2
1
c.43_47del
r.(43_47del), r.(?)
p.(Trp15Profs*37), p.(Trp15ProfsTer37)
ACMG
pathogenic, pathogenic (recessive)
g.94586557_94586561del
g.94121001_94121005del
c.43_48del6insC
-
ABCA4_000257
-
PubMed: Cornelis 2017
,
Journal: Cornelis 2017
,
Journal: Cornelis 2023
,
PubMed: Zhao 2015
-
-
Germline, SUMMARY record
?
-
-
-
-
Stéphanie Cornelis
+/., +?/.
21
1
c.45G>A
r.(45g>a), r.(?)
p.(Trp15*), p.(Trp15Ter)
ACMG
likely pathogenic, pathogenic, pathogenic (recessive)
g.94586557C>T
g.94121001C>T
c.45G>A, c.45G>A (p.Trp15*), c.45G>A p.(W15*), c.45G>A, p.Trp15Stop Heterozygous, p.Trp15*,
5 more items
-
ABCA4_000259
ACMG PM2, PVS1, PP5, no segregation analysis done, no variant 2nd chromosome
PubMed: Birtel 2018
,
PubMed: Cornelis 2017
,
Journal: Cornelis 2017
,
Journal: Cornelis 2023
,
12 more items
-
-
Germline, SUMMARY record, Unknown
-, ?, yes
-
-
-
-
Johan den Dunnen
,
Stéphanie Cornelis
+/.
2
1
c.46_47del
r.(?)
p.(Thr16Profs*37), p.(Thr16ProfsTer37)
ACMG
pathogenic, pathogenic (recessive)
g.94586555_94586556del
g.94120999_94121000del
c.46_47del
-
ABCA4_002513
ACMG PVS1, PM2_sup, PP3_m; severity category not assesed
Journal: Cornelis 2023
,
PubMed: Khan 2020
-
-
SUMMARY record, Unknown
-
-
-
-
-
Stéphanie Cornelis
+/., +?/., ?/.
45
1
c.52C>T
r.(52c>u), r.(?)
p.(Arg18Trp)
ACMG
likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive), VUS
g.94586550G>A
g.94120994G>A
52C>T, Arg18Trp, C > T transition at nucleotide 52, c.52C> T, p.R18W, c.52C>T, c.52C>T,
14 more items
-
ABCA4_000037
ACMG PP3, PM2, PM5, PP2, PP5_STRONG, combination of variants not reported, no variant 2nd chromosome,
1 more item
PubMed: Birtel 2018
,
PubMed: Braun 2013
,
PubMed: Bravo-Gil 2017
,
PubMed: Chacón-Camacho 2013
,
24 more items
1048166
-
Germline, Germline/De novo (untested), SUMMARY record, Unknown
-, ?, yes
3, 121392, 0, 0.00002471, ExAC 3, 121392, 0, 0.00002471
-
-
-
Johan den Dunnen
,
Frans Cremers
,
Stéphanie Cornelis
,
Nereida Bravo Gil
,
Zelia Corradi
,
Oscar Francisco Chacón Camacho
+/., +?/.
11
1
c.53G>A
r.(?)
p.(Arg18Gln)
ACMG
likely pathogenic, likely pathogenic (recessive), pathogenic (recessive)
g.94586549C>T
g.94120993C>T
c.4773+1 G>Ac.53 G>A, c.53G>A, c.53G>A p.(Arg18Gln), c.53G>A p.Arg18Gln,
1 more item
-
ABCA4_001028
ACMG PS4, PM3_sup, PM5, PP3; severity category severe
Journal: Cornelis 2023
,
PubMed: Cornelis 2024
,
Journal: Cornelis 2024
,
PubMed: Georgiou 2019
,
5 more items
-
rs868543294
Germline, Germline/De novo (untested), SUMMARY record, Unknown
yes
-
-
-
-
Frans Cremers
,
Stéphanie Cornelis
,
Marco Nassisi
,
Lu Tian
+/., +?/., ?/.
17
1
c.53G>C
r.(?)
p.(Arg18Pro)
ACMG
likely pathogenic (recessive), pathogenic, pathogenic (recessive), VUS
g.94586549C>G
g.94120993C>G
ABCA4 c.53G>C, p.R18P, c.53G>C, c.53G>C p.(Arg18Pro), p.R18P
-
ABCA4_001329
ACMG PS4, PM3_S, PM5, PP3_m; severity category moderate,
1 more item
Zixi Sun 2020, submitted,
Journal: Cornelis 2023
,
PubMed: Corradi 2023
,
Journal: Corradi 2023
,
6 more items
-
-
Germline, Germline/De novo (untested), SUMMARY record, Unknown
?, yes
-
-
-
-
Stéphanie Cornelis
,
Fangyuan Hu
,
Zixi Sun
,
Lu Tian
,
Zelia Corradi
+/., +?/.
