Global Variome shared LOVD
PRPH2 (peripherin 2 (retinal degeneration, slow))
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Curator:
Manon Peeters
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Unique 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.
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%)
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Column type
Example
Matches
Text
Arg
all entries containing 'Arg'
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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"
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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
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>
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.
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all entries containing 'Asian' but not containing 'Caucasian'
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all entries containing 'Asian' or 'African', but not containing 'Caucasian'
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all entries containing 'South Asian', but not containing 'South East Asian'
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370 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
+/+
2
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
-/., -?/., ?/-
4
1
c.-11A>C
r.(=), r.(?)
p.(=), p.?
ACMG
benign, likely benign (dominant), VUS
g.42690083T>G
g.42722345T>G
c.-11A>C
-
PRPH2_000042
ACMG BS1, BS4, BP4, VKGL data sharing initiative Nederland
PubMed: Anasagasti-2013
,
PubMed: Boon 2007
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
1 more item
-
rs114062933
CLASSIFICATION record, Germline, SUMMARY record
yes
0.03, 1/15
-
-
-
VKGL-NL_Nijmegen
,
Manon Peeters
+/., +?/.
2
_1_1i
c.-286_(581+1_582-1){0}
r.(?), r.0?
p.0?, p.?
ACMG
likely pathogenic (dominant), pathogenic
g.(42672350_42689491)_(42690353_?)del
g.(42704612_42721753)_(42722615_?)del
c.1-c581+?del
-
PRPH2_000152
ACMG PVS1, PM1, PM2
PubMed: Boulanger-Scemama 2015
,
PubMed: Boulanger-Scemama 2019
,
1 more item
-
-
Germline, SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
+?/.
1
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
+/+, +/.
2
1
c.1A>T
r.(?)
p.(Met1?)
ACMG
pathogenic, pathogenic (dominant)
g.42690072T>A
g.42722334T>A
Met1Leu
-
PRPH2_000295
ACMG PVS1, PM1, PM2, PP5,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
1 more item
-
-
SUMMARY record, Unknown
-
1/82
-
-
-
Manon Peeters
+/+, +/., +?/+, +?/.
11
1
c.2T>C
r.(?)
p.(Met1?)
ACMG
likely pathogenic, likely pathogenic (dominant), pathogenic, pathogenic (dominant)
g.42690071A>G
g.42722333A>G
c.2T>C; p.M1T, c.2T>C; p.Met1?, PRPH2 c.2T>C, Met1?, PRPH2 c.2T>C, p.Met1?
-
PRPH2_000128
ACMG PVS1, PM1, PM2, PM5, PP5, heterozygous
PubMed: Birtel 2018
,
PubMed: Felbor 1997
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
2 more items
-
rs121918565
Germline, SUMMARY record, Unknown
?
1/147, 1/28
-NlaIII
-
-
Manon Peeters
+?/., ?/.
2
1
c.4G>T
r.(?)
p.(Ala2Ser)
ACMG
likely pathogenic, 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
,
PubMed: Ramkumar 2017
-
-
Germline, SUMMARY record
-
-
-
-
-
LOVD
-/.
2
1
c.13C>T
r.?
p.?
-
benign
g.42690060G>A
-
13C>T
-
PRPH2_000343
64/250 healthy controls, 8/250 healthy controls
PubMed: Bardak 2016
-
rs361524
Unknown
-
-
-
-
-
LOVD
+/., +/?, ?/.
4
1
c.37C>T
r.(?)
p.(Arg13Trp)
ACMG
pathogenic, pathogenic (dominant), VUS
g.42690036G>A
g.42722298G>A
c.37C>T; p.(R13W)
-
PRPH2_000070
ACMG PM5, PP3; ACMG criteria not strong enough to label as pathogenic
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
2 more items
-
rs61754402
Germline, SUMMARY record, Unknown
-
1/1204 cases with retinitis pigmentosa, 1/88
AciI-
-
-
Julia Lopez
,
Yoshito Koyanagi
,
Manon Peeters
+?/+?, +?/., ?/.
3
1
c.38G>A
r.(?)
p.(Arg13Gln)
ACMG
likely pathogenic, likely pathogenic (dominant), VUS
g.42690035C>T
g.42722297C>T
c.38G>A; p.Arg13Gln
-
PRPH2_000294
ACMG PM2, PM5_SUPPORTING, PP2, PP5, ACMG PS4, PM5, PP3
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
PubMed: Weisschuh 2024
,
1 more item
-
-
Germline, SUMMARY record
-
1/298
-
-
-
Johan den Dunnen
,
Manon Peeters
+?/.
1
-
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
?/., ?/?
3
1
c.44A>G
r.(?)
p.(Lys15Arg)
ACMG
VUS
g.42690029T>C
g.42722291T>C
c.44A>G; p.K15R
-
PRPH2_000069
ACMG PS4; ACMG criteria not strong enough to label as pathogenic
PubMed: Arai 2015
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
2 more items
-
rs555112175
Germline, SUMMARY record
-
1/1204 cases with retinitis pigmentosa, 1/349
-
-
-
Yoshito Koyanagi
,
Manon Peeters
+/+, +/.
9
1
c.63G>A
r.(?)
p.(Trp21*), p.(Trp21Ter)
ACMG
pathogenic, pathogenic (dominant)
g.42690010C>T
g.42722272C>T
c.63G>A; p.W21X, Chr6(GRCh37):g.42690010C>T; p.(Trp21*)
-
PRPH2_000041
ACMG PVS1, PM2, PP1, VKGL data sharing initiative Nederland
PubMed: Boon 2007
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
2 more items
-
-
CLASSIFICATION record, Germline, SUMMARY record
yes
2/30, 3/147
-
-
-
VKGL-NL_Nijmegen
,
Manon Peeters
,
Rebekkah Hitti-Malin
?/.
2
-
c.68del
r.(?)
p.(Met23Argfs*15)
ACMG
likely pathogenic (dominant)
g.42690005del
-
-
-
PRPH2_000352
-
PubMed: Bianco 2023
-
-
Unknown
-
-
-
-
-
Lorenzo Bianco
+/., +?/+
3
1
c.73_74del
r.(?)
p.(Trp25Valfs*19)
ACMG
likely pathogenic (dominant), pathogenic
g.42689999_42690000del
g.42722261_42722262del
2-bp deletion codon 25
-
PRPH2_000293
ACMG PVS1, PM1, PM2
PubMed: Kajiwara 1993
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
1 more item
-
rs61755765
Germline, SUMMARY record
yes
2/6
+MaeII
-
-
Manon Peeters
+/., +?/+
2
1
c.74_77del
r.(?)
p.(Trp25Serfs*12)
ACMG
likely pathogenic (dominant), pathogenic
g.42689997_42690000del
g.42722259_42722262del
c.74_77delGGTT, p.W25SfsX12
-
PRPH2_000291
ACMG PVS1, PM1, PM2, PP1
PubMed: Maertz 2015
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
1 more item
-
-
Germline, SUMMARY record
yes
1/3
-
-
-
Manon Peeters
+?/?, ?/., ?/?
