Global Variome shared LOVD
LCA5 (Leber congenital amaurosis 5)
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Global Variome, with Curator vacancy
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Unique variants in the LCA5 gene
This database is one of the
"Eye disease"
gene variant databases.
The variants shown are described using the
NM_181714.3
NM_001122769.2
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'
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.
106 entries on 2 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.-298+13T>G
r.(=)
p.(=)
-
VUS
g.80246820A>C
g.79537103A>C
-
-
LCA5_000070
9 heterozygous, no homozygous;
Clinindb (India)
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs9343917
Germline
-
9/2795 individuals
-
-
-
Mohammed Faruq
-/.
1
-
c.-297-18C>T
r.(=)
p.(=)
-
benign
g.80235004G>A
g.79525287G>A
-
-
LCA5_000067
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/.
2
2i
c.-192+748_-192+2345del
r.(=)
p.(=)
-
likely pathogenic
g.80232537_80234134del
g.79522820_79524417del
g.[-19612_-18015]del1598
-
LCA5_000004
-
PubMed: den Hollander 2007
,
OMIM:var0004
,
PubMed: Hollander 2007
,
OMIM:var0004
-
-
Germline
yes
-
-
-
-
Frans Cremers
-/.
1
-
c.-62A>G
r.(?)
p.(=)
-
benign
g.80228673T>C
g.79518956T>C
-
-
LCA5_000052
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/.
5
-
c.?
r.(?)
p.0?
-
likely pathogenic
g.80232537_80234134del
g.79522820_79524417del
LCA5
-
LCA5_000004
1 more item
PubMed: Mohamed 2003
-
-
Germline
yes
-
-
-
-
LOVD
+?/+?, +?/.
2
3
c.3G>A
r.(?)
p.(Met1?)
-
likely pathogenic
g.80228609C>T
g.79518892C>T
Met1Ile
-
LCA5_000013
-
PubMed: den Hollander 2007
PubMed: Mackay 2013
,
PubMed: Mackay 2013
,
Journal: Mackay 2013
-
-
Germline, Unknown
?
-
-
-
-
Frans Cremers
-?/.
1
-
c.28A>G
r.(?)
p.(Thr10Ala)
-
likely benign
g.80228584T>C
g.79518867T>C
LCA5(NM_181714.3):c.28A>G (p.T10A)
-
LCA5_000066
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/., +?/+?, +?/.
3
3
c.42_45del
r.(?)
p.(Lys15Glnfs*95), p.(Lys15GlnfsTer95)
-
likely pathogenic, pathogenic
g.80228574_80228577del
g.79518857_79518860del
35_38delAAGA, 42_45delAAAG
-
LCA5_000021, LCA5_000028
-
PubMed: Mackay 2013
,
Journal: Mackay 2013
,
PubMed: Wang 2015
-
-
Germline
-
-
-
-
-
Frans Cremers
+/., +?/+?
2
3
c.69C>G
r.(?)
p.(Tyr23*)
ACMG
likely pathogenic, pathogenic
g.80228543G>C
g.79518826G>C
LCA5 NM_001122769: g.18633C>G, c.69C>G, p.Y23X
-
LCA5_000030
-
PubMed: Mackay 2013
,
Journal: Mackay 2013
,
PubMed: Xu 2020
-
-
Germline
yes
-
-
-
-
Frans Cremers
-/., ?/.
3
-
c.71T>C
r.(?)
p.(Leu24Ser)
-
benign, VUS
g.80228541A>G
g.79518824A>G
LCA5 c.71T>C (p.L24S), LCA5(NM_181714.4):c.71T>C (p.L24S)
-
LCA5_000051
heterozygous, VKGL data sharing initiative Nederland
PubMed: Seong 2009
-
rs2655655
CLASSIFICATION record, Unknown
?
-
-
-
-
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
-/., ?/.
2
-
c.77A>C
r.(?)
p.(Asp26Ala)
-
benign, VUS
g.80228535T>G
g.79518818T>G
LCA5 c.77A>C (p.D26A)
-
LCA5_000050
heterozygous, VKGL data sharing initiative Nederland
PubMed: Seong 2009
-
rs3406846
CLASSIFICATION record, Unknown
?
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
-
c.91C>T
r.(?)
p.(Gln31*)
ACMG
pathogenic
g.80228521G>A
g.79518804G>A
LCA5 NM_001122769: g.18655C>T, c.91C>T, p.Q31X
-
LCA5_000093
-
PubMed: Xu 2020
-
-
Germline
yes
-
-
-
-
LOVD
+/., +?/., ?/.
