Global Variome shared LOVD
RPE65 (retinal pigment epithelium-specific protein ...)
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Global Variome, with Curator vacancy
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Unique variants in the RPE65 gene
This database is one of the
"Eye disease"
gene variant databases.
The variants shown are described using the NM_000329.2 transcript reference sequence.
Legend
Please note that a short description of a certain column can be displayed when you move your mouse cursor over the column's header and hold it still. Below, a more detailed description is shown per column.
Effect
: The variant's effect on the function of the gene/protein, displayed in the format 'R/C'. R is the value reported by the source (publication, submitter) and this classification may vary between records. C is the value concluded by the curator. Note that in some database the curator uses Summary records to give details on the classification of the variant.Values used: '+' indicating the variant affects function, '+?' probably affects function, '-' does not affect function, '-?' probably does not affect function, '?' effect unknown, '.' effect was not classified.
Reported
: The number of times this variant has been reported in the database.
Exon
: number of exon/intron containing variant; 2 = exon 2, 12i = intron 12, 2i_7i = from intron 2 to intron 7, 8i_9 = intron 8/exon 9 boundary, _1 = 5' to exon 1, 18_ = 3' of exon 18, _1_18_ = encompassing the entire 18-exon gene
DNA change (cDNA)
: description of variant at DNA level, based on a coding DNA reference sequence (following HGVS recommendations); e.g. c.123C>T, c.123_145del, c.123_126dup. For deletions/duplications extending beyond the reference transcript resp. {0}/{2} is used to replace del/dup. Extent of the deletion/duplication should be specified using the genomic description (g.). "-" indicates the variant described on genomic level does not affect the coding DNA reference sequence.
RNA change
: description of variant at RNA level (following HGVS recommendations).
r.123c>u
r.? = unknown
r.(?) = RNA not analysed but probably transcribed copy of DNA variant
r.spl? = RNA not analysed but variant probably affects splicing
r.(spl?) = RNA not analysed but variant may affect splicing
r.0? = change expected to abolish transcription
Protein
: description of variant at protein level (following HGVS recommendations).
p.(Arg345Pro) = change predicted from DNA (RNA not analysed)
p.Arg345Pro = change derived from RNA analysis
p.? = unknown effect
p.0? = probably no protein produced
Classification method
: The method used for the clinical classification of this variant.
All options:
ACMG
ACGS
EAHAD-CFDB
ENIGMA
IARC
InSiGHT
kConFab
other
Clinical classification
: Clinical classification of variant, preferably based on standardised criteria (e.g. ACMG), directed on the clinical consequences as published/submitted, indicated using an enriched system including inheritance: e.g. pathogenic, pathogenic (dominant), pathogenic (recessive), pathogenic (!), pathogenic (maternal), pathogenic (paternal). Standard inheritance is covered by dominant/recessive, imprinting by maternal/paternal. A '!' warns for exceptional circumstances to be explained in the 'Remarks' field (low penetrance, variants pathogenic in heterozygous state only, hypomorphic/hypermorphic variants, protective variants, etc.). Non-disease consequences (e.g. drug metabolism (pharmacogenetics), risk factor, blood group, tasting bitter) are indicated using additions to the benign classification; benign (dominant), benign (recessive), benign (!), etc. The value 'association' is used for variants associated with a phenotype and 'NA' for variants from in vitro/in silico records. NOTE: classification may differ from the opinion of the curator as given in a variant SUMMARY-record or the 'Functional effect concluded'). NOTE: pathogenic/likely pathogenic should go together with "variant (probably) affects function" In ClassFunctional.
All options:
pathogenic
pathogenic (dominant)
pathogenic (recessive)
pathogenic (!)
pathogenic (maternal)
pathogenic (paternal)
likely pathogenic
likely pathogenic (dominant)
likely pathogenic (recessive)
likely pathogenic (!)
likely pathogenic (maternal)
likely pathogenic (paternal)
VUS
VUS (!)
likely benign
likely benign (dominant)
likely benign (recessive)
likely benign (!)
likely benign (maternal)
likely benign (paternal)
benign
benign (dominant)
benign (recessive)
benign (!)
benign (maternal)
benign (paternal)
conflicting
association
NA
DNA change (genomic) (hg19)
: HGVS description of variant at DNA level, based on the genomic (chromosomal) DNA reference sequence; e.g. g.12345678C>T, g.12345679del, g.12345678_12345890dup
DNA change (hg38)
: HGVS description of variant at DNA level, based on the hg38 genomic (chromosomal) eference sequence; e.g. g.12345678C>T, g.12345679del, g.12345678_12345890dup
Published as
: listed only when different from "DNA change"; variant as reported originally (e.g. 521delT). Variants seen in animal models, tested in vitro, predicted from RNA analysis, etc. are described between brackets like c.(456C>G)
ISCN
: description of the variant according to ISCN nomenclature
DB-ID
: database ID of variant, grouping multiple observations of the same variant together, starting with the HGNC gene symbol, followed by an underscore (_) and a six digit number (e.g. DMD_012345). _000000 is used for variants where DNA was not analysed (change predicted from RNA analysis), variants seen in animal models or variants not seen in humans but functionally tested in vitro
Variant remarks
: remarks regarding variant described, e.g. germline mosaicism in mother, 345 kb deletion, muscle RNA analysed, not in 200 control chromosomes tested, on founder haplotype, etc.
Reference
: publication describing the variant submitted, incl. links to OMIM, PubMed or other source, e.g. "den Dunnen ASHG2003 P2346"
ClinVar ID
: ID of variant in ClinVar database
dbSNP ID
: the dbSNP ID
Origin
: Origin of variant/record: Germline = in all cells, De novo = in all cells, but not in either parent, Germline/De novo (untested) = in all cells, parents not tested (use only when De novo is likely, e.g. isolated/sporadic cases with dominant disease), Somatic = present in a subset of cells, but not in either parent, Uniparental disomy = from parental disomy (maternal or paternal), CLASSIFICATION record = submitter only sharing variant classification (note another report may share Individual data), SUMMARY record = master summary record from curator (may link to another database), In vitro (cloned) = data resulting from in vitro functional assays, animal model = data from animal model, Artefact = false positive variant call, DUPLICATE record = variant already described on another chromosome (e.g. unbalanced translocation, duplicating transposition, 2nd fusion transcript, etc.)
All options:
Germline
De novo
Germline/De novo (untested)
Somatic
Uniparental disomy
Uniparental disomy, maternal allele
Uniparental disomy, paternal allele
CLASSIFICATION record
SUMMARY record
In vitro (cloned)
In silico
animal model
Artefact
DUPLICATE record
Unknown
Not applicable
Segregation
: Indicates whether the variant segregates with the phenotype (yes), does not segregate with the phenotype (no) or segregation is unknown (?)
