Global Variome shared LOVD
PDE6A (phosphodiesterase 6A, cGMP-specific, rod, alpha)
LOVD v.3.0 Build 30b [
Current LOVD status
]
Register as submitter
|
Log in
Curator:
Global Variome, with Curator vacancy
View all genes
View PDE6A gene homepage
View graphs about the PDE6A gene database
Create a new gene entry
View all transcripts
View all transcripts of gene PDE6A
Create a new transcript information entry
View all variants
View all variants affecting transcripts
View unique variants in gene PDE6A
View all variants in gene PDE6A
Full data view for gene PDE6A
Create a new data submission
View active genomic custom columns
Enable more genomic custom columns
View all individuals
View all individuals with variants in gene PDE6A
Create a new data submission
View active custom columns
Enable more custom columns
View all diseases
View all diseases associated with gene PDE6A
Create a new disease information entry
View available phenotype columns
View all screenings
View all screenings for gene PDE6A
Create a new data submission
View active custom columns
Enable more custom columns
Submit new data
Unique variants in the PDE6A gene
This database is one of the
"Eye disease"
gene variant databases.
The variants shown are described using the NM_000440.2 transcript reference sequence.
Legend
Please note that a short description of a certain column can be displayed when you move your mouse cursor over the column's header and hold it still. Below, a more detailed description is shown per column.
Effect
: The variant's effect on the function of the gene/protein, displayed in the format 'R/C'. R is the value reported by the source (publication, submitter) and this classification may vary between records. C is the value concluded by the curator. Note that in some database the curator uses Summary records to give details on the classification of the variant.Values used: '+' indicating the variant affects function, '+?' probably affects function, '-' does not affect function, '-?' probably does not affect function, '?' effect unknown, '.' effect was not classified.
Reported
: The number of times this variant has been reported in the database.
Exon
: number of exon/intron containing variant; 2 = exon 2, 12i = intron 12, 2i_7i = from intron 2 to intron 7, 8i_9 = intron 8/exon 9 boundary, _1 = 5' to exon 1, 18_ = 3' of exon 18, _1_18_ = encompassing the entire 18-exon gene
DNA change (cDNA)
: description of variant at DNA level, based on a coding DNA reference sequence (following HGVS recommendations); e.g. c.123C>T, c.123_145del, c.123_126dup. For deletions/duplications extending beyond the reference transcript resp. {0}/{2} is used to replace del/dup. Extent of the deletion/duplication should be specified using the genomic description (g.). "-" indicates the variant described on genomic level does not affect the coding DNA reference sequence.
RNA change
: description of variant at RNA level (following HGVS recommendations).
r.123c>u
r.? = unknown
r.(?) = RNA not analysed but probably transcribed copy of DNA variant
r.spl? = RNA not analysed but variant probably affects splicing
r.(spl?) = RNA not analysed but variant may affect splicing
r.0? = change expected to abolish transcription
Protein
: description of variant at protein level (following HGVS recommendations).
p.(Arg345Pro) = change predicted from DNA (RNA not analysed)
p.Arg345Pro = change derived from RNA analysis
p.? = unknown effect
p.0? = probably no protein produced
Classification method
: The method used for the clinical classification of this variant.
All options:
ACMG
ACGS
EAHAD-CFDB
ENIGMA
IARC
InSiGHT
kConFab
other
Clinical classification
: Clinical classification of variant, preferably based on standardised criteria (e.g. ACMG), directed on the clinical consequences as published/submitted, indicated using an enriched system including inheritance: e.g. pathogenic, pathogenic (dominant), pathogenic (recessive), pathogenic (!), pathogenic (maternal), pathogenic (paternal). Standard inheritance is covered by dominant/recessive, imprinting by maternal/paternal. A '!' warns for exceptional circumstances to be explained in the 'Remarks' field (low penetrance, variants pathogenic in heterozygous state only, hypomorphic/hypermorphic variants, protective variants, etc.). Non-disease consequences (e.g. drug metabolism (pharmacogenetics), risk factor, blood group, tasting bitter) are indicated using additions to the benign classification; benign (dominant), benign (recessive), benign (!), etc. The value 'association' is used for variants associated with a phenotype and 'NA' for variants from in vitro/in silico records. NOTE: classification may differ from the opinion of the curator as given in a variant SUMMARY-record or the 'Functional effect concluded'). NOTE: pathogenic/likely pathogenic should go together with "variant (probably) affects function" In ClassFunctional.
All options:
pathogenic
pathogenic (dominant)
pathogenic (recessive)
pathogenic (!)
pathogenic (maternal)
pathogenic (paternal)
likely pathogenic
likely pathogenic (dominant)
likely pathogenic (recessive)
likely pathogenic (!)
likely pathogenic (maternal)
likely pathogenic (paternal)
VUS
VUS (!)
likely benign
likely benign (dominant)
likely benign (recessive)
likely benign (!)
likely benign (maternal)
likely benign (paternal)
benign
benign (dominant)
benign (recessive)
benign (!)
benign (maternal)
benign (paternal)
conflicting
association
NA
DNA change (genomic) (hg19)
: HGVS description of variant at DNA level, based on the genomic (chromosomal) DNA reference sequence; e.g. g.12345678C>T, g.12345679del, g.12345678_12345890dup
DNA change (hg38)
: HGVS description of variant at DNA level, based on the hg38 genomic (chromosomal) eference sequence; e.g. g.12345678C>T, g.12345679del, g.12345678_12345890dup
Published as
: listed only when different from "DNA change"; variant as reported originally (e.g. 521delT). Variants seen in animal models, tested in vitro, predicted from RNA analysis, etc. are described between brackets like c.(456C>G)
ISCN
: description of the variant according to ISCN nomenclature
DB-ID
: database ID of variant, grouping multiple observations of the same variant together, starting with the HGNC gene symbol, followed by an underscore (_) and a six digit number (e.g. DMD_012345). _000000 is used for variants where DNA was not analysed (change predicted from RNA analysis), variants seen in animal models or variants not seen in humans but functionally tested in vitro
Variant remarks
: remarks regarding variant described, e.g. germline mosaicism in mother, 345 kb deletion, muscle RNA analysed, not in 200 control chromosomes tested, on founder haplotype, etc.