11
1
c.61C>T
r.(61c>u), r.(?)
p.(Gln21*), p.(Gln21Ter)
ACMG
likely pathogenic, pathogenic, pathogenic (recessive)
g.94586541G>A
g.94120985G>A
c.61C>T, c.61C>T p.(Gln21*), c.61C>T; p.Gln21Ter, Het NM_000350.2: c.61C>T, ;Q21X
-
ABCA4_000256
VKGL data sharing initiative Nederland
PubMed: Abed 2018
,
PubMed: Cornelis 2017
,
Journal: Cornelis 2017
,
Journal: Cornelis 2023
,
6 more items
-
-
CLASSIFICATION record, Germline, SUMMARY record, Unknown
-, yes
0 (in-house database, ~5000 samples), 1, 121388, 0, 0.000008238
-
-
-
Stéphanie Cornelis
,
VKGL-NL_Nijmegen
,
Anna Tracewska
,
Zelia Corradi
+/., +?/., ?/.
9
1
c.66G>A
r.(?), r.spl, r.spl?
p.(=), p.(Lys22=), p.(Lys22=,?), p.[(Lys21=, ?)]
ACMG
likely pathogenic, pathogenic, pathogenic (recessive), VUS
g.94586536C>T
g.94120980C>T
c.66G>A (p.?), c.66G>A, p.Lys22Lys Heterozygous, c.[66G4A;859-9T4C], c.[66G>A;859-9T>C],
2 more items
-
ABCA4_000255, ABCA4_000871
ACMG PS4, PM3_S, PP3_m; severity category mild/moderate, no variant 2nd chromosome
Journal: Cornelis 2023
,
PubMed: Chen 2019
,
PubMed: Duncker 2015
,
PubMed: Gliem 2020
,
PubMed: Lee 2017
,
4 more items
-
-
Germline, SUMMARY record, Unknown
-, yes
0 (in-house database, ~5000 samples)
-
-
-
Jana Zernant
,
Stéphanie Cornelis
,
Anna Tracewska
+?/., ?/.
5
1
c.66G>T
r.(?), r.spl?
p.(Lys22Asn), p.(Lys22Asn,?)
ACMG
likely pathogenic, likely pathogenic (recessive), VUS
g.94586536C>A
g.94120980C>A
c.66G>T p.Lys22Asn, p.Lys22Ans
-
ABCA4_002247
ACMG PM2_sup, PP3; severity category not assesed
Journal: Cornelis 2023
,
PubMed: Hitti-Malin 2024
,
Journal: Hitti-Malin 2024
,
PubMed: Salles 2017
,
2 more items
-
-
Germline, SUMMARY record, Unknown
yes
-
-
-
-
Stéphanie Cornelis
,
Rebekkah Hitti-Malin
?/.
2
1i
c.66+3A>C
r.spl?
p.(?), p.?
ACMG
VUS
g.94586533T>G
g.94120977T>G
c.66+3A>C
-
ABCA4_000254
1 more item
PubMed: Cornelis 2017
,
Journal: Cornelis 2017
,
Journal: Cornelis 2023
,
PubMed: Ritter 2013
-
-
Germline, SUMMARY record
-
1, 121380, 0, 0.000008239
-
-
-
Stéphanie Cornelis
+?/., ?/.
2
1i
c.66+520_67-389dup
r.(?)
p.(?), p.?
ACMG
likely pathogenic (recessive), VUS
g.94579013_94586018dup
g.94113457_94120462dup
[66+520_67-389dup ]
-
ABCA4_002246
ACMG PM2_sup; severity category uncertain, no segregation analysis done
Journal: Cornelis 2023
,
PubMed: Bauwens 2019
-
-
SUMMARY record, Unknown
-
-
-
-
-
Stéphanie Cornelis
-?/., ?/.
2
1i
c.66+2044G>A
r.spl?
p.(?), p.?
ACMG
likely benign, VUS
g.94584492C>T
g.94118936C>T
m36: c.66+2044G>A; p.?
-
ABCA4_002281
ACMG BP4_m; severity category not assesed
Journal: Cornelis 2023
,
PubMed: Gonzalez del Pozo 2018
-
-
Germline, SUMMARY record
yes
-
-
-
-
Stéphanie Cornelis
+?/., ?/.
6
1i
c.67-2023T>G
r.(66_67ins67-2266_67-2024,=), r.[(66_67ins67-2266_67-2024,=)], r.[66_67ins67-2266_67-2024,=]
p.(IIe23IIefs*30,=), p.[(IIe23IIefs*30,=)], p.[IIe23IIefs*30,=], p.[IIe23IIefsTer30,=]
ACMG
likely pathogenic (recessive), VUS
g.94580645A>C
g.94115089A>C
c.67-2023T>G
-
ABCA4_002512
ACMG PS3_sup, PM2_sup, PM3, PP3; severity category moderate
Khan 2020,
Journal: Cornelis 2023
,
PubMed: Cornelis 2024
,
Journal: Cornelis 2024
-
-
Germline/De novo (untested), SUMMARY record, Unknown
-
-
-
-
-
Frans Cremers
,
Stéphanie Cornelis
+/., +?/.
2
1i, 1i_2i
c.67-1845_160+345del
r.(?)
p.(?), p.?
ACMG
likely pathogenic, pathogenic (recessive)
g.94578188_94580471del
g.94112632_94114915del
c.67-1845_c.160+345del (p.?)