All options:
? = unknown
yes = segregates with phenotype
no = does not segregate with phenotype
- = not applicable
Frequency
: frequency in which the variant was found; e.g 5/760 chromosomes (in 5 of 760 chromosomes tested), 1/33 patients (in 1 of 33 patients analysed in study), 0.05 controls (in 5% of control cases tested)
Re-site
: restriction enzyme recognition site created (+) or destroyed (-); e.g. BglII+;BamHI-
VIP
: variant VIP-status was requested for matchmaking - need collaboration(s) to crack the case - please contact the submitter/curator. NOTE: to get VIP status ask the curator.
Methylation
: result of methylation test; GOM (gain of methylation), LOM (loss of methylation), 30% (30% methylated). NOTE: when several tests were done mention the method as well (e.g. MS-PCR 75%)
How to query this table
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Operator
Column type
Example
Matches
Text
Arg
all entries containing 'Arg'
space
Text
Arg Ser
all entries containing 'Arg' and 'Ser'
|
Text
Arg|Ser
all entries containing 'Arg' or 'Ser'
!
Text
!fs
all entries not containing 'fs'
^
Text
^p.(Arg
all entries beginning with 'p.(Arg'
$
Text
Ser)$
all entries ending with 'Ser)'
=""
Text
=""
all entries with this field empty
=""
Text
="p.0"
all entries exactly matching 'p.0'
!=""
Text
!=""
all entries with this field not empty
!=""
Text
!="p.0"
all entries not exactly matching 'p.0?'
combination
Text
*|Ter !fs
all entries containing '*' or 'Ter' but not containing 'fs'
Date
2020
all entries matching the year 2020
|
Date
2020-03|2020-04
all entries matching March or April, 2020
!
Date
!2020-03
all entries not matching March, 2020
<
Date
<2020
all entries before the year 2020
<=
Date
<=2020-06
all entries in or before June, 2020
>
Date
>2020-06
all entries after June, 2020
>=
Date
>=2020-06-15
all entries on or after June 15th, 2020
combination
Date
2019|2020 <2020-03
all entries in 2019 or 2020, and before March, 2020
Numeric
23
all entries exactly matching 23
|
Numeric
23|24
all entries exactly matching 23 or 24
!
Numeric
!23
all entries not exactly matching 23
<
Numeric
<23
all entries lower than 23
<=
Numeric
<=23
all entries lower than, or equal to, 23
>
Numeric
>23
all entries higher than 23
>=
Numeric
>=23
all entries higher than, or equal to, 23
combination
Numeric
>=20 <30 !23
all entries with values from 20 to 29, but not equal to 23
Some more advanced examples:
Example
Matches
Asian
all entries containing 'Asian', 'asian', including 'Caucasian', 'caucasian', etc.
Asian !Caucasian
all entries containing 'Asian' but not containing 'Caucasian'
Asian|African !Caucasian
all entries containing 'Asian' or 'African', but not containing 'Caucasian'
"South Asian"
all entries containing 'South Asian', but not containing 'South East Asian'
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364 entries on 4 pages. Showing entries 1 - 100.
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Effect
Reported
Exon
DNA change (cDNA)
RNA change
Protein
Classification method
Clinical classification
DNA change (genomic) (hg19)
DNA change (hg38)
Published as
ISCN
DB-ID
Variant remarks
Reference
ClinVar ID
dbSNP ID
Origin
Segregation
Frequency
Re-site
VIP
Methylation
Owner
+?/.
1
-
c.(?_-1)_1128+1_1129-1)del
r.spl
p.(?)
-
likely pathogenic
g.?
g.?
Allele 1 c.(?_-1)_(1128+1_1129���������1)del, Allele 2 c
-
NPHS2_000000
homozygous
PubMed: Khan 2019
-
-
Germline/De novo (untested)
?
-
-
-
-
LOVD
+?/.
1
-
c.-330_1128+252del
r.spl
p.(?)
-
likely pathogenic
g.68903618_68915918del
g.68437935_68450235del
RPE65 chr1:68903619_68915919del
-
RPE65_000318
start loss, range 11717-92433 bp in various techniques, homozygous
PubMed: Zampaglione 2020
-
-
Unknown
?
-
-
-
-
LOVD
?/?
1
1
c.-4G>A
r.(?)
p.(?)
-
VUS
g.68915592C>T
g.68449909C>T
IVS1(-4) G>A
-
RPE65_000137
unknown variant 2ndchromosome
PubMed: Dharmaraj 2000
-
-
Germline
yes
1/100 cases
-
-
-
Muhammad Ajmal
+?/.
1
_1_14_
c.-54_*952{0}
r.?
p.?
-
likely pathogenic (recessive)
g.(?_68895454)_(68915593_?)del
-
c.(?_-1)_(*1_?)del
-
RPE65_000320
-
PubMed: Ellingsford 2018
-
-
Germline
-
-
-
-
-
LOVD
+/., -/.
3
-
c.?
r.(?), r.?
p.?
-
benign, pathogenic (dominant)
g.?
-
Asp477Gly, gi3077820:1056G>A, gi3077820:IVS12+20A>C
-
NPHS2_000000
-
PubMed: Daiger 2014
,
PubMed: Morimura 1998
;
PubMed: Booij 2005
-
-
Germline
-
-
-
-
-
Julia Lopez
?/?
3
1
c.2T>C
r.(?)
p.(Met1?)
-
VUS
g.68915587A>G
g.68449904A>G
56T->c, M1T
-
RPE65_000136
-
PubMed: Morimura 1998
,
PubMed: Thompson 2000
-
-
Germline
yes
-
HpyCH4IV+
-
-
Muhammad Ajmal
+?/.
1
-
c.10C>T
r.(?)
p.(Gln4Ter)
ACMG
likely pathogenic (recessive)
g.68915579G>A
g.68449896G>A
-
-
RPE65_000347
ACMG PM2, PVS1
PubMed: Weisschuh 2024
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.10del
r.(?)
p.(Gln4Argfs*27)
-
likely pathogenic
g.68915579del
g.68449896del
c.10delC
-
RPE65_000205
-
PubMed: Holtan 2020
-
-
Germline
-
1/899 cases
-
-
-
Global Variome, with Curator vacancy
+/+
1
1i
c.11+1G>T
r.spl
p.?
-
pathogenic (recessive)
g.68915577C>A
g.68449894C>A
-
-
RPE65_000135
-
PubMed: Jacobson 2012
-
-
Germline
yes
-
-
-
-
Muhammad Ajmal
+?/.
1
-
c.11+2T>G
r.spl
p.?
-
likely pathogenic (recessive)
g.68915576A>C
g.68449893A>C
-
-
RPE65_000204
-
PubMed: Holtan 2020
-
-
Germline
-
2/899 cases
-
-
-
Global Variome, with Curator vacancy
+/.
1
-
c.11+2T>V
r.spl
p.?
-
pathogenic (recessive)
g.68915576A>B
g.68449893A>B
c.11+2T>?
-
RPE65_000000
-
PubMed: Chung 2019
-
-
Germline
-
-
-
-
-
LOVD
+/+, +/., +?/+?, +?/., ?/., ?/?