4
1
c.75G>C
r.(?)
p.(Trp25Cys)
ACMG
likely pathogenic, VUS
g.42689998C>G
g.42722260C>G
c.75G>C; p.Trp25Cys, p.W25C
-
PRPH2_000292
ACMG PP3; ACMG criteria not strong enough to label as pathogenic
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
2 more items
-
-
SUMMARY record, Unknown
-
1/187, 1/9
-
-
-
Manon Peeters
+?/+?, +?/.
4
1
c.80C>T
r.(?)
p.(Ser27Phe)
ACMG
likely pathogenic, likely pathogenic (dominant)
g.42689993G>A
g.42722255G>A
C>T transition in the second nucleotide of codon 27; p.Phe27Ser
-
PRPH2_000290
ACMG PS4, PP1, PP3
PubMed: Fishman 1997
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
1 more item
-
rs61755766
Germline, SUMMARY record
yes
3/7
-
-
-
Manon Peeters
+?/-?, +?/., -/-?, -?/., ?/-?, ?/.
15
1
c.94A>G
r.(?)
p.(Ile32Val)
ACMG
benign, likely benign, likely pathogenic, likely pathogenic (dominant), VUS
g.42689979T>C
g.42722241T>C
c.94A>G, c.94A>G; p.Ile32Val, c.A94G; p.I32V,
1 more item
-
PRPH2_000068
ACMG BP4, VKGL data sharing initiative Nederland
PubMed: Barbazetto 2007
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
PubMed: Maggi_2021
,
6 more items
-
rs61755767
CLASSIFICATION record, Germline, SUMMARY record, Unknown
yes
1/1204 cases with retinitis pigmentosa, 1/28, 1/4740, 2/187, 4/4
-SfaNI
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
,
Yoshito Koyanagi
,
MobiDetails
,
Manon Peeters
,
Daan Panneman
+/+, +/., +?/+
5
1
c.96dup
r.(?)
p.(Ile33Hisfs*12)
ACMG
likely pathogenic (dominant), pathogenic, pathogenic (dominant)
g.42689977dup
g.42722239dup
1-bp insertion at codon 32, c.96dupC; p.Ile33Hisfs*12
-
PRPH2_000289
ACMG PVS1, PM1, PM2
PubMed: Jacobson 1996
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
2 more items
-
rs61755768
Germline, SUMMARY record
yes
1/3 cases, 3/27
-
-
-
Manon Peeters
+?/+?, +?/.
2
1
c.112G>T
r.(?)
p.(Gly38*)
ACMG
likely pathogenic, likely pathogenic (dominant)
g.42689961C>A
g.42722223C>A
c.112G>T; p.G38*
-
PRPH2_000287
ACMG PVS1, PM2
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
-
Germline, SUMMARY record
-
1/147
-
-
-
Manon Peeters
+/+, +/.
13
1
c.113del
r.(?)
p.(Gly38Aspfs*4)
ACMG
pathogenic, pathogenic (dominant)
g.42689961del
g.42722223del
c.113delG; p.Gly38Aspfs*4, Guanine deletion at position 112, PRPH2 c.113del, p.Gly38AspfsTer4,
1 more item
-
PRPH2_000288
ACMG PVS1, PM1, PM12,PP1, heterozygous
PubMed: Moshfeghi 2006
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
PubMed: Zampaglione 2020
,
3 more items
-
rs61755769
Germline, SUMMARY record, Unknown
?, yes
1/187, 2/3, 8/12
-
-
-
Manon Peeters
+/., +?/?, ?/.
3
1
c.116T>C
r.(?)
p.(Leu39Pro)
ACMG
likely pathogenic (dominant), pathogenic, VUS
g.42689957A>G
g.42722219A>G
p.PRPH2-L39P
-
PRPH2_000286
ACMG PM2, PP3; ACMG criteria not strong enough to label as pathogenic
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
PubMed: Schorderet-2013
,
1 more item
-
-
Germline, SUMMARY record
yes
1/23
-
-
-
Manon Peeters
?/., ?/?
3
1
c.122T>C
r.(?)
p.(Leu41Pro)
ACMG
VUS
g.42689951A>G
g.42722213A>G
Chr6(GRCh37):g.42689951A>G; p.(Leu41Pro)
-
PRPH2_000285
ACMG PM2, PP3; ACMG criteria not strong enough to label as pathogenic
PubMed: Bianco 2023
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
-
Germline, SUMMARY record, Unknown
-
1/147
-
-
-
Manon Peeters
,
Lorenzo Bianco
+/., +?/., +?/?, -/., -?/., ?/., ?/?
9
1
c.133C>T
r.(?)
p.(Leu45Phe)
ACMG
benign, likely benign, likely pathogenic, likely pathogenic (dominant), pathogenic, VUS
g.42689940G>A
g.42722202G>A
c133C>T; p.Leu45Phe, C133T; L45F, Chr.6: 42666061:C > 42666061:T, p.L45F (c133C>T),
3 more items
-
PRPH2_000079
ACMG PP3; ACMG criteria not strong enough to label as pathogenic, Common Caribbean variant,
2 more items
PubMed: Abouelhoda 2016
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
PubMed: Habibi 2020
,
6 more items
-
rs61755770
CLASSIFICATION record, De novo, Germline, SUMMARY record
no, yes
1/23, 1/45, 1/4740
+ApoI
-
-
Julia Lopez
,
VKGL-NL_AMC
,
Manon Peeters
+/+, +/., +?/+, +?/., ?/+, ?/.