-
ABCA4_002236
ACMG PVS1, PM2_sup; severity category severe, no segregation analysis done
Journal: Cornelis 2023
,
PubMed: Zernant 2017
-
-
SUMMARY record, Unknown
-
-
-
-
-
Stéphanie Cornelis
+/., ?/.
2
1i_6i
c.67-975_769-4582dup
r.(?)
p.(?), p.?
ACMG
pathogenic (recessive), VUS
g.94553580_94579598dup
g.94088024_94114042dup
[67-975_769-4582dup{insA}]
-
ABCA4_002166
ACMG PM2_sup, PM3, PM4; severity category severe
Journal: Cornelis 2023
,
PubMed: Bauwens 2019
-
-
SUMMARY record, Unknown
yes
-
-
-
-
Stéphanie Cornelis
+/., +?/.
3
1i_2i
c.67-140_161-355del
r.(67_160del)
p.(Ile23Alafs*24), p.(Ile23AlafsTer24)
ACMG
likely pathogenic, pathogenic, pathogenic (recessive)
g.94577492_94578764del
g.94111936_94113208del
-
-
ABCA4_002315
ACMG PVS1, PM2_sup; severity category not assesed
Journal: Cornelis 2023
,
PubMed: Tian 2022
,
PubMed: Tian 2022
,
PubMed: Tian 2024
-
-
Germline, SUMMARY record
-
-
-
-
-
Stéphanie Cornelis
,
Lu Tian
+/., +?/., ?/.
3
1i
c.67-16T>A
r.spl, r.spl?
p.(?), p.(Ile23AlafsTer24), p.?
ACMG
likely pathogenic (recessive), pathogenic (recessive), VUS
g.94578638A>T
g.94113082A>T
c.67-16T>A
-
ABCA4_002245
ACMG PM2_sup, PM3, PP3; severity category uncertain, no segregation analysis done
Journal: Cornelis 2023
,
PubMed: Jiang 2016
,
PubMed: Tian 2024
-
-
Germline, SUMMARY record, Unknown
yes
-
-
-
-
Stéphanie Cornelis
,
Lu Tian
+/., +?/.
9
1i
c.67-2A>G
r.spl
p.?
ACMG
likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive)
g.94578624T>C
g.94113068T>C
c.67-2A>G p.(?), c.67-2A>G(;)769-784C>T
-
ABCA4_001055
ACMG PM2, PVS1, PP5, ACMG PVS1, PM2_sup; severity category severe, no segregation analysis done,
1 more item
Journal: Cornelis 2023
,
PubMed: Cornelis 2024
,
Journal: Cornelis 2024
,
PubMed: Khan 2019
,
3 more items
92871
-
Germline, Germline/De novo (untested), SUMMARY record, Unknown
no
-
-
-
-
Johan den Dunnen
,
Stéphanie Cornelis
,
Rebekkah Hitti-Malin
+/.
8
1i
c.67-1del
r.spl
p.?
ACMG
pathogenic, pathogenic (recessive)
g.94578623del
g.94113067del
67-1delG, c.[5714+5G>A];[67-1delG]
-
ABCA4_001093
67-1delG, ACMG PVS1, PM2_sup, PP3_m; severity category severe
Journal: Cornelis 2023
,
PubMed: Green 2020
,
PubMed: Green 2020
,
Journal: Green 2020
-
-
Germline, SUMMARY record
yes
-
-
-
-
Terry-Lynn Young
,
Stéphanie Cornelis
,
Darren O'Rielly
+/.
1
-
c.67-1dup
r.spl?
p.?
ACMG
pathogenic (recessive)
g.94578623dup
g.94113067dup
-
-
ABCA4_002879
combination of variants not reported
-
-
-
Germline
-
-
-
-
-
Oscar Francisco Chacón Camacho
+/., +?/.
7
1i
c.67-1G>C
r.spl
p.?
ACMG
likely pathogenic, likely pathogenic (recessive), pathogenic (recessive)
g.94578623C>G
g.94113067C>G
c.67-1G>C IVS1-1 G>C, c.67-1G>C p.(I23Afs*24), c.67-1G>C, Heterozygous, IVS1-1G>C,
1 more item
-
ABCA4_001481
ACMG PVS1, PM2_sup; severity category severe, no variant 2nd chromosome
Journal: Cornelis 2023
,
PubMed: Cornelis 2024
,
Journal: Cornelis 2024
,
PubMed: Fujinami 2019
,
3 more items
-
-
Germline, SUMMARY record, Unknown
yes
-
-
-
-
Frans Cremers
,
Stéphanie Cornelis
+?/., ?/.
2
2
c.69T>G
r.spl?
p.(Ile23Met)
ACMG
likely pathogenic (recessive), VUS
g.94578620A>C
g.94113064A>C
c.69T>G, p.Ile23Met Heterozygous
-
ABCA4_002244
ACMG PM2_sup; severity category not assesed, no variant 2nd chromosome
Journal: Cornelis 2023
,
PubMed: Goetz 2020
-
-
SUMMARY record, Unknown
-
-
-
-
-
Stéphanie Cornelis
+/., +?/+?, +?/., ?/.