94
1i
c.11+5G>A
r.spl, r.spl?, r.[=|red,11_12ins[guaaa;11+6_11+124]]
p.(?), p.?
ACMG
likely pathogenic, likely pathogenic (recessive), NA, pathogenic, pathogenic (recessive), VUS
g.68915573C>T
g.68449890C>T
12+4G>A, SPLICE, 1590delC, 1:68915573C>T ENST00000262340.5:c.11+5G>A, 65 + 5g>a,
13 more items
-
RPE65_000058
ACMG PP3, PM2, PP5_STRONG, PS4_MODERATE, heterozygous, no second chromosome; uniparental isodisomy,
6 more items
Tracewska 2021, MolVis in press,
PubMed: Booij 2005
,
PubMed: Carss 2017
,
PubMed: Chung 2019
,
38 more items
98825
-
CLASSIFICATION record, Germline, Germline/De novo (untested), In vitro (cloned), Unknown,
1 more item
?, yes
0,0002 (in-house database, ~5000 samples), 1/143 cases, 2/110 cases, 2/18 chromosomes, 2/8 chromosomes,
1 more item
-, ?
-
-
Johan den Dunnen
,
Muhammad Ajmal
,
VKGL-NL_Nijmegen
,
VKGL-NL_AMC
,
Daan Panneman
+/+
1
1i
c.12-2A>G
r.spl
p.?
-
pathogenic (recessive)
g.68914391T>C
g.68448708T>C
-
-
RPE65_000134
-
PubMed: Ripamonti 2014
-
-
Germline
yes
1/18 chromosomes
-
-
-
Muhammad Ajmal
+/+
2
2
c.16G>T
r.(?)
p.(Glu6*)
-
pathogenic (recessive)
g.68914385C>A
g.68448702C>A
-
-
RPE65_000133
unknown variant 2ndchromosome
PubMed: Henderson 2007
,
PubMed: Ripamonti 2014
-
-
Germline
yes
1/18 chromosomes
-
-
-
Muhammad Ajmal
?/.
1
-
c.20A>G
r.(?)
p.(His7Arg)
ACMG
VUS
g.68914381T>C
g.68448698T>C
-
-
RPE65_000346
ACMG PP3, PM2, PP2
PubMed: Weisschuh 2024
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
2
c.46_49del
r.(?)
p.(Phe16Lysfs*14)
-
likely pathogenic (recessive)
g.68914354_68914357del
g.68448671_68448674del
46_49delTTTG
-
RPE65_000009
-
-
-
-
Germline
-
-
-
-
-
Soumittra Nagasamy
+?/.
1
-
c.47T>C
r.(?)
p.(Phe16Ser)
ACMG
likely pathogenic
g.68914354A>G
g.68448671A>G
RPE65 NM_000329: g.1289T>C, c.47T>C, p.F16S
-
RPE65_000316
-
PubMed: Xu 2020
-
-
Germline
yes
-
-
-
-
LOVD
-/., -?/.
2
-
c.48T>C
r.(?)
p.(Phe16=)
-
benign, likely benign
g.68914353A>G
g.68448670A>G
RPE65(NM_000329.2):c.48T>C (p.F16=), RPE65(NM_000329.3):c.48T>C (p.F16=)
-
RPE65_000199
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
+/+
2
2
c.57_58del
r.(?)
p.(Glu20Glyfs*31)
-
pathogenic (recessive)
g.68914343_68914344del
g.68448660_68448661del
p.(Val19Valfs*31), V19del2bp
-
RPE65_000132
-
PubMed: Dharmaraj 2000
,
PubMed: Koenekoop 2014
-
-
Germline
yes
1/100 cases, 2/14 chromosomes
SfcI+;BsaXI (2) -, SfcI+;BsaXI (2)-
-
-
Muhammad Ajmal
+/.
2
2
c.61del
r.(?)
p.(Glu21Asnfs*10)
ACMG
pathogenic, pathogenic (recessive)
g.68914341del
g.68448658del
61delG
-
RPE65_000192
ACMG PVS1, PM2, PM3, PP2, PP4
PubMed: Zenteno 2020
-
-
Germline
-
1/143 cases
-
-
-
Johan den Dunnen
,
Juan Carlos Zenteno
+/.
2
-
c.61G>T
r.(?)
p.(Glu21*)
ACMG
pathogenic, pathogenic (recessive)
g.68914340C>A
g.68448657C>A
RPE65 c.61G>T; p.GIu21Ter
-
RPE65_000272
heterozygous
PubMed: Chung 2019
,
PubMed: Sallum 2020
-
-
Germline, Unknown
?
-
-
-
-
LOVD
+/., +?/+?, ?/.
6
2
c.65T>C
r.(?)
p.(Leu22Pro)
ACMG
likely pathogenic (recessive), pathogenic, VUS
g.68914336A>G
g.68448653A>G
119T>C, RPE65 c.65T>C; p.Leu22Pro, RPE65(NM_000329.3):c.65T>C (p.L22P)
-
RPE65_000151
heterozygous, VKGL data sharing initiative Nederland
PubMed: Bernardis 2016
,
PubMed: Marlhens 1998
,
PubMed: Hamel 2001
,
PubMed: Sallum 2020
,
1 more item
-
-
CLASSIFICATION record, Germline, Unknown
?, yes
1/184
NlaIV+
-
-
Muhammad Ajmal
,
VKGL-NL_AMC
+/., +?/+?, +?/.
6
2
c.74C>T
r.(?)
p.(Pro25Leu)
-
likely pathogenic, likely pathogenic (recessive), pathogenic (recessive)
g.68914327G>A
g.68448644G>A
1:68914327G>A ENST00000262340.5:c.74C>T (Pro25Leu), n.128C>T, RPE65 c.74C>T, p.Pro25Leu
-
RPE65_000150
heterozygous
PubMed: Carss 2017
,
PubMed: Chung 2019
,
PubMed: Lorenz 2008
,
PubMed: Turro 2020
-
-
Germline, Germline/De novo (untested)
?, yes
-
ApeKI+;BbvI+;TseI+;AciI-;BsrBI-
-
-
Muhammad Ajmal
+/+
1
2
c.89dup
r.(?)
p.(Thr31Asnfs*21)
-
pathogenic (recessive)
g.68914312dup
g.68448629dup
Ins144T
-
RPE65_000148
-
PubMed: Gu 1997
-
-
Germline
yes
-
HincII+;HpaI+;Hpy166II+;MseI+
-
-
Muhammad Ajmal
?/., ?/?
2
2
c.89T>C
r.(?)
p.(Val30Ala)
-
VUS
g.68914312A>G
g.68448629A>G
-
-
RPE65_000149
-
PubMed: Wang 2014
-
-
Germline
yes
-
HpyCH4V+
-
-
Muhammad Ajmal
+/+, +?/.
