Global Variome shared LOVD
CNGB3 (cyclic nucleotide gated channel beta 3)
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Curator:
Global Variome, with Curator vacancy
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Unique variants in the CNGB3 gene
This database is one of the
"Eye disease"
gene variant databases.
The variants shown are described using the NM_019098.4 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.
303 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
13i_18_
c.(1578+1_1579-1)_*1869{0}
r.?
p.?
-
pathogenic (recessive)
g.(?_87586163)_(87623900_87638210)del
g.(?_86573935)_(86611672_86625982)del
del ex14-18
-
CNGB3_000210
-
PubMed: Mayer 2017
SCV000575868
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
3i_18_
c.(338+1_339-1)_*1869{0}
r.?
p.?
-
pathogenic (recessive)
g.(?_87586163)_(87683327_87738758)del
g.(?_86573935)_(86671099_86726530)del
del ex4-18
-
CNGB3_000211
-
PubMed: Mayer 2017
SCV000575865
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
_1_1i
c.-48_(129+1_130-1){0}
r.0?
p.0?
-
pathogenic (recessive)
g.(87751965_87755726)_(87755903_?)del
g.(86739737_86743498)_(86743675_?)del
del ex1
-
CNGB3_000262
-
PubMed: Mayer 2017
SCV000575861
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
6
c.782A<G
r.(?)
p.(Asp261Gly)
-
likely pathogenic
g.87679223A>G
-
-
-
CNGB3_000053
1 more item
Sharon, submitted
-
-
Germline
-
-
-
-
-
Dror Sharon
-/.
1
-
c.-36T>G
r.(?)
p.(=)
-
benign
g.87755891A>C
g.86743663A>C
-
-
CNGB3_000049
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/.
1
-
c.?
r.(?)
p.?
-
likely pathogenic
g.?
g.?
CNGB3 R296fs
-
RP1_000000
1 more item
PubMed: Kellner 2004
-
-
Unknown
?
-
-
-
-
LOVD
+/.
3
1
c.2T>C
p.?, r.(?)
p.(Met1?)
-
pathogenic (recessive)
g.87755854A>G
g.86743626A>G
-
-
CNGB3_000207
no variant 2nd chromosome (qPCR analysis negative)
PubMed: Mayer 2017
,
PubMed: Weisschuh 2020
SCV000575803
-
Germline
yes
-
-
-
-
Johan den Dunnen
+/., +?/.
3
-
c.3G>A
r.(?), r.0?
p.(Met1?)
ACMG
likely pathogenic, pathogenic (recessive)
g.87755853C>T
g.86743625C>T
CNGB3 c.3G>A, (p.Met1?), CNGB3 c.[3G>A];[680T>C]
-
CNGB3_000107
heterozygous; protein change not reported
PubMed: Matet 2018
,
PubMed: Mayer 2017
,
PubMed: Matet 2018
,
PubMed: Sun 2020
SCV000575804
-
Germline, Unknown
?, yes
-
-
-
-
LOVD
+?/.
1
-
c.11C>A
r.(?)
p.(Ser4*)
-
likely pathogenic
g.87755845G>T
g.86743617G>T
CNGB3 c.[11C>A];[11C>A]
-
CNGB3_000266
homozygous; protein change not reported
PubMed: Sun 2020
-
-
Unknown
yes
-
-
-
-
LOVD
+/.
1
1
c.29dup
r.(?)
p.(Val11GlyfsTer9)
-
pathogenic (recessive)
g.87755828dup
g.86743600dup
29dupA
-
CNGB3_000265
-
PubMed: Mayer 2017
SCV000575773
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
1
c.31dup
r.(?)
p.(Val11GlyfsTer9)
-
pathogenic (recessive)
g.87755826dup
g.86743598dup
31dupG
-
CNGB3_000264
-
PubMed: Mayer 2017
SCV000575774
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.31G>A
r.(?)
p.(Val11Met)
-
VUS
g.87755825C>T
g.86743597C>T
-
-
CNGB3_000048
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/.
