Global Variome shared LOVD
NRL (neural retina leucine zipper)
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Curator:
Global Variome, with Curator vacancy
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Unique variants in the NRL gene
This database is one of the
"Eye disease"
gene variant databases.
The variants shown are described using the NM_006177.3 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.
76 entries on 1 page. Showing entries 1 - 76.
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Legend
How to query
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.-19344G>A
r.(?)
p.(=)
-
likely benign
g.24573045C>T
g.24103836C>T
PCK2(NM_001308054.1):c.1393C>T (p.P465S)
-
DCAF11_000010
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/.
1
-
c.-19290C>T
r.(?)
p.(=)
-
benign
g.24572991G>A
g.24103782G>A
PCK2(NM_001308054.1):c.1339G>A (p.A447T)
-
DCAF11_000006
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.-19222A>G
r.(?)
p.(=)
-
VUS
g.24572923T>C
g.24103714T>C
PCK2(NM_001308054.1):c.1271T>C (p.I424T)
-
DCAF11_000009
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.-19156C>T
r.(?)
p.(=)
-
VUS
g.24572857G>A
-
PCK2(NM_004563.4):c.1607G>A (p.R536Q)
-
DCAF11_000026
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
?/.
1
-
c.-19110G>A
r.(?)
p.(=)
-
VUS
g.24572811C>T
-
PCK2(NM_001308054.1):c.1159C>T (p.R387C)
-
DCAF11_000020
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.-19050G>A
r.(?)
p.(=)
-
VUS
g.24572751C>T
-
PCK2(NM_004563.4):c.1501C>T (p.R501W)
-
DCAF11_000025
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-?/.
1
-
c.-19009dup
r.(?)
p.(=)
-
likely benign
g.24572716dup
-
PCK2(NM_001308054.1):c.1067-3dupC
-
DCAF11_000022
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.-15722C>A
r.(?)
p.(=)
-
VUS
g.24569423G>T
-
PCK2(NM_001018073.2):c.1235G>T (p.G412V), PCK2(NM_004563.3):c.1234+1G>T
-
DCAF11_000027
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.-15645C>T
r.(?)
p.(=)
-
likely benign
g.24569346G>A
-
PCK2(NM_001308054.1):c.756G>A (p.V252=)
-
DCAF11_000023
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.-15642G>A
r.(?)
p.(=)
-
likely benign
g.24569343C>T
-
PCK2(NM_001308054.1):c.753C>T (p.G251=)
-
DCAF11_000019
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.-14661A>C
r.(?)
p.(=)
-
VUS
g.24568362T>G
g.24099153T>G
PCK2(NM_001018073.1):c.769T>G (p.(Ser257Ala))
-
PCK2_000002
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
-
c.-14183G>A
r.(?)
p.(=)
-
VUS
g.24567884C>T
-
PCK2(NM_004563.4):c.661C>T (p.(Gln221Ter))
-
DCAF11_000028
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
3
-
c.-14099G>A
r.(?)
p.(=)
-
VUS
g.24567800C>T
-
PCK2(NM_004563.4):c.577C>T (p.(Arg193Ter), p.R193*)
-
DCAF11_000018
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
+/.
1
-
c.-13895_-13894insTCTCATCCACAGC
r.(?)
p.(=)
-
pathogenic
g.24567596_24567597insCTGTGGATGAGAG
-
PCK2(NM_001308054.1):c.58_59insCTGTGGATGAGAG (p.G20Afs*31)
-
DCAF11_000017
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.-13892_-13891insGAAACCTCT
r.(?)
p.(=)
-
VUS
g.24567592_24567593insAGAGGTTTC
-
PCK2(NM_001308054.1):c.54_55insAGAGGTTTC (p.M18_Q19insRGF)
-
DCAF11_000016
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.-13803G>A
r.(?)
p.(=)
-
likely benign
g.24567504C>T
g.24098295C>T
PCK2(NM_004563.3):c.368C>T (p.P123L)
-
DCAF11_000008
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/.
