Global Variome shared LOVD
RPGR (retinitis pigmentosa GTPase regulator)
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Curator:
Global Variome, with Curator vacancy
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Unique variants in the RPGR gene
This database is one of the
"Eye disease"
gene