1
-
c.43G>C
r.(?)
p.(Gly15Arg)
-
likely benign
g.87755813C>G
g.86743585C>G
CNGB3(NM_019098.4):c.43G>C (p.G15R)
-
CNGB3_000028
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.77G>A
r.(?)
p.(Arg26Gln)
-
likely benign
g.87755779C>T
g.86743551C>T
CNGB3(NM_019098.4):c.77G>A (p.R26Q)
-
CNGB3_000076
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/., ?/.
4
-
c.80A>G
r.(?)
p.(Asn27Ser)
-
benign, VUS
g.87755776T>C
g.86743548T>C
CNGB3(NM_019098.4):c.80A>G (p.N27S)
-
CNGB3_000027
conflicting interpretations of pathogenicity; 1 homozygous;
Clinindb (India)
,
2 more items
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs35807406
CLASSIFICATION record, Germline
-
1/2792 individuals, 58/2792 individuals
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Nijmegen
,
Mohammed Faruq
+/.
1
1
c.95dup
r.(?)
p.(His32GlnfsTer4)
-
pathogenic (recessive)
g.87755761dup
g.86743533dup
c.95dupA
-
CNGB3_000263
-
PubMed: Mayer 2017
SCV000575775
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
-
c.105_114del
r.(?)
p.(Gln36Lysfs*44), p.(Gln36LysfsTer44)
ACMG
pathogenic, pathogenic (recessive)
g.87755742_87755751del
g.86743514_86743523del
c.105_114delTCAGTCTCAG
-
CNGB3_000082
-
PubMed: Aweidah 2021
,
PubMed: Sharon 2019
-
-
Germline
-
1/2420 IRD families
-
-
-
Global Variome, with Curator vacancy
,
Johan den Dunnen
+/.
6
1
c.112C>T
r.(?)
p.(Gln38*), p.(Gln38Ter)
-
pathogenic, pathogenic (recessive)
g.87755744G>A
g.86743516G>A
CNGB3: c.[112C>T];[1208G>A;1673G>T], p.[Q38*];[R403Q;G558V], CNGA3: c.[=];[=]
-
CNGB3_000134
-
PubMed: Burkhard 2018
,
PubMed: Mayer 2017
,
PubMed: Varsanyi 2005
SCV000575819
-
Germline
yes
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.129+1G>A
r.spl
p.?
-
likely pathogenic (recessive)
g.87755726C>T
g.86743498C>T
-
-
CNGB3_000118
-
PubMed: Huang 2013
,
PubMed: Huang 2016
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., +?/.
4
-
c.129+2T>C
r.(?), r.spl
p.(?), p.?
ACMG
likely pathogenic, pathogenic (recessive)
g.87755725A>G
g.86743497A>G
CNGB3 c.129+2T>C, p.(?)
-
CNGB3_000106
-
PubMed: Matet 2018
,
PubMed: Mayer 2017
,
PubMed: Matet 2018
SCV000575839
-
Germline, Unknown
?, yes
-
-
-
-
LOVD
-?/.
1
-
c.130-1161A>C
r.(?)
p.(=)
-
likely benign
g.87753125T>G
g.86740897T>G
-
-
CNGB3_000206
-
PubMed: Weisschuh 2020
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., +?/.
3
-
c.130-1G>T
r.spl
p.(?), p.?
ACMG
likely pathogenic, pathogenic (recessive)
g.87751965C>A
g.86739737C>A
CNGB3 c.130-1G>T, p.(?)
-
CNGB3_000105
-
PubMed: Matet 2018
,
PubMed: Mayer 2017
,
PubMed: Matet 2018
SCV000575840
-
Germline, Unknown
?
-
-
-
-
LOVD
+/.
2
-
c.133G>T
r.(?)
p.(Glu45Ter)
ACMG
pathogenic
g.87751961C>A
g.86739733C>A
c.133G>T; p.(Glu45*), CNGB3:NM_019098 c.G133T, p.E45X
-
CNGB3_000144
heterozygous, individual solved, variant causal
PubMed: Rodriguez-Munoz 2020
-
-
Germline
yes
-
-
-
-
LOVD
-?/.
