Global Variome shared LOVD
PRPH2 (peripherin 2 (retinal degeneration, slow))
LOVD v.3.0 Build 30b [
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Curator:
Manon Peeters
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All variants in the PRPH2 gene
This database is one of the
"Eye disease"
gene variant databases.
The variants shown are described using the NM_000322.4 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.
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
Allele
: On which allele is the variant located? Does not necessarily imply inheritance! 'Paternal' (confirmed or inferred), 'Maternal' (confirmed or inferred), 'Parent #1' or #2 for compound heterozygosity without having screened the parents, 'Unknown' for heterozygosity without having screened the parents, 'Both' for homozygozity.
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%)
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Operator
Column type
Example
Matches
Text
Arg
all entries containing 'Arg'
space
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|
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!
Text
!fs
all entries not containing 'fs'
^
Text
^p.(Arg
all entries beginning with 'p.(Arg'
$
Text
Ser)$
all entries ending with 'Ser)'
=""
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=""
all entries with this field empty
=""
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="p.0"
all entries exactly matching 'p.0'
!=""
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!=""
all entries with this field not empty
!=""
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!="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
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all entries containing 'Asian', 'asian', including 'Caucasian', 'caucasian', etc.
Asian !Caucasian
all entries containing 'Asian' but not containing 'Caucasian'
Asian|African !Caucasian
all entries containing 'Asian' or 'African', but not containing 'Caucasian'
"South Asian"
all entries containing 'South Asian', but not containing 'South East Asian'
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2482 entries on 25 pages. Showing entries 1 - 100.
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Effect
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.-281_(581+1_582-1){0}
r.?
p.?
-
pathogenic (dominant)
g.(42672350_42689491)_(42690353_?)del
g.(42704612_42721753)_(42722615_?)del
c.1-?_581+?del
-
PRPH2_000152
-
PubMed: Boulanger-Scemama 2015
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
-
Germline
yes
1/96
-
-
-
Manon Peeters
+/+
1
c.-281_(581+1_582-1){0}
r.?
p.?
-
pathogenic (dominant)
g.(42672350_42689491)_(42690353_?)del
g.(42704612_42721753)_(42722615_?)del
c.1-?_581+?del
-
PRPH2_000152
-
PubMed: Boulanger-Scemama 2015
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
-
Germline
yes
1/96
-
-
-
Manon Peeters
-/.
-
c.-11A>C
r.(?)
p.(=)
-
benign
g.42690083T>G
g.42722345T>G
-
-
PRPH2_000042
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/.
1
c.-11A>C
r.(?)
p.(=)
ACMG
benign
g.42690083T>G
g.42722345T>G
-
-
PRPH2_000042
ACMG BS1, BS4, BP4
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
rs114062933
SUMMARY record
-
-
-
-
-
LOVD
?/-
1
c.-11A>C
r.(?)
p.?
-
VUS
g.42690083T>G
g.42722345T>G
-
-
PRPH2_000042
-
PubMed: Boon 2007
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
rs114062933
Germline
-
1/15
-
-
-
Manon Peeters
-?/.
1
c.-11A>C
r.(=)
p.(=)
-
likely benign (dominant)
g.42690083T>G
-
c.-11A>C
-
PRPH2_000042
-
PubMed: Anasagasti-2013
-
rs114062933
Germline
yes
0.03
-
-
-
LOVD
+?/.
_1_1i
c.-286_(581+1_582-1){0}
r.(?)
p.?
-
likely pathogenic (dominant)
g.(42672350_42689491)_(42690353_?)del
g.(42704612_42721753)_(42722615_?)del
c.1-c581+?del
-
PRPH2_000152
-
PubMed: Boulanger-Scemama 2015
,
PubMed: Boulanger-Scemama 2019
-
-
Germline
-
-
-
-
-
Global Variome, with Curator vacancy
+/.
_1_1i
c.-286_(581+1_582-1){0}
r.0?
p.0?
ACMG
pathogenic
g.(42672350_42689491)_(42690353_?)del
g.(42704612_42721753)_(42722615_?)del
-
-
PRPH2_000152
ACMG PVS1, PM1, PM2
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
-
SUMMARY record
-
-
-
-
-
LOVD
+?/.