1
-
c.-13797T>G
r.(?)
p.(=)
-
benign
g.24567498A>C
g.24098289A>C
PCK2(NM_004563.4):c.362A>C (p.Q121P)
-
PCK2_000001
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+?/.
1
-
c.-27-2A>G
r.spl?
p.(?)
-
likely pathogenic
g.24552086T>C
-
c.-27-2A>G
-
NRL_000044
-
PubMed: Panneman 2023
-
-
Unknown
-
-
-
-
-
Daan Panneman
+/.
3
-
c.?
r.?
p.?
-
pathogenic, pathogenic (dominant)
g.?
-
p.Ala129fs, p.Ser6fs, Pro51Ala
-
SERPINA1_000009
-
PubMed: Carrigan 2016
,
PubMed: Daiger 2014
-
-
Germline
-
-
-
-
-
LOVD
+/.
1
-
c.16del
r.(?)
p.(Ser6Alafs*13)
-
pathogenic
g.24552042del
g.24082833del
16delA
-
NRL_000025
-
PubMed: Carrigan 2016
-
-
Germline
-
-
-
-
-
LOVD
+/.
1
-
c.23del
r.(?)
p.(Leu8ArgfsTer11)
-
pathogenic
g.24552035del
g.24082826del
23delT
-
NRL_000028
-
PubMed: Oishi 2014
-
-
Germline
-
-
-
-
-
LOVD
?/.
1
-
c.41A>C
r.(?)
p.(Asn14Thr)
-
VUS
g.24552017T>G
-
NRL(NM_001354768.3):c.41A>C (p.(Asn14Thr))
-
DCAF11_000029
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
2
-
c.73C>T
r.(?)
p.(Arg25Trp)
-
VUS
g.24551985G>A
g.24082776G>A
-
-
NRL_000016
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
-
Germline
-
1/1204 cases with retinitis pigmentosa, 6/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
?/.
1
-
c.74G>A
r.(?)
p.(Arg25Gln)
-
VUS
g.24551984C>T
-
NRL(NM_006177.5):c.74G>A (p.R25Q)
-
DCAF11_000021
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
+/., +?/.
30
2
c.91C>T
r.(?)
p.(Arg31*)
ACMG
likely pathogenic, pathogenic
g.24551967G>A
g.24082758G>A
NRL p.R31X, NRL, variant 1: c.91C>T/p.R31*, variant 2: c.223dup/p.L75Pfs*19,
1 more item
-
NRL_000011
heterozygous, homozygous, solved, compound heterozygous
Sharon, submitted,
PubMed: Braverman 2017
,
PubMed: Ehrenberg 2019
,
PubMed: Sharon 2019
,
1 more item
-
-
Germline, Unknown
?, yes
1/2420 IRD families, 3/2420 IRD families
-
-
-
Global Variome, with Curator vacancy
,
Dror Sharon
+/.
3
-
c.104dup
r.(?)
p.(Thr36Tyrfs*20)
-
pathogenic
g.24551954dup, g.24551959dup
g.24082750dup
c.104dup
-
NRL_000009
-
PubMed: Haer-Wigman 2017
,
PubMed: Panneman 2023
-
-
Germline, Unknown
-
-
-
-
-
Daan Panneman
+/.
7
3
c.146C>T
r.(?)
p.(Pro49Leu)
-
pathogenic
g.24551912G>A
g.24082703G>A
NRL c.146 C>T, p.P49L
-
NRL_000043
heterozygous
PubMed: Gao 2016
-
-
Germline
yes
-
-
-
-
LOVD
+/.