1
-
c.183G>A
r.(?)
p.(Thr61=)
-
likely benign
g.87751911C>T
g.86739683C>T
CNGB3(NM_019098.4):c.183G>A (p.T61=)
-
CNGB3_000026
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/., +?/.
2
2
c.190del
r.(?)
p.(Glu64Serfs*19), p.(Glu64SerfsTer19)
-
likely pathogenic (recessive), pathogenic (recessive)
g.87751904del
g.86739676del
190delG
-
CNGB3_000098
-
PubMed: Mayer 2017
,
PubMed: Taylor 2017
SCV000575776
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
3
2
c.208C>T
r.(?)
p.(Gln70Ter)
-
pathogenic (recessive)
g.87751886G>A
g.86739658G>A
-
-
CNGB3_000261
-
PubMed: Mayer 2017
SCV000575820
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
2
c.210A>G
r.[(130_211del,=)]
p.[(Glu44fs,Gln70=)]
ACMG
likely pathogenic
g.87751884T>C
g.86739656T>C
-
-
CNGB3_000293
1 more item
Rawnsley 2025, submitted
-
-
Germline
-
-
-
-
-
Susanne Kohl
-/., -?/.
3
-
c.211+13T>G
r.(=)
p.(=)
-
benign, likely benign
g.87751870A>C
g.86739642A>C
-
-
CNGB3_000047
11 homozygous;
Clinindb (India)
, 233 heterozygous;
Clinindb (India)
,
1 more item
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs66881636
CLASSIFICATION record, Germline
-
11/2795 individuals, 233/2795 individuals
-
-
-
VKGL-NL_Nijmegen
,
Mohammed Faruq
-/.
1
-
c.211+34del
r.(=)
p.(=)
-
benign
g.87751866del
g.86739638del
CNGB3(NM_019098.5):c.211+34delA
-
CNGB3_000025
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-?/.
1
-
c.211+2085T>G
r.(?)
p.(=)
-
likely benign
g.87749798A>C
g.86737570A>C
-
-
CNGB3_000205
-
PubMed: Weisschuh 2020
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.211+2449G>A
r.(?)
p.(=)
-
likely benign
g.87749434C>T
g.86737206C>T
-
-
CNGB3_000204
-
PubMed: Weisschuh 2020
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.212-5347G>C
r.(?)
p.(=)
-
likely benign
g.87744232C>G
g.86732004C>G
-
-
CNGB3_000203
-
PubMed: Weisschuh 2020
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.212-4549T>C
r.(?)
p.(=)
-
likely benign
g.87743434A>G
g.86731206A>G
-
-
CNGB3_000202
-
PubMed: Weisschuh 2020
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.212-3599T>A
r.(=)
p.(=)
-
likely benign
g.87742484A>T
g.86730256A>T
-
-
CNGB3_000201
effect variant predicted form mini-gene splicing analysis in HEK293T cells
PubMed: Weisschuh 2020
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
2i_3i
c.(211+1_212-3180)_(338+2728_339-1)del
r.?
p.?
ACMG
likely pathogenic (recessive)
g.(87683327_87736031)_(87742065_87751882)del
g.(86671099_86723803)_(86729837_86739654)del
arr[hg19]8q21.3 (87736031-87742065=x0
-
CNGB3_000294
-
PubMed: Andersen 2023
-
-
Germline
-
-
-
-
-
Susanne Kohl
+/.
2
2i_3i
c.212-2527_338+2854del
r.(?)
p.(Asp71Alafs*12)
ACMG
pathogenic (recessive)
g.87735908_87741415del
g.86723680_86729187del
del ex3 (g.86723677_86729184del)
-
CNGB3_000103
-
PubMed: Mayer 2017
,
PubMed: Matet 2018
SCV000575862
-
Germline
yes
-
-
-
-
LOVD
-?/.