15
2
c.70C>T
r.(70c>u), r.(?)
p.(Arg24Cys), p.[IIe23IIefs*30,=]
ACMG
likely pathogenic, likely pathogenic (recessive), pathogenic (recessive), VUS
g.94578619G>A
g.94113063G>A
70C>T, c.70 C>T, c.70C>T Arg24Cys CGC>TGC, c.70C>T p.(Arg24Cys), c.70C>T p.Arg24Cys,
5 more items
-
ABCA4_000253
no segregation analysis done, no variant 2nd chromosome, VKGL data sharing initiative Nederland
Zixi Sun 2020, submitted,
PubMed: Cornelis 2017
,
Journal: Cornelis 2017
,
Journal: Cornelis 2023
,
10 more items
-
-
CLASSIFICATION record, Germline, Germline/De novo (untested), SUMMARY record, Unknown
?
5, 120400, 0, 0.00004153, ExAC 5, 120400, 0, 0.00004153
-
-
-
Johan den Dunnen
,
Stéphanie Cornelis
,
VKGL-NL_Nijmegen
,
Zixi Sun
+/., +?/., -?/., ?/.
55
1, 2
c.71G>A
r.(71g>a), r.(?)
p.(Arg24Cys), p.(Arg24His), p.(Met1Ile)
ACMG
likely benign, likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive),
1 more item
g.94578618C>T
g.94113062C>T
ABCA4 c.71G>A(;)1804C>T, V2: c.71G>A, (p.Arg24His), ABCA4 c.71G>A, p.R24H, c.71 G>A,
15 more items
-
ABCA4_000252
alleles in cis or trans; heterozygous, case unsolved, heterozygous, no segregation analysis done,
5 more items
Prevention Genetics,
PubMed: Bauwens 2014
,
PubMed: Bauwens 2019
,
PubMed: Chacón-Camacho 2013
,
28 more items
-
-
CLASSIFICATION record, Germline, Germline/De novo (untested), SUMMARY record, Unknown
-, ?, yes
2/899 cases, 23, 120444, 0, 0.000191, ExAC 23, 120444, 0, 0.000191, frequency 0.016,
1 more item
-
-
-
Global Variome, with Curator vacancy
,
Johan den Dunnen
,
Frans Cremers
,
Stéphanie Cornelis
,
VKGL-NL_Rotterdam
,
VKGL-NL_Nijmegen
,
Lu Tian
,
Zelia Corradi
+?/.
1
-
c.83A>C
r.(?)
p.(Glu28Ala)
-
likely pathogenic (recessive)
g.94578606T>G
g.94113050T>G
[83A>C;1610G>A]
-
ABCA4_002454
-
PubMed: Tian 2024
-
-
Germline
yes
-
-
-
-
Lu Tian
+?/., ?/.
2
2
c.85C>T
r.(?)
p.(Leu29Phe)
ACMG
likely pathogenic (recessive), VUS
g.94578604G>A
g.94113048G>A
c.85C>T, p.Leu29Phe Heterozygous
-
ABCA4_002243
ACMG PM2_sup; severity category not assesed, no variant 2nd chromosome
Journal: Cornelis 2023
,
PubMed: Goetz 2020
-
-
SUMMARY record, Unknown
-
-
-
-
-
Stéphanie Cornelis
+?/., ?/.
4
2
c.86T>G
r.(?)
p.(Arg24His), p.(Leu29Arg)
ACMG
likely pathogenic (recessive), VUS
g.94578603A>C
g.94113047A>C
c.86T>G p.(L29R)
-
ABCA4_002242
ACMG PM2_sup, PP3_m; severity category not assesed, no variant 2nd chromosome
Journal: Cornelis 2023
,
PubMed: Cornelis 2024
,
Journal: Cornelis 2024
,
PubMed: Schulz 2017
-
-
SUMMARY record, Unknown
-
-
-
-
-
Frans Cremers
,
Stéphanie Cornelis
-/.
1
-
c.87C>T
r.(?)
p.(Leu29=)
-
benign
g.94578602G>A
g.94113046G>A
ABCA4(NM_000350.3):c.87C>T (p.L29=)
-
ABCA4_001246
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
4
-
c.88G>A
r.(?)
p.(Val30Met)
-
VUS
g.94578601C>T
g.94113045C>T
-
-
ABCA4_002757
-
PubMed: Cornelis 2024
,
Journal: Cornelis 2024
-
-
Germline, Unknown
yes
-
-
-
-
Frans Cremers
+?/.
9
2
c.91T>C
r.(91u>c), r.(?)
p.(Trp31Arg)
ACMG
likely pathogenic, likely pathogenic (recessive)
g.94578598A>G
g.94113042A>G
c.91T>C, c.91T>C (p.Trp31Arg), c.91T>C, p.Trp31Arg Heterozygous, p.(W31*), p.(W31R), p.W31*
-
ABCA4_000251
no segregation analysis done
PubMed: Cornelis 2017
,
Journal: Cornelis 2017
,
Journal: Cornelis 2023
,
PubMed: Goetz 2020
,
5 more items
-
-
Germline, SUMMARY record, Unknown
?