2
2
c.90_91insT
r.(?)
p.(Thr31Tyrfs*21), p.(Thr31TyrfsTer21)
-
likely pathogenic, pathogenic (recessive)
g.68914310_68914311insA
g.68448627_68448628insA
144insT, RPE65 p.T31NfsX21 (c.90insT)
-
RPE65_000147
1 more item
PubMed: Preising 2007
,
PubMed: Thompson 2000
-
-
Germline
yes
-
MseI+
-
-
Muhammad Ajmal
+/.
4
-
c.94G>T
r.(?), r.spl?
p.(Gly32Cys)
-
pathogenic (recessive)
g.68914307C>A
g.68448624C>A
G32C
-
RPE65_000227
-
PubMed: Chung 2019
,
PubMed: Li 2019
,
PubMed: Zhong 2019
-
-
Germline
-
-
-
-
-
LOVD
?/?
1
2i
c.94+10A>G
r.spl?
p.(?)
-
VUS
g.68914297T>C
g.68448614T>C
-
-
RPE65_000146
1 more item
PubMed: Henderson 2007
-
-
Germline
yes
-
-
-
-
Muhammad Ajmal
-?/.
2
-
c.95-10T>A
r.(=)
p.(=)
-
likely benign
g.68912553A>T
g.68446870A>T
RPE65(NM_000329.2):c.95-10T>A, RPE65(NM_000329.3):c.95-10T>A
-
RPE65_000198
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
+?/+?
3
2i
c.95-3C>G
r.spl?
p.?
-
likely pathogenic (recessive)
g.68912546G>C
g.68446863G>C
-
-
RPE65_000144
-
PubMed: Jakobsson 2014
-
-
Germline
yes
8/362 chromosomes
-
-
-
Muhammad Ajmal
+/+
4
2i
c.95-2A>C
r.spl
p.?
-
pathogenic (recessive)
g.68912545T>G
g.68446862T>G
-
-
RPE65_000139
-
PubMed: Jacobson 2008
,
PubMed: Jacobson 2009
-
-
Germline
-
-
-
-
-
Muhammad Ajmal
+/+, +/.
12
2i
c.95-2A>T
r.spl, r.spl?
p.?
ACMG
pathogenic, pathogenic (recessive)
g.68912545T>A
g.68446862T>A
c.95-2A>T
-
RPE65_000141
-
Sharon, submitted,
PubMed: Avila Fernandez 2010
,
PubMed: Chung 2019
,
PubMed: Hanein 2004
,
4 more items
-
-
Germline
yes
1/143 cases, 1/2420 IRD families, 14/2420 IRD families
-, ?
-
-
Global Variome, with Curator vacancy
,
Johan den Dunnen
,
Dror Sharon
,
Muhammad Ajmal
+/+, +/.
5
2i
c.95-1G>A
r.spl
p.?
-
pathogenic (recessive)
g.68912544C>T
g.68446861C>T
-
-
RPE65_000116
-
PubMed: Kabir 2013
,
PubMed: Li 2017
-
-
Germline
yes
16/440 chromosomes
-
-
-
Muhammad Ajmal
,
James Hejtmancik
+?/+?
1
3
c.95G>T
r.(?)
p.(Gly32Val)
-
likely pathogenic (recessive)
g.68912543C>A
g.68446860C>A
-
-
RPE65_000115
-
PubMed: Sitorus 2003
-
-
Germline
yes
-
Hpy188III+;HpyCH4III+
-
-
Muhammad Ajmal
+/+
1
3
c.97_116del
r.(?)
p.(Ile34Glnfs*11)
-
pathogenic (recessive)
g.68912523_68912542del
g.68446840_68446859del
97del20bp
-
RPE65_000113
-
PubMed: Jacobson 2009
-
-
Germline
-
-
;DpnII-;HphI-;MboI-;MnlI-;NlaIV-;Sau3AI-
-
-
Muhammad Ajmal
-?/.
2
-
c.102C>A
r.(?)
p.(Ile34=)
-
likely benign
g.68912536G>T
g.68446853G>T
RPE65(NM_000329.2):c.102C>A (p.I34=), RPE65(NM_000329.3):c.102C>A (p.I34=)
-
RPE65_000197
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
+/.
1
3
c.104C>T
r.(?)
p.(Pro35Leu)
-
pathogenic
g.68912534G>A
-
c.104C>T
-
RPE65_000327
-
PubMed: Salmaninejad-202
-
-
Unknown
-
-
-
-
-
LOVD
+?/.
1
3
c.106del
r.(?)
p.(Leu36Serfs*58)
-
likely pathogenic
g.68912532del
-
c.106del (p.(Leu36Serfs*58))
-
RPE65_000329
-
PubMed: SkorczykWerner-2020
-
-
Germline
-
-
-
-
-
LOVD
+/+
1
3
c.106_114del
r.(?)
p.(Leu36_Leu38del)
-
pathogenic (recessive)
g.68912525_68912533del
g.68446842_68446850del
(106del9bp)
-
RPE65_000114
-
PubMed: Sitorus 2003
-
-
Germline
yes
-
HphI-;MnlI-
-
-
Muhammad Ajmal
?/.
1
-
c.110G>C
r.(?)
p.(Trp37Ser)
ACMG
VUS
g.68912528C>G
g.68446845C>G
allele 1: c.110G>C/p.W37S, allele 2: c.722A>G/p.H241R
-
RPE65_000298
heterozygous
PubMed: Weisschuh 2018
-
-
Germline
?
-
-
-
-
LOVD
-?/.
1
-
c.117C>T
r.(?)
p.(Thr39=)
-
likely benign
g.68912521G>A
-
RPE65(NM_000329.2):c.117C>T (p.T39=)
-
RPE65_000202
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/., +?/+?, +?/.
27
3
c.(118G>A), c.118G>A
-, r.(?)
p.(Gly40Ser), p.Gly40Ser
ACMG
likely pathogenic, likely pathogenic (recessive), NA, pathogenic
g.(68912520C>T), g.68912520C>T
g.(68446837C>T), g.68446837C>T
G40S, RPE65 c.118G>A, p.Gly40Ser, RPE65 c.[118G>A];[118G>A], V1: c.118G>A, (p.Gly40Ser),
1 more item
-
RPE65_000049
expression cloning activity <0.02, heterozygous, homozygous, unknown variant 2ndchromosome,
1 more item
PubMed: Chen 2021
,
PubMed: Chen 2021
,
PubMed: Ellingford 2016
,
PubMed: Galvin 2005
,
PubMed: Maeda 2018
,
15 more items
-
rs61751281
Germline, In vitro (cloned), Unknown
?, yes
2/110 cases, 2/18 chromosomes, Taiwan Biobank: 0; GnomAD_exome_East: 0; GnomAD_All: 0.0000239
BsrFI-;HpaII-, BsrFI-;HpaII-;MspI-, MspI-
-
-
Muhammad Ajmal
+/., +?/.