1
-
c.212-12T>G
r.(=)
p.(=)
-
likely benign
g.87738897A>C
g.86726669A>C
CNGB3(NM_019098.5):c.212-12T>G
-
CNGB3_000024
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/.
1
-
c.212-3T>C
r.spl?
p.?
-
benign
g.87738888A>G
g.86726660A>G
CNGB3(NM_019098.5):c.212-3T>C
-
CNGB3_000072
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+?/.
3
-
c.(211+1_212-1)_(338+1_339-1)del
r.(?), r.spl
p.(?)
ACMG
likely pathogenic
g.?
g.?
CNGB3 c.(211+1_212-1) _(338+1_339-1)del, c.(211+1_212-1) _(338+1_339-1)del, CNGB3 ex 3 deletion, p.(?)
-
RP1_000000
homozygous
PubMed: Jespersgaar 2019
,
PubMed: Matet 2018
-
-
Germline, Unknown
?
-
-
-
-
LOVD
-?/.
1
-
c.221C>T
r.(?)
p.(Ser74Phe)
-
likely benign
g.87738876G>A
-
CNGB3(NM_019098.4):c.221C>T (p.S74F)
-
CNGB3_000104
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
3
c.257del
r.(?)
p.(Pro86LeufsTer39)
-
pathogenic (recessive)
g.87738842del
g.86726614del
257delC
-
CNGB3_000260
-
PubMed: Mayer 2017
SCV000575777
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
3
c.265C>T
r.(?)
p.(Gln89Ter)
-
pathogenic (recessive)
g.87738832G>A
g.86726604G>A
-
-
CNGB3_000200
no variant 2nd chromosome (no qPCR analysis)
PubMed: Mayer 2017
,
PubMed: Weisschuh 2020
SCV000575821
-
Germline
yes
-
-
-
-
Johan den Dunnen
+/.
1
3
c.281_284del
r.(?)
p.(Pro94LeufsTer30)
-
pathogenic (recessive)
g.87738816_87738819del
g.86726588_86726591del
281_284delCAAC
-
CNGB3_000259
-
PubMed: Mayer 2017
SCV000575778
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.283A>C
r.(?)
p.(Thr95Pro)
-
VUS
g.87738814T>G
g.86726586T>G
CNGB3(NM_019098.4):c.283A>C (p.T95P)
-
CNGB3_000071
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
3
3
c.301C>T
r.(?)
p.(Gln101Ter)
-
pathogenic, pathogenic (recessive)
g.87738796G>A
g.86726568G>A
-
-
CNGB3_000046
VKGL data sharing initiative Nederland
PubMed: Mayer 2017
SCV000575822
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Nijmegen
?/.
1
-
c.319G>A
r.(?)
p.(Gly107Arg)
-
VUS
g.87738778C>T
g.86726550C>T
CNGB3(NM_019098.4):c.319G>A (p.G107R)
-
CNGB3_000023
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.338+465G>T
r.(?)
p.(=)
-
likely benign
g.87738294C>A
g.86726066C>A
-
-
CNGB3_000199
-
PubMed: Weisschuh 2020
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.338+1820G>C
r.(?)
p.(=)
-
likely benign
g.87736939C>G
g.86724711C>G
-
-
CNGB3_000198
-
PubMed: Weisschuh 2020
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.338+4727G>T
r.(?)
p.(=)
-
likely benign
g.87734032C>A
g.86721804C>A
-
-
CNGB3_000197
-
PubMed: Weisschuh 2020
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.338+12250C>T
r.(?)
p.(=)
-
likely benign
g.87726509G>A
g.86714281G>A
-
-
CNGB3_000196
-
PubMed: Weisschuh 2020
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.338+14712G>T
r.(?)
p.(=)
-
likely benign
g.87724047C>A
g.86711819C>A
-
-
CNGB3_000195
-
PubMed: Weisschuh 2020
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.338+16163G>A
r.(?)
p.(=)
-
likely benign
g.87722596C>T
g.86710368C>T
-
-
CNGB3_000194
-
PubMed: Weisschuh 2020
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.338+18721C>T
r.(?)