3
3
c.103C>T
r.(?)
p.(Arg35*)
-
likely pathogenic, pathogenic, VUS
g.80228509G>A
g.79518792G>A
1 more item
-
LCA5_000005
heterozygous, ACMG unclassified - no access to supplementary table 2
PubMed: Corton 2014
,
Journal: Corton 2014
,
PubMed: Gerber 2007
,
PubMed: Hull 2020
-
-
Germline
?, yes
-
-
-
-
Marta Corton
,
Frans Cremers
?/.
1
3
c.131C>A
r.(?)
p.(Ala44Glu)
-
VUS
g.80228481G>T
-
c.131C>A
-
LCA5_000110
-
PubMed: Panneman 2023
-
-
Unknown
-
-
-
-
-
Daan Panneman
?/.
2
-
c.137T>A
r.(?)
p.(Val46Asp)
-
VUS
g.80228475A>T
g.79518758A>T
LCA5(NM_181714.3):c.137T>A (p.V46D), LCA5(NM_181714.4):c.137T>A (p.V46D)
-
LCA5_000055
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
+?/+?, +?/.
4
3
c.142A>T
r.(?)
p.(Arg48*)
-
likely pathogenic
g.80228470T>A
g.79518753T>A
-
-
LCA5_000016
-
PubMed: Mackay 2013
,
PubMed: Mackay 2013
,
Journal: Mackay 2013
-
-
Germline, Unknown
?
-
-
-
-
Frans Cremers
+/.
1
3
c.149del
r.(?)
p.(Asn50Ilefs*61)
-
pathogenic
g.80228468del
g.79518751del
-
-
LCA5_000037
-
PubMed: Corton 2014
,
Journal: Corton 2014
-
-
Germline
yes
-
-
-
-
Marta Corton
-/.
1
-
c.191-9del
r.(=)
p.(=)
-
benign
g.80223467del
g.79513750del
LCA5(NM_181714.4):c.191-9delC
-
LCA5_000049
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/.
1
-
c.191-3del
r.spl?
p.?
-
likely benign
g.80223466del
g.79513749del
LCA5(NM_181714.3):c.191-3delT
-
LCA5_000065
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/.
1
-
c.194del
r.(?)
p.(Pro65Leufs*46)
ACMG
likely pathogenic
g.80223458del
g.79513741del
1 more item
-
LCA5_000097
heterozygous, ACMG classified, novel (Table 2)
PubMed: Hull 2020
-
-
Germline
?
-
-
-
-
LOVD
-/.
1
-
c.197G>A
r.(?)
p.(Arg66Gln)
-
benign
g.80223452C>T
g.79513735C>T
-
-
LCA5_000064
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
3
4
c.238C>T
r.(?)
p.(Arg80*), p.(Arg80Ter)
ACMG
pathogenic
g.80223411>T, g.80223411G>A
g.79513694G>A
-
-
LCA5_000053
-
Sharon, submitted,
PubMed: Sharon 2019
,
PubMed: Wang 2015
-
-
Germline
-
1/2420 IRD families
-
-
-
Global Variome, with Curator vacancy
,
Dror Sharon
+/., +?/+?
4
4
c.256del
r.(?)
p.(Gln86Argfs*25)
-
likely pathogenic, pathogenic
g.80223395del
g.79513678del
-
-
LCA5_000001, LCA5_000033
-
PubMed: Abu-Safieh-2013
-
-
Germline
yes
-
-
-
-
Leen Abu Safieh
,
Frans Cremers
-?/.
1
4
c.301A>G
r.(?)
p.(Thr101Ala)
-
likely benign
g.80223348T>C
-
c.301A>G
-
LCA5_000085
-
PubMed: Chen-2013
-
-
Unknown
-
-
-
-
-
LOVD
-?/.
1
-
c.325C>T
r.(?)
p.(Leu109=)
-
likely benign
g.80223324G>A
-
LCA5(NM_181714.3):c.325C>T (p.L109=)
-
LCA5_000104
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
4
c.334A>G
r.(?)
p.(Ile112Val)
-
VUS
g.80223315T>C
-
c.334A>G (p.I112V)
-
LCA5_000103
-
-
-
-
Germline
-
-
-
-
-
LOVD
?/.