46
1
c.136C>T
r.(?)
p.(Arg46*), p.(Arg46Ter)
ACMG
likely pathogenic, likely pathogenic (dominant), pathogenic, pathogenic (dominant), VUS,
1 more item
g.42689937G>A
g.42722199G>A
C-to-T transition (CGA for TGA) at codon 46, C-to-T transition at codon 46 (CGA—TGA), c.136C>T,
10 more items
-
PRPH2_000067
ACMG PVS1, PS4, heterozygous, solved, heterozygous, VKGL data sharing initiative Nederland
PubMed: Abu-Safieh 2013
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
PubMed: Abu-Safieh-2013
,
22 more items
-
rs61755771
CLASSIFICATION record, De novo, Germline, SUMMARY record, Unknown
?, no, yes
1/147, 1/160, 2/1204 cases with retinitis pigmentosa, 2/5, 2/63, 2/7, 3/187, 3/310 cases, 4/24, 4/45
-
-
-
Julia Lopez
,
VKGL-NL_Nijmegen
,
VKGL-NL_AMC
,
Yoshito Koyanagi
,
Manon Peeters
,
Lorenzo Bianco
+?/.
1
-
c.144G>T
r.(?)
p.(Arg48Ser)
-
likely pathogenic
g.42689929C>A
g.42722191C>A
PRPH2 c.144G>T, p.Arg48Ser
-
PRPH2_000329
heterozygous
PubMed: Liu 2020
-
-
Germline/De novo (untested)
?
1/64
-
-
-
LOVD
+/., +?/., ?/., ?/?
7
1
c.148G>C
r.(?)
p.(Asp50His)
ACMG
likely pathogenic, pathogenic (dominant), VUS
g.42689925C>G
g.42722187C>G
c.148G>C, D50H
-
PRPH2_000112
ACMG PM2, PP1, PP3; ACMG criteria not strong enough to label as pathogenic
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
PubMed: Zhou 2018
,
PubMed: Zhou-2011
,
1 more item
-
-
Germline, SUMMARY record, Unknown
yes
4/12
-
-
-
Manon Peeters
+/+, +/., ?/+
9
1
c.163del
r.(?)
p.(Ser55Leufs*10)
ACMG
pathogenic, pathogenic (dominant), VUS
g.42689911del
g.42722173del
c.163delT; p.Asn54fsX9, c.163delT; p.S55Lfs*10, c.163delT; p.Ser55Leufs*10
-
PRPH2_000284
ACMG PVS1, PM2, PP1
PubMed: Boon 2007
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
3 more items
-
rs62645886
Germline, SUMMARY record, Unknown
yes
2/187, 2/39, 4/30
-
-
-
Manon Peeters
+/., +?/.
2
1
c.178del
r.(?)
p.(Val60Cysfs*5)
ACMG
likely pathogenic, pathogenic (dominant)
g.42689895del
g.42722157del
-
-
PRPH2_000127
ACMG PVS1, PM2
PubMed: Birtel 2018
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
-
Germline, SUMMARY record
-
-
-
-
-
LOVD
+?/.
2
-
c.192dup
r.(?)
p.(Ile65AspfsTer112)
-
likely pathogenic
g.42689881dup
g.42722143dup
PRPH2 c.192dup, p.(Ile65AspfsTer112)
-
PRPH2_000336
-
PubMed: Sodi 2021
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
1
1
c.195del
r.(?)
p.(Ile65MetfsTer34)
ACMG
likely pathogenic
g.42689878del
g.42722140del
-
-
PRPH2_000368
-
PubMed: Hitti-Malin 2024
,
Journal: Hitti-Malin 2024
-
-
Germline
-
-
-
-
-
Rebekkah Hitti-Malin
+?/?, ?/.
5
1
c.198_202delinsAAGACACA
r.(?)
p.(Met67_Gly68delinsArgHisArg)
ACMG
likely pathogenic (dominant), VUS
g.42689871_42689875delinsTGTGTCTT
g.42722133_42722137delinsTGTGTCTT
p.67-69 del/ins
-
PRPH2_000281
ACMG PM2, PM4, PP3, BS4; contradictory ACMG categories
PubMed: Jacobson 1996
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
1 more item
-
-
Germline, SUMMARY record
yes
4/27
-
-
-
Manon Peeters
?/., ?/?
2
1
c.199_201del
r.(?)
p.(Met67del)
ACMG
VUS
g.42689873_42689875del
g.42722135_42722137del
?
-
PRPH2_000283
ACMG PM2, PM4, PP3; ACMG criteria not strong enough to label as pathogenic
PubMed: Jacobson 1994
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
1 more item
-
rs61755773
SUMMARY record, Unknown
-
-
-
-
-
Manon Peeters
+?/+?, +?/., ?/+?
5
1
c.202G>C
r.(?)
p.(Gly68Arg)
ACMG
likely pathogenic, likely pathogenic (dominant), VUS
g.42689871C>G
g.42722133C>G
p.G68R, p.Gly68Arg
-
PRPH2_000280
ACMG PS4, PM1, PP3
PubMed: Dryja 1997
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
2 more items
-
rs61755774
Germline, SUMMARY record
yes
1/227 cases
-
-
-
Manon Peeters
+/., ?/+
2
1
c.205del
r.(?)
p.(Val69Cysfs*30)
ACMG
pathogenic, VUS
g.42689872del
g.42722134del
c.205delG; p.Val69Cysfs*30
-
PRPH2_000282
ACMG PVS1, PM1, PM2, PP1
PubMed: Manes 2015
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
1 more item
-
-
Germline, SUMMARY record
yes
1/310 cases
-
-
-
Manon Peeters
+/.
1
1
c.205delG
r.(?)
p.(Val69Cysfs*30)
ACMG
pathogenic
g.42722134del
g.42722134del
PRPH2 c.205delG, p.Val69CysfsX30, heterozygous
-
PRPH2_000314
-
PubMed: Sun 2020
-
-
Unknown
?
-
-
-
-
LOVD
+/+, +/.
2
1
c.209dup
r.(?)
p.(Ser71Ilefs*106)
ACMG
pathogenic, pathogenic (dominant)
g.42689864dup
g.42722126dup
c.209dup / p.S71Ifs*106
-
PRPH2_000279
ACMG PVS1, PM1, PM2
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
-
SUMMARY record, Unknown
-
1/147
-
-
-
Manon Peeters
?/.
1
-
c.215G>A
r.(?)
p.(Cys72Tyr)
ACMG
VUS
g.42689858C>T
g.42722120C>T
-
-
PRPH2_000360
ACMG PM2, PP2
PubMed: Weisschuh 2024
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.229C>G
r.(?)
p.(Leu77Val)
-
VUS
g.42689844G>C
-
PRPH2(NM_000322.5):c.229C>G (p.L77V)
-
PRPH2_000369
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/+, +/.