2
3
c.119G>A
r.(?)
p.(Gly40Asp)
ACMG
likely pathogenic, pathogenic (recessive)
g.68912519C>T
g.68446836C>T
RPE65 NM_000329: g.3124G>A, c.119G>A, p.G40D
-
RPE65_000156
-
PubMed: Li 2017
,
PubMed: Xu 2020
-
-
Germline, Unknown
?, yes
-
-
-
-
James Hejtmancik
+/.
1
-
c.124C>T
r.(?)
p.(Leu42Phe)
-
pathogenic (recessive)
g.68912514G>A
g.68446831G>A
L42F
-
RPE65_000271
-
PubMed: Zhong 2019
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/.
16
-
c.130C>T
r.(?)
p.(Arg44*)
ACMG
likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive)
g.68912508G>A
g.68446825G>A
R44X, RPE65 c.130C>T, p.(Arg44*), BBS1 c.1169T>G, p.(Met390Arg), RPE65 c.130C>T, p.Arg44*,
3 more items
-
RPE65_000229
compound heterozygous, probably causal, single heterozygous variant (recessive),
1 more item
PubMed: Chung 2019
,
PubMed: Jespersgaar 2019
,
PubMed: Thompson 2017
,
PubMed: Weisschuh 2020
,
3 more items
-
-
Germline, Germline/De novo (untested), Unknown
?, yes
-
-
-
-
Johan den Dunnen
+/., +?/+?, +?/.
33
3, 9
c.(131G>A), c.131G>A
-, r.(?)
p.(Arg44Gln), p.Arg44Gln
ACMG
likely pathogenic, likely pathogenic (recessive), NA, pathogenic, pathogenic (recessive)
g.(68912507C>T), g.68912507C>T
g.(68446824C>T), g.68446824C>T
c.131C>T, p.(Arg44Gln), R44Q, RPE65 c.131G>A, p.(Arg44Gln),
1 more item
-
RPE65_000036
error in annotation: c.131C>T instead of G>A, compound heterozygous, homozygous,
4 more items
PubMed: Chung 2019
,
PubMed: Coppieters 2010
,
PubMed: Dan 2020
,
PubMed: Ellingford 2016
,
17 more items
-
-
Germline, In vitro (cloned), Unknown
?, yes
1/143 cases, 1/3 cases, 1/91 cases
TaqI-
-
-
Johan den Dunnen
,
Muhammad Ajmal
+?/., ?/?
2
3
c.133T>C
r.(?)
p.(Cys45Arg)
-
likely pathogenic, VUS
g.68912505A>G
g.68446822A>G
-
-
RPE65_000112
-
PubMed: Oishi 2014
-
-
Germline
yes
-
BmgBI+
-
-
Muhammad Ajmal
+/., ?/.
3
-
c.137G>A
r.(?)
p.(Gly46Glu)
ACMG
pathogenic (recessive), VUS
g.68912501C>T
g.68446818C>T
RPE65 c.137G>A; p.GIy46GIu
-
RPE65_000270
heterozygous
PubMed: Chung 2019
,
PubMed: Sallum 2020
-
-
Germline, Unknown
?
-
-
-
-
LOVD
+/+, +/.
8
3
c.138del
r.(?)
p.(Pro47Glnfs*47)
-
pathogenic (recessive)
g.68912502del
g.68446819del
1 bp del 46, 1-bp del of (g) codon 46, 138delG, Gly46 del1, tgtG, Gly46 del1tgtG
-
RPE65_000047
unknown variant 2ndchromosome
PubMed: Chung 2019
,
PubMed: Galvin 2005
,
PubMed: Lotery 2000
,
PubMed: Pasadhika 2010
,
2 more items
-
-
Germline
yes
1/110 cases
EaeI+;MscI+;Sau96I-, Sau96I-
-
-
Muhammad Ajmal
+/+, +/.
2
3
c.144_145insT
r.(?)
p.(Leu49Serfs*3)
-
pathogenic (recessive)
g.68912493_68912494insA
g.68446810_68446811insA
Ins144T
-
RPE65_000046
-
PubMed: Chung 2019
,
PubMed: Lorenz 2000
,
PubMed: Lorenz 2004
-
-
Germline
yes
-
HinfI-;MlyI-;PleI-
-
-
Muhammad Ajmal
+/.
1
-
c.149T>C
r.(?)
p.(Phe50Ser)
-
pathogenic (recessive)
g.68912489A>G
g.68446806A>G
F50S
-
RPE65_000269
-
PubMed: Zhong 2019
-
-
Germline
-
-
-
-
-
LOVD
+?/+?, +?/., ?/.
3
3
c.177C>G
r.(?)
p.(His59Gln)
-
likely pathogenic, likely pathogenic (recessive), VUS
g.68912461G>C
g.68446778G>C
-
-
RPE65_000045
-
PubMed: Katagiri 2014
,
PubMed: Maeda 2018
-
-
Germline
yes
2/14 chromosomes
AluI+;MspA1I+;PvuII+
-
-
Johan den Dunnen
,
Muhammad Ajmal
+/., +?/+?, ?/.
10
3
c.179T>C
r.(?)
p.(Leu60Pro)
-
likely pathogenic (recessive), pathogenic, pathogenic (recessive), VUS
g.68912459A>G
g.68446776A>G
RPE65 c.179T>C, p.Leu60Pro,
1 more item
-
RPE65_000043
heterozygous, ACMG unclassified - no access to supplementary table 2, homozygous
PubMed: Henderson 2007
,
PubMed: Hull 2020
,
PubMed: Kabir 2013
,
PubMed: Li 2017
,
PubMed: Scholl 2015
-
-
Germline, Unknown
?, yes
9/440 chromosomes
BslI+;LpnPI-
-
-
Muhammad Ajmal
,
James Hejtmancik
+/+
1
3
c.183dup
r.(?)
p.(Asp62*)
-
pathogenic (recessive)
g.68912457dup
g.68446774dup
183_184insT
-
RPE65_000042
-
PubMed: Katagiri 2014
-
-
Germline
yes
3/14 chromosomes
CspCI+;XcmI-
-
-
Muhammad Ajmal
?/.
1
-
c.184G>A
r.(?)
p.(Asp62Asn)
ACMG
VUS
g.68912454C>T
g.68446771C>T
RPE65 c.184G>A p.Asp62Asn
-
RPE65_000324
heterozygous
PubMed: Sallum 2020
-
-
Unknown
?
-
-
-
-
LOVD
+?/.
2
3
c.188G>A
r.(?)
p.(Gly63Glu)
ACMG
likely pathogenic, likely pathogenic (recessive)
g.68912450C>T
g.68446767C>T
c.188G>A, NM_000329.2:c.188G>A, NP_000320.1:p.(Gly63Glu), NC_000001.10:g.68912450C>T
-
RPE65_000294
-
PubMed: Liu-2020
,
PubMed: Wang 2018
-
-
Germline
?