2
3
c.147_149del
r.(?)
p.Ser50del
-
pathogenic
g.24551910_24551912del
g.24082701_24082703del
NRL c.147_149del, p.Ser50del
-
NRL_000042
heterozygous
PubMed: Qin 2017
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
13
-
c.148T>A
r.(?)
p.(Ser50Thr)
-
likely pathogenic, likely pathogenic (dominant)
g.24551910A>T
g.24082701A>T
NRL c.148T>A, p.(Ser50Thr), NRL c.148T>A, p.S50T
-
NRL_000034
heterozygous,
1 more item
PubMed: Bessant 1999
,
PubMed: Bessant 2000
,
PubMed: Kanda 2007
-
-
Germline, In vitro (cloned)
?, yes
-
-
-
-
LOVD
+/., +?/.
6
3
c.148T>C
r.(?)
p.(Ser50Pro)
-
likely pathogenic, pathogenic
g.24551910A>G
g.24082701A>G
NRL c.148T>C, p.S50P, NRL TCA -> CCA, Ser50Pro
-
NRL_000019
no nucleotide annotation, extrapolated from protein; heterozygous,
2 more items
PubMed: DeAngelis 2002
,
PubMed: Kanda 2007
,
PubMed: Zenteno 2020
-
-
CLASSIFICATION record, Germline, In vitro (cloned)
?, yes
1/143 cases
-
-
-
Johan den Dunnen
,
VKGL-NL_Nijmegen
+/., +?/., ?/.
14
3
c.149C>T
r.(?)
p.(Ser50Leu)
ACMG
likely pathogenic, pathogenic, VUS
g.24551909G>A
g.24082700G>A
NRL c.149C>T, p.S50L, NRL c.C149T, p.S50L, NRL TCA -> TTA Ser50Leu
-
NRL_000015
heterozygous, marked as causative, heterozygous,
2 more items
Tracewska 2021, MolVis in press,
PubMed: DeAngelis 2002
,
PubMed: Jauregui 2020
,
PubMed: Kanda 2007
,
2 more items
-
-
Germline, In vitro (cloned), Unknown
?, yes
0 (in-house database, ~5000 samples), 1/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
+?/.
4
3
c.151C>A
r.(?)
p.(Pro51Thr)
-
likely pathogenic
g.24551907G>T
g.24082698G>T
NRL c.151C>A, p.P51T, NRL CCC -> ACC, Pro51Thr
-
NRL_000041
no nucleotide annotation, extrapolated from protein; heterozygous,
1 more item
PubMed: DeAngelis 2002
,
PubMed: Kanda 2007
-
-
Germline, In vitro (cloned)
?, yes
-
-
-
-
LOVD
+/., +?/.
4
-
c.151C>T
r.(?)
p.(Pro51Ser)
-
likely pathogenic, pathogenic
g.24551907G>A
g.24082698G>A
NRL 151C>T, P51S, NRL c.151C>T, p.P51S
-
NRL_000017
1 heterozygous, no homozygous;
Clinindb (India)
, heterozygous, VKGL data sharing initiative Nederland,
1 more item
PubMed: Kanda 2007
,
PubMed: Narang 2020
,
Journal: Narang 2020
,
PubMed: Nishiguchi 2004
-
rs794727281
CLASSIFICATION record, Germline, In vitro (cloned), Unknown
?
1/2793 individuals
-
-
-
VKGL-NL_Nijmegen
,
Mohammed Faruq
+?/.
1
-
c.152C>A
r.(?)
p.(Pro51His)
-
likely pathogenic
g.24551906G>T
g.24082697G>T
-
-
NRL_000008
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/.
1
-
c.152C>G
r.(?)
p.(Pro51Arg)
ACMG
likely pathogenic
g.24551906G>C
g.24082697G>C
NRL nucleotide 1, protein 1:c.152C>G, p.Pro51Arg nucleotide 2, protein 2:-,
-
NRL_000032
heterozygous, ACMG classified, novel (Table 2)
PubMed: Hull 2020
-
-
Germline
?
-
-
-
-
LOVD
+/., +?/., ?/.