2
1
c.232_233insT
r.(?)
p.(Ala78Valfs*99)
ACMG
pathogenic, pathogenic (dominant)
g.42689840_42689841insA
g.42722102_42722103insA
c.232GC;c.232_233insT; p.Ala78LeufsX99
-
PRPH2_000278
ACMG PVS1, PM1, PM2, PM6
PubMed: Lim 2009
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
1 more item
-
-
SUMMARY record, Unknown
-
1/76
-
-
-
Manon Peeters
?/.
1
-
c.239A>G
r.(?)
p.(Lys80Arg)
ACMG
VUS
g.42689834T>C
g.42722096T>C
-
-
PRPH2_000359
ACMG PM2, PP2
PubMed: Weisschuh 2024
-
-
Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
+/+?, +?/+?, +?/.
3
1
c.246C>A
r.(?)
p.(Cys82*)
ACMG
likely pathogenic, likely pathogenic (dominant), pathogenic (dominant)
g.42689827G>T
g.42722089G>T
c.246C>A; p.Cys82*, c.246C>A; p.Cys82X
-
PRPH2_000277
ACMG PVS1, PM2
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
2 more items
-
-
SUMMARY record, Unknown
-
1/187, 1/79
-
-
-
Manon Peeters
-/., -?/.
2
-
c.249C>T
r.(?)
p.(Tyr83=)
-
benign, likely benign
g.42689824G>A
g.42722086G>A
PRPH2(NM_000322.4):c.249C>T (p.Y83=), PRPH2(NM_000322.5):c.249C>T (p.Y83=)
-
PRPH2_000016
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
-/.
1
-
c.252C>T
r.(?)
p.(Asp84=)
-
benign
g.42689821G>A
g.42722083G>A
PRPH2(NM_000322.5):c.252C>T (p.D84=)
-
PRPH2_000046
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/., ?/?
2
1
c.253G>A
r.(?)
p.(Ala85Thr)
ACMG
VUS
g.42689820C>T
g.42722082C>T
Chr6(GRCh37):g.42689820C>T; p.(Ala85Thr)
-
PRPH2_000276
ACMG PS4, BP4; contradictory ACMG categories
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
-
SUMMARY record, Unknown
-
1/147
-
-
-
Manon Peeters
+/+, +/., ?/+
7
1
c.259_266del
r.(?)
p.(Asp87Glnfs*87)
ACMG
pathogenic, pathogenic (dominant), VUS
g.42689807_42689814del
g.42722069_42722076del
6:42689806GGCTGGGTC>G ENST00000230381.5:c.259_266delGACCCAGC (Asp87GlnfsTer87),
2 more items
-
PRPH2_000111
ACMG PVS1, PM1, PM2, PP1, heterozygous
PubMed: Ba-Abbad 2014
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
PubMed: Carss 2017
,
4 more items
-
rs1554270834
Germline, Germline/De novo (untested), SUMMARY record, Unknown
?, yes
2/187
-
-
-
Manon Peeters
+/+, +/.
19
1
c.265_268delinsAGGGCC
r.(?)
p.(Ala89Argfs*11)
ACMG
pathogenic, pathogenic (dominant)
g.42689805_42689808delinsGGCCCT
g.42722067_42722070delinsGGCCCT
del265-268GCCA ins AGGGCC
-
PRPH2_000275
ACMG PVS1, PM1, PM2, PP1
PubMed: Pajic 2006
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
1 more item
-
-
Germline, SUMMARY record
yes
18/59
HaeIII+
-
-
Manon Peeters
+?/+?, +?/., ?/+?
7
1
c.271T>A
r.(?)
p.(Tyr91Asn)
ACMG
likely pathogenic, likely pathogenic (dominant), VUS
g.42689802A>T
g.42722064A>T
c.271T>A; p.Tyr91Asn (Alapati) / c.271T>A;p.Tyr91Asn (Reeves), c.271T>A;p.Tyr91Asn, p.Y91N
-
PRPH2_000274
ACMG PS4, PP1, PP3, PP5
PubMed: Alapati 2014
,
PubMed: Reeves 2020
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
4 more items
-
rs747893076
Germline, SUMMARY record, Unknown
no, yes
2/187, 2/39
-
-
-
Manon Peeters
+?/.
2
1
c.271T>C
r.(?)
p.(Tyr91His)
ACMG
likely pathogenic
g.42689802A>G
g.42722064A>G
-
-
PRPH2_000142
ACMG PM2, PP3, PP5
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
PubMed: Stone 2017
-
-
Germline, SUMMARY record
-
-
-
-
-
LOVD
+?/.
1
-
c.273T>A
r.(?)
p.(Tyr91*)
-
likely pathogenic
g.42689800A>T
g.42722062A>T
PRPH2, variant 1: c.273T>A/p.Y91*
-
PRPH2_000328
solved, heterozygous
PubMed: Weisschuh 2020
-
-
Unknown
?
-
-
-
-
LOVD
+/., +?/+, +?/.
3
1
c.276dup
r.(?)
p.(Arg93Glnfs*84)
ACMG
likely pathogenic, likely pathogenic (dominant), pathogenic
g.42689798dup
g.42722060dup
c.276dup p.(Arg93Glnfs*84), PRPH2 c.276dup, p.(Arg93Glnfs*84)
-
PRPH2_000273
ACMG PVS1, PM1, PM2
PubMed: Jespersgaar 2019
,
PubMed: Jespersgaard 2019
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
1 more item
-
-
Germline, SUMMARY record, Unknown
?
1/677
-
-
-
Manon Peeters
+/.
2
-
c.(281^282)G>A
r.(?)
p.(Trp94*)
-
pathogenic
g.(42689791^42689792)C>T
-
p.W94X
-
PRPH2_000310
-
PubMed: Meunier 2011
-
-
Germline
-
-
-
-
-
LOVD
+/+, +/., +?/+
9
1
c.281G>A
r.(?)
p.(Trp94*)
ACMG
likely pathogenic (dominant), pathogenic, pathogenic (dominant)
g.42689792C>T
g.42722054C>T
c.281G>A, c.281G>A ; p.W94*, c.281G>A change (p.Trp94X), p.W94X
-
PRPH2_000126
ACMG PVS1, PM2, PP1
PubMed: Birtel 2018
,
PubMed: Leroy 2007
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
3 more items
-
-
Germline, SUMMARY record, Unknown
yes
1/147, 2/44, 3/8
-
-
-
Manon Peeters
,
Daan Panneman
?/.