2i_3_
c.828+1797_*1700{0}ins(?)
r.?
p.?
-
likely pathogenic
g.42663546_42670306delinsN[?]
-
del ex3 insertion ALU Chr6:42663546-42670306del_insALU
-
PRPH2_000131
-
PubMed: Stone 2017
-
-
Germline
-
-
-
-
-
LOVD
+/.
1
c.1A>T
r.(?)
p.(Met1?)
ACMG
pathogenic
g.42690072T>A
g.42722334T>A
-
-
PRPH2_000295
ACMG PVS1, PM1, PM2, PP5,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
-
SUMMARY record
-
-
-
-
-
LOVD
+/+
1
c.1A>T
r.(?)
p.(Met1?)
-
pathogenic (dominant)
g.42690072T>A
g.42722334T>A
Met1Leu
-
PRPH2_000295
-
PubMed: Zhao 2015
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
-
Unknown
-
1/82
-
-
-
Manon Peeters
+/.
1
c.2T>C
r.(?)
p.(Met1?)
-
pathogenic (dominant)
g.42690071A>G
g.42722333A>G
-
-
PRPH2_000128
-
PubMed: Birtel 2018
-
-
Germline
-
-
-
-
-
LOVD
+/.
1
c.2T>C
r.(?)
p.(Met1?)
-
pathogenic (dominant)
g.42690071A>G
g.42722333A>G
-
-
PRPH2_000128
-
PubMed: Birtel 2018
-
-
Germline
-
-
-
-
-
LOVD
+/.
1
c.2T>C
r.(?)
p.(Met1?)
-
pathogenic (dominant)
g.42690071A>G
g.42722333A>G
-
-
PRPH2_000128
-
PubMed: Birtel 2018
-
-
Germline
-
-
-
-
-
LOVD
+/.
1
c.2T>C
r.(?)
p.(Met1?)
ACMG
pathogenic
g.42690071A>G
g.42722333A>G
-
-
PRPH2_000128
ACMG PVS1, PM1, PM2, PM5, PP5
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
rs121918565
SUMMARY record
-
-
-
-
-
LOVD
+/+
1
c.2T>C
r.(?)
p.(Met1?)
-
pathogenic (dominant)
g.42690071A>G
g.42722333A>G
c.2T>C; p.Met1?
-
PRPH2_000128
-
PubMed: Felbor 1997
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
rs121918565
Unknown
-
1/28
-NlaIII
-
-
Manon Peeters
+?/+
1
c.2T>C
r.(?)
p.(Met1?)
-
likely pathogenic (dominant)
g.42690071A>G
g.42722333A>G
c.2T>C; p.M1T
-
PRPH2_000128
-
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
rs121918565
Unknown
-
1/147
-NlaIII
-
-
Manon Peeters
+?/.
1
c.2T>C
r.(?)
p.(Met1?)
-
likely pathogenic
g.42690071A>G
g.42722333A>G
PRPH2 c.2T>C, p.Met1?
-
PRPH2_000128
heterozygous
PubMed: Gliem 2020
-
-
Unknown
?
-
-
-
-
LOVD
+?/.
1
c.2T>C
r.(?)
p.(Met1?)
-
likely pathogenic
g.42690071A>G
g.42722333A>G
PRPH2 c.2T>C, p.Met1?
-
PRPH2_000128
heterozygous
PubMed: Gliem 2020
-
-
Unknown
?
-
-
-
-
LOVD
+?/.
1
c.2T>C
r.(?)
p.(Met1?)
-
likely pathogenic
g.42690071A>G
g.42722333A>G
PRPH2 c.2T>C, Met1?
-
PRPH2_000128
heterozygous
PubMed: Gliem 2020
-
-
Unknown
?
-
-
-
-
LOVD
+?/.
1
c.2T>C
r.(?)
p.(Met1?)
-
likely pathogenic
g.42690071A>G
g.42722333A>G
PRPH2 c.2T>C, p.Met1?
-
PRPH2_000128
heterozygous
PubMed: Gliem 2020
-
-
Unknown
?
-
-
-
-
LOVD
+?/.
1
c.2T>C
r.(?)
p.(Met1?)
-
likely pathogenic
g.42690071A>G
g.42722333A>G
PRPH2 c.2T>C, p.Met1?