2
4
c.338A>G
r.(?)
p.(Asn113Ser)
-
VUS
g.80223311T>C
g.79513594T>C
A338G, LCA5(NM_181714.3):c.338A>G (p.N113S)
-
LCA5_000063
VKGL data sharing initiative Nederland
PubMed: Katagiri 2014
-
rs181890907
CLASSIFICATION record, Germline
-
-
-
-
-
VKGL-NL_Rotterdam
+?/+?
1
4
c.367C>T
r.(?)
p.(Gln123*)
-
likely pathogenic
g.80223282G>A
g.79513565G>A
-
-
LCA5_000025
-
PubMed: Mackay 2013
,
Journal: Mackay 2013
-
-
Germline
-
-
-
-
-
Frans Cremers
+/+, +/.
2
4
c.393del
r.(?)
p.(Glu132Lysfs*5)
-
pathogenic
g.80223259del
g.79513542del
828delA
-
LCA5_000034
Contact: Carmen Ayuso: CAyuso@fjd.es
PubMed: Corton 2014
,
Journal: Corton 2014
,
PubMed: Vallespin 2010
-
-
Germline
yes
-
-
-
-
Leen Abu Safieh
,
Marta Corton
+?/., ?/.
2
-
c.401A>C
r.(?)
p.(Lys134Thr)
-
likely pathogenic, VUS
g.80223248T>G
g.79513531T>G
LCA5, variant 1: c.516_519del/p.K172Nfs*3, variant 2: c.401A>C/p.K134T
-
LCA5_000073
possibly solved, compound heterozygous
Doucette 2021, submitted,
PubMed: Weisschuh 2020
-
rs200395970
Germline, Unknown
?, yes
-
-
-
-
Lance P Doucette
+?/+?
1
4
c.439_449dup
r.(?)
p.(Glu151*)
-
likely pathogenic
g.80223200_80223210dup
g.79513483_79513493dup
-
-
LCA5_000032
-
PubMed: Mackay 2013
,
Journal: Mackay 2013
-
-
Germline
-
-
-
-
-
Frans Cremers
+/., +?/+?
2
4
c.491A>G
r.(?)
p.(His164Arg)
-
likely pathogenic, pathogenic
g.80223158T>C
g.79513441T>C
c.491T>C, p.(His164Arg)
-
LCA5_000031
error in annotation: c.491T>C instead of A>G, compound heterozygous
PubMed: Mackay 2013
,
Journal: Mackay 2013
,
PubMed: Wang 2019
-
-
Germline
?
-
-
-
-
Frans Cremers
+?/.
1
-
c.516_519del
r.(?)
p.(Lys172Asnfs*3)
-
likely pathogenic
g.80223132_80223135del
g.79513415_79513418del
LCA5, variant 1: c.516_519del/p.K172Nfs*3, variant 2: c.401A>C/p.K134T
-
LCA5_000096
possibly solved, compound heterozygous
PubMed: Weisschuh 2020
-
-
Unknown
?
-
-
-
-
LOVD
?/.
1
-
c.521G>A
r.(?)
p.(Arg174His)
-
VUS
g.80223128C>T
g.79513411C>T
LCA5(NM_181714.3):c.521G>A (p.R174H)
-
LCA5_000048
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
4
c.535_536insT
r.(?)
p.(Gln179Leufs*8)
-
pathogenic
g.80223113_80223114insA
-
c.535_536insT
-
LCA5_000109
-
PubMed: Panneman 2023
-
-
Unknown
-
-
-
-
-
Daan Panneman
?/.
1
-
c.547C>T
r.(?)
p.(Arg183Trp)
-
VUS
g.80223102G>A
g.79513385G>A
LCA5(NM_181714.4):c.547C>T (p.R183W)
-
LCA5_000047
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/.
1
-
c.586C>T
r.(?)
p.(Leu196=)
-
benign
g.80223063G>A
g.79513346G>A
LCA5(NM_181714.4):c.586C>T (p.L196=)
-
LCA5_000062
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/+?, +/., +?/.
4
4
c.604T>C
r.(?)
p.(Ser202Pro)
-
likely pathogenic, pathogenic
g.80223045A>G
g.79513328A>G
1039T>C, LCA5 c.1039T>C, p.Ser202Pro
-
LCA5_000035
obsolete nucleotide annotation, extrapolated from sequence and databases,
2 more items
PubMed: Corton 2014
,
Journal: Corton 2014
,
PubMed: Vallespin 2010
-
-
Germline, Unknown
?, yes
-
-
-
-
Leen Abu Safieh
,
Marta Corton
+/.