Reference
: publication describing the variant submitted, incl. links to OMIM, PubMed or other source, e.g. "den Dunnen ASHG2003 P2346"
ClinVar ID
: ID of variant in ClinVar database
dbSNP ID
: the dbSNP ID
Origin
: Origin of variant/record: Germline = in all cells, De novo = in all cells, but not in either parent, Germline/De novo (untested) = in all cells, parents not tested (use only when De novo is likely, e.g. isolated/sporadic cases with dominant disease), Somatic = present in a subset of cells, but not in either parent, Uniparental disomy = from parental disomy (maternal or paternal), CLASSIFICATION record = submitter only sharing variant classification (note another report may share Individual data), SUMMARY record = master summary record from curator (may link to another database), In vitro (cloned) = data resulting from in vitro functional assays, animal model = data from animal model, Artefact = false positive variant call, DUPLICATE record = variant already described on another chromosome (e.g. unbalanced translocation, duplicating transposition, 2nd fusion transcript, etc.)
All options:
Germline
De novo
Germline/De novo (untested)
Somatic
Uniparental disomy
Uniparental disomy, maternal allele
Uniparental disomy, paternal allele
CLASSIFICATION record
SUMMARY record
In vitro (cloned)
In silico
animal model
Artefact
DUPLICATE record
Unknown
Not applicable
Segregation
: Indicates whether the variant segregates with the phenotype (yes), does not segregate with the phenotype (no) or segregation is unknown (?)
All options:
? = unknown
yes = segregates with phenotype
no = does not segregate with phenotype
- = not applicable
Frequency
: frequency in which the variant was found; e.g 5/760 chromosomes (in 5 of 760 chromosomes tested), 1/33 patients (in 1 of 33 patients analysed in study), 0.05 controls (in 5% of control cases tested)
Re-site
: restriction enzyme recognition site created (+) or destroyed (-); e.g. BglII+;BamHI-
VIP
: variant VIP-status was requested for matchmaking - need collaboration(s) to crack the case - please contact the submitter/curator. NOTE: to get VIP status ask the curator.
Methylation
: result of methylation test; GOM (gain of methylation), LOM (loss of methylation), 30% (30% methylated). NOTE: when several tests were done mention the method as well (e.g. MS-PCR 75%)
How to query this table
All list views have search fields which can be used to search data. You can search for a complete word or you can search for a part of a search term. If you enclose two or more words in double quotes, LOVD will search for the combination of those words only exactly in the order you specify. Note that search terms are case-insensitive and that wildcards such as * are treated as normal text! For all options, like "and", "or", and "not" searches, or searching for prefixes or suffixes, see the table below.
Operator
Column type
Example
Matches
Text
Arg
all entries containing 'Arg'
space
Text
Arg Ser
all entries containing 'Arg' and 'Ser'
|
Text
Arg|Ser
all entries containing 'Arg' or 'Ser'
!
Text
!fs
all entries not containing 'fs'
^
Text
^p.(Arg
all entries beginning with 'p.(Arg'
$
Text
Ser)$
all entries ending with 'Ser)'
=""
Text
=""
all entries with this field empty
=""
Text
="p.0"
all entries exactly matching 'p.0'
!=""
Text
!=""
all entries with this field not empty
!=""
Text
!="p.0"
all entries not exactly matching 'p.0?'
combination
Text
*|Ter !fs
all entries containing '*' or 'Ter' but not containing 'fs'
Date
2020
all entries matching the year 2020
|
Date
2020-03|2020-04
all entries matching March or April, 2020
!
Date
!2020-03
all entries not matching March, 2020
<
Date
<2020
all entries before the year 2020
<=
Date
<=2020-06
all entries in or before June, 2020
>
Date
>2020-06
all entries after June, 2020
>=
Date
>=2020-06-15
all entries on or after June 15th, 2020
combination
Date
2019|2020 <2020-03
all entries in 2019 or 2020, and before March, 2020
Numeric
23
all entries exactly matching 23
|
Numeric
23|24
all entries exactly matching 23 or 24
!
Numeric
!23
all entries not exactly matching 23
<
Numeric
<23
all entries lower than 23
<=
Numeric
<=23
all entries lower than, or equal to, 23
>
Numeric
>23
all entries higher than 23
>=
Numeric
>=23
all entries higher than, or equal to, 23
combination
Numeric
>=20 <30 !23
all entries with values from 20 to 29, but not equal to 23
Some more advanced examples:
Example
Matches
Asian
all entries containing 'Asian', 'asian', including 'Caucasian', 'caucasian', etc.
Asian !Caucasian
all entries containing 'Asian' but not containing 'Caucasian'
Asian|African !Caucasian
all entries containing 'Asian' or 'African', but not containing 'Caucasian'
"South Asian"
all entries containing 'South Asian', but not containing 'South East Asian'
To sort on a certain column, click on the column header or on the arrows. If that column is already selected to sort on, the sort order will be swapped. The column currently sorted on has a darker blue background color than the other columns. The up and down arrows next to the column name indicate the current sorting direction. When sorting on any field other than the default, LOVD will sort secondarily on the default sort column.
215 entries on 3 pages. Showing entries 1 - 100.
10 per page
25 per page
50 per page
100 per page
Legend
How to query
« First
Prev
1
2
3
Next
Last »
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
?/.
2
1
c.-42C>T
r.(?)
p.(=)
-
VUS
g.149324278G>A
-
c.-42C>T
-
PDE6A_000151
-
PubMed: Anasagasti-2013
-
rs113137904
Germline
yes
<0.01
-
-
-
LOVD
+?/.
1
-
c.?
r.?
p.?
ACMG
likely pathogenic (recessive)
g.149230182_149270203dup
-
-
-
RAD50_000000
ACMG PM2, PVS1; no variant 2nd chromosome
PubMed: Weisschuh 2024
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.1A>G
r.(?)
p.(Met1?)
ACMG
likely pathogenic
g.149324236T>C
g.149944673T>C
PDE6A c.1A>G, p.(Met1?), c.2332_2335del, p.(Asp778Leufs*42)
-
PDE6A_000140
-
PubMed: Jespersgaar 2019
-
-
Germline
?
-
-
-
-
LOVD
+?/.
1
-
c.38T>A
r.(?)
p.(Leu13Gln)
-
likely pathogenic
g.149324199A>T
g.149944636A>T
PDE6A, variant 1: c.38T>A/p.L13Q, variant 2: c.2053G>A/p.V685M
-
PDE6A_000166
possibly solved, compound heterozygous
PubMed: Weisschuh 2020
-
-
Unknown
?