-
-
-
-
Jana Zernant
,
Stéphanie Cornelis
+/.
3
2
c.92G>A
r.(?)
p.(Trp31*), p.(Trp31Ter)
ACMG
pathogenic, pathogenic (recessive)
g.94578597C>T
g.94113041C>T
c.92G>A, c.92G>A, p.Trp31Stop Heterozygous
-
ABCA4_000250
ACMG PVS1, PS4, PM3_sup, PP3_m; severity category severe
Journal: Cornelis 2023
,
PubMed: Goetz 2020
,
PubMed: Khan 2020
-
-
SUMMARY record, Unknown
-
-
-
-
-
Stéphanie Cornelis
+/., +?/.
21
2
c.93G>A
r.(93g>a), r.(?)
p.(Gln8Hisfs*33), p.(Trp31*), p.(Trp31Ter)
ACMG
likely pathogenic, pathogenic (recessive)
g.94578596C>T, g.94578597C>T
g.94113040C>T
c.93G>A p.W31X, c.93G>A (p.Trp31*), c.93G>A p.(Trp31*), c.93G>A, p.Tyr31*, p.(W31*)
-
ABCA4_000250
-
PubMed: Cornelis 2017
,
Journal: Cornelis 2017
,
Journal: Cornelis 2023
,
PubMed: Fakin 2016
,
8 more items
-
-
Germline, Germline/De novo (untested), SUMMARY record, Unknown
-, ?, yes
-
-
-
-
Johan den Dunnen
,
Jana Zernant
,
Frans Cremers
,
Stéphanie Cornelis
+/., +?/., ?/.
3
2
c.94C>A
r.(?)
p.(Pro32Thr)
ACMG
likely pathogenic (recessive), pathogenic (recessive), VUS
g.94578595G>T
g.94113039G>T
c.94C>A
-
ABCA4_001328
ACMG PM2_sup, PP3_m; severity category mild/moderate
Journal: Cornelis 2023
,
PubMed: Hu 2019
-
-
Germline, SUMMARY record, Unknown
-
-
-
-
-
Stéphanie Cornelis
,
Fangyuan Hu
+/., +?/., ?/.
5
2
c.95C>T
r.(?)
p.(Pro32Leu)
ACMG
likely pathogenic (recessive), pathogenic (recessive), VUS
g.94578594G>A
g.94113038G>A
c.95C>T, c.95C>T, p.Pro32Leu Heterozygous
-
ABCA4_001327
ACMG PM2_sup, PM3_sup, PP3_m; severity category mild/moderate
Journal: Cornelis 2023
,
PubMed: Goetz 2020
,
PubMed: Hu 2019
-
-
Germline, SUMMARY record, Unknown
-
-
-
-
-
Stéphanie Cornelis
,
Fangyuan Hu
+/., +?/., ?/.
88
2
c.101_106del
r.(101_106del), r.(?)
p.(Ser34_Leu35del)
ACMG, other
likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive), VUS
g.94578583_94578588del, g.94578589_94578594del
g.94113033_94113038del
101_106delCTTTAT, ABCA4 c.101_106del(;)1804C>T, V2: c.101_106delCTTTAT, (p.Ser34_Leu35del),
14 more items
-
ABCA4_000249
alleles in cis or trans; heterozygous, no segregation analysis done, no variant 2nd chromosome,
3 more items
Zixi Sun 2020, submitted,
PubMed: Chen 2021
,
PubMed: Consugar 2015
,
PubMed: Dan 2019
,
PubMed: Gao 2019
,
14 more items
-
rs745925619
Germline, Germline/De novo (untested), SUMMARY record, Unknown
?, yes
-
-
-
-
Johan den Dunnen
,
Stéphanie Cornelis
,
Fangyuan Hu
,
Handong Dan
,
Zixi Sun
,
Qing Zhu
,
Lu Tian
+?/.
3
-
c.101_106delCTTTAT
r.(?)
p.(Ser34_Leu35del)
-
likely pathogenic
g.94578589_94578594del
g.94113033_94113038del
ABCA4 c.101_106del(;)1804C>T; p.(Ser34_Leu35del), ABCA4 c.[101_106del];[?]; p.(Ser34_Leu35del),
1 more item
-
ABCA4_000249
heterozygous, heterozygous; single variant in a recessive gene, no second allele found
PubMed: Chen 2021
-
-
Germline, Germline/De novo (untested)
?, yes
Taiwan Biobank: 0.000334; GnomAD_exome_East: 0.000272; GnomAD_All: 0.0000199
-
-
-
LOVD
+/., +?/.
11
2
c.108del
r.(108del), r.(?)
p.(Leu37Trpfs*3), p.(Leu37TrpfsTer3)
ACMG
likely pathogenic, pathogenic (recessive)
g.94578583del
g.94113027del
106delT, 108delT, 1bpdel(T) Co 36 which creates stop at Co 38, c.106del (p.Leu37fs),
2 more items
-
ABCA4_000248
no segregation analysis done
PubMed: Cornelis 2017
,
Journal: Cornelis 2017
,
Journal: Cornelis 2023
,
PubMed: Genead 2009
,
7 more items
-
-
Germline, Germline/De novo (untested), SUMMARY record, Unknown
?, yes
-
-
-
-
Stéphanie Cornelis
-?/.