1
-
c.609A>G
r.(?)
p.(Leu203=)
-
pathogenic
g.80223040T>C
g.79513323T>C
-
-
LCA5_000078
-
PubMed: Costa 2017
-
-
Germline
-
-
-
-
-
LOVD
+?/.
5
4
c.610C>T
r.(?)
p.(Gln204*)
-
likely pathogenic
g.80223039G>A
g.79513322G>A
-
-
LCA5_000006
-
PubMed: Gerber 2007
-
-
Germline
yes
-
-
-
-
Frans Cremers
+/., +?/+?, +?/., ?/.
6
4
c.633_639del
r.(?)
p.(Glu211Aspfs*13)
-
likely pathogenic, pathogenic (recessive), VUS
g.80223010_80223016del, g.80223012_80223018del
g.79513295_79513301del
633_639delAGCTAGA, 6:80223009GTCTAGCT>G ENST00000392959.1:c.633_639delAGCTAGA (Glu211AspfsTer13),
1 more item
-
LCA5_000012
solved, homozygous
PubMed: Carss 2017
,
PubMed: Ellingford 2016
,
PubMed: Mackay 2013
,
PubMed: Weisschuh 2020
,
1 more item
-
-
Germline, Unknown
?, yes
-
-
-
-
Frans Cremers
+?/.
2
-
c.634G>C
r.(?)
p.(Ala212Pro)
-
likely pathogenic
g.80223015C>G
g.79513298C>G
LCA5 c.[634G>C];[1322A>G], p.[Ala212Pro];[Tyr441Cys]
-
LCA5_000100
heterozygous
PubMed: Chen 2016
-
-
Germline
yes
-
-
-
-
LOVD
+?/+?, -/.
2
4
c.634G>T
r.(?)
p.(Ala212Ser)
-
benign, likely pathogenic
g.80223015C>A
g.79513298C>A
634G>T
-
LCA5_000024
-
PubMed: li 2011
-
-
Germline, Unknown
?
1/87 cases; 0/96 controls
-
-
-
Frans Cremers
+?/.
4
4
c.643del
r.(?)
p.(Leu215Tyrfs*11)
-
likely pathogenic
g.80223007del
g.79513290del
-
-
LCA5_000009
-
PubMed: Ahmad 2011
-
-
Germline
yes
-
-
-
-
Frans Cremers
+/.
1
4
c.652C>G
r.(?)
p.(Arg218Gly)
-
pathogenic
g.80222997G>C
g.79513280G>C
-
-
LCA5_000042
-
PubMed: Li 2017
-
-
Germline
yes
-
-
-
-
James Hejtmancik
?/.
1
-
c.661T>G
r.(?)
p.(Leu221Val)
-
VUS
g.80222988A>C
g.79513271A>C
NM_001122769.2:c.661T>G
-
LCA5_000079
-
PubMed: Tiwari 2016
-
-
Germline
-
-
-
-
-
LOVD
?/.
1
4
c.664G>C
r.(?)
p.(Ala222Pro)
-
VUS
g.80222985C>G
-
c.664G>C
-
LCA5_000108
-
PubMed: Panneman 2023
-
-
Unknown
-
-
-
-
-
Daan Panneman
?/.
1
-
c.669G>T
r.(?)
p.(Lys223Asn)
ACMG
VUS
g.80222980C>A
-
-
-
LCA5_000072
ACMG grading: PM2,PP3
-
-
-
Germline
-
-
-
-
-
Andreas Laner
+/., +?/.
2
4, 4i
c.720+1G>A
r.(?), r.0
p.(?), p.0
-
likely pathogenic, pathogenic
g.80222928C>T
g.79513211C>T
c.720+1G>A, p.?
-
LCA5_000039
Homozygous, no mRNA detected
PubMed: Corton 2014
,
Journal: Corton 2014
,
PubMed: Tayebi 2019
-
-
Germline
yes
-
-
-
-
Marta Corton
+/.
1
-
c.721-2A>G
r.spl
p.(?)
-
pathogenic
g.80203469T>C
g.79493752T>C
c.721-2T>C, p.?
-
LCA5_000092
error in annotation: c.721-2T>C instead of A>G, compound heterozygous
PubMed: Wang 2019
-
-
Germline
yes
-
-
-
-
LOVD
+/.
1
5
c.763C>T
r.(?)
p.(Arg255*)
-
pathogenic
g.80203425G>A
-
c.763C>T
-
LCA5_000087
-
PubMed: Eisenberger-2013
-
rs151017794
Germline
-
-
-
-
-
LOVD
+?/.