-
PRPH2_000128
heterozygous
PubMed: Gliem 2020
-
-
Unknown
?
-
-
-
-
LOVD
+?/.
1
c.4G>T
r.(?)
p.(Ala2Ser)
-
likely pathogenic
g.42690069C>A
g.42722331C>A
-
-
PRPH2_000147
-
PubMed: Ramkumar 2017
-
-
Germline
-
-
-
-
-
LOVD
?/.
1
c.4G>T
r.(?)
p.(Ala2Ser)
ACMG
VUS
g.42690069C>A
g.42722331C>A
-
-
PRPH2_000147
ACMG PM2, PP3; ACMG criteria not strong enough to label as pathogenic
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
-
SUMMARY record
-
-
-
-
-
LOVD
-/.
1
c.13C>T
r.?
p.?
-
benign
g.42690060G>A
-
13C>T
-
PRPH2_000343
8/250 healthy controls
PubMed: Bardak 2016
-
rs361524
Unknown
-
-
-
-
-
LOVD
-/.
1
c.13C>T
r.?
p.?
-
benign
g.42690060G>A
-
13C>T
-
PRPH2_000343
64/250 healthy controls
PubMed: Bardak 2016
-
rs361524
Unknown
-
-
-
-
-
LOVD
?/.
-
c.37C>T
r.(?)
p.(Arg13Trp)
-
VUS
g.42690036G>A
g.42722298G>A
-
-
PRPH2_000070
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
rs61754402
Germline
-
1/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
+/.
1
c.37C>T
r.(?)
p.(Arg13Trp)
-
pathogenic
g.42690036G>A
-
-
-
PRPH2_000070
-
PubMed: Sohocki 2001
-
-
Germline
-
-
-
-
-
Julia Lopez
?/.
1
c.37C>T
r.(?)
p.(Arg13Trp)
ACMG
VUS
g.42690036G>A
g.42722298G>A
-
-
PRPH2_000070
ACMG PM5, PP3; ACMG criteria not strong enough to label as pathogenic
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
rs61754402
SUMMARY record
-
-
-
-
-
LOVD
+/?
1
c.37C>T
r.(?)
p.(Arg13Trp)
-
pathogenic (dominant)
g.42690036G>A
g.42722298G>A
c.37C>T; p.(R13W)
-
PRPH2_000070
-
PubMed: Zaneveld 2015
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
rs61754402
Unknown
-
1/88
AciI-
-
-
Manon Peeters
+?/.
1
c.38G>A
r.(?)
p.(Arg13Gln)
ACMG
likely pathogenic
g.42690035C>T
g.42722297C>T
-
-
PRPH2_000294
ACMG PS4, PM5, PP3
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
-
SUMMARY record
-
-
-
-
-
LOVD
+?/+?
1
c.38G>A
r.(?)
p.(Arg13Gln)
-
likely pathogenic (dominant)
g.42690035C>T
g.42722297C>T
c.38G>A; p.Arg13Gln
-
PRPH2_000294
-
PubMed: Sun 2015
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
-
Germline
-
1/298
-
-
-
Manon Peeters
?/.
-
c.38G>A
r.(?)
p.(Arg13Gln)
ACMG
VUS
g.42690035C>T
g.42722297C>T
-
-
PRPH2_000294
ACMG PM2, PM5_SUPPORTING, PP2, PP5
PubMed: Weisschuh 2024
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
-
c.40_41insGT
r.(?)
p.(Val14Glyfs*11)
-
likely pathogenic (recessive)
g.42690032_42690033insAC
g.42722294_42722295insAC
c.40_41insGT
-
PRPH2_000095
-
PubMed: Holtan 2020
-
-
Germline
-
1/899 cases
-
-
-
Global Variome, with Curator vacancy
?/.
-
c.44A>G
r.(?)
p.(Lys15Arg)
-
VUS
g.42690029T>C
g.42722291T>C
-
-
PRPH2_000069
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
rs555112175
Germline
-
1/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
?/.
1
c.44A>G
r.(?)
p.(Lys15Arg)
ACMG
VUS
g.42690029T>C
g.42722291T>C
-
-
PRPH2_000069
ACMG PS4; ACMG criteria not strong enough to label as pathogenic
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
-
SUMMARY record
-
-
-
-
-
LOVD
?/?