1
-
c.290G>A
r.(?)
p.(Trp97*)
ACMG
likely pathogenic (dominant)
g.42689783C>T
-
-
-
PRPH2_000351
-
PubMed: Bianco 2023
-
-
Unknown
-
-
-
-
-
Lorenzo Bianco
-?/.
1
-
c.299C>T
r.(?)
p.(Pro100Leu)
-
likely benign
g.42689774G>A
g.42722036G>A
-
-
PRPH2_000296
-
PubMed: Fernandez-San Jose 2015
-
-
Germline
-
-
-
-
-
LOVD
+/+?, +/., +?/+?, +?/.
4
1
c.303C>G
r.(?)
p.(Tyr101*), p.(Tyr101Ter)
ACMG
likely pathogenic, likely pathogenic (dominant), pathogenic, pathogenic (dominant)
g.42689770G>C
g.42722032G>C
c.303C>G; p.Tyr101*, Chr6(GRCh37):g.42689770G>C; p.(Tyr101*)
-
PRPH2_000078
ACMG PVS1, PM2, VKGL data sharing initiative Nederland
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
1 more item
-
rs61755776
CLASSIFICATION record, Germline, SUMMARY record, Unknown
-
1/147, 1/187
-
-
-
VKGL-NL_Nijmegen
,
Manon Peeters
-/?, ?/.
2
1
c.303C>T
r.(?)
p.(Tyr101=)
ACMG
VUS
g.42689770G>A
g.42722032G>A
p.Tyr10lTyr
-
PRPH2_000272
ACMG PM2, BS4, BP4; contradictory ACMG categories
PubMed: Dryja 1997
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
1 more item
-
-
SUMMARY record, Unknown
-
1/227 cases
-
-
-
Manon Peeters
+/+, +/., +?/.
7
1
c.310_313del
r.(?)
p.(Ile104Valfs*34)
ACMG
likely pathogenic, pathogenic, pathogenic (dominant)
g.42689762_42689765del
g.42722024_42722027del
c.310-313del(AT); p.Ile104Val (Alapati) / c.310_313del; p.Ile104Valfs*34 (Reeves),
2 more items
-
PRPH2_000125
ACMG PVS1, PM1, PM2, heterozygous
PubMed: Alapati 2014
,
PubMed: Reeves 2020
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
5 more items
-
-
Germline, SUMMARY record, Unknown
?, no
2/187
-
-
-
Manon Peeters
+/., +?/+?, +?/., -/.
4
1
c.318del
r.(?)
p.(Leu107Serfs*32), p.(Leu107SerfsTer32)
ACMG
benign, likely pathogenic, likely pathogenic (dominant), pathogenic (dominant)
g.42689755del, g.42689756del
g.42722018del
c.318delT; p.Leu107Serfs*32
-
PRPH2_000271, PRPH2_000301
ACMG PM2, PVS1, PP5, ACMG PVS1, PM2
PubMed: Lim 2009
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
PubMed: Weisschuh 2024
,
1 more item
-
rs7764439
Germline/De novo (untested), SUMMARY record, Unknown
-
1/187
-
-
-
Johan den Dunnen
,
Manon Peeters
-/., -?/.
9
1
c.318T>C
r.(=), r.(?)
p.(=), p.(Val106=)
-
benign, likely benign
g.42689755A>G
g.42722017A>G
318C>T (GTC?GTT) Val106Val, c.318T>C, RDS:c.555C>T (V106V) / rs7764439,
1 more item
-
PRPH2_000015
120/250 healthy controls, 87/250 healthy controls, VKGL data sharing initiative Nederland
PubMed: Bardak 2016
,
PubMed: Sohocki 2001
,
PubMed: Yi-2012
,
PubMed: Zhuk 2006
-
rs7764439
CLASSIFICATION record, Germline, Unknown
-
-66.70%, 0.02
-
-
-
Julia Lopez
,
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
?/.
1
-
c.318_332del
r.(?)
p.(Asn109_Phe113del)
-
VUS
g.42689742_42689756del
g.42722004_42722018del
PRPH2 c.318_332del, p.(Asn109_Phe113del)
-
PRPH2_000335
-
PubMed: Sodi 2021
-
-
Unknown
?
-
-
-
-
LOVD
+?/.
1
-
c.331del
r.(?)
p.(Ile111Serfs*28)
-
likely pathogenic
g.42689742del
g.42722004del
PRPH2, variant 1: c.331del/p.I111Sfs*28
-
PRPH2_000327
solved, heterozygous
PubMed: Weisschuh 2020
-
-
Unknown
?
-
-
-
-
LOVD
+?/., ?/., ?/?
4
1
c.346G>T
r.(?)
p.(Ala116Ser)
ACMG
likely pathogenic (dominant), VUS
g.42689727C>A
g.42721989C>A
c.346G>T, c.346G>T; p.A116S
-
PRPH2_000066
ACMG PS4, BP4; contradictory ACMG categories
PubMed: Arai 2015
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
PubMed: Liu-2020
,
2 more items
-
rs140227298
Germline, SUMMARY record, Unknown
-
1/349, 13/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
,
Manon Peeters
+?/.
2
1
c.[352_353del;356del]
r.(?)
p.[(Cys118Leufs*58);(Cys119Serfs*20)]
-
likely pathogenic
g.42689717del, g.42689720_42689721del
-
c.[352_353del;356del]
-
PRPH2_000345
-
PubMed: Panneman 2023
-
-
Unknown
-
-
-
-
-
Daan Panneman
+?/+?, +?/.
15
1
c.356_358del
r.(?)
p.(Cys119del)
ACMG
likely pathogenic, likely pathogenic (dominant)
g.42689720_42689722del
g.42721982_42721984del
3 bp deletion at codon 118/119,
1 more item
-
PRPH2_000270
ACMG PM1, PM2, PM4, PP1_moderate, PP3
PubMed: Farrar 1991
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
2 more items
-
-
Germline, SUMMARY record
yes
1/58 cases, 1/60 cases
-
-
-
Manon Peeters
+/.
2
-
c.356_365del
r.(?)
p.(Cys119Phefs*17)
ACMG
pathogenic
g.42689714_42689723del
g.42721976_42721985del
c.356_365del; p.(Cys119Phefs*17), PRPH2:NM_000322 c.356_365del, p.C119fs
-
PRPH2_000306
heterozygous, individual solved, variant causal
PubMed: Rodriguez-Munoz 2020
-
-
Germline
yes
-
-
-
-
LOVD
+?/+?, +?/., ?/.