-
-
-
-
LOVD
+?/.
1
-
c.63_68del
r.(?)
p.(Lys21_Tyr23delinsAsn)
ACMG
likely pathogenic
g.149324170_149324175del
g.149944607_149944612del
PDE6A c.63_68del/ p.K21_Y23delinsN
-
PDE6A_000198
HGMD: none; heterozygous
PubMed: Kuehlewein 2020
-
-
Germline
yes
-
-
-
-
LOVD
+/., +?/.
2
-
c.84C>G
r.(?)
p.(Tyr28*), p.(Tyr28Ter)
ACMG
likely pathogenic, pathogenic
g.149324153G>C
g.149944590G>C
PDE6A c.84C>G/p.Y28*
-
PDE6A_000050
HGMD: none; homozygous, VKGL data sharing initiative Nederland
PubMed: Kuehlewein 2020
-
-
CLASSIFICATION record, Unknown
?
-
-
-
-
VKGL-NL_Nijmegen
+?/., ?/.
2
1
c.85C>T
r.(?)
p.(Arg29Trp)
-
likely pathogenic, VUS
g.149324152G>A
g.149944589G>A
PDE6A Ex.1 c.85C>T p.(Arg29Trp), Ex.1 c.85C>T p.(Arg29Trp),
1 more item
-
PDE6A_000093
homozygous, VKGL data sharing initiative Nederland
PubMed: Martin Merida 2019
-
-
CLASSIFICATION record, Germline/De novo (untested)
?
-
-
-
-
VKGL-NL_Rotterdam
+?/.
1
-
c.86G>A
r.(?)
p.(Arg29Gln)
-
VUS
g.149324151C>T
g.149944588C>T
-
-
PDE6A_000213
-
PubMed: Peng 2017
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.88G>A
r.(?)
p.(Ala30Thr)
-
VUS
g.149324149C>T
g.149944586C>T
PDE6A(NM_000440.2):c.88G>A (p.A30T)
-
PDE6A_000028
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/.
1
-
c.102C>T
r.(?)
p.(Ser34=)
-
benign
g.149324135G>A
g.149944572G>A
PDE6A(NM_000440.3):c.102C>T (p.S34=)
-
PDE6A_000027
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
1
-
c.103G>A
r.(?)
p.(Asp35Asn)
-
VUS
g.149324134C>T
g.149944571C>T
PDE6A(NM_000440.2):c.103G>A (p.D35N)
-
PDE6A_000092
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.156G>A
r.(?)
p.(Pro52=)
-
likely benign
g.149324081C>T
-
PDE6A(NM_000440.2):c.156G>A (p.P52=)
-
PDE6A_000191
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
1
c.166G>T
r.(?)
p.(Glu56*)
-
pathogenic (recessive)
g.149324071C>A
-
c.166G>T:p.E56X
-
PDE6A_000174
-
PubMed: Numa-2020
-
-
Unknown
-
-
-
-
-
LOVD
?/.
1
-
c.174C>A
r.(?)
p.(Ser58Arg)
-
VUS
g.149324063G>T
g.149944500G>T
PDE6A(NM_000440.3):c.174C>A (p.S58R)
-
PDE6A_000091
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/., -?/.
2
-
c.177A>G
r.(?)
p.(Glu59=)
-
benign, likely benign
g.149324060T>C
g.149944497T>C
PDE6A(NM_000440.2):c.177A>G (p.E59=), PDE6A(NM_000440.3):c.177A>G (p.E59=)
-
PDE6A_000026
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
?/.
3
-
c.196C>T
r.(?)
p.(Arg66Trp)
-
VUS
g.149324041G>A
g.149944478G>A
PDE6A(NM_000440.2):c.196C>T (p.R66W)
-
PDE6A_000069
VKGL data sharing initiative Nederland
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
rs149945348
CLASSIFICATION record, Germline
-
1/1204 cases with retinitis pigmentosa
-
-
-
VKGL-NL_Rotterdam
,
Yoshito Koyanagi
+/., ?/.
3
1
c.205C>T
r.(?)
p.(Gln69*), p.?
ACMG
pathogenic, VUS
g.149324032G>A
g.149944469G>A
c.205C>T, PDE6A c.205C>T, p.(Gln69*),
1 more item
-
PDE6A_000129
heterozygous, ACMG unclassified - no access to supplementary table 2,
1 more item
PubMed: Hull 2020
,
PubMed: Khateb 2019
,
PubMed: Wang-2013
-
-
Germline, Unknown
?, yes
-
-
-
-
LOVD
-?/.
1
1
c.251A>T
r.(?)
p.(Lys84Met)
-
likely benign
g.149323986T>A
-
c.251A>T
-
PDE6A_000188
-
PubMed: Men-2017
-
-
Germline
-
-
-
-
-
LOVD
+/.
1
-
c.268C>T
r.(?)
p.(Gln90Ter)
-
pathogenic
g.149323969G>A
g.149944406G>A
-
-
PDE6A_000049
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
2
-
c.282G>A
r.(?)
p.(Met94Ile)
-
VUS
g.149323955C>T
g.149944392C>T
PDE6A(NM_000440.2):c.282G>A (p.M94I), PDE6A(NM_000440.3):c.282G>A (p.M94I)
-
PDE6A_000025
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
+?/.
2
1
c.285C>A
r.(?), r.?
p.(Ser95Arg)
ACMG
likely pathogenic, likely pathogenic (recessive)
g.149323952G>T
g.149944389G>T
c.285C>A, NM_000440.2:c.285C>A, NP_000431.2:p.(Ser95Arg), NC_000005.9:g.149323952G>T
-
PDE6A_000126
-
PubMed: Liu-2020
,
PubMed: Wang 2018
-
-
Germline
?
-
-
-
-
LOVD
+?/.
1
1
c.298C>T
r.(?)
p.(Arg100Trp)
-
likely pathogenic
g.149323939G>A
-
c.298C>T
-
PDE6A_000131
-
PubMed: Eisenberger-2013
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/., ?/.