1
-
c.117G>A
r.(?)
p.(Leu39=)
-
likely benign
g.94578572C>T
g.94113016C>T
-
-
ABCA4_001245
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/., +?/., ?/.
12
2
c.122G>A
r.(122g>a), r.(?)
p.(Trp41*), p.(Trp41Ter)
ACMG
likely pathogenic, pathogenic, pathogenic (recessive), VUS
g.94578567C>T
g.94113011C>T
c.121G>A, c.122G>A, c.122G>A (p.Trp41*), c.122G>A (p.Trp41Ter), c.122G>A p.(W41*),
2 more items
-
ABCA4_000247
ACMG PM2, PVS1, PP5, no segregation analysis done, no variant 2nd chromosome,
1 more item
Prevention Genetics,
PubMed: Bauwens 2019
,
PubMed: Kellner 2009
,
PubMed: Khan 2019
,
PubMed: Khan 2020
,
6 more items
-
-
CLASSIFICATION record, Germline, SUMMARY record, Unknown
-, ?, no, yes
-
-
-
-
Johan den Dunnen
,
Stéphanie Cornelis
,
VKGL-NL_Nijmegen
+/., +?/.
4
2
c.123G>A
r.(123g>a), r.(?)
p.(Trp41*), p.(Trp41Ter)
ACMG
likely pathogenic, pathogenic (recessive)
g.94578566C>T
g.94113010C>T
c.123G>A p.Trp41* Het, W41X
-
ABCA4_000246
-
Bianco 2023, submitted, Prevention Genetics,
PubMed: Cideciyan 2009
,
1 more item
-
-
Germline, SUMMARY record, Unknown
-, yes
1, 120980, 0, 0.000008266
-
-
-
Stéphanie Cornelis
,
Lorenzo Bianco
?/.
2
2
c.140C>T
r.(?)
p.(Pro47Leu)
ACMG
VUS
g.94578549G>A
g.94112993G>A
c.140C>T, p.Pro47Leu Heterozygous
-
ABCA4_002241
ACMG PP3; severity category benign
Journal: Cornelis 2023
,
PubMed: Goetz 2020
-
-
SUMMARY record, Unknown
-
-
-
-
-
Stéphanie Cornelis
-/.
1
-
c.141=
r.(=)
p.(Pro47=)
-
benign
g.94578548T>C
-
ABCA4(NM_000350.3):c.141A>G (p.P47=)
-
ABCA4_000950
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-/., ?/.
6
2
c.141G>A
r.(?)
p.(Pro47=)
ACMG
benign, VUS
g.94578548=, g.94578548C>T
g.94112992=, g.94112992C>T
c.141A>G, c.141A>G, p.Pro47 Homozygous, c.141A>G, p.Pro47= Homozygous
-
ABCA4_002240
ACMG BS1, BP4_m, BP7
Journal: Cornelis 2023
,
PubMed: Goetz 2020
-
-
SUMMARY record, Unknown
-
-
-
-
-
Stéphanie Cornelis
+?/., ?/.
3
2
c.156T>G
r.(?)
p.(His52Gln)
ACMG
likely pathogenic (recessive), VUS
g.94578533A>C
g.94112977A>C
c.156T>G, p.His52Gln Heterozygous
-
ABCA4_002239
ACMG PP3; severity category not assesed
Journal: Cornelis 2023
,
PubMed: Corradi 2023
,
Journal: Corradi 2023
,
PubMed: Goetz 2020
-
-
Germline, SUMMARY record, Unknown
-
-
-
-
-
Stéphanie Cornelis
,
Zelia Corradi
+/., +?/.
7
2
c.157G>T
r.(?)
p.(Glu53*), p.(Glu53Ter)
ACMG
likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive)
g.13174G>T, g.94578532C>A
g.94112976C>A
c.157G>T, c.157G>T p.E53*, c.157G>T, p.E53*, p.E53X
-
ABCA4_002238
ACMG PVS1, PS4, PM3_sup, PP3_m; severity category severe, compound heterozygous
Journal: Cornelis 2023
,
PubMed: Groselj 2012
,
PubMed: Jiang 2016
,
PubMed: Liu 2020
,
PubMed: Liu-2020
,
1 more item
-
-
Germline, SUMMARY record, Unknown
yes
-
-
-
-
Stéphanie Cornelis
,
Lu Tian
+/., +?/., ?/.
15
2
c.160T>G
r.(160u>g), r.(?), r.spl?
p.(Cys54Gly), p.(Cys54Gly, ?), p.(Cys54Gly,?)