7
1
c.367C>T
r.(?)
p.(Arg123Trp)
ACMG
likely pathogenic, likely pathogenic (dominant), VUS
g.42689706G>A
g.42721968G>A
(Arg123Trp), c.367CT; p.Arg123Trp, Chr6(GRCh37):g.42689706G>A; p.(Arg123Trp),
1 more item
-
PRPH2_000040
ACMG PS4, PM1, PP3, PP5, heterozygous, VKGL data sharing initiative Nederland
PubMed: Mezer-2006
,
PubMed: Ng 2019
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
1 more item
-
rs563581127
CLASSIFICATION record, Germline, SUMMARY record
no, yes
1/147, 2/22
-
-
-
VKGL-NL_Nijmegen
,
Manon Peeters
+/., +?/+
5
1
c.371del
r.(?)
p.(Gly124Alafs*15), p.(Gly124AlafsTer15)
ACMG
likely pathogenic (dominant), pathogenic
g.42689705del
g.42721967del
c.368delG; p.Gly124AlafsX14, PRPH2 c.371del, p.(Gly124AlafsTer15)
-
PRPH2_000269
ACMG PVS1, PM1, PM2
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
PubMed: Sodi 2021
,
1 more item
-
-
Germline, SUMMARY record, Unknown
?, yes
1/22
-
-
-
Manon Peeters
+/.
1
-
c.372_381delCTCGCTGGAGinsAAGCTGA
r.(?)
p.(Leu126Ter)
-
pathogenic
g.42689692_42689701delinsTCAGCTT
g.42721954_42721963delinsTCAGCTT
PRPH2 c.[372_381del];[372_381=]; p.(Leu126Ter)
-
PRPH2_000334
heterozygous
PubMed: Chen 2021
-
-
Germline
yes
Taiwan Biobank: 0; GnomAD_exome_East: 0; GnomAD_All: 0
-
-
-
LOVD
+/.
1
-
c.372_381delinsAAGCTGA
r.(?)
p.(Leu126*)
ACMG
pathogenic
g.42689692_42689701delinsTCAGCTT
g.42721954_42721963delinsTCAGCTT
PRPH2 c.[372_381del];[372_381=], V1: c.372_381delCTCGCTGGAGinsAAGCTGA, (p.Leu126Ter)
-
PRPH2_000334
heterozygous
PubMed: Chen 2021
-
-
Unknown
?
-
-
-
-
LOVD
+?/+?, +?/.
5
1
c.374C>T
r.(?)
p.(Ser125Leu)
ACMG
likely pathogenic, likely pathogenic (dominant)
g.42689699G>A
g.42721961G>A
C>T change at codon 125; p.Ser125Leu, PRPH2 c.374C>T, p.(Ser125Leu)
-
PRPH2_000094
ACMG PS4, PM1, PP3
PubMed: Holtan 2020
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
PubMed: Sodi 2021
,
1 more item
-
-
Germline, SUMMARY record, Unknown
?, yes
2/7, 3/899 cases
-
-
-
Global Variome, with Curator vacancy
,
Manon Peeters
+/+?, +?/+?, +?/.
7
1
c.377T>C
r.(?)
p.(Leu126Pro)
ACMG
likely pathogenic, likely pathogenic (dominant), pathogenic (dominant)
g.42689696A>G
g.42721958A>G
c.377T>C; p.Leu126Pro, c.377TC; p.Leu126Pro, Chr6(GRCh37):g.42689696A>G; p.(Leu126Pro),
1 more item
-
PRPH2_000077
ACMG PM1, PM2, PM5, PP3, PP5, solved, heterozygous, VKGL data sharing initiative Nederland
PubMed: Manes 2015
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
PubMed: Weisschuh 2020
,
2 more items
-
-
CLASSIFICATION record, Germline, SUMMARY record, Unknown
?, no, yes
1/147, 1/310 cases, 2/22
-
-
-
VKGL-NL_Nijmegen
,
Manon Peeters
+/., +?/+?, +?/.
8
1
c.377T>G
r.(?)
p.(Leu126Arg)
ACMG
likely pathogenic, likely pathogenic (dominant), pathogenic, pathogenic (dominant)
g.42689696A>C
g.42721958A>C
p.Leu126Arg, RDS: 377T>G
-
PRPH2_000106
ACMG PM1, PM2, PP1, PP3
PubMed: Dryja 1997
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
PubMed: Sohocki 2001
,
2 more items
-
-
Germline, SUMMARY record
yes
1/227 cases
-
-
-
Julia Lopez
,
Manon Peeters
+?/+?, +?/., ?/+?
5
1
c.380A>G
r.(?)
p.(Glu127Gly)
ACMG
likely pathogenic, likely pathogenic (dominant), VUS
g.42689693T>C
g.42721955T>C
c.380A>G; p.Glu127Gly
-
PRPH2_000268
ACMG PS4, PM1
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
2 more items
-
-
Germline, Germline/De novo (untested), SUMMARY record
yes
1/62, 3/187
-
-
-
Manon Peeters
+/.
2
1
c.382_385dup
r.(?)
p.(Thr129Lysfs*49)
ACMG
pathogenic, pathogenic (dominant)
g.42689688_42689691dup
g.42721950_42721953dup
-
-
PRPH2_000146
ACMG PVS1, PM1, PM2
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
PubMed: Van Cauwenbergh 2017
-
-
Germline, SUMMARY record
yes
-
-
-
-
LOVD
+/+?, +/., +?/+?, +?/.
12
1
c.389T>C
r.(?)
p.(Leu130Pro)
ACMG
likely pathogenic, likely pathogenic (dominant), pathogenic, pathogenic (dominant)
g.42689684A>G
g.42721946A>G
c.389T>C, c.389T>C (p.Leu130Pro), c.389T>C; p.Leu130Pro
-
PRPH2_000141
ACMG PM1, PM2,PP1, PP3
PubMed: Fakin 2012
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
PubMed: Stone 2017
,
4 more items
-
-
Germline, SUMMARY record, Unknown
yes
1/170, 4/187, 4/4 families
-
-
-
Manon Peeters
+/+, +/., +?/+, +?/.
6
1
c.394del
r.(?)
p.(Gln132Lysfs*7)
ACMG
likely pathogenic, likely pathogenic (dominant), pathogenic, pathogenic (dominant)
g.42689679delG, g.42689680del
g.42721942del
c.394delC, c.394delC, p.Q132fs, c.394delC; p.Gln132Lysfs*7
-
PRPH2_000267
ACMG PVS1, PS4, PM1
PubMed: Ba-Abbed 2014
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
PubMed: O'Sullivan-2012
,
2 more items
-
rs769723975
SUMMARY record, Unknown
-
3/187
-
-
-
Manon Peeters
?/., ?/?