53
1
c.304C>A
r.(?)
p.(Arg102Cys), p.(Arg102Ser)
ACMG
likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive), VUS
g.149323933G>T
g.149944370G>T
c.304C>A, c.304C>A , p.Arg102Ser, c.304C>A, p.Arg102Ser, PDE6A Arg102Ser (CGC to AGC),
9 more items
-
PDE6A_000048
ACMG PM2, PM5, PP5_STRONG, heterozygous, HGMD: CM994742; heterozygous, HGMD: CM994742; homozygous,
6 more items
PubMed: Abu-Safieh-2013
,
PubMed: Avila Fernandez 2010
,
PubMed: Birtel 2018
,
PubMed: Booij-2011
,
15 more items
193099
rs141252097
CLASSIFICATION record, Germline, Unknown
?, yes
1/2420 IRD families, 2/899 cases
-
-
-
Global Variome, with Curator vacancy
,
Johan den Dunnen
,
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
,
Daan Panneman
+/., +?/.
6
1
c.304C>T
r.(?)
p.(Arg102Cys)
-
likely pathogenic, pathogenic
g.149323933G>A
g.149944370G>A
c.304C>T, c.304C>T, p.Arg102Cys, PDE6A 304C>T, R102C
-
PDE6A_000047
heterozygous, VKGL data sharing initiative Nederland,
1 more item
PubMed: Panneman 2023
,
PubMed: Tsang 2008
,
PubMed: Zampaglione 2020
-
-
CLASSIFICATION record, Germline, Unknown
?, yes
-
-
-
-
VKGL-NL_Nijmegen
,
Daan Panneman
+/., +?/., ?/.
14
1
c.305G>A
r.(?)
p.(Arg102His)
ACMG
likely pathogenic, likely pathogenic (recessive), pathogenic, VUS
g.149323932C>T
g.149944369C>T
c.305G>A, PDE6A Arg102His (CGC to CAC), PDE6A c.305G > A, p.Arg102His, PDE6A c.305G>A/p.R102H,
3 more items
-
PDE6A_000102
heterozygous, HGMD: CM994741; heterozygous, HGMD: CM994741; homozygous, homozygous,
2 more items
PubMed: Avila Fernandez 2010
,
PubMed: Collin-2011
,
PubMed: Dryja 1999
,
PubMed: Holtan 2020
,
7 more items
-
-
Germline, Germline/De novo (untested), In vitro (cloned), Unknown
?, yes
1/899 cases
-
-
-
Global Variome, with Curator vacancy
,
Daan Panneman
?/.
1
-
c.316G>A
r.(?)
p.(Ala106Thr)
-
VUS
g.149323921C>T
g.149944358C>T
-
-
PDE6A_000068
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
rs200696413
Germline
-
3/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
-/.
1
-
c.331A>C
r.(?)
p.(Arg111=)
-
benign
g.149323906T>G
g.149944343T>G
-
-
PDE6A_000046
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/.
1
-
c.357del
r.(?)
p.(Val120SerfsTer7)
-
likely pathogenic
g.149323880del
g.149944317del
357delT
-
PDE6A_000119
-
PubMed: Huang 2015
-
-
Germline
yes
-
-
-
-
LOVD
?/.
2
1
c.367G>T
r.(?)
p.(Asp123Tyr)
-
VUS
g.149323870C>A
g.149944307C>A
p.Asp123Tyr
-
PDE6A_000113
-
PubMed: Anasagasti-2013
,
PubMed: Ellingford 2016
-
rs147010346
Germline
yes
<0.01
-
-
-
LOVD
?/.
1
-
c.397G>A
r.(?)
p.(Val133Ile)
-
VUS
g.149323840C>T
g.149944277C>T
PDE6A(NM_000440.3):c.397G>A (p.V133I)
-
PDE6A_000090
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
2
1
c.409delinsCT
r.(?)
p.(Asp137Leufs*12)
ACMG
pathogenic
g.149323828delinsAG, g.149323828delinsCT
-
-
-
PDE6A_000054
1 more item
Sharon, submitted,
PubMed: Sharon 2019
-
-
Germline
-
1/2420 IRD families
-
-
-
Global Variome, with Curator vacancy
,
Dror Sharon
?/.
1
-
c.425G>A
r.(?)
p.(Gly142Asp)
ACMG
VUS
g.149323812C>T
g.149944249C>T
PDE6A c.G425A, p.G142D
-
PDE6A_000169
marked as causative, heterozygous
PubMed: Ma 2021
-
-
Unknown
?
-
-
-
-
LOVD
-?/.
1
-
c.456C>T
r.(?)
p.(Asn152=)
-
likely benign
g.149323781G>A
-
PDE6A(NM_000440.2):c.456C>T (p.N152=)
-
PDE6A_000106
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.457G>A
r.(?)
p.(Val153Ile)
-
VUS
g.149323780C>T
g.149944217C>T
-
-
PDE6A_000117
-
PubMed: Xu 2014
-
-
Germline
-
1/314 case chromosomes
-
-
-
LOVD
-/.
1
-
c.465C>T
r.(?)
p.(Asn155=)
-
benign
g.149323772G>A
g.149944209G>A
-
-
PDE6A_000045
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
-
c.472G>A
r.(?)
p.(Glu158Lys)
-
VUS
g.149323765C>T
g.149944202C>T
-
-
PDE6A_000067
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
-
Germline
-
1/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
-?/.
1
-
c.474+11C>T
r.(=)
p.(=)
-
likely benign
g.149323752G>A
-
PDE6A(NM_000440.3):c.474+11C>T
-
PDE6A_000206
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
1
-
c.(474+1_475-1)_(627+1_628-1)del
r.?
p.(Asp159_Glu209del)
-
VUS
g.?
-
c.(474+1_475-1)_(627+1_628-1)del
-
RAD50_000000
-
PubMed: Panneman 2023
-
-
Unknown
-
-
-
-
-
Daan Panneman
?/.
1
-
c.497T>C
r.(?)
p.(Val166Ala)
-
VUS
g.149314259A>G
-
PDE6A(NM_000440.2):c.497T>C (p.V166A)
-
PDE6A_000190
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/., ?/.
3
-
c.498G>A
r.(?)
p.(Val166=)
-
benign, VUS
g.149314258C>T
g.149934695C>T
PDE6A(NM_000440.2):c.498G>A (p.V166=), PDE6A(NM_000440.3):c.498G>A (p.V166=, p.(Val166=))
-
PDE6A_000024
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
?/.