-
-
-
-
LOVD
+/.
1
-
c.190C>G
r.(?)
p.(Gln64Glu)
-
pathogenic (recessive)
g.68912448G>C
g.68446765G>C
-
-
RPE65_000268
-
PubMed: Li 2019
-
-
Germline
-
-
-
-
-
LOVD
+/+, +?/+?
2
3
c.190C>T
r.(?)
p.(Gln64*)
-
likely pathogenic (recessive), pathogenic (recessive)
g.68912448G>A
g.68446765G>A
244C>T
-
RPE65_000041
-
PubMed: Hanein 2004
,
PubMed: Perrault 1999
-
-
Germline
yes
-
Cac8I-
-
-
Muhammad Ajmal
+/.
2
3
c.190del
r.(?)
p.(Gln64Lysfs*30)
ACMG
pathogenic, pathogenic (recessive)
g.68912448del
g.68446765del
190delC
-
RPE65_000191
ACMG PVS1, PM2, PM3, PP4
PubMed: Zenteno 2020
-
-
Germline
-
1/143 cases
-
-
-
Johan den Dunnen
,
Juan Carlos Zenteno
+?/.
1
-
c.200T>A
r.(?)
p.(Leu67Gln)
ACMG
likely pathogenic (recessive)
g.68912438A>T
g.68446755A>T
-
-
RPE65_000345
ACMG PP3, PM2, PM5, PM1, PP2
PubMed: Weisschuh 2024
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., +?/+?, +?/., ?/.
18
3
c.200T>G
r.(?)
p.(Leu67Arg)
ACMG
likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive), VUS
g.68912438A>C
g.68446755A>C
c.200T>G, c.[200T>G];[430T>C], L67R, RPE65 NM_000329: g.3205T>G, c.200T>G, p.L67R
-
RPE65_000039
unknown variant 2nd chromosome
PubMed: Chen 2013
,
PubMed: Li 2029
,
PubMed: Li 2020
,
PubMed: Chen-2013
,
PubMed: Fu 2013
,
9 more items
-
-
Germline, Unknown
?, yes
0/384 controls, 1/100, 2/14 chromosomes
HpaII+, HpaII+;MspI+;BsgI-;HpyCH4V-
-
-
Muhammad Ajmal
+?/+?
1
3
c.201_202delinsTT
r.(?)
p.(His68Tyr)
-
likely pathogenic (recessive)
g.68912436_68912437delinsAA
g.68446753_68446754delinsAA
255 and 256 G to T and C to T
-
RPE65_000038
-
PubMed: Marlhens 1998
,
PubMed: Hamel 2001
-
-
Germline
yes
1/184
BsgI-;HpyCH4V-
-
-
Muhammad Ajmal
+/.
1
-
c.(202C>T)
-
p.His68Tyr
-
NA
g.(68912436G>A)
g.(68446753G>A)
H68Y
-
RPE65_000225
expression cloning activity <0.02
PubMed: Redmond 2005
-
-
In vitro (cloned)
-
-
-
-
-
LOVD
+/.
1
-
c.(202_203delinsGC)
-
p.His68Ala
-
NA
g.(68912435_68912436delinsGC)
g.(68446752_68446753delinsGC)
H68A
-
RPE65_000224
expression cloning activity <0.02
PubMed: Redmond 2005
-
-
In vitro (cloned)
-
-
-
-
-
LOVD
?/.
1
-
c.203A>C
r.(?)
p.(His68Pro)
ACMG
VUS
g.68912435T>G
g.68446752T>G
allele 1: c.203A>C/p.H68P, allele 2: c.825C>G/p.Y275*
-
RPE65_000297
heterozygous
PubMed: Weisschuh 2018
-
-
Germline
?
-
-
-
-
LOVD
+/., +?/+?, +?/., ?/.
5
3
c.208T>G
r.(?)
p.(Phe70Val)
-
likely pathogenic (recessive), pathogenic (recessive), VUS
g.68912430A>C
g.68446747A>C
-
-
RPE65_000002
predicted to affect function, but insufficient evidence for definite conclusion,
1 more item
PubMed: Chung 2019
,
PubMed: Neveling 2012
,
PubMed: Pierrache 2020
,
PubMed: Simonelli 2007
-
-
CLASSIFICATION record, Germline
yes
-
BslI+;EcoNI+;HincII+;Hpy166II+
-
-
Kornelia Neveling
,
Muhammad Ajmal
,
VKGL-NL_Nijmegen
+/.
2
-
c.209T>C
r.(?)
p.(Phe70Ser)
ACMG
pathogenic (recessive)
g.68912429A>G
g.68446746A>G
-
-
RPE65_000267
ACMG PP3, PM2, PM5_SUPPORTING, PM1, PP2, PP5_STRONG
PubMed: Chung 2019
,
PubMed: Weisschuh 2024
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/+
3
3
c.219dup
r.(?)
p.(Glu74Argfs*14)
-
pathogenic (recessive)
g.68912421dup
g.68446738dup
271insA
-
RPE65_000035
-
PubMed: Lorenz 2004
-
-
Germline
yes
-
-
-
-
Muhammad Ajmal
?/.
2
-
c.224G>A
r.(?)
p.(Gly75Glu)
-
VUS
g.68912414C>T
g.68446731C>T
RPE65(NM_000329.2):c.224G>A (p.G75E)
-
RPE65_000165
VKGL data sharing initiative Nederland
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
rs201062742
CLASSIFICATION record, Germline
-
17/1204 cases with retinitis pigmentosa
-
-
-
VKGL-NL_Rotterdam
,
Yoshito Koyanagi
+/., +?/.
3
3
c.227A>C
r.(?)
p.(His76Pro)
ACMG
likely pathogenic (recessive), pathogenic
g.68912411T>G
g.68446728T>G
-
-
RPE65_000173
-
Sharon, submitted,
PubMed: Beryozkin 2015
,
PubMed: Sharon 2019
-
-
Germline
-
3/2420 IRD families
-
-
-
Global Variome, with Curator vacancy
,
Johan den Dunnen
,
Dror Sharon
+/., +?/+?, +?/.
4
3
c.235T>C
r.(?)
p.(Tyr79His)
-
likely pathogenic, likely pathogenic (recessive), pathogenic (recessive)
g.68912403A>G
g.68446720A>G
289T->C, RPE65 p.Y79H (c.235T>C)
-
RPE65_000034
heterozygous
PubMed: Chung 2019
,
PubMed: Preising 2007
,
PubMed: Thompson 2000
-
-
Germline
yes
-
-
-
-
Muhammad Ajmal
+?/.
9
-
c.242G>T
r.(242g>u), r.242g>u
p.(Arg81Ile)
-
likely pathogenic, likely pathogenic (recessive)
g.68912396C>A
g.68446713C>A
-
-
RPE65_000177
-
-
000803387
-
Germline
yes
-
-
-
-
Bailey Glen
+/.