p.(=)
-
likely benign
g.87720038G>A
g.86707810G>A
-
-
CNGB3_000193
-
PubMed: Weisschuh 2020
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.339-12423_339-12418dup
r.(?)
p.(=)
-
likely benign
g.87695745_87695750dup
g.86683517_86683522dup
-
-
CNGB3_000192
-
PubMed: Weisschuh 2020
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.339-9886C>T
r.(?)
p.(=)
-
likely benign
g.87693212G>A
g.86680984G>A
-
-
CNGB3_000191
-
PubMed: Weisschuh 2020
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.339-5537G>A
r.(?)
p.(=)
-
likely benign
g.87688863C>T
g.86676635C>T
-
-
CNGB3_000190
-
PubMed: Weisschuh 2020
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.339-2593A>G
r.(?)
p.(=)
-
likely benign
g.87685919T>C
g.86673691T>C
-
-
CNGB3_000189
-
PubMed: Weisschuh 2020
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.339-1034C>T
r.(?)
p.(=)
-
likely benign
g.87684360G>A
g.86672132G>A
-
-
CNGB3_000188
-
PubMed: Weisschuh 2020
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.339-760C>T
r.(?)
p.(=)
-
likely benign
g.87684086G>A
g.86671858G>A
-
-
CNGB3_000187
-
PubMed: Weisschuh 2020
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.339-10del
r.(=)
p.(=)
-
likely benign
g.87683343del
-
CNGB3(NM_019098.5):c.339-10delT
-
CNGB3_000280
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+?/.
1
-
c.(338+1_339-1)_(*1869_?)del
r.spl
p.(?)
-
likely pathogenic
g.?
g.?
CNGB3, variant 1 :Deletion exon 4-18, variant 2 :Deletion exon 4-18
-
RP1_000000
solved, homozygous
PubMed: Weisschuh 2020
-
-
Unknown
?
-
-
-
-
LOVD
+?/.
1
-
c.372_375del
r.(?)
p.(Ile124Metfs*12)
-
likely pathogenic
g.87683292_87683295del
g.86671064_86671067del
Allele 1 c.372_375del (p.Ile124Metfs*12), Allele 2 c.372_375del (p.Ile124Metfs*12)
-
CNGB3_000141
homozygous
PubMed: Khan 2019
-
-
Germline/De novo (untested)
?
-
-
-
-
LOVD
?/.
1
-
c.385G>A
r.(?)
p.(Asp129Asn)
-
VUS
g.87683280C>T
-
CNGB3(NM_019098.4):c.385G>A (p.D129N)
-
CNGB3_000275
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/., +?/.
3
4
c.391C>T
r.(?)
p.(Gln131*), p.(Gln131Ter)
-
likely pathogenic, pathogenic (recessive)
g.87683274G>A
g.86671046G>A
CNGB3 Q131X, p.Q131X
-
CNGB3_000090
no nucleotide annotation, extrapolated from protein and databases; heterozygous
PubMed: Kellner 2004
,
PubMed: Mayer 2017
,
PubMed: Nishiguchi 2005
SCV000575823
-
Germline, Unknown
?
-
-
-
-
Johan den Dunnen
,
Julia Lopez
+/.
2
4
c.393_394delinsTCCTGGTGA
r.(?)
p.(Gln131HisfsTer50)
-
pathogenic (recessive)
g.87683271_87683272delinsTCACCAGGA
g.86671043_86671044delinsTCACCAGGA
393_394delGCinsTCCTGGTGA
-
CNGB3_000256
-
PubMed: Mayer 2017
,
PubMed: Wawrocka 2014
SCV000575779
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.397C>T
r.(?)
p.(His133Tyr)
-
VUS
g.87683268G>A
g.86671040G>A
-
-
CNGB3_000091
-
PubMed: Zenteno 2020
-
-
Germline
-
1/143 cases
-
-
-
Johan den Dunnen
?/.