2
1
c.403A>G
r.(?)
p.(Lys135Glu)
ACMG
VUS
g.42689670T>C
g.42721932T>C
c.403A>G; p.Lys135Glu
-
PRPH2_000266
ACMG PM2, PP3; ACMG criteria not strong enough to label as pathogenic
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
1 more item
-
-
SUMMARY record, Unknown
-
1/187
-
-
-
Manon Peeters
-/.
1
1
c.409G>A
r.(?)
p.(Gly137Ser)
-
VUS
g.42689664C>T
-
RDS: 409G>A
-
PRPH2_000145
Family has another pathogenic mutation
PubMed: Sullivan 2006
-
-
Germline
no
-
-
-
-
Julia Lopez
+/?, +?/., ?/., ?/?
6
1
c.410G>A
r.(?)
p.(Gly137Asp)
ACMG
likely pathogenic, pathogenic (dominant), VUS
g.42689663C>T
g.42721925C>T
c.410G>A, c.410G>A; G137D, c.410G>A; p.G137D (hetero)
-
PRPH2_000265
ACMG PM1, PM2, PP3; ACMG criteria not strong enough to label as pathogenic
PubMed: Jin 2008
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
PubMed: Midgley 2024
,
3 more items
-
rs527236097
Germline, SUMMARY record, Unknown
-
1/203, 2/329
-
-
-
Johan den Dunnen
,
Manon Peeters
?/.
1
-
c.413T>G
r.(?)
p.(Met138Arg)
ACMG
VUS
g.42689660A>C
-
-
-
PRPH2_000350
-
PubMed: Bianco 2023
-
-
Unknown
-
-
-
-
-
Lorenzo Bianco
+?/+?, +?/.
3
1
c.415_417del
r.(?)
p.(Lys139del)
ACMG
likely pathogenic, likely pathogenic (dominant)
g.42689657_42689659del
g.42721919_42721921del
c.415_417del; p.K139del
-
PRPH2_000264
ACMG PM1, PM2, PM4, PP3
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
-
SUMMARY record, Unknown
-
2/147
-
-
-
Manon Peeters
+/+
1
1
c.(417_421)ins(1)
r.?
p.?
-
pathogenic
g.(42689652_42689656)insN[1]
-
140ins (1 bp)
-
PRPH2_000308
ACMG PVS1, PM1, PM2
PubMed: Payne 1998
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
-
Germline
yes
1/300
-
-
-
Manon Peeters
+/., +?/+
7
1
c.418_421dup
r.(?)
p.(Tyr141Leufs*37)
ACMG
likely pathogenic (dominant), pathogenic
g.42689652_42689655dup
g.42721914_42721917dup
4-bp insertion (TACT) at codon 140
-
PRPH2_000263
ACMG PVS1, PM1, PM2, PP1_strong
PubMed: Keen 1994
,
PubMed: Kim 1995
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
1 more item
-
rs672601326
Germline, SUMMARY record
yes
6/13
-
-
-
Manon Peeters
+/., +?/+, +?/.
13
1
c.421T>C
r.(?)
p.(Tyr141His)
ACMG
likely pathogenic, likely pathogenic (dominant), pathogenic
g.42689652A>G
g.42721914A>G
658 TAC>CAC; Tyr141His, g.661T>C; Y141H, PRPH2 c.421T>C, p.(Tyr141His)
-
PRPH2_000262
ACMG PS3, PM1, PM2,PP1, PP3, heterozygous
PubMed: Dineiro 2020
,
PubMed: Gamundi 2007
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
2 more items
-
rs61755780
Germline, SUMMARY record
?, yes
1/61 cases, 10/30
-
-
-
Manon Peeters
+/+, +/., +?/+, +?/., ?/.
68
1
c.422A>G
r.(?)
p.(Tyr141Cys)
ACMG
likely pathogenic, likely pathogenic (dominant), pathogenic, pathogenic (dominant), VUS
g.42689651T>C
g.42721913T>C
422A.G (Tyr141Cys), c.422A>G, c.422A>G (p.Y141C), c.422A>G, p.Tyr141Cys, p.Y141C, Tyr141Cys,
5 more items
-
PRPH2_000105
ACMG PS3, PM1, PM2,PP1, PP3, Heterozygous
PubMed: Alapati 2014
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
PubMed: Birtel 2018
,
14 more items
-
rs61755781
Germline, SUMMARY record, Unknown
?, yes
1/65, 14/187, 14/188, 14/189, 14/190, 14/191, 14/192, 14/193, 14/194, 14/195, 14/196, 14/197, 14/198,
6 more items
-
-
-
Julia Lopez
,
Manon Peeters
,
Rebekkah Hitti-Malin
+?/.
1
-
c.422delA
r.(?)
p.(Tyr141Serfs*12)
ACMG
likely pathogenic
g.42689651del
g.42721913del
PRPH2 c.422delA, p.(Tyr141Serfs*12)
-
PRPH2_000316
heterozygous
PubMed: Dineiro 2020
-
-
Germline
?
-
-
-
-
LOVD
+/+, +/.
2
1
c.423C>A
r.(?)
p.(Tyr141*)
ACMG
pathogenic, pathogenic (dominant)
g.42689650G>T
g.42721912G>T
c.423C>A; p.Y141*
-
PRPH2_000261
ACMG PVS1, PM1, PM2
PubMed: Peeters 2021
,
Journal: Peeters 2021
-
-
SUMMARY record, Unknown
-
1/147
MspI-
-
-
Manon Peeters
+/+, +/., +?/., ?/.
161
1
c.424C>T
r.(424c>u), r.(?)
p.(Arg142Trp)
ACMG
likely pathogenic, pathogenic, pathogenic (dominant), VUS
g.42689649G>A
g.42721911G>A
661 CGG>TGG; Arg142 Trp, Arg142Trp, c.424C > T; p.R142W, c.424C>T, c.424C>T p.(Arg142Trp),
13 more items
-
PRPH2_000001
ACMG PS4, PM1, PP1_strong, PP3, heterozygous, no zygosity and pathogenicity classification indicated,
2 more items
PubMed: Birtel 2018
,
PubMed: Boon 2009
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
PubMed: Ng 2021
,
24 more items
-
rs61755783
CLASSIFICATION record, De novo, Germline, Germline/De novo (untested), SUMMARY record, Unknown
?, no, yes
1/170, 1/22, 1/677, 1/86, 12/20, 2/2420 IRD families, 2/61 cases, 21/103, 29/62, 3/30, 3/351, 39/147,
1 more item
MspI-
-
-
Global Variome, with Curator vacancy
,
Johan den Dunnen
,
Kornelia Neveling
,
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
,
Oscar F Chacon-Camacho
,
Manon Peeters
,
Rebekkah Hitti-Malin
+?/+?, +?/.