1
3i
c.246-11A>G
r.245_246ins246-10_246-1
p.Arg82fs
-
pathogenic (recessive)
g.68910577T>C
-
IVS3-11A>G
-
RPE65_000209
effect RNA analysed using IPS cells, RNA expression reduced
PubMed: Tucker 2015
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., +?/.
6
-
c.247T>C
r.(?)
p.(Phe83Leu)
ACMG
likely pathogenic, pathogenic (recessive)
g.68910565A>G
g.68444882A>G
RPE65 c.247T>C; p.Phe83Leu, SS c.247T>C; p.Phe83Leu
-
RPE65_000266
homozygous
PubMed: Chung 2019
,
PubMed: Sallum 2020
-
-
Germline, Unknown
?
-
-
-
-
LOVD
+?/+?
1
4
c.249C>G
r.(?)
p.(Phe83Leu)
-
likely pathogenic (recessive)
g.68910563G>C
g.68444880G>C
-
-
RPE65_000033
-
PubMed: Koenekoop 2014
-
-
Germline
yes
2/14 chromosomes
DpnI+;DpnII+;MboI+;BtsCI-;FokI-
-
-
Muhammad Ajmal
+/.
1
-
c.250A>T
r.(?)
p.(Ile84Phe)
-
pathogenic
g.68910562T>A
g.68444879T>A
RPE65 c.250A>T, p.Ile84Phe
-
RPE65_000250
heterozygous
PubMed: Scholl 2015
-
-
Unknown
?
-
-
-
-
LOVD
+?/+?, ?/.
2
4
c.253C>T
r.(?)
p.(Arg85Cys)
-
likely pathogenic (recessive), VUS
g.68910559G>A
g.68444876G>A
c.253C>T
-
RPE65_000154
unknown variant 2ndchromosome
PubMed: Colombo-2020
,
PubMed: Stone 2007
-
rs763317722
Germline
-
-
BsgI+;HpyCH4V+;AciI-;BtsCI-;FokI-
-
-
Muhammad Ajmal
+?/+?
1
4
c.254G>A
r.(?)
p.(Arg85His)
-
likely pathogenic (recessive)
g.68910558C>T
g.68444875C>T
308G->A
-
RPE65_000153
unknown variant 2ndchromosome
PubMed: Thompson 2000
-
-
Germline
-
-
MslI+
-
-
Muhammad Ajmal
?/.
2
-
c.259G>A
r.(?)
p.(Asp87Asn)
ACMG
VUS
g.68910553C>T
g.68444870C>T
RPE65 c.259G>A(;)1543C>T, V2: c.259G>A, (p.Asp87Asn),
1 more item
-
RPE65_000328
alleles in cis or trans; heterozygous, heterozygous
PubMed: Chen 2021
,
PubMed: Chen 2021
-
-
Germline/De novo (untested), Unknown
?
Taiwan Biobank: 0; GnomAD_exome_East: 0; GnomAD_All: 0
-
-
-
LOVD
+/., +?/+?, +?/., ?/.
104
4
c.(271C>T), c.271C>T
-, r.(?), r.271c>u
p.(Arg91Trp), p.Arg91Trp
ACMG
likely pathogenic, likely pathogenic (recessive), NA, pathogenic, pathogenic (recessive), VUS
g.(68910541G>A), g.68910541G>A
g.(68444858G>A), g.68444858G>A
325C->T, 325C>T, c.271C>T, c.C271T p.R91W, R01W, RPE65 c.271C>T, p.R91W,
4 more items
-
RPE65_000003
6 heterozygous, no homozygous;
Clinindb (India)
, heterozygous, heterozygous; no second allele found,
7 more items
PubMed: Abu-Safieh-2013
,
PubMed: Bereta 2008
,
PubMed: Chebil 2016
,
PubMed: Chung 2019
,
PubMed: Li 2009
,
33 more items
-
rs61752871
animal model, CLASSIFICATION record, Germline, In vitro (cloned), Unknown
?, yes
1/110 cases, 1/20 cases, 1/3 cases, 2/18 chromosomes, 3/117 cases, 4/46 chromosomes,
1 more item
BsiWI-, BsiWI-;CviQI-;RsaI-
-
-
Johan den Dunnen
,
Kornelia Neveling
,
Muhammad Ajmal
,
VKGL-NL_Nijmegen
,
Mohammed Faruq
+?/.
3
-
c.271_272insA
r.(?)
p.(Arg91GlnfsTer5)
-
likely pathogenic
g.68910540_68910541insT
g.68444857_68444858insT
RPE65 p.R91TfsX5 (c.271insA)
-
RPE65_000333
error in annotation: should be c.271_272insA and not c.271insA, homozygous,
2 more items
PubMed: Preising 2007
-
-
Germline
yes
-
-
-
-
LOVD
+/., +?/+?, +?/., -/-
36
4
c.272G>A
r.(?)
p.(Arg91Gln), p.(Arg91Gly)
ACMG
benign, likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive)
g.68910540C>T
g.68444857C>T
326G->A, R91Q, RPE65 c.272G>A, p.Arg91Gln, RPE65 c.272G>A; p.Arg91GIn,
1 more item
-
RPE65_000081
ACMG PP3, PM2, PM5, PM1, PP2, PP5_STRONG, heterozygous, homozygous, not in 400 controls,
1 more item
0,
PubMed: Chung 2019
,
PubMed: Jacobson 2007
,
PubMed: Jacobson 2007
,
PubMed: Jacobson 2008
,
13 more items
-
-
Germline, Unknown
?, yes
2/14 chromosomes
LpnPI +;BsiWI-, LpnPI+;BsiWI-, SunI+
-
-
Johan den Dunnen
,
Muhammad Ajmal
+?/+?, ?/.
2
4
c.272G>C
r.(?)
p.(Arg91Pro)
ACMG
likely pathogenic (recessive), VUS
g.68910540C>G
g.68444857C>G
RPE65 c.272G>C; p.Arg91Pro
-
RPE65_000152
heterozygous
PubMed: Sallum 2020
,
PubMed: Simonelli 2007
-
-
Germline, Unknown
?, yes
-
BsrFI+;HpaII+;LpnPI+;MspI+;BsiWI-
-
-
Muhammad Ajmal
?/.
1
-
c.275C>A
r.(?)
p.(Ala92Glu)
-
VUS
g.68910537G>T
g.68444854G>T
RPE65(NM_000329.3):c.275C>A (p.A92E)
-
RPE65_000164
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+?/.
2
-
c.283G>C
r.(?)
p.(Glu95Gln)
ACMG
likely pathogenic, likely pathogenic (recessive)
g.68910529C>G
g.68444846C>G
RPE65 p.E95Q (c.283G>C)
-
RPE65_000332
ACMG PM2, PM1, PP2, PP5, heterozygous
PubMed: Preising 2007
,
PubMed: Weisschuh 2024
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.284A>G
r.(?)
p.(Glu95Gly)
-
likely pathogenic
g.68910528T>C
g.68444845T>C
RPE65 c.284A>G, p.E95G
-
RPE65_000331
homozygous
PubMed: Wiszniewski 2011
-
-
Unknown
?