1
c.44A>G
r.(?)
p.(Lys15Arg)
-
VUS
g.42690029T>C
g.42722291T>C
c.44A>G; p.K15R
-
PRPH2_000069
-
PubMed: Arai 2015
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
-
Germline
-
1/349
-
-
-
Manon Peeters
+/.
-
c.63G>A
r.(?)
p.(Trp21Ter)
-
pathogenic
g.42690010C>T
g.42722272C>T
-
-
PRPH2_000041
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
c.63G>A
r.(?)
p.(Trp21*)
ACMG
pathogenic
g.42690010C>T
g.42722272C>T
-
-
PRPH2_000041
ACMG PVS1, PM2, PP1
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
-
SUMMARY record
-
-
-
-
-
LOVD
+/+
1
c.63G>A
r.(?)
p.(Trp21*)
-
pathogenic (dominant)
g.42690010C>T
g.42722272C>T
c.63G>A; p.W21X
-
PRPH2_000041
-
PubMed: Boon 2007
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
-
Germline
yes
2/30
-
-
-
Manon Peeters
+/+
1
c.63G>A
r.(?)
p.(Trp21*)
-
pathogenic (dominant)
g.42690010C>T
g.42722272C>T
c.63G>A; p.W21X
-
PRPH2_000041
-
PubMed: Boon 2007
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
-
Germline
yes
2/30
-
-
-
Manon Peeters
+/+
1
c.63G>A
r.(?)
p.(Trp21*)
-
pathogenic (dominant)
g.42690010C>T
g.42722272C>T
Chr6(GRCh37):g.42690010C>T; p.(Trp21*)
-
PRPH2_000041
-
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
-
Germline
-
3/147
-
-
-
Manon Peeters
+/+
1
c.63G>A
r.(?)
p.(Trp21*)
-
pathogenic (dominant)
g.42690010C>T
g.42722272C>T
Chr6(GRCh37):g.42690010C>T; p.(Trp21*)
-
PRPH2_000041
-
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
-
Germline
-
3/147
-
-
-
Manon Peeters
+/+
1
c.63G>A
r.(?)
p.(Trp21*)
-
pathogenic (dominant)
g.42690010C>T
g.42722272C>T
Chr6(GRCh37):g.42690010C>T; p.(Trp21*)
-
PRPH2_000041
-
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
-
Germline
yes
3/147
-
-
-
Manon Peeters
+/+
1
c.63G>A
r.(?)
p.(Trp21*)
-
pathogenic (dominant)
g.42690010C>T
g.42722272C>T
Chr6(GRCh37):g.42690010C>T; p.(Trp21*)
-
PRPH2_000041
-
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
-
Germline
yes
3/147
-
-
-
Manon Peeters
+/.
1
c.63G>A
r.(?)
p.(Trp21Ter)
ACMG
pathogenic
g.42690010C>T
g.42722272C>T
-
-
PRPH2_000041
-
PubMed: Hitti-Malin 2024
,
Journal: Hitti-Malin 2024
-
-
Germline
-
-
-
-
-
Rebekkah Hitti-Malin
?/.
-
c.68del
r.(?)
p.(Met23Argfs*15)
ACMG
likely pathogenic (dominant)
g.42690005del
-
-
-
PRPH2_000352
-
PubMed: Bianco 2023
-
-
Unknown
-
-
-
-
-
Lorenzo Bianco
?/.
-
c.68del
r.(?)
p.(Met23Argfs*15)
ACMG
likely pathogenic (dominant)
g.42690005del
-
-
-
PRPH2_000352
-
PubMed: Bianco 2023
-
-
Unknown
-
-
-
-
-
Lorenzo Bianco
+/.