ACMG
likely pathogenic, likely pathogenic (recessive), pathogenic, VUS
g.94578529A>C
g.94112973A>C
c.160T>G, c.160T>G p.(C54G), c.160T>G(;)587C>T, C54T, p.C54G, [c.160T>G],
1 more item
-
ABCA4_000245
no segregation analysis done, no variant 2nd chromosome, segregation analysis done when possible,
1 more item
PubMed: Chen 2010
,
PubMed: Cornelis 2017
,
Journal: Cornelis 2017
,
Journal: Cornelis 2023
,
7 more items
-
-
Germline, SUMMARY record, Unknown
?, no, yes
-
-
-
-
Frans Cremers
,
Stéphanie Cornelis
,
Rebekkah Hitti-Malin
+?/.
2
2i
c.160+1G>A
r.spl
p.?
ACMG
likely pathogenic, likely pathogenic (recessive)
g.94578528C>T
g.94112972C>T
160+1g > a
-
ABCA4_000244
-
PubMed: Cornelis 2017
,
Journal: Cornelis 2017
,
Journal: Cornelis 2023
,
PubMed: Webster 2001
-
-
Germline, SUMMARY record
-
-
-
-
-
Stéphanie Cornelis
+/., +?/.
6
2i
c.160+2T>C
r.spl, r.spl?
p.(?), p.?
ACMG
likely pathogenic, pathogenic, pathogenic (recessive)
g.94578527A>G
g.94112971A>G
c.160+2T4C, c.160+2T>C, c.160+2T>C p.(?)
-
ABCA4_000243
no variant 2nd chromosome, unknown variant 2nd allele
PubMed: Cornelis 2017
,
Journal: Cornelis 2017
,
Journal: Cornelis 2023
,
PubMed: Khan 2019
,
4 more items
-
-
Germline, SUMMARY record, Unknown
?, no
-
-
-
-
Stéphanie Cornelis
+?/., ?/.
4
2i
c.160+5G>A
r.(?)
p.(?), p.?
ACMG
likely pathogenic, VUS
g.94578524C>T
g.94112968C>T
c.160+5G>A, splice sitealteration, c.IVS2+5G>Ac.160+5G>A, Heterozygous
-
ABCA4_002237
ACMG PS4, PM3, PP3; causative variant of unknown severity
Journal: Cornelis 2023
,
PubMed: Fujinami 2019
,
PubMed: Goetz 2020
-
-
SUMMARY record, Unknown
-
-
-
-
-
Stéphanie Cornelis
+?/., +?/?
7
2i
c.160+5G>C
r.spl?, r.[67_160del,161_302delins161+1_161+14,=], r.[=,67_160del,161_302delinsguaaccauagcagg]
p.(?), p.[(=,Ile23Alafs∗24,His55Asnfs∗63)], p.[Ile23AlafsTer24,His55AsnfsTer63,=],
1 more item
ACMG
likely pathogenic, likely pathogenic (recessive), NA
g.94578524C>G
g.94112968C>G
c.160+5G>C (p.?), c.160+5G>C, Heterozygous, c.160+5G>C, splice sitealteration
-
ABCA4_000068
expression cloning midigene splicing construct: 0.343 correctly spliced RNA,
2 more items
PubMed: Cornelis 2017
,
Journal: Cornelis 2017
,
Journal: Cornelis 2023
,
PubMed: Fujinami 2019
,
4 more items
-
-
Germline, In vitro (cloned), SUMMARY record, Unknown
-
-
-
-
-
Monika Oldak
,
Frans Cremers
,
Stéphanie Cornelis
+/., +?/., ?/.
4
2i
c.161-395G>A
r.spl, r.[161_302del,=,160_161ins161-484_161–394]
p.[(Cys54Serfs*14,=,Cys54Cysfs*75)], p.[(His55ProfsTer136,=)], p.[Cys54Serfs*14,=,Cys54Cysfs*75]
ACMG
NA, pathogenic (recessive), VUS
g.94577530C>T
g.94111974C>T
-
-
ABCA4_002453
expression cloning midi-gene splicing assay, 0.36 normally spliced mRNA; predicted severity moderate,
1 more item
PubMed: Corradi 2023
,
Journal: Corradi 2023
,
PubMed: Tian 2024
-
-
Germline, In vitro (cloned)
yes
-
-
-
-
Johan den Dunnen
,
Lu Tian
,
Zelia Corradi
+?/., ?/.
4
2i
c.161-23T>A
r.spl?
p.(?), p.?
ACMG
likely pathogenic (recessive), VUS
g.94577158A>T
g.94111602A>T
c.161-23T>A p.[=,Cys54Serfs*14]
-
ABCA4_001065
ACMG PM2_sup, PM3_sup, PP3; severity category uncertain, no segregation analysis done
Journal: Cornelis 2023
,
PubMed: Khan 2019
-
-
SUMMARY record, Unknown
no
-
-
-
-
Stéphanie Cornelis
+?/., ?/.
3
2i
c.161-23T>G
r.[=,161_302del]
p.[=,Cys54Serfs*14], p.[=,Cys54SerfsTer14]
ACMG
likely pathogenic (recessive), VUS
g.94577158A>C
g.94111602A>C
c.161-23T>G, [161-23T>G]
-
ABCA4_002235
ACMG PS4, BP4_m; severity category mild, no segregation analysis done
Bauwens 2019,
Journal: Cornelis 2023
,
PubMed: Bauwens 2019
-
-
SUMMARY record, Unknown
?