-
-
-
-
LOVD
+/+, +/., +?/+?
3
4
c.289dup
r.(?)
p.(Arg97Lysfs*34)
-
likely pathogenic (recessive), pathogenic (recessive)
g.68910526dup
g.68444843dup
289dupA, 289_290dupA
-
RPE65_000080
unknown variant 2ndchromosome
PubMed: Chung 2019
,
PubMed: den Hollander 2007
,
PubMed: Stone 2007
-
-
Germline
yes
-
-
-
-
Muhammad Ajmal
+/+
3
4
c.289_309del
r.(?)
p.(Arg97_Phe103del)
-
pathogenic (recessive)
g.68910503_68910523del
g.68444820_68444840del
20 bp del codon 97, 97del20bp
-
RPE65_000029
-
PubMed: Jacobson 2005
,
PubMed: Jacobson 2007
,
PubMed: Lotery 2000
-
-
Germline
yes
-
ApoI-;MluCI-
-
-
Muhammad Ajmal
+/+, +/., +?/.
30
4
c.292_311del
r.(?)
p.(Ile98Hisfs*26), p.(Ile98HisfsTer26)
ACMG
likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive)
g.68910501_68910520del, g.68910503_68910522del
g.68444820_68444839del
20 bp deletion in exon 4 nt 344 to 363, 344del20, 97del20bp, Arg97 del20, aGGATCGTCATAACAGAATTT,
2 more items
-
RPE65_000028
DNA change not mentioned, heterozygous, unknown variant 2ndchromosome
PubMed: Jacobson 2007
,
PubMed: Jacobson 2008
,
PubMed: Jacobson 2012
,
PubMed: Panneman 2023
,
6 more items
000803386
-
Germline, Unknown
?, yes
-
ApoI-, ApoI-;MluCI-
-
-
Muhammad Ajmal
,
Bailey Glen
,
Daan Panneman
+/., -/-, -/., ?/.
9
4
c.295G>A
r.(?)
p.(Val99Ile)
-
benign, pathogenic, pathogenic (recessive), VUS
g.68910517C>T
g.68444834C>T
295G>A, RPE65(NM_000329.2):c.295G>A (p.V99I)
-
RPE65_000079
reported as digenic with GUCY2D variant; unknown variant 2ndchromosome; not in 96 controls,
2 more items
PubMed: Li 2011
,
PubMed: Li 2019
,
PubMed: Xu 2014
-
rs143056561
CLASSIFICATION record, Germline
-
1/314 case chromosomes, 4/87 cases, 4/87 cases; 0/96 controls
-
-
-
Muhammad Ajmal
,
VKGL-NL_Rotterdam
+/+
1
4
c.297del
r.(?)
p.(Ile100*)
-
pathogenic (recessive)
g.68910515del
g.68444832del
-
-
RPE65_000078
-
PubMed: Jacobson 2005
-
-
Germline
yes
-
-
-
-
Muhammad Ajmal
+/+
1
4
c.297_308del
r.(?)
p.(Ile100_Phe103del)
-
pathogenic (recessive)
g.68910505_68910516del
g.68444822_68444833del
297del1bp
-
RPE65_000030
-
PubMed: Jacobson 2009
-
-
Germline
-
-
ApoI-;MluCI-
-
-
Muhammad Ajmal
+?/+?
2
4
c.302C>T
r.(?)
p.(Thr101Ile)
-
likely pathogenic (recessive)
g.68910510G>A
g.68444827G>A
-
-
RPE65_000077
not in 400 controls, unknown variant 2ndchromosome
PubMed: Philpa 2009
,
PubMed: Stone 2007
-
-
Germline
yes
-
-
-
-
Muhammad Ajmal
+/., +?/+?
3
4
c.304G>A
r.(?)
p.(Glu102Lys)
-
likely pathogenic (recessive), pathogenic (recessive)
g.68910508C>T
g.68444825C>T
-
-
RPE65_000031
-
PubMed: Chung 2019
,
PubMed: Morimura 1998
,
PubMed: Simonelli 2007
-
-
Germline
yes
-
-
-
-
Muhammad Ajmal
+/+, +/., +?/+?, +?/.
24
4
c.304G>T
r.(?)
p.(Glu102*), p.(Glu102Ter)
ACMG
likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive)
g.68910508C>A
g.68444825C>A
358G->T, c.304G>T (p.Glu102*), RPE65 c.304G>T, p.Glu102X, RPE65 p.E102X (c.304G>T)
-
RPE65_000032
ACMG PM2, PVS1, PP5, homozygous, not in 200 controls, unknown variant 2ndchromosome
Tracewska 2021, MolVis in press,
PubMed: Chung 2019
,
PubMed: Dharmaraj 2000
,
PubMed: Ellingford 2016
,
10 more items
98863
-
Germline, Unknown
?, yes
0,00041 (in-house database, ~5000 samples), 1/100 cases, 1/110 cases
ApoI-
-
-
Johan den Dunnen
,
Muhammad Ajmal
+/., +?/.
3
4
c.310G>A
r.(?)
p.(Gly104Ser)
-
likely pathogenic (recessive), pathogenic, pathogenic (recessive)
g.68910502C>T
g.68444819C>T
c.310G>A
-
RPE65_000265
-
PubMed: Chung 2019
,
PubMed: Ge 2015
,
PubMed: Liu-2020
-
-
Germline
-
-
-
-
-
LOVD
+/+?, +?/.
2
4
c.310G>C
r.(?)
p.(Gly104Arg)
-
likely pathogenic, pathogenic (recessive)
g.68910502C>G
g.68444819C>G
-
-
RPE65_000005
-
PubMed: Abu-Safieh-2013
,
PubMed: Patel 2016
-
-
Germline
yes
-
BanI-
-
-
Leen Abu Safieh
+?/+?, +?/.
2
4
c.311G>A
r.(?)
p.(Gly104Asp)
-
likely pathogenic, likely pathogenic (recessive)
g.68910501C>T
g.68444818C>T
-
-
RPE65_000027
-
PubMed: Huang 2015
,
PubMed: Jacobson 2009
-
-
Germline
yes
-
BanI-;NlaIV-
-
-
Muhammad Ajmal
+/.
1
-
c.311G>T
r.(?)
p.(Gly104Val)
-
pathogenic (recessive)
g.68910501C>A
g.68444818C>A
-
-
RPE65_000280
-
PubMed: Villanueva 2018
-
-
Germline
-
-
-
-
-
LOVD
?/.
1
4
c.314C>A
r.(?)
p.(Thr105Asn)
-
VUS
g.68910498G>T
-
c.314C>A
-
RPE65_000341
-
PubMed: Panneman 2023
-
-
Unknown
-
-
-
-
-
Daan Panneman
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