1
c.73_74del
r.(?)
p.(Trp25Valfs*19)
ACMG
pathogenic
g.42689999_42690000del
g.42722261_42722262del
-
-
PRPH2_000293
ACMG PVS1, PM1, PM2
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
rs61755765
SUMMARY record
-
-
-
-
-
LOVD
+?/+
1
c.73_74del
r.(?)
p.(Trp25Valfs*19)
-
likely pathogenic (dominant)
g.42689999_42690000del
g.42722261_42722262del
2-bp deletion codon 25
-
PRPH2_000293
-
PubMed: Kajiwara 1993
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
rs61755765
Germline
yes
2/6
+MaeII
-
-
Manon Peeters
+?/+
1
c.73_74del
r.(?)
p.(Trp25Valfs*19)
-
likely pathogenic (dominant)
g.42689999_42690000del
g.42722261_42722262del
2-bp deletion codon 25
-
PRPH2_000293
-
PubMed: Kajiwara 1993
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
rs61755765
Germline
yes
2/6
+MaeII
-
-
Manon Peeters
+/.
1
c.74_77del
r.(?)
p.(Trp25Serfs*12)
ACMG
pathogenic
g.42689997_42690000del
g.42722259_42722262del
-
-
PRPH2_000291
ACMG PVS1, PM1, PM2, PP1
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
-
SUMMARY record
-
-
-
-
-
LOVD
+?/+
1
c.74_77del
r.(?)
p.(Trp25Serfs*12)
-
likely pathogenic (dominant)
g.42689997_42690000del
g.42722259_42722262del
c.74_77delGGTT, p.W25SfsX12
-
PRPH2_000291
-
PubMed: Maertz 2015
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
-
Germline
yes
1/3
-
-
-
Manon Peeters
?/.
1
c.75G>C
r.(?)
p.(Trp25Cys)
ACMG
VUS
g.42689998C>G
g.42722260C>G
-
-
PRPH2_000292
ACMG PP3; ACMG criteria not strong enough to label as pathogenic
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
-
SUMMARY record
-
-
-
-
-
LOVD
?/?
1
c.75G>C
r.(?)
p.(Trp25Cys)
-
VUS
g.42689998C>G
g.42722260C>G
c.75G>C; p.Trp25Cys
-
PRPH2_000292
-
PubMed: Reeves 2020
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
-
Unknown
-
1/187
-
-
-
Manon Peeters
+?/?
1
c.75G>C
r.(?)
p.(Trp25Cys)
-
likely pathogenic
g.42689998C>G
g.42722260C>G
p.W25C
-
PRPH2_000292
-
PubMed: Strom 2012
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
-
Unknown
-
1/9
-
-
-
Manon Peeters
+?/?
1
c.75G>C
r.(?)
p.(Trp25Cys)
-
likely pathogenic
g.42689998C>G
g.42722260C>G
p.W25C
-
PRPH2_000292
-
PubMed: Strom 2012
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
-
Unknown
-
1/9
-
-
-
Manon Peeters
+?/.
1
c.80C>T
r.(?)
p.(Ser27Phe)
ACMG
likely pathogenic
g.42689993G>A
g.42722255G>A
-
-
PRPH2_000290
ACMG PS4, PP1, PP3
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
rs61755766
SUMMARY record
-
-
-
-
-
LOVD
+?/+?
1
c.80C>T
r.(?)
p.(Ser27Phe)
-
likely pathogenic (dominant)
g.42689993G>A
g.42722255G>A
C>T transition in the second nucleotide of codon 27; p.Phe27Ser
-
PRPH2_000290
-
PubMed: Fishman 1997
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
rs61755766
Germline
yes
3/7
-
-
-
Manon Peeters
+?/+?
1
c.80C>T
r.(?)
p.(Ser27Phe)
-
likely pathogenic (dominant)
g.42689993G>A
g.42722255G>A
C>T transition in the second nucleotide of codon 27; p.Phe27Ser
-
PRPH2_000290
-
PubMed: Fishman 1997
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
rs61755766
Germline
yes
3/7
-
-
-
Manon Peeters
+?/+?
1
c.80C>T
r.(?)
p.(Ser27Phe)
-
likely pathogenic (dominant)
g.42689993G>A
g.42722255G>A
C>T transition in the second nucleotide of codon 27; p.Phe27Ser
-
PRPH2_000290
-
PubMed: Fishman 1997
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
rs61755766
Germline
yes
3/7
-
-
-
Manon Peeters
?/.
-
c.94A>G
r.(?)
p.(Ile32Val)
-
VUS
g.42689979T>C
g.42722241T>C
-
-
PRPH2_000068
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
rs61755767
Germline
-
1/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
?/.