1
-
c.442A>G
r.(?)
p.(Lys148Glu)
-
VUS
g.87683223T>C
-
-
-
CNGB3_000208
-
PubMed: Rojas 2002
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.446_447insT
r.(?)
p.(Lys149Asnfs*30)
-
pathogenic (recessive)
g.87683218_87683219insA
-
492_493insT
-
CNGB3_000209
-
PubMed: Rojas 2002
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
+/., +?/., ?/.
10
4
c.467C>T
r.(?)
p.(Ser156Phe)
ACMG
likely pathogenic, likely pathogenic (recessive), pathogenic (recessive), VUS
g.87683198G>A
g.86670970G>A
-
-
CNGB3_000055
ACMG PS4_mod, PM2_sup, BP4
Sharon, submitted,
PubMed: Andersen 2023
,
PubMed: Aweidah 2021
,
PubMed: Mayer 2017
,
1 more item
SCV000575805
-
Germline
-
1/2420 IRD families
-
-
-
Global Variome, with Curator vacancy
,
Johan den Dunnen
,
Dror Sharon
,
Susanne Kohl
+?/.
1
p.(Pro158His)
c.473C>A
r.(?)
p.(Pro158His)
-
likely pathogenic
g.87683192G>T
g.86670964G>T
CNGB3 473C>A, Pro158His
-
CNGB3_000271
heterozygous
PubMed: Georgiou 2019
-
-
Unknown
?
-
-
-
-
LOVD
?/.
1
-
c.489A>T
r.(?)
p.(Gln163His)
-
VUS
g.87683176T>A
g.86670948T>A
CNGB3(NM_019098.4):c.489A>T (p.Q163H)
-
CNGB3_000022
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.494-1082C>T
r.(?)
p.(=)
-
likely benign
g.87681478G>A
g.86669250G>A
-
-
CNGB3_000186
-
PubMed: Weisschuh 2020
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
1
-
c.494-11del
r.(=)
p.(=)
-
benign
g.87680417del
g.86668189del
CNGB3(NM_019098.5):c.494-11delT
-
CNGB3_000021
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/., -?/.
2
-
c.494-11T>C
r.(=)
p.(=)
-
benign, likely benign
g.87680407A>G
-
CNGB3(NM_019098.5):c.494-11T>C
-
CNGB3_000139
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
,
VKGL-NL_AMC
+/.
2
4i
c.494-2A>T
r.spl
p.?
-
pathogenic (recessive)
g.87680398T>A
g.86668170T>A
-
-
CNGB3_000255
-
PubMed: Mayer 2017
,
PubMed: Wawrocka 2014
SCV000575841
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.503C>T
r.(?)
p.(Thr168Met)
-
pathogenic (recessive)
g.87680387G>A
g.86668159G>A
-
-
CNGB3_000254
-
PubMed: Langlo 2014
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
5
c.589_590del
r.(?)
p.(Leu197AsnfsTer8)
ACMG
pathogenic (recessive)
g.87680301_87680302del
g.86668073_86668074del
589_590delTT
-
CNGB3_000253
ACMG PVS1, PS4_sup, PM2_sup
PubMed: Andersen 2023
,
PubMed: Mayer 2017
SCV000575780
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Susanne Kohl
+/.
6
5
c.595del
r.(?)
p.(Glu199Serfs*3), p.(Glu199SerfsTer3)
-
pathogenic, pathogenic (recessive)
g.87680295del
g.86668067del
1148delC/595delG, 595delG, c.595delG
-
CNGB3_000087
-
PubMed: Dubis 2015
,
PubMed: Johnson 2004
,
PubMed: Sundaram_2014
-
-
Germline
-
-
-
-
-
Julia Lopez
+/., +?/.