13
0, 2, 3
c.152C>T
r.(?)
p.(Pro51Leu)
-
likely pathogenic, pathogenic, VUS
g.24551906G>A
g.24082697G>A
c.152C>T, NRL 2316C>T, Pro51Leu, NRL c.152C>T, p.P51L, NRL, variant 1: c.152C>T/p.P51L,
1 more item
-
NRL_000014
obsolete nucleotide annotation 2316C>T; extrapolated from sequence; heterozygous,
2 more items
PubMed: Booij-2011
,
PubMed: Holtan 2020
,
PubMed: Kanda 2007
,
PubMed: Martin-Merida 2018
,
5 more items
-
-
Germline, In vitro (cloned), Unknown
?, no, yes
1/1204 cases with retinitis pigmentosa, 2/258, 3/899 cases
-
-
-
Global Variome, with Curator vacancy
,
Julia Lopez
,
Yoshito Koyanagi
?/.
2
-
c.187G>A
r.(?)
p.(Glu63Lys)
-
VUS
g.24551871C>T
g.24082662C>T
NRL 187G>A, E63K, NRL c.187G>A, p.E63K
-
NRL_000040
heterozygous,
1 more item
PubMed: Kanda 2007
,
PubMed: Nishiguchi 2004
-
-
In vitro (cloned), Unknown
?
-
-
-
-
LOVD
?/.
1
-
c.197G>A
r.(?)
p.(Arg66Gln)
-
VUS
g.24551861C>T
-
NRL(NM_006177.5):c.197G>A (p.R66Q)
-
DCAF11_000024
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
1
-
c.197G>T
r.(?)
p.(Arg66Leu)
-
VUS
g.24551861C>A
-
-
-
DCAF11_000015
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/., -?/.
3
-
c.199C>T
r.(?)
p.(Pro67Ser)
-
benign, likely benign
g.24551859G>A
g.24082650G>A
NRL c.199C>T, p.P67S, NRL(NM_006177.5):c.199C>T (p.P67S), P67S
-
NRL_000007
VKGL data sharing initiative Nederland,
1 more item
PubMed: Kanda 2007
,
PubMed: Ziviello 2005
-
-
CLASSIFICATION record, Germline, In vitro (cloned)
?, no
-
-
-
-
Julia Lopez
,
VKGL-NL_AMC
+/., +?/.
3
-
c.223dup
r.(?)
p.(Leu75Profs*19), p.(Leu75ProfsTer19)
-
likely pathogenic, pathogenic (recessive)
g.24551835dup
g.24082626dup
223dupC, NRL, variant 1: c.91C>T/p.R31*, variant 2: c.223dup/p.L75Pfs*19
-
NRL_000027
solved, compound heterozygous
PubMed: Consugar 2015
,
PubMed: Weisschuh 2020
-
-
Germline, Unknown
?, yes
-
-
-
-
LOVD
+?/.
3
-
c.224_225insC
r.(?)
p.(Ala76GlyfsTer18)
-
likely pathogenic
g.24551833_24551834insG
g.24082624_24082625insG
NRL 224_225insC, L75fs, NRL c.224_225insC, p.L75fs
-
NRL_000039
heterozygous,
1 more item
PubMed: Kanda 2007
,
PubMed: Nishiguchi 2004
-
-
Germline, In vitro (cloned)
?, yes
-
-
-
-
LOVD
-?/., ?/.
4
-
c.227C>T
r.(?)
p.(Ala76Val)
-
likely benign, VUS
g.24551831G>A
g.24082622G>A
NRL 227C>T, A76V, NRL c.227C>T, p.A76V, p.A76V
-
NRL_000033
not present in one RP affected member and present in an unaffected member of this family,
3 more items
PubMed: Borràs 2013
,
PubMed: Kanda 2007
,
PubMed: Nishiguchi 2004
-
-
CLASSIFICATION record, Germline, In vitro (cloned), Unknown
?
0.008
-
-
-
VKGL-NL_Nijmegen
?/.