1
-
c.499G>A
r.(?)
p.(Asp167Asn)
-
VUS
g.149314257C>T
-
PDE6A(NM_000440.3):c.499G>A (p.D167N)
-
PDE6A_000201
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+?/.
1
2
c.533T>A
r.(?)
p.(Leu178*)
-
likely pathogenic
g.149314223A>T
g.149934660A>T
PDE6A Ex.2 c.533T>A p.(Leu178*), Ex.16 c.1957C>T p.(Arg653*)
-
PDE6A_000154
compound heterozygous
PubMed: Martin Merida 2019
-
-
Germline/De novo (untested)
?
-
-
-
-
LOVD
+?/.
2
-
c.612del
r.(?)
p.(Lys205Argfs*16)
ACMG
likely pathogenic
g.149314145del
g.149934582del
PDE6A c.612del, p.(Lys205Argfs*16), c.612del, p.(Lys205Argfs*16), PDE6A c.612del/p.K205Rfs*16
-
PDE6A_000139
HGMD: CD192430; homozygous, homozygous
PubMed: Jespersgaar 2019
,
PubMed: Kuehlewein 2020
-
-
Germline, Unknown
?
-
-
-
-
LOVD
+?/.
2
-
c.627+2T>G
r.(?), r.spl
p.(?), p.?
ACMG
likely pathogenic
g.149314127A>C
g.149934564A>C
PDE6A c.627 + 2T>G/p.?, PDE6A c.627+2T>G, p.(?), c.627+2T>G, p.(?)
-
PDE6A_000138
HGMD: none; homozygous, homozygous
PubMed: Jespersgaar 2019
,
PubMed: Kuehlewein 2020
-
-
Germline, Unknown
?
-
-
-
-
LOVD
+/., +?/., -/.
6
2
c.647A>G
r.(?)
p.(Asn216Ser)
-
benign, likely benign, likely pathogenic, pathogenic
g.149313563T>C
g.149934000T>C
c.647A>G, PDE6A , N216S
-
PDE6A_000044
VKGL data sharing initiative Nederland,
1 more item
PubMed: Booij-2011
,
PubMed: Holtan 2020
,
PubMed: Tsang 2008
-
-
CLASSIFICATION record, Germline
yes
1/899 cases
-
-
-
Global Variome, with Curator vacancy
,
VKGL-NL_Nijmegen
+/., +?/.
3
2
c.676del
r.(?)
p.(His226Thrfs*2)
ACMG
likely pathogenic, pathogenic (recessive)
g.149313534del, g.149313535del
g.149933972del
c.676del, PDE6A c.676del/p.H226Tfs*2,
1 more item
-
PDE6A_000165
HGMD: CD140529; heterozygous, solved, compound heterozygous
PubMed: Colombo-2020
,
PubMed: Kuehlewein 2020
,
PubMed: Weisschuh 2020
-
-
Germline, Unknown
?
-
-
-
-
LOVD
?/.
1
-
c.717+5G>A
r.spl?
p.?
-
VUS
g.149313488C>T
g.149933925C>T
PDE6A(NM_000440.3):c.717+5G>A
-
PDE6A_000023
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/.
3
-
c.718-4dup
r.(?), r.spl?
p.?
-
likely benign, VUS
g.149310740dup
g.149931177dup
PDE6A(NM_000440.2):c.718-4dupT, PDE6A(NM_000440.3):c.718-4dupT
-
PDE6A_000089
heterozygous variant only, VKGL data sharing initiative Nederland
PubMed: Arno 2017
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
?/.
1
-
c.722T>C
r.(?)
p.(Leu241Pro)
-
VUS
g.149310727A>G
-
PDE6A(NM_000440.3):c.722T>C (p.L241P)
-
PDE6A_000212
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
1
-
c.733G>A
r.(?)
p.(Gly245Arg)
-
VUS
g.149310716C>T
g.149931153C>T
-
-
PDE6A_000066
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
-
Germline
-
1/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
+?/., ?/.
2
-
c.743T>A
r.(?)
p.(Val248Asp)
ACMG
likely pathogenic, VUS
g.149310706A>T
g.149931143A>T
PDE6A c.743T>A/p.V248D, PDE6A, variant 1: c.1610T>C/p.I537T, variant 2: c.743T>A/p.V248D
-
PDE6A_000164
HGMD: none; heterozygous, possibly solved, compound heterozygous
PubMed: Kuehlewein 2020
,
PubMed: Weisschuh 2020
-
-
Unknown
?
-
-
-
-
LOVD
-?/.
1
-
c.759G>A
r.(?)
p.(Thr253=)
-
likely benign
g.149310690C>T
-
PDE6A(NM_000440.3):c.759G>A (p.T253=)
-
PDE6A_000200
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
1
-
c.763A>G
r.(?)
p.(Ile255Val)
ACMG
VUS
g.149310686T>C
g.149931123T>C
PDE6A:NM_000440 c.A763G, p.I255V
-
PDE6A_000158
heterozygous, individual solved, variant non-causal
PubMed: Rodriguez-Munoz 2020
-
-
Germline
?
-
-
-
-
LOVD
+/., +?/.
20
3, 4
c.769C>T
r.(?)
p.(Arg257*), p.(Arg257Ter)
ACMG
likely pathogenic, pathogenic, pathogenic (recessive)
g.149310680G>A
g.149931117G>A
5:149310680G>A ENST00000255266.5:c.769C>T (Arg257Ter), c.769C>T, c.769C>T, p.Arg257Ter,
3 more items
-
PDE6A_000003
ACMG PM2, PVS1, PP5, heterozygous, HGMD: CM066961; homozygous, solved, homozygous,
2 more items
PubMed: Bocquet 2013
,
PubMed: Bocquet-2013
,
PubMed: Carss 2017
,
PubMed: Kuehlewein 2020
,
7 more items
-
-
CLASSIFICATION record, Germline, Unknown
?, yes
1/2420 IRD families
-
-
-
Global Variome, with Curator vacancy
,
Johan den Dunnen
,
James Hejtmancik
,
VKGL-NL_Nijmegen
,
Daan Panneman
+/., +?/., ?/.