9
5
c.607C>T
r.(?)
p.(Arg203*), p.(Arg203Ter)
-
likely pathogenic, pathogenic, pathogenic (recessive)
g.87680283G>A
g.86668055G>A
CNGB3, variant 1: c.1148del/p.T383Ifs*13, variant 2: c.607C>T/p.R203*
-
CNGB3_000077
1 heterozygous, no homozygous;
Clinindb (India)
, solved, compound heterozygous,
1 more item
PubMed: Habibi 2016
,
PubMed: Kohl 2000
,
PubMed: Mayer 2017
,
PubMed: Narang 2020
,
Journal: Narang 2020
,
3 more items
SCV000575824
rs267606739
CLASSIFICATION record, Germline, Unknown
?, yes
1/2795 individuals
-
-
-
Johan den Dunnen
,
VKGL-NL_Nijmegen
,
Mohammed Faruq
+/.
2
5
c.607_608insT
r.(?)
p.(Arg203Leufs*3), p.(Arg203LeufsTer3)
-
pathogenic, pathogenic (recessive)
g.87680282_87680283insA
g.86668054_86668055insA
c.607-608insT
-
CNGB3_000121
-
PubMed: Dubis 2015
,
PubMed: Sundaram_2014
-
-
Germline
-
-
-
-
-
LOVD
-/.
1
-
c.608G>A
r.(?)
p.(Arg203Gln)
-
benign
g.87680282C>T
g.86668054C>T
-
-
CNGB3_000045
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
-
c.615del
r.(?)
p.(Lys205AsnfsTer6)
-
pathogenic
g.87680277del
-
CNGB3(NM_019098.5):c.615delA (p.K205Nfs*6)
-
CNGB3_000069
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
+?/.
3
5
c.643G>C
r.(?), r.494_643del
p.(Asp215His), p.Ala165_Thr214del
-
likely pathogenic, likely pathogenic (recessive), NA
g.87680247C>G
g.86668019C>G
CNGB3, variant 1: c.643G>C/p.D215H, variant 2: c.1148del/p.T383Ifs*13
-
CNGB3_000185
solved, compound heterozygous,
1 more item
Rawnsley 2025, submitted,
PubMed: Mayer 2017
,
PubMed: Weisschuh 2020
SCV000575806
-
Germline, In vitro (cloned)
yes
-
-
-
-
Johan den Dunnen
,
Susanne Kohl
+/.
1
5i
c.643+2T>C
r.spl
p.?
-
pathogenic (recessive)
g.87680245A>G
g.86668017A>G
-
-
CNGB3_000252
-
PubMed: Mayer 2017
SCV000575842
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
14
5i
c.644-1G>C
r.spl
p.?
ACMG
pathogenic, pathogenic (recessive)
g.87679362C>G
g.86667134C>G
-
-
CNGB3_000054
no variant 2nd chromosome (qPCR analysis negative)
Sharon, submitted,
PubMed: Aweidah 2021
,
PubMed: Mayer 2017
,
PubMed: Sharon 2019
,
1 more item
SCV000575843
-
Germline
-
2/2420 IRD families
-
-
-
Global Variome, with Curator vacancy
,
Johan den Dunnen
,
Dror Sharon
+/.
6
6
c.646C>T
r.(?)
p.(Arg216*), p.(Arg216Ter)
-
pathogenic, pathogenic (recessive)
g.87679359G>A
g.86667131G>A
-
-
CNGB3_000075
no variant 2nd chromosome (no qPCR analysis), VKGL data sharing initiative Nederland
PubMed: Mayer 2017
,
PubMed: Saqib 2015
,
Journal: Saqib 2015
,
PubMed: Weisschuh 2020
SCV000575825
-
CLASSIFICATION record, Germline
yes
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Nijmegen
?/.
1
-
c.647G>T
r.(?)
p.(Arg216Leu)
-
VUS
g.87679358C>A
g.86667130C>A
CNGB3(NM_019098.4):c.647G>T (p.R216L)
-
CNGB3_000018
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.648A>G
r.(?)
p.(Arg216=)
-
likely benign
g.87679357T>C
-
CNGB3(NM_019098.4):c.648A>G (p.R216=)
-
CNGB3_000138
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.677C>A
r.(?)
p.(Thr226Asn)
ACMG
VUS
g.87679328G>T
g.86667100G>T
CNGB3:NM_019098 c.C677A, p.T226N
-
CNGB3_000143
heterozygous, individual solved, variant non-causal
PubMed: Rodriguez-Munoz 2020
-
-
Germline
?