1
5
c.764G>A
r.(?)
p.(Arg255Gln)
-
likely pathogenic
g.80203424C>T
-
c.764G>A
-
LCA5_000086
-
PubMed: Eisenberger-2013
-
-
Germline
-
-
-
-
-
LOVD
+?/.
1
-
c.787del
r.(?)
p.(Arg263Glyfs*23)
-
likely pathogenic
g.80203404del
g.79493687del
LCA5 c.828delA, p.Lys131fs
-
LCA5_000099
obsolete nucleotide annotation, extrapolated from sequence and databases
PubMed: Vallespin 2010
-
-
Unknown
?
-
-
-
-
LOVD
+/., +?/+?, +?/., ?/.
8
5
c.795T>G
r.(?)
p.(Tyr265*), p.(Tyr265Ter)
ACMG
likely pathogenic, pathogenic, VUS
g.80203393A>C
g.79493676A>C
c.795A>C, p.(Tyr265*), c.[795T>G];[795T>G],
1 more item
-
LCA5_000019
compound heterozygous
PubMed: Chen-2013
,
PubMed: Mackay 2013
,
PubMed: Mackay 2013
,
Journal: Mackay 2013
,
PubMed: Wang 2015
,
2 more items
-
-
Germline, Unknown
?, yes
0/384 controls
-
-
-
Frans Cremers
+?/.
4
5
c.[823C>A;829del]
r.(?)
p.[(Gln275Lys);(Glu277Argfs*9)]
-
likely pathogenic
g.80203359del, g.80203365G>T
-
c.[823C>A;829del]
-
LCA5_000107
-
PubMed: Panneman 2023
-
-
Unknown
-
-
-
-
-
Daan Panneman
+/., +?/+?, +?/., ?/.
16
5
c.835C>T
r.(?)
p.(Gln279*)
ACMG
likely pathogenic, pathogenic, VUS
g.80203353>T, g.80203353G>A
g.79493636G>A
c.835C>T, LCA5 c.835C>T, p.Q279X, LCA5 Gln279Stop
-
LCA5_000003
homozygous
Jacobson 2009, Sharon, submitted,
PubMed: den Hollander 2007
,
PubMed: Mackay 2013
,
PubMed: Mackay 2013
,
6 more items
-
rs121918165
Germline, Unknown
?, yes
3/2420 IRD families
-
-
-
Global Variome, with Curator vacancy
,
Johan den Dunnen
,
Dror Sharon
,
Frans Cremers
+/., +?/.
8
5
c.838C>T
r.(?)
p.(Arg280*), p.(Arg280Ter)
ACMG
likely pathogenic, pathogenic, pathogenic (recessive)
g.80203350G>A
g.79493633G>A
6:80203350G>A ENST00000392959.1:c.838C>T (Arg280Ter), LCA5 c.838C>T, p.Arg280Ter,
2 more items
-
LCA5_000041
homozygous
PubMed: Carss 2017
,
PubMed: Consugar 2015
,
PubMed: Sallum 2020
,
PubMed: Turro 2020
-
-
Germline, Germline/De novo (untested), Unknown
?, yes
-
-
-
-
Soumittra Nagasamy
-?/.
1
-
c.858+5G>C
r.spl?
p.?
-
likely benign
g.80203325C>G
g.79493608C>G
LCA5(NM_181714.3):c.858+5G>C
-
LCA5_000061
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.858+77C>T
r.(?)
p.(?)
-
VUS
g.80203253G>A
g.79493536G>A
LCA5 c.858+77C>T
-
LCA5_000102
heterozygous
PubMed: Seong 2009
-
rs9443675
Unknown
?
-
-
-
-
LOVD
?/.
1
-
c.858+127_858+129delATC
r.(?)
p.(?)
-
VUS
g.80203201_80203203del
g.79493484_79493486del
LCA5 c.858+127 129delATC
-
LCA5_000101
heterozygous
PubMed: Seong 2009
-
-
Unknown
?
-
-
-
-
LOVD
-/.
1
-
c.859-12G>C
r.(=)
p.(=)
-
benign
g.80202376C>G
g.79492659C>G
-
-
LCA5_000060
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
-
c.871_872del
r.(?)
p.(Glu291ThrfsTer9)
-
pathogenic
g.80202357_80202358del
g.79492640_79492641del
-
-
LCA5_000083
-
PubMed: Wang 2015
-
-
Germline
-
-
-
-
-
LOVD
+/.