1
3
c.242_244del
r.(?)
p.(Gln81del)
ACMG
VUS
g.24551822_24551824del
g.24082613_24082615del
-
-
NRL_000029
-
Tracewska 2021, MolVis in press
-
-
Germline
-
0 (in-house database, ~5000 samples)
-
-
-
LOVD
+/., +?/.
5
0, 2
c.287T>C
r.(?)
p.(Met96Thr)
-
likely pathogenic, pathogenic
g.24551771A>G
g.24082562A>G
c.287T>C, NRL c.287T>C, p.M96T
-
NRL_000030
heterozygous, incomplete penetrance
PubMed: Borràs 2013
,
PubMed: Hernan 2011
,
PubMed: Martin-Merida 2018
-
-
Germline
?, yes
1/258, Novel
-
-
-
LOVD
?/.
1
-
c.293T>C
r.(?)
p.(Leu98Pro)
-
VUS
g.24551765A>G
g.24082556A>G
NRL(NM_006177.4):c.293T>C (p.L98P)
-
DCAF11_000002
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.315C>T
r.(?)
p.(Val105=)
-
likely benign
g.24551743G>A
-
NRL(NM_006177.4):c.315C>T (p.V105=)
-
DCAF11_000014
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/.
3
2
c.339C>G
r.(?)
p.(Tyr113*)
ACMG
likely pathogenic, pathogenic
g.24551719G>C
g.24082510G>C
Allele 1 c.339C>G (p.Tyr113*), Allele 2 c.339C>G (p.Tyr113*)
-
NRL_000003
homozygous
PubMed: de Castro-Miró 2016
,
PubMed: Khan 2019
-
-
Germline, Germline/De novo (untested)
?, yes
-
-
-
-
Marta de Castro-Miró
+?/., ?/.
3
3
c.365G>A
r.(?)
p.(Gly122Glu)
-
likely pathogenic, VUS
g.24551693C>T
g.24082484C>T
NRL 2529G>A, Gly122Glu, NRL c.365G>A, p.G122E, NRL(NM_006177.4):c.365G>A (p.G122E)
-
DCAF11_000013
obsolete nucleotide annotation 2529G>A; extrapolated from sequence; heterozygous,
2 more items
PubMed: Kanda 2007
,
PubMed: Martinez-Gimeno 2001
-
-
CLASSIFICATION record, In vitro (cloned), Unknown
?
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.366_368del
r.(?)
p.(Ala123del)
-
VUS
g.24551690_24551692del
g.24082481_24082483del
-
-
NRL_000024
no genotypes reported
PubMed: Sergouniotis 2016
-
-
Germline
-
1/486 individuals
-
-
-
LOVD
?/.
1
-
c.371A>C
r.(?)
p.(Gln124Pro)
-
VUS
g.24551687T>G
g.24082478T>G
-
-
NRL_000013
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
-
Germline
-
1/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
?/.
1
-
c.375C>G
r.(?)
p.(His125Gln)
-
VUS
g.24551683G>C
g.24082474G>C
NRL c.375C>G, p.H125Q
-
NRL_000038
1 more item
PubMed: Kanda 2007
-
-
In vitro (cloned)
?
-
-
-
-
LOVD
+/.
1
-
c.386del
r.(?)
p.(Ala129Glufs*17)
-
pathogenic
g.24550773del
g.24081564del
386delC
-
NRL_000023
-
PubMed: Carrigan 2016
-
-
Germline
-
-
-
-
-
LOVD
-?/.
2
-
c.399C>T
r.(?)
p.(Ser133=)
-
likely benign
g.24550760G>A
g.24081551G>A
NRL(NM_006177.4):c.399C>T (p.S133=), NRL(NM_006177.5):c.399C>T (p.S133=)
-
NRL_000006
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
+/.
1
-
c.416C>G
r.(?)
p.(Ser139Trp)
-
pathogenic (recessive)
g.24550743G>C
-
-
-
NRL_000021
-
PubMed: Porto 2017
-
-
Germline
-
-
-
-
-
LOVD
?/.