5
3, 4
c.784G>A
r.(?)
p.(Ala262Thr)
-
likely pathogenic, pathogenic, VUS
g.149310665C>T
g.149931102C>T
c.784G>A, p.Ala262Thr,
1 more item
-
PDE6A_000043
homozygous, VKGL data sharing initiative Nederland
PubMed: Anasagasti-2013
,
PubMed: Eisenberger-2013
,
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
,
1 more item
-
rs148938083
,
rs14898083
CLASSIFICATION record, Germline
yes
1/1204 cases with retinitis pigmentosa, <0.01
-
-
-
VKGL-NL_Nijmegen
,
Yoshito Koyanagi
+/.
1
-
c.816T>A
r.(?)
p.(Cys272Ter)
ACMG
pathogenic (recessive)
g.149310633A>T
g.149931070A>T
-
-
PDE6A_000210
ACMG PM2, PVS1, PP5
PubMed: Weisschuh 2024
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
4
c.823_824del
r.(?)
p.(Tyr275Leufs*15)
ACMG
pathogenic
g.149310626_149310627del
g.149931063_149931064del
PDE6A c.823_824del, p.(Tyr275Leufs*15)
-
PDE6A_000186
heterozygous; mother - het c.2366C>T p.(Ser789Phe)
PubMed: Khateb 2019
-
-
Germline
yes
-
-
-
-
LOVD
?/.
1
-
c.858+5G>T
r.spl?
p.?
ACMG
VUS
g.149310586C>A
g.149931023C>A
-
-
PDE6A_000209
ACMG PP3, PM2
PubMed: Weisschuh 2024
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
5
3i
c.859-63A>G
r.(=)
p.(=)
-
benign (recessive)
g.149301335T>C
-
c.859-63A>G
-
PDE6A_000150
-
PubMed: Anasagasti-2013
-
rs11167487
Germline
yes
0.33
-
-
-
LOVD
+?/., -/., -?/., ?/.
9
5
c.878C>T
r.(?)
p.(Pro293Leu)
ACMG
benign, likely benign, likely pathogenic, likely pathogenic (recessive), VUS
g.149301253G>A
g.149921690G>A
p.Pro293Leu, PDE6A c.878C>T, p.(Pro293Leu), PDE6A(NM_000440.3):c.878C>T (p.P293L),
1 more item
-
PDE6A_000021
12 heterozygous, no homozygous;
Clinindb (India)
, compound heterozygous,
2 more items
PubMed: Anasagasti-2013
,
PubMed: Holtan 2020
,
PubMed: Jespersgaar 2019
,
PubMed: Martin Merida 2019
,
3 more items
-
rs114973968
CLASSIFICATION record, Germline, Germline/De novo (untested)
?, yes
12/2795 individuals, 16/1204 cases with retinitis pigmentosa, 3/899 cases, <0.01
-
-
-
Global Variome, with Curator vacancy
,
VKGL-NL_Groningen
,
VKGL-NL_AMC
,
Yoshito Koyanagi
,
Mohammed Faruq
+/.
1
-
c.906C>G
r.(?)
p.(Tyr302Ter)
-
pathogenic
g.149301225G>C
g.149921662G>C
PDE6A(NM_000440.2):c.906C>G (p.Y302*)
-
PDE6A_000020
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/., -?/.
8
-
c.908C>G
r.(?)
p.(Asn216Ser), p.(Ser303Cys)
ACMG
likely benign, likely pathogenic
g.149301223G>C, g.149313563T>C
g.149921660G>C, g.149934000T>C
PDE6A 908C>G, S303C, PDE6A c.908C>G, p.S303C,
1 more item
-
PDE6A_000044, PDE6A_000088
compound heterozygous, VKGL data sharing initiative Nederland,
1 more item
PubMed: Jauregui 2020
,
PubMed: Sharon 2019
,
PubMed: Tsang 2008
-
-
CLASSIFICATION record, Germline, Unknown
?, yes
1/2420 IRD families
-
-
-
Global Variome, with Curator vacancy
,
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
-?/.
1
-
c.918G>A
r.(?)
p.(Arg306=)
-
likely benign
g.149301213C>T
g.149921650C>T
PDE6A(NM_000440.3):c.918G>A (p.R306=)
-
PDE6A_000087
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+?/.
1
5
c.923C>T
r.(?)
p.(Pro308Leu)
-
likely pathogenic
g.149301208G>A
-
c.923C>T
-
PDE6A_000130
-
PubMed: Eisenberger-2013
-
-
Germline
-
-
-
-
-
LOVD
-?/.
1
-
c.924G>A
r.(?)
p.(Pro308=)
-
likely benign
g.149301207C>T
g.149921644C>T
PDE6A(NM_000440.2):c.924G>A (p.P308=)
-
PDE6A_000019
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/., -/.
5
5i
c.933+4C>T
r.spl?
p.(?), p.?
-
benign, likely pathogenic, likely pathogenic (recessive)
g.149301194G>A
g.149921631G>A
c.933+4C>T, PDE6A(NM_000440.3):c.933+4C>T
-
PDE6A_000096
heterozygous, VKGL data sharing initiative Nederland
PubMed: Anasagasti-2013
,
PubMed: Gao 2019
-
rs12109444
CLASSIFICATION record, Germline
?, yes
0.01
-
-
-
VKGL-NL_AMC
+?/.
1
-
c.948C>G
r.(?)
p.(Tyr316*)
ACMG
pathogenic
g.149294556G>C
-
-
-
PDE6A_000122
-
-
-
-
Germline/De novo (untested)
-
-
-
-
-
Jinu Han
?/.
2
-
c.959A>G
r.(?)
p.(Asp320Gly)
ACMG
VUS
g.149294545T>C
g.149914982T>C
PDE6A c.959A>G/p.D320G
-
PDE6A_000041
HGMD: none; heterozygous, VKGL data sharing initiative Nederland
PubMed: Kuehlewein 2020
-
-
CLASSIFICATION record, Unknown
?
-
-
-
-
VKGL-NL_Nijmegen
+/., +?/.
24
6, 7i
c.998+1G>A
r.(?), r.spl, r.spl?
p.(?), p.?
ACMG
likely pathogenic, pathogenic
g.149294505C>T
g.149914942C>T
c.998+1G>A, PDE6A c.998 + 1G>A/p.?, PDE6A c.998+1G>A, PDE6A c.998+1G>A, p.(?),
2 more items
-
PDE6A_000040
heterozygous, HGMD: CS994749; homozygous, homozygous, VKGL data sharing initiative Nederland,
1 more item
PubMed: Avila Fernandez 2010
,
PubMed: Dryja 1999
,
PubMed: Khateb 2019
,
PubMed: Kjellstrom 2016
,
3 more items
-
-
CLASSIFICATION record, Germline, Unknown
?, yes
-
-
-
-
VKGL-NL_Nijmegen
,
Daan Panneman
+/., +?/.