-
c.94A>G
r.(?)
p.(Ile32Val)
-
VUS
g.42689979T>C
g.42722241T>C
PRPH2(NM_000322.4):c.94A>G (p.I32V), PRPH2(NM_000322.5):c.94A>G (p.I32V)
-
PRPH2_000068
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
c.94A>G
r.(?)
p.(Ile32Val)
ACMG
likely benign
g.42689979T>C
g.42722241T>C
-
-
PRPH2_000068
ACMG BP4
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
rs61755767
SUMMARY record
-
-
-
-
-
LOVD
?/-?
1
c.94A>G
r.(?)
p.(Ile32Val)
-
VUS
g.42689979T>C
g.42722241T>C
c.94A>G; p.Ile32Val
-
PRPH2_000068
-
PubMed: Reeves 2020
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
rs61755767
Unknown
-
2/187
-SfaNI
-
-
Manon Peeters
?/-?
1
c.94A>G
r.(?)
p.(Ile32Val)
-
VUS
g.42689979T>C
g.42722241T>C
c.94A>G; p.Ile32Val
-
PRPH2_000068
-
PubMed: Reeves 2020
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
rs61755767
Unknown
-
2/187
-SfaNI
-
-
Manon Peeters
-/-?
1
c.94A>G
r.(?)
p.(Ile32Val)
-
benign
g.42689979T>C
g.42722241T>C
c.A94G; p.I32V
-
PRPH2_000068
-
PubMed: de Breuk 2020
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
rs61755767
Unknown
-
1/4740
-SfaNI
-
-
Manon Peeters
?/-?
1
c.94A>G
r.(?)
p.(Ile32Val)
-
VUS
g.42689979T>C
g.42722241T>C
c.94A>G; p.Ile32Val
-
PRPH2_000068
-
PubMed: Daftarian 2019
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
rs61755767
Germline
yes
4/4
-SfaNI
-
-
Manon Peeters
?/-?
1
c.94A>G
r.(?)
p.(Ile32Val)
-
VUS
g.42689979T>C
g.42722241T>C
c.94A>G; p.Ile32Val
-
PRPH2_000068
-
PubMed: Daftarian 2019
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
rs61755767
Germline
yes
4/4
-SfaNI
-
-
Manon Peeters
?/-?
1
c.94A>G
r.(?)
p.(Ile32Val)
-
VUS
g.42689979T>C
g.42722241T>C
c.94A>G; p.Ile32Val
-
PRPH2_000068
-
PubMed: Daftarian 2019
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
rs61755767
Germline
yes
4/4
-SfaNI
-
-
Manon Peeters
?/-?
1
c.94A>G
r.(?)
p.(Ile32Val)
-
VUS
g.42689979T>C
g.42722241T>C
c.94A>G; p.Ile32Val
-
PRPH2_000068
-
PubMed: Daftarian 2019
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
rs61755767
Germline
yes
4/4
-SfaNI
-
-
Manon Peeters
+?/-?
1
c.94A>G
r.(?)
p.(Ile32Val)
-
likely pathogenic (dominant)
g.42689979T>C
g.42722241T>C
c.94A>G; p.Ile32Val
-
PRPH2_000068
-
PubMed: Barbazetto 2007
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
rs61755767
Unknown
-
1/28
-SfaNI
-
-
Manon Peeters
?/.
-
c.94A>G
r.(?)
p.(Ile32Val)
-
VUS
g.42689979T>C
-
-
-
PRPH2_000068
-
-
-
rs61755767
CLASSIFICATION record
-
-
-
-
-
MobiDetails
+?/.
1
c.94A>G
r.(?)
p.(Ile32Val)
-
likely pathogenic
g.42689979T>C
-
c.94A>G
-
PRPH2_000068
-
PubMed: Maggi_2021
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
1
c.94A>G
r.(?)
p.(Ile32Val)
-
likely pathogenic
g.42689979T>C
-
c.94A>G
-
PRPH2_000068
-
PubMed: Panneman 2023
-
-
Unknown
-
-
-
-
-
Daan Panneman
-?/.