1
-
c.878del
r.(?)
p.(Asp293ValfsTer2)
-
pathogenic
g.80202345del
g.79492628del
-
-
LCA5_000082
-
PubMed: Wang 2015
-
-
Germline
-
-
-
-
-
LOVD
?/.
1
-
c.902G>A
r.(?)
p.(Arg301His)
-
VUS
g.80202321C>T
-
LCA5(NM_181714.3):c.902G>A (p.R301H)
-
LCA5_000090
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/.
1
-
c.923del
r.(?)
p.(Asn308Ilefs*8)
-
likely pathogenic
g.80202302del
g.79492585del
Allele 1 c.923del (p.Asn308Ilefs*8), Allele 2 c.923del (p.Asn308Ilefs*8)
-
LCA5_000091
homozygous
PubMed: Khan 2019
-
-
Germline/De novo (untested)
?
-
-
-
-
LOVD
+/., +?/., ?/.
3
6
c.955G>A
r.(?), r.(spl?)
p.(Ala319Thr), p.[Ala319Thr;?]
ACMG
likely pathogenic, pathogenic, VUS
g.80202268C>T
g.79492551C>T
LCA7 c.955G>A; p.AIa319Thr/p.?
-
LCA5_000008
homozygous
PubMed: Corton 2014
,
Journal: Corton 2014
,
PubMed: Ramprasad 2008
,
PubMed: Sallum 2020
-
-
Germline, Unknown
?, yes
-
-
-
-
Marta Corton
,
Frans Cremers
+?/.
1
-
c.1020del
r.(?)
p.(Lys340AsnfsTer7)
-
likely pathogenic
g.80201383del
g.79491666del
-
-
LCA5_000080
-
PubMed: Patel 2016
-
-
Germline
-
-
-
-
-
LOVD
+/.
1
7
c.1062_1068del
r.(?)
p.(Tyr354*)
-
pathogenic
g.80201335_80201341del
-
c.1062_1068del
-
LCA5_000088
-
PubMed: Beryozkin-2014
-
-
Germline
-
-
-
-
-
LOVD
?/.
1
-
c.1097T>C
r.(?)
p.(Leu366Ser)
-
VUS
g.80201306A>G
g.79491589A>G
LCA5(NM_181714.4):c.1097T>C (p.L366S)
-
LCA5_000059
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
1
-
c.1098+3G>T
r.spl?
p.?
-
VUS
g.80201302C>A
-
LCA5(NM_181714.3):c.1098+3G>T
-
LCA5_000074
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/.
1
-
c.1144_1147dup
r.(?)
p.(Asn383Thrfs*15)
-
likely pathogenic (recessive)
g.80198885_80198888dup
g.79489168_79489171dup
-
-
LCA5_000075
-
PubMed: Thompson 2017
-
-
Germline
-
-
-
-
-
LOVD
+/+, +/., +?/+?, +?/.
22
8
c.1151del
r.(?)
p.(Pro384Glnfs*18)
ACMG
likely pathogenic, pathogenic
g.80198881del, g.80198883del
g.79489166del
1151delC, c.1151delC (p.Pro384GlnfsX17), LCA5: c.1151delC (NM_001122769.2, p.P384fs (NP_001116241.1)
-
LCA5_000001, LCA5_000043
ACMG grading: PVS1,PM2,PM3, homozygous
den Hollander et al. 2007. Nat Genet 39: 889; Maranhao et al. 2015. PLoS One 10: 136561,
6 more items
-
rs386834252
Germline, Unknown
?, yes
-
-
-
needs Curator approval
Johan den Dunnen
,
Andreas Laner
,
Frans Cremers
,
James Hejtmancik
+/.
1
-
c.1171A>T
r.(?)
p.(Lys391*)
ACMG
pathogenic
g.80198861T>A
-
-
-
LCA5_000071
-
PubMed: Sharon 2019
-
-
Germline
-
1/2420 IRD families
-
-
-
Global Variome, with Curator vacancy
+?/.
1
8
c.1186G>T
r.(?)
p.(Glu396*)
-
likely pathogenic
g.80198846C>A
g.79489129C>A
-
-
LCA5_000007
-
PubMed: Gerber 2007
-
-
Germline
yes
-
-
-
-
Frans Cremers
+/., +?/+?, +?/.
3
8
c.1207C>T
r.(?)
p.(Gln403*), p.(Gln403Ter)
-
likely pathogenic, pathogenic
g.80198825G>A
g.79489108G>A
-
-
LCA5_000022
-
PubMed: Mackay 2013
,
Journal: Mackay 2013
,
PubMed: Wang 2015
-
-
Germline
-
-
-
-
-
Frans Cremers
-?/.