2
6i
c.998+2T>G
r.(?), r.spl
p.(?), p.?
ACMG
likely pathogenic, pathogenic
g.149294504A>C
g.149914941A>C
PDE6A c.998 + 2T>G/p.?, PDE6A IVS6 c.998+2T>G p.(?), Ex.16 c.1957C>T p.(Arg653*)
-
PDE6A_000153
compound heterozygous, HGMD: none; heterozygous
PubMed: Kuehlewein 2020
,
PubMed: Martin Merida 2019
-
-
Germline
yes
-
-
-
-
LOVD
-?/.
1
7i
c.998+11C>T
r.(=)
p.(=)
-
likely benign (recessive)
g.149294495G>A
-
c.998+11C>T
-
PDE6A_000149
-
PubMed: Anasagasti-2013
-
rs75319698
Germline
yes
0.01
-
-
-
LOVD
+/.
1
-
c.999-2A>G
r.spl
p.?
ACMG
pathogenic (recessive)
g.149286943T>C
g.149907380T>C
-
-
PDE6A_000208
ACMG PM2, PVS1, PP5
PubMed: Weisschuh 2024
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
3
-
c.1032C>A
r.(?)
p.(Ser334Arg), p.(Ser344Arg)
-
likely pathogenic
g.149286908G>T
g.149907345G>T
PDE6A Ser334Arg
-
PDE6A_000039
no nucleotide annotation, extrapolated from protein, sequence and databases; heterozygous,
1 more item
PubMed: Huang 1995
-
-
CLASSIFICATION record, Germline
yes
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
-
c.1032C>T
r.(=)
p.(=)
-
pathogenic
g.149286908G>A
g.149907345G>A
-
-
PDE6A_000065
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
rs121918577
Germline
-
1/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
+/.
1
-
c.1054C>T
r.(?)
p.(Gln352*)
ACMG
pathogenic
g.149286886G>A
g.149907323G>A
PDE6A c.1287G>A, p.(Trp429*), c.1054C>T, p.(Gln352*)
-
PDE6A_000137
-
PubMed: Jespersgaar 2019
-
-
Germline
?
-
-
-
-
LOVD
+/.
1
7
c.1065+1G>T
r.spl
p.(?)
ACMG
pathogenic
g.149286874C>A
g.149907311C>A
PDE6A c.1065+1G>T, p.(?)
-
PDE6A_000185
heterozygous
PubMed: Khateb 2019
-
-
Germline
yes
-
-
-
-
LOVD
+/., +?/.
7
7, 7i
c.1065+2T>A
r.(?), r.spl, r.spl?
p.(?), p.?
ACMG
likely pathogenic, pathogenic
g.149286873A>T
g.149907310A>T
c.1065+2T>A, PDE6A c.1065 + 2T>A/p.?, PDE6A c.1065+2T>A, p.(?),
1 more item
-
PDE6A_000163
heterozygous; mother het c.998+1G>A unaffected sister - het c.1065+2T, HGMD: none; heterozygous,
1 more item
PubMed: Khateb 2019
,
PubMed: Kuehlewein 2020
,
PubMed: Panneman 2023
,
PubMed: Weisschuh 2020
-
-
Germline, Unknown
?, yes
-
-
-
-
Daan Panneman
-?/.
1
6i
c.1066-57A>G
r.(=)
p.(=)
-
likely benign (recessive)
g.149283315T>C
-
c.1066-57A>G
-
PDE6A_000148
-
PubMed: Anasagasti-2013
-
rs115072370
Germline
yes
0.03
-
-
-
LOVD
+/.
1
8
c.1072A>T
r.(?)
p.(Asn358Tyr)
ACMG
pathogenic
g.149283252T>A
g.149903689T>A
PDE6A c.1072A>T, p.(Asn358Tyr)
-
PDE6A_000184
heterozygous; mother het c.1072A>T p.(Asn358Tyr)
PubMed: Khateb 2019
-
-
Germline
yes
-
-
-
-
LOVD
-?/.
1
-
c.1086G>A
r.(?)
p.(Ala362=)
-
likely benign
g.149283238C>T
g.149903675C>T
PDE6A(NM_000440.2):c.1086G>A (p.A362=)
-
PDE6A_000086
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
-
c.1113+1G>A
r.spl
p.(?)
ACMG
pathogenic
g.149283210C>T
g.149903647C>T
PDE6A:NM_000440 c.1113+1G>A, p.?
-
PDE6A_000157
heterozygous, individual solved, variant non-causal
PubMed: Rodriguez-Munoz 2020
-
-
Germline
?
-
-
-
-
LOVD
+?/.
3
8
c.1166C>T
r.(?)
p.(Pro389Leu)
ACMG
likely pathogenic, likely pathogenic (recessive)
g.149279035G>A
g.149899472G>A
c.1166C>T, PDE6A c.1166C>T/p.P389L
-
PDE6A_000121
HGMD: CM112500; homozygous
PubMed: Collin-2011
,
PubMed: Colombo-2020
,
PubMed: Kuehlewein 2020
-
rs1406110455
Germline, Unknown
?
0/360 controls
-
-
-
LOVD
-?/.
1
8
c.1214A>G
r.(?)
p.(Asn405Ser)
-
likely benign
g.149278987T>C
-
c.1214A>G
-
PDE6A_000187
-
PubMed: Men-2017
-
rs145107955
Germline
-
-
-
-
-
LOVD
?/.
1
-
c.1235T>C
r.(?)
p.(Phe412Ser)
ACMG
VUS
g.149278966A>G
g.149899403A>G
PDE6A c.1235T>C/p.F412S
-
PDE6A_000197
HGMD: none; heterozygous
PubMed: Kuehlewein 2020
-
-
Germline
yes
-
-
-
-
LOVD
+/.