-
-
-
-
Stéphanie Cornelis
-?/., ?/.
3
2i
c.161-5T>C
r.(?)
p.(?), p.?
ACMG
likely benign, VUS
g.94577140A>G
g.94111584A>G
– c.161-5T>C
-
ABCA4_001520
ACMG PM2_sup, BP4_m; severity category not assesed, no variant 2nd chromosome
Journal: Cornelis 2023
,
PubMed: Tsipi 2016
-
-
Germline, SUMMARY record, Unknown
-
-
-
-
-
Stéphanie Cornelis
+/., +?/.
3
2i
c.161-2A>G
r.spl
p.?
ACMG
likely pathogenic, pathogenic (recessive)
g.94577137T>C
g.94111581T>C
c.161-2A>G
-
ABCA4_000242
not statistically tested, classification unknown
PubMed: Cornelis 2017
,
Journal: Cornelis 2017
,
Journal: Cornelis 2023
,
PubMed: Xin 2015
-
-
Germline, SUMMARY record
-
-
-
-
-
Stéphanie Cornelis
+/., +?/.
8
2i
c.161-1G>A
r.spl
p.?
ACMG
likely pathogenic, pathogenic (recessive)
g.94577136C>T
g.94111580C>T
c.161-1G.A (p.?), c.161-1G>A (p.?), c.161-1G>A IVS2-1 G>A, c.161G>A, IVS2-1 G>A,
2 more items
-
ABCA4_000116
no segregation analysis done
PubMed: Collison 2019
,
PubMed: Cornelis 2017
,
Journal: Cornelis 2017
,
Journal: Cornelis 2023
,
5 more items
-
-
Germline, SUMMARY record, Unknown
-, ?, yes
-
-
-
-
Stéphanie Cornelis
+?/.
1
2i
c.161-1G>T
r.spl?
p.?
ACMG
pathogenic
g.94577136C>A
-
-
-
ABCA4_001444
-
Mena et al., 2020 submitted.
-
-
Germline
yes
-
-
-
-
Marcela Mena
+/., +?/., ?/.
155
3, ?
c.161G>A
r.(?), r.spl, r.spl?, r.[(161_302del,161g>a)], r.[161_302del,161g>a]
p.(Cys54Tyr), p.(Cys54Tyr, ?), p.[(Cys54Serfs*14,Cys54Tyr)], p.[Cys54Serfs*14,Cys54Tyr],
1 more item
ACMG
likely pathogenic, likely pathogenic (recessive), NA, pathogenic, pathogenic (recessive), VUS
g.94577135C>T
g.94111579C>T
161G > A, 161G>A, 2 c.156T>G p.His52Gln Heterozygous, ABCA4 c.161G>A p.(Cys54Tyr) het,
25 more items
-
ABCA4_000240
compound heterozygous, probably causal, no segregation analysis done, no variant 2nd chromosome,
2 more items
Bianco 2023, submitted, Fadaie 2019, Prevention Genetics,
PubMed: Alabduljalil 2019
,
PubMed: Chen 2019
,
49 more items
-
-
Germline, Germline/De novo (untested), In vitro (cloned), SUMMARY record, Unknown
-, ?, no, yes
2, 121210, 0, 0.0000165, 2/899 cases, ExAC 2, 121210, 0, 0.0000165
-
-
-
Global Variome, with Curator vacancy
,
Stéphanie Cornelis
,
Zeinab Fadaie
,
Zeinab Fadaie
,
Lorenzo Bianco
,
Zelia Corradi
+/., +?/.
6
3
c.161G>T
r.(?), r.161_302del, r.spl
p.(Cys54Phe), p.(Cys54SerfsTer14), p.(Cys54Serfs∗14), p.Cys54SerfsTer14
ACMG
likely pathogenic, NA, pathogenic (recessive)
g.94577135C>A
g.94111579C>A
-
-
ABCA4_000241
expression cloning midigene splicing construct: no correctly spliced RNA,
1 more item
PubMed: Cornelis 2017
,
Journal: Cornelis 2017
,
Journal: Cornelis 2023
,
PubMed: Oishi 2014
,
2 more items
-
-
Germline, In vitro (cloned), SUMMARY record
-, yes
ExAC 1, 121210, 0, 0.00000825
-
-
-
Frans Cremers
,
Stéphanie Cornelis
,
Lu Tian
+?/., ?/.
3
3
c.164A>C
r.(?)
p.(His55Pro)
ACMG
likely pathogenic, likely pathogenic (recessive), VUS
g.94577132T>G
g.94111576T>G
164A>C (H55P), 164A?C H55P
-
ABCA4_001545
ACMG PM2_sup, PM5, PP3_m; severity category not assesed, no variant 2nd chromosome
Journal: Cornelis 2023
,
PubMed: Downs 2007
-
-
Germline, SUMMARY record, Unknown
-
-
-
-
-
Julia Lopez
,
Stéphanie Cornelis
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