-
c.94A>G
r.(?)
p.(Ile32Val)
-
likely benign
g.42689979T>C
-
PRPH2(NM_000322.4):c.94A>G (p.I32V), PRPH2(NM_000322.5):c.94A>G (p.I32V)
-
PRPH2_000068
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
1
c.96dup
r.(?)
p.(Ile33Hisfs*12)
ACMG
pathogenic
g.42689977dup
g.42722239dup
-
-
PRPH2_000289
ACMG PVS1, PM1, PM2
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
rs61755768
SUMMARY record
-
-
-
-
-
LOVD
+?/+
1
c.96dup
r.(?)
p.(Ile33Hisfs*12)
-
likely pathogenic (dominant)
g.42689977dup
g.42722239dup
1-bp insertion at codon 32
-
PRPH2_000289
-
PubMed: Jacobson 1996
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
rs61755768
Germline
yes
3/27
-
-
-
Manon Peeters
+?/+
1
c.96dup
r.(?)
p.(Ile33Hisfs*12)
-
likely pathogenic (dominant)
g.42689977dup
g.42722239dup
1-bp insertion at codon 32
-
PRPH2_000289
-
PubMed: Jacobson 1996
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
rs61755768
Germline
yes
3/27
-
-
-
Manon Peeters
+?/+
1
c.96dup
r.(?)
p.(Ile33Hisfs*12)
-
likely pathogenic (dominant)
g.42689977dup
g.42722239dup
1-bp insertion at codon 32
-
PRPH2_000289
-
PubMed: Jacobson 1996
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
rs61755768
Germline
yes
3/27
-
-
-
Manon Peeters
+/+
1
c.96dup
r.(?)
p.(Ile33Hisfs*12)
-
pathogenic (dominant)
g.42689977dup
g.42722239dup
c.96dupC; p.Ile33Hisfs*12
-
PRPH2_000289
-
PubMed: Palma 2019
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
rs61755768
Germline
yes
1/3 cases
-
-
-
Manon Peeters
+?/.
1
c.112G>T
r.(?)
p.(Gly38*)
ACMG
likely pathogenic
g.42689961C>A
g.42722223C>A
-
-
PRPH2_000287
ACMG PVS1, PM2
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
-
SUMMARY record
-
-
-
-
-
LOVD
+?/+?
1
c.112G>T
r.(?)
p.(Gly38*)
-
likely pathogenic (dominant)
g.42689961C>A
g.42722223C>A
c.112G>T; p.G38*
-
PRPH2_000287
-
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
-
Germline
-
1/147
-
-
-
Manon Peeters
+/.
1
c.113del
r.(?)
p.(Gly38Aspfs*4)
ACMG
pathogenic
g.42689961del
g.42722223del
-
-
PRPH2_000288
ACMG PVS1, PM1, PM12,PP1
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
rs61755769
SUMMARY record
-
-
-
-
-
LOVD
+/+
1
c.113del
r.(?)
p.(Gly38Aspfs*4)
-
pathogenic (dominant)
g.42689961del
g.42722223del
c.113delG; p.Gly38Aspfs*4
-
PRPH2_000288
-
PubMed: Reeves 2020
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
rs61755769
Unknown
-
1/187
-
-
-
Manon Peeters
+/+
1
c.113del
r.(?)
p.(Gly38Aspfs*4)
-
pathogenic (dominant)
g.42689961del
g.42722223del
Guanine deletion at position 112
-
PRPH2_000288
-
PubMed: Moshfeghi 2006
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
rs61755769
Germline
yes
2/3
-
-
-
Manon Peeters
+/+
1
c.113del
r.(?)
p.(Gly38Aspfs*4)
-
pathogenic (dominant)
g.42689961del
g.42722223del
Guanine deletion at position 112
-
PRPH2_000288
-
PubMed: Moshfeghi 2006
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
rs61755769
Germline
yes
2/3
-
-
-
Manon Peeters
+/+
1
c.113del
r.(?)
p.(Gly38Aspfs*4)
-
pathogenic (dominant)
g.42689961del
g.42722223del
Guanine deletion at nucleotide position 112 leading to a premature termination of the gene product at amino acid 38.
-
PRPH2_000288
-
PubMed: Yang 2003
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
rs61755769
Germline
yes
8/12
-
-
-
Manon Peeters
+/+
1
c.113del
r.(?)
p.(Gly38Aspfs*4)
-
pathogenic (dominant)
g.42689961del
g.42722223del
Guanine deletion at nucleotide position 112 leading to a premature termination of the gene product at amino acid 38.