1
9
c.1236del
r.(?)
p.(Phe412Leufs*12)
ACMG
pathogenic
g.149278967del
g.149899404del
PDE6A c.1236del, p.(Phe412Leufs*12)
-
PDE6A_000183
heterozygous; father het c.1236del p.(Phe412Leufs*12) mother - het c.823_824del p.(Tyr275Leufs*1
PubMed: Khateb 2019
-
-
Germline
yes
-
-
-
-
LOVD
?/.
1
-
c.1237G>T
r.(?)
p.(Asp413Tyr)
-
VUS
g.149278964C>A
g.149899401C>A
PDE6A(NM_000440.3):c.1237G>T (p.D413Y)
-
PDE6A_000017
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+?/.
1
-
c.1246G>A
r.(?)
p.(Asp416Asn)
-
likely pathogenic
g.149278955C>T
-
-
-
PDE6A_000199
not in 100 controls
PubMed: Li 2022
,
Journal: Li 2022
-
-
Germline/De novo (untested)
-
1/8 patients
-
-
-
Alaaeldin Fayez
+?/.
1
9
c.1250A>G
r.(?)
p.(Glu417Gly)
-
likely pathogenic
g.149278951T>C
g.149899388T>C
PDE6A Ex.9 c.1250A>G p.(Glu417Gly), Ex.9 c.1250A>G p.(Glu417Gly)
-
PDE6A_000152
homozygous
PubMed: Martin Merida 2019
-
-
Germline/De novo (untested)
?
-
-
-
-
LOVD
?/.
1
8
c.1253C>T
r.(?)
p.(Thr418Met)
-
VUS
g.149278948G>A
-
p.Tre418Met
-
PDE6A_000147
-
PubMed: Anasagasti-2013
-
-
Germline
yes
-
-
-
-
LOVD
+/.
1
-
c.1263+1G>A
r.spl
p.?
ACMG
pathogenic
g.149278937C>T
g.149899374C>T
PDE6A c.1263 + 1G>A/p.?
-
PDE6A_000196
HGMD: none; heterozygous
PubMed: Kuehlewein 2020
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
1
-
c.1264-30_1264-25del
r.(=)
p.(=)
-
likely pathogenic (recessive)
g.149278098_149278103del
g.149898535_149898540del
delCAGAGA
-
PDE6A_000215
-
PubMed: Anasagasti-2013
-
rs34656337
Germline
yes
0.03
-
-
-
LOVD
-/.
1
-
c.1264-16T>A
r.(=)
p.(=)
-
benign
g.149278085A>T
g.149898522A>T
PDE6A(NM_000440.3):c.1264-16T>A
-
PDE6A_000085
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/., +?/.
6
10
c.1268del
r.(?)
p.(Leu423*), p.(Leu423Ter)
ACMG
likely pathogenic, likely pathogenic (recessive), pathogenic
g.149278067del
g.149898504del
1268delT, PDE6A c.1268del, p.(Leu423*)
-
PDE6A_000110
heterozygous, not in 100 controls
PubMed: Khateb 2019
,
PubMed: Riera 2017
,
PubMed: Yang 2015
-
-
Germline, Unknown
?, yes
-
-
-
-
LOVD
+?/.
1
-
c.1268delT
r.(?)
p.(Leu423*)
-
likely pathogenic
g.149278067del
g.149898504del
PDE6A c.1268delT (p.Leu423Ter)
-
PDE6A_000110
heterozygous
PubMed: Riera 2019
-
-
In vitro (cloned)
?
-
-
-
-
LOVD
+?/.
5
-
c.1283G>A
r.(?)
p.(Gly428Asp)
ACMG
likely pathogenic, likely pathogenic (recessive), VUS
g.149278050C>T
g.149898487C>T
PDE6A c.1283G>A, p.G428GD
-
PDE6A_000101
-
PubMed: Kim 2019
,
PubMed: Kim 2019
-
-
Germline, Germline/De novo (untested)
?
1/86 cases
-
-
-
Global Variome, with Curator vacancy
,
Jinu Han
+/.
1
-
c.1287G>A
r.(?)
p.(Trp429*)
ACMG
pathogenic
g.149278046C>T
g.149898483C>T
PDE6A c.1287G>A, p.(Trp429*), c.1054C>T, p.(Gln352*)
-
PDE6A_000136
-
PubMed: Jespersgaar 2019
-
-
Germline
?
-
-
-
-
LOVD
+/.
1
-
c.1336del
r.(?)
p.(Arg446GlyfsTer8)
-
pathogenic (recessive)
g.149277997del
g.149898434del
1336delA
-
PDE6A_000118
-
PubMed: Consugar 2015
-
-
Germline
-
-
-
-
-
LOVD
-/., ?/.
2
-
c.1349T>C
r.(?)
p.(Phe450Ser)
-
benign, VUS
g.149277984A>G
g.149898421A>G
-
-
PDE6A_000064
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
,
PubMed: Li 2022
,
Journal: Li 2022
-
rs185588836
Germline, Germline/De novo (untested)
-
3/1204 cases with retinitis pigmentosa
-
-
-
Johan den Dunnen
,
Yoshito Koyanagi
+/.
3
10
c.1351C>T
r.(?)
p.(Gln451*)
ACMG
pathogenic
g.149277982G>A
g.149898419G>A
PDE6A c.1351C>T, p.(Gln451*)
-
PDE6A_000182
heterozygous, heterozygous; one unaffected brother - het c.1724T>C , p.(Leu575Pro)
PubMed: Khateb 2019
-
-
Germline
yes
-
-
-
-
LOVD
+/., +?/.
2
-
c.1359_1361delinsCC
r.(?)
p.(Val454Glnfs*5)
ACMG
likely pathogenic, pathogenic
g.149277972_149277974delinsGG
g.149898409_149898411delinsGG
PDE6A c.1359_1361delinsCC/ p.V454Qfs*5,
1 more item
-
PDE6A_000162
HGMD: none; heterozygous, solved, compound heterozygous
PubMed: Kuehlewein 2020
,
PubMed: Weisschuh 2020
-
-
Germline, Unknown
?, yes
-
-
-
-
LOVD
10 per page
25 per page
50 per page
100 per page
Legend
How to query
« First
Prev
1
2
3
Next
Last »
Screenscraping/webscraping (downloading large amounts of data using scripts) is strictly prohibited.
Use our
APIs
to retrieve data.
Powered by
LOVD v.3.0
Build 30b
LOVD software ©2004-2024
Leiden University Medical Center
Database contents © by their respective submitters and curators