-
-
-
-
LOVD
+?/.
3
-
c.680T>C
r.(?)
p.(Leu227Pro)
-
likely pathogenic, likely pathogenic (recessive)
g.87679325A>G
g.86667097A>G
CNGB3 c.[3G>A];[680T>C], CNGB3 c.[680T>C];[1155G>T]
-
CNGB3_000117
heterozygous; protein change not reported
PubMed: Huang 2016
,
PubMed: Sun 2020
-
-
Germline, Unknown
yes
-
-
-
-
Johan den Dunnen
+/.
2
6
c.682dup
r.(?)
p.(Ala228GlyfsTer3)
-
pathogenic (recessive)
g.87679323dup
g.86667095dup
682dupG
-
CNGB3_000251
-
PubMed: Mayer 2017
SCV000575781
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.692G>A
r.(?)
p.(Trp231*)
-
pathogenic
g.87679313C>T
-
CNGB3(NM_019098.5):c.692G>A (p.W231*)
-
CNGB3_000279
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
1
6
c.701G>T
r.(?)
p.(Cys234Phe)
-
VUS
g.87679304C>A
g.86667076C>A
G701T
-
CNGB3_000124
-
PubMed: Katagiri 2014
-
-
Germline
-
-
-
-
-
LOVD
+/.
1
-
c.701_702delinsAG
r.(?)
p.(Cys234*)
ACMG
pathogenic
g.87679303_87679304delinsCT
g.86667075_86667076delinsCT
-
-
CNGB3_000282
-
Villafuerte-de la Cruz RA, et al., 2023. Submitted
ClinVar-935306
rs1823755123
Germline
yes
-
-
-
-
Rocio Villafuerte-de la Cruz
+/.
4
6
c.702T>A
r.(?)
p.(Cys234Ter)
ACMG
pathogenic (recessive)
g.87679303A>T
g.86667075A>T
originally reported as Trp234*
-
CNGB3_000250
ACMG PVS1, PM2_sup, PM3
PubMed: Andersen 2023
,
PubMed: Mayer 2017
SCV000575826
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Susanne Kohl
-/.
2
-
c.702T>G
r.(?)
p.(Cys234Trp)
-
benign
g.87679303A>C
g.86667075A>C
CNGB3(NM_019098.5):c.702T>G (p.C234W)
-
CNGB3_000017
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
+/.
1
6
c.702_706delinsGTTTTT
r.(?)
p.(Cys234TrpfsTer28)
-
pathogenic (recessive)
g.87679299_87679303delinsAAAAAC
g.86667071_86667075delinsAAAAAC
-
-
CNGB3_000249
-
PubMed: Mayer 2017
SCV000575782
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., +?/.
2
6
c.706delinsTT
r.(?)
p.(Ile236PhefsTer26)
-
likely pathogenic, pathogenic (recessive)
g.87679299delinsAA
g.86667071delinsAA
CNGB3 I236 fs
-
CNGB3_000248
no nucleotide annotation, extrapolated from protein and databases; heterozygous
PubMed: Kellner 2004
,
PubMed: Mayer 2017
SCV000575783
-
Germline, Unknown
?
-
-
-
-
Johan den Dunnen
?/.
1
-
c.731A>G
r.(?)
p.(Tyr244Cys)
ACMG
VUS
g.87679274T>C
g.86667046T>C
CNGB3:NM_019098 c.A731G, p.Y244C
-
CNGB3_000142
heterozygous, individual unsolved, causality of variants unknown
PubMed: Rodriguez-Munoz 2020
-
-
Germline
?
-
-
-
-
LOVD
-?/.
1
-
c.738C>T
r.(?)
p.(Thr246=)
-
likely benign
g.87679267G>A
g.86667039G>A
CNGB3(NM_019098.4):c.738C>T (p.T246=)
-
CNGB3_000068
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
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