-
PRPH2_000288
-
PubMed: Yang 2003
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
rs61755769
Germline
yes
8/12
-
-
-
Manon Peeters
+/+
1
c.113del
r.(?)
p.(Gly38Aspfs*4)
-
pathogenic (dominant)
g.42689961del
g.42722223del
Guanine deletion at nucleotide position 112 leading to a premature termination of the gene product at amino acid 38.
-
PRPH2_000288
-
PubMed: Yang 2003
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
rs61755769
Germline
yes
8/12
-
-
-
Manon Peeters
+/+
1
c.113del
r.(?)
p.(Gly38Aspfs*4)
-
pathogenic (dominant)
g.42689961del
g.42722223del
Guanine deletion at nucleotide position 112 leading to a premature termination of the gene product at amino acid 38.
-
PRPH2_000288
-
PubMed: Yang 2003
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
rs61755769
Germline
yes
8/12
-
-
-
Manon Peeters
+/+
1
c.113del
r.(?)
p.(Gly38Aspfs*4)
-
pathogenic (dominant)
g.42689961del
g.42722223del
Guanine deletion at nucleotide position 112 leading to a premature termination of the gene product at amino acid 38.
-
PRPH2_000288
-
PubMed: Yang 2003
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
rs61755769
Germline
yes
8/12
-
-
-
Manon Peeters
+/+
1
c.113del
r.(?)
p.(Gly38Aspfs*4)
-
pathogenic (dominant)
g.42689961del
g.42722223del
Guanine deletion at nucleotide position 112 leading to a premature termination of the gene product at amino acid 38.
-
PRPH2_000288
-
PubMed: Yang 2003
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
rs61755769
Germline
yes
8/12
-
-
-
Manon Peeters
+/+
1
c.113del
r.(?)
p.(Gly38Aspfs*4)
-
pathogenic (dominant)
g.42689961del
g.42722223del
Guanine deletion at nucleotide position 112 leading to a premature termination of the gene product at amino acid 38.
-
PRPH2_000288
-
PubMed: Yang 2003
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
rs61755769
Germline
yes
8/12
-
-
-
Manon Peeters
+/+
1
c.113del
r.(?)
p.(Gly38Aspfs*4)
-
pathogenic (dominant)
g.42689961del
g.42722223del
Guanine deletion at nucleotide position 112 leading to a premature termination of the gene product at amino acid 38.
-
PRPH2_000288
-
PubMed: Yang 2003
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
rs61755769
Germline
yes
8/12
-
-
-
Manon Peeters
+/.
-
c.113del
r.(?)
p.(Gly38Aspfs*4)
-
pathogenic
g.42689961del
g.42722223del
PRPH2 c.113del, p.Gly38AspfsTer4
-
PRPH2_000288
heterozygous
PubMed: Zampaglione 2020
-
-
Unknown
?
-
-
-
-
LOVD
?/.
1
c.116T>C
r.(?)
p.(Leu39Pro)
ACMG
VUS
g.42689957A>G
g.42722219A>G
-
-
PRPH2_000286
ACMG PM2, PP3; ACMG criteria not strong enough to label as pathogenic
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
-
SUMMARY record
-
-
-
-
-
LOVD
+?/?
1
c.116T>C
r.(?)
p.(Leu39Pro)
-
likely pathogenic (dominant)
g.42689957A>G
g.42722219A>G
p.PRPH2-L39P
-
PRPH2_000286
-
PubMed: Schorderet 2013
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
-
Germline
-
1/23
-
-
-
Manon Peeters
+/.
1
c.116T>C
r.(?)
p.(Leu39Pro)
-
pathogenic
g.42689957A>G
-
p.PRPH2-L39P
-
PRPH2_000286
-
PubMed: Schorderet-2013
-
-
Germline
yes
-
-
-
-
LOVD
?/.
1
c.122T>C
r.(?)
p.(Leu41Pro)
ACMG
VUS
g.42689951A>G
g.42722213A>G
-
-
PRPH2_000285
ACMG PM2, PP3; ACMG criteria not strong enough to label as pathogenic
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
-
SUMMARY record
-
-
-
-
-
LOVD
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