1
-
c.1215T>G
r.(?)
p.(Val405=)
-
likely benign
g.80198817A>C
g.79489100A>C
LCA5(NM_181714.3):c.1215T>G (p.V405=)
-
LCA5_000069
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/+?, +?/.
2
8i
c.1231+2dup
r.spl?
p.?
-
likely pathogenic
g.80198799dup
g.79489082dup
1231+2insT
-
LCA5_000000, LCA5_000026
-
PubMed: Mackay 2013
,
PubMed: Mackay 2013
,
Journal: Mackay 2013
-
-
Germline, Unknown
?
-
-
-
-
Frans Cremers
-/.
1
-
c.1232-15T>A
r.(=)
p.(=)
-
benign
g.80197598A>T
g.79487881A>T
LCA5(NM_181714.4):c.1232-15T>A
-
LCA5_000058
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+?/.
4
-
c.1243G>T
r.(?)
p.(Glu415Ter)
-
likely pathogenic
g.80197572C>A
g.79487855C>A
LCA5 c.1243G>T; c.1243G>T
-
LCA5_000095
no protein change given, homozygous
PubMed: Zanolli 2020
-
-
Unknown
?
-
-
-
-
LOVD
-?/.
1
-
c.1260G>A
r.(?)
p.(Lys420=)
-
likely benign
g.80197555C>T
g.79487838C>T
LCA5(NM_181714.3):c.1260G>A (p.K420=)
-
LCA5_000057
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
9
c.1261C>T
r.(?)
p.(Gln421*)
-
pathogenic
g.80197554G>A
g.79487837G>A
-
-
LCA5_000036
-
-
-
-
Germline
-
-
-
-
-
Amy Turriff
-?/.
1
9
c.1316G>A
r.(?)
p.(Gly439Glu)
-
likely benign
g.80197499C>T
g.79487782C>T
-
-
LCA5_000010
-
PubMed: Wiszniewski 2010
-
-
Unknown
?
-
-
-
-
Frans Cremers
+?/.
2
-
c.1322A>G
r.(?)
p.(Tyr441Cys)
-
likely pathogenic
g.80197493T>C
g.79487776T>C
LCA5 c.[634G>C];[1322A>G], p.[Ala212Pro];[Tyr441Cys]
-
LCA5_000098
heterozygous
PubMed: Chen 2016
-
-
Germline
yes
-
-
-
-
LOVD
-?/., ?/.
2
-
c.1323C>T
r.(?)
p.(=), p.(Tyr441=)
-
likely benign, VUS
g.80197492G>A
g.79487775G>A
LCA5(NM_181714.4):c.1323C>T (p.Y441=)
-
LCA5_000077
VKGL data sharing initiative Nederland
PubMed: Costa 2017
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
VKGL-NL_AMC
?/.
1
9
c.1418A>C
r.(?)
p.(Glu473Ala)
-
VUS
g.80197397T>G
g.79487680T>G
-
-
LCA5_000038
predicted to affected function, unknown second allele; not found in 100 controls
PubMed: Corton 2014
,
Journal: Corton 2014
-
-
Germline
?
-
-
-
-
Marta Corton
+?/.
1
9
c.1422del
r.(?)
p.(Ile474Metfs*11)
-
likely pathogenic
g.80197394del
g.79487677del
-
-
LCA5_000040
-
-
-
-
Germline
yes
-
-
-
-
Soumittra Nagasamy
+?/.
1
9
c.1443_1444delinsA
r.(?)
p.(Arg482Glufs*3)
-
likely pathogenic
g.80197371_80197372delinsT
g.79487654_79487655delinsT
-
-
LCA5_000017
-
PubMed: Mackay 2013
,
Journal: Mackay 2013
-
-
Germline
-
-
-
-
-
Frans Cremers
+/.
1
-
c.1466del
r.(?)
p.(Leu489CysfsTer104)
-
pathogenic
g.80197352del
g.79487635del
-
-
LCA5_000081
-
PubMed: Wang 2015
-
-
Germline
-
-
-
-
-
LOVD
+?/.
1
9
c.1476dup
r.(?)
p.(Pro493Thrfs*2)
-
likely pathogenic
g.80197339dup
g.79487622dup
-
-
LCA5_000002
-
PubMed: Hollander 2007
,
OMIM:var0002
-
-
Germline
yes
-
-
-
-
Frans Cremers
?/.