1
-
c.425T>A
r.(?)
p.(Val142Glu)
ACMG
VUS
g.24550734A>T
g.24081525A>T
-
-
NRL_000045
ACMG PP3, PM2, PP2
PubMed: Weisschuh 2024
-
-
Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
-/., -?/.
3
-
c.441G>A
r.(?)
p.(=), p.(Arg147=)
-
benign, likely benign
g.24550718C>T
g.24081509C>T
441G>A, NRL(NM_006177.4):c.441G>A (p.R147=), NRL(NM_006177.5):c.441G>A (p.R147=)
-
NRL_000005
Silent substitution, VKGL data sharing initiative Nederland
PubMed: Sullivan 2006
-
-
CLASSIFICATION record, Germline
yes
-
-
-
-
Julia Lopez
,
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
+/.
1
-
c.444_445insGCTGCGGG
r.(?)
p.(Leu149Alafs*15)
ACMG
pathogenic (recessive)
g.24550714_24550715insCCCGCAGC
-
444_445insGCTGCGGG
-
NRL_000018
variant not 444_45idup?
PubMed: Beryozkin 2015
,
PubMed: Sharon 2019
-
-
Germline
-
1/2420 IRD families
-
-
-
Global Variome, with Curator vacancy
+/., +?/.
2
3
c.452_459dup
r.(?)
p.(Arg154Alafs*10), p.(Arg154AlafsTer10)
-
likely pathogenic, pathogenic
g.24550693_24550700dup, g.24550708_24550715dup
-
-
-
NRL_000010
VKGL data sharing initiative Nederland,
1 more item
Sharon, submitted
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Dror Sharon
,
VKGL-NL_Nijmegen
?/.
2
-
c.459_477dup
r.(?)
p.(Leu160AlafsTer67)
-
VUS
g.24550689_24550707dup
g.24081480_24081498dup
NRL 459_477dup, L160fs, NRL c.459_477dup, p.L160fs
-
NRL_000037
heterozygous,
1 more item
PubMed: Kanda 2007
,
PubMed: Nishiguchi 2004
-
-
In vitro (cloned), Unknown
?
-
-
-
-
LOVD
+?/.
4
-
c.479T>C
r.(?)
p.(Leu160Pro)
-
likely pathogenic, likely pathogenic (recessive)
g.24550680A>G
g.24081471A>G
NRL 479T>C, L160P, NRL c.479T>C, p.L160P
-
NRL_000020
heterozygous,
1 more item
PubMed: Kanda 2007
,
PubMed: Nishiguchi 2004
,
PubMed: Taylor 2017
-
-
Germline, In vitro (cloned)
?, yes
-
-
-
-
LOVD
+?/.
7
0, 3
c.508C>A
r.(?)
p.(Arg170Ser)
-
likely pathogenic
g.24550651G>T
g.24081442G>T
c.508C>A, NRL c.508C>A, p.Arg170Ser
-
NRL_000001
compound heterozygous, homozygous, VKGL data sharing initiative Nederland,
1 more item
PubMed: Collin-2011
,
PubMed: Littink 2018
,
PubMed: Neveling 2012
,
PubMed: Panneman 2023
-
-
CLASSIFICATION record, Germline, Unknown
yes
0/360 controls
-
-
-
Kornelia Neveling
,
VKGL-NL_Nijmegen
,
Daan Panneman
+?/.
1
-
c.520C>A
r.(?)
p.(Gln174Lys)
ACMG
likely pathogenic
g.24550639G>T
g.24081430G>T
NRL NM_006177: g.33585C>A, c.520C>A, p.Q174K
-
NRL_000031
-
PubMed: Xu 2020
-
-
Germline
yes
-
-
-
-
LOVD
-/.
1
-
c.521A>G
r.(?)
p.(Gln174Arg)
-
benign
g.24550638T>C
-
521A>G
-
NRL_000022
-
PubMed: Sullivan 2006
-
-
Germline
no
-
-
-
-
Julia Lopez
?/.