3
1
c.425G>A
r.(?)
p.(Arg142Gln)
ACMG
likely pathogenic, likely pathogenic (dominant)
g.42689648C>T
g.42721910C>T
c.425G>A; p.Arg142Gln
-
PRPH2_000140
ACMG PS4, PM1, PM5, PP3
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
PubMed: Stone 2017
,
1 more item
-
rs554945964
Germline, SUMMARY record, Unknown
-
1/187
-
-
-
Manon Peeters
+/., +?/+
3
1
c.433_434del
r.(?)
p.(Asp145Hisfs*31)
ACMG
likely pathogenic (dominant), pathogenic
g.42689640_42689641del
g.42721902_42721903del
c.433_434delGA; p.D145fsX30
-
PRPH2_000039
ACMG PVS1, PM1, PM2, BS4
PubMed: Boon 2007
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
1 more item
-
-
Germline, SUMMARY record
yes
2/30
-
-
-
Manon Peeters
+/.
1
-
c.440del
r.(?)
p.(Pro147Leufs*6)
ACMG
pathogenic
g.42689636del
g.42721898del
PRPH2 c.[440del];[440=], V1: c.440delC, (p.Pro147LeufsTer6)
-
PRPH2_000333
heterozygous
PubMed: Chen 2021
-
-
Unknown
?
-
-
-
-
LOVD
+/.
1
-
c.440delC
r.(?)
p.(Pro147LeufsTer6)
-
pathogenic
g.42689636del
g.42721898del
PRPH2 c.[440del];[440=]; p.(Pro147LeufsTer6)
-
PRPH2_000333
heterozygous
PubMed: Chen 2021
-
-
Germline
yes
Taiwan Biobank: 0; GnomAD_exome_East: 0.0000544; GnomAD_All: 0.00000398
-
-
-
LOVD
+/+, +/., +?/+, +?/.
31
1
c.441del
r.(?)
p.(Gly148Alafs*5), p.(Gly148AlafsTer5)
ACMG
likely pathogenic, likely pathogenic (dominant), pathogenic, pathogenic (dominant)
g.42689632del
g.42721894del
c.441del; p.G148Afs*5, c.441delT, c.441delT; p.Gly148Alafs*5, c.441delT; p.P147fsX4,
5 more items
-
PRPH2_000002
ACMG PVS1, PS4, PM1, PP1, heterozygous, solved, heterozygous, VKGL data sharing initiative Nederland
PubMed: Birtel 2018
,
PubMed: Boon 2007
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
8 more items
-
rs61755784
CLASSIFICATION record, Germline, SUMMARY record, Unknown
?, yes
1/187, 1/2420 IRD families, 1/61 cases, 1/93 cases, 5/147, 7/30
MspI+;MvaI-
-
-
Global Variome, with Curator vacancy
,
Kornelia Neveling
,
VKGL-NL_Rotterdam
,
Manon Peeters
?/., ?/?
6
1
c.454A>G
r.(?)
p.(Met152Val)
ACMG
VUS
g.42689619T>C
g.42721881T>C
A454G, c.454A>G; M152V
-
PRPH2_000065
ACMG PS4, PM1, BP4; contradictory ACMG categories
PubMed: Jin 2008
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
PubMed: Katagiri 2014
,
2 more items
-
rs146703538
Germline, SUMMARY record, Unknown
-
11/1204 cases with retinitis pigmentosa, 3/203
-
-
-
Yoshito Koyanagi
,
Manon Peeters
+?/+?, +?/.
2
1
c.457A>G
r.(?)
p.(Lys153Glu)
ACMG
likely pathogenic, likely pathogenic (dominant)
g.42689616T>C
g.42721878T>C
c.457A>G (p.Lys153Glu)
-
PRPH2_000260
ACMG PM1, PM2, PM5, PP3
PubMed: Lee 2015
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
1 more item
-
-
SUMMARY record, Unknown
-
1/26
-
-
-
Manon Peeters
?/., ?/?
2
1
c.458A>C
r.(?)
p.(Lys153Thr)
ACMG
VUS
g.42689615T>G
g.42721877T>G
c.458A>C; p.Lys153Thr
-
PRPH2_000259
ACMG PM1, PM2, PP3; ACMG criteria not strong enough to label as pathogenic
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
1 more item
-
-
SUMMARY record, Unknown
-
1/187
-
-
-
Manon Peeters
+/., +?/+?, +?/., ?/+?
12
1
c.458A>G
r.(?)
p.(Lys153Arg)
ACMG
likely pathogenic, likely pathogenic (dominant), pathogenic (dominant), VUS
g.42689615T>C
g.42721877T>C
c.458A>G, c.458A>G; p.Lys153Arg, Chr6(GRCh37):g.42689615T>C; p.(Lys153Arg), K153R,
1 more item
-
PRPH2_000038
ACMG PM1, PM2, PP1, PP3, VKGL data sharing initiative Nederland
PubMed: Colombo-2020
,
PubMed: Jacobson 1996
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
3 more items
-
rs61755785
CLASSIFICATION record, Germline, SUMMARY record, Unknown
yes
1/147, 1/22
-
-
-
Sandro Banfi
,
VKGL-NL_Nijmegen
,
VKGL-NL_AMC
,
Manon Peeters
+?/., ?/., ?/?
5
1
c.460A>C
r.(?)
p.(Lys154Gln)
ACMG
likely pathogenic, VUS
g.42689613T>G
g.42721875T>G
c.460A>C, p.K154Q, c.460A>C, p.Lys154Gln, c.460A>C; p.K154Q, c.460A>C; p.Lys154Gln
-
PRPH2_000258
ACMG PS4, PM1, BP4; contradictory ACMG categories, heterozygous
PubMed: Arai 2015
,
PubMed: Peeters 2021
,
Journal: Peeters 2021
,
PubMed: Gao 2019
,
3 more items
-
-
Germline, SUMMARY record, Unknown
?
1/145 cases, 1/349, 1/3944
-
-
-
Manon Peeters
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