1
-
c.1533C>A
r.(?)
p.(Phe511Leu)
-
VUS
g.80197282G>T
g.79487565G>T
LCA5(NM_181714.3):c.1533C>A (p.F511L)
-
LCA5_000068
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/., -?/.
2
-
c.1642C>T
r.(?)
p.(Pro548Ser)
-
benign, likely benign
g.80197173G>A
g.79487456G>A
LCA5 c.1642C>T (p.P548S), LCA5(NM_181714.3):c.1642C>T (p.P548S)
-
LCA5_000046
heterozygous, VKGL data sharing initiative Nederland
PubMed: Seong 2009
-
-
CLASSIFICATION record, Unknown
?
-
-
-
-
VKGL-NL_Rotterdam
+/., +?/+?, +?/.
4
9
c.1676C>A
r.(?)
p.(Ser559*)
-
likely pathogenic, pathogenic, pathogenic (recessive)
g.80197139G>T
g.79487422G>T
6:80197139G>T ENST00000392959.1:c.1676C>A (Ser559Ter), LCA5 c.1676C>A, p.Ser559Ter
-
LCA5_000014
homozygous
PubMed: Carss 2017
,
PubMed: Mackay 2013
,
PubMed: Mackay 2013
,
Journal: Mackay 2013
,
PubMed: Turro 2020
-
-
Germline, Germline/De novo (untested), Unknown
?
-
-
-
-
Frans Cremers
+/.
1
9
c.1714C>T
r.(?)
p.(Gln572*)
-
pathogenic
g.80197101G>A
g.79487384G>A
-
-
LCA5_000054
-
Sharon, submitted
-
-
Germline
-
-
-
-
-
Dror Sharon
+?/+?, +?/.
2
9
c.1730dup
r.(?)
p.(Leu577Phefs*12)
-
likely pathogenic
g.80197090dup
g.79487373dup
-
-
LCA5_000018
no variant found on other allele
PubMed: Mackay 2013
,
PubMed: Mackay 2013
,
Journal: Mackay 2013
-
-
Germline, Unknown
?
-
-
-
-
Frans Cremers
-/.
1
-
c.1746C>T
r.(?)
p.(Asn582=)
-
benign
g.80197069G>A
g.79487352G>A
LCA5(NM_181714.4):c.1746C>T (p.N582=)
-
LCA5_000045
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/., +?/+?
2
9
c.1756A>T
r.(?)
p.(Lys586*)
-
likely pathogenic, pathogenic
g.80197059T>A
g.79487342T>A
NM_001122769.2:c.1756A>T
-
LCA5_000011
-
PubMed: Carrigan 2016
,
PubMed: Mackay 2013
,
Journal: Mackay 2013
-
-
Germline
-
-
-
-
-
Frans Cremers
+/., +?/+?, +?/.
3
9
c.1759_1760insAG
r.(?)
p.(Leu587Glnfs*7), p.(Leu587GlnfsTer7)
-
likely pathogenic, pathogenic
g.80197055_80197056insCT
g.79487338_79487339insCT
-
-
LCA5_000020
-
PubMed: Mackay 2013
,
Journal: Mackay 2013
,
PubMed: Wang 2015
-
-
Germline, Unknown
?
-
-
-
-
Frans Cremers
+/., +?/.
5
9
c.1819_1820del
r.(?)
p.(Gln607Valfs*7)
ACMG
likely pathogenic, likely pathogenic (recessive), pathogenic
g.80196995_80196996del, g.80196996_80196997del
g.79487279_79487280del
1818_1819delAC, c.1818_1819delAC, c.1819_1820delAC, p.(Gln607Valfs*7),
1 more item
-
LCA5_000023
error in annotation: c.1819_1820delAC instead of c.1819_1820del, Homozygous
PubMed: Liu-2020
,
PubMed: Stone 2017
,
PubMed: Wang 2018
,
PubMed: Wang 2019
-
-
Germline, Unknown
?
-
-
-
-
Frans Cremers
+/.
1
9
c.1819_1820delCA
r.(?)
p.(Gln607Valfs*7)
-
pathogenic
g.80196995_80196996delTG
-
1819_1820delCA
-
LCA5_000084
-
PubMed: li 2011
-
-
Germline
-
1/87 cases; 0/96 controls
-
-
-
LOVD
?/.
1
9
c.1825T>C
r.(?)
p.(Phe609Leu)
-
VUS
g.80196990A>G
-
c.1825T>C
-
LCA5_000106
-
PubMed: Panneman 2023
-
-
Unknown
-
-
-
-
-
Daan Panneman
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