1
-
c.586_627dup
r.(?)
p.(Ala196_Ala209dup)
-
VUS
g.24550544_24550585dup
g.24081335_24081376dup
586_627dupGCCCAGCTGGACGCGCTGCGGGCCGAGGTGGCCCGCCTGGCC
-
NRL_000026
-
PubMed: Ellingford 2016
-
-
Germline
-
-
-
-
-
LOVD
?/.
1
-
c.605G>A
r.(?)
p.(Arg202Gln)
-
VUS
g.24550554C>T
g.24081345C>T
-
-
DCAF11_000007
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
-
c.613G>A
r.(?)
p.(Val205Met)
-
VUS
g.24550546C>T
-
NRL(NM_006177.4):c.613G>A (p.V205M)
-
DCAF11_000011
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.652C>T
r.(?)
p.(Arg218Cys)
-
VUS
g.24550507G>A
g.24081298G>A
-
-
NRL_000012
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
-
Germline
-
1/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
+/., +?/.
9
0, 3
c.654del
r.(?)
p.(Cys219Valfs*4), p.(Cys219ValfsTer4)
-
likely pathogenic, pathogenic, pathogenic (recessive)
g.24550505del
g.24081296del
654delC, c.654del, NRL c.654del, p.Cys219Valfs*4,
1 more item
-
NRL_000002
compound heterozygous, predicted to affect function, but insufficient evidence for definite conclusion,
1 more item
PubMed: Littink 2018
,
PubMed: Neveling 2012
,
PubMed: Panneman 2023
,
PubMed: Porto 2017
-
-
CLASSIFICATION record, Germline, Unknown
yes
-
-
-
-
Kornelia Neveling
,
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
VKGL-NL_Nijmegen
,
VKGL-NL_AMC
,
Daan Panneman
?/.
2
-
c.654delC
r.(?)
p.(Cys219ValfsTer4)
-
VUS
g.24550505del
g.24081296del
NRL 654delC, R218fs, NRL c.654delC, p.R218fs
-
NRL_000002
2 more items
PubMed: Kanda 2007
,
PubMed: Nishiguchi 2004
-
-
Germline, In vitro (cloned)
?, no
-
-
-
-
LOVD
?/.
2
-
c.674G>A
r.(?)
p.(Ser225Asn)
-
VUS
g.24550485C>T
g.24081276C>T
NRL 674G>A, S225N, NRL c.674G>A, p.S225N
-
NRL_000036
heterozygous,
1 more item
PubMed: Kanda 2007
,
PubMed: Nishiguchi 2004
-
-
In vitro (cloned), Unknown
?
-
-
-
-
LOVD
?/.
1
-
c.684_694del
r.(?)
p.(Ser229Leufs*15)
-
VUS
g.24550465_24550475del
-
NRL(NM_006177.5):c.684_694delGTCCGGGGACC (p.S229Lfs*15)
-
DCAF11_000012
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
1
-
c.687del
r.(?)
p.(Asp231Thrfs*87)
-
pathogenic
g.24550473del
-
NRL(NM_006177.4):c.687delC (p.D231Tfs*87)
-
CPNE6_000004
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.703C>T
r.(?)
p.(Leu235Phe)
-
likely benign
g.24550456G>A
g.24081247G>A
NRL c.703C>T, p.L235F
-
NRL_000035
1 more item
PubMed: Kanda 2007
-
-
In vitro (cloned)
?
-
-
-
-
LOVD
-/.
2
-
c.711C>G
r.(?)
p.(Leu237=)
-
benign
g.24550448G>C
g.24081239G>C
NRL(NM_006177.4):c.711C>G (p.L237=), NRL(NM_006177.5):c.711C>G (p.L237=)
-
NRL_000004
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
?/.
1
-
c.*3860G>A
r.(=)
p.(=)
-
VUS
g.24546585C>T
g.24077376C>T
-
-
DCAF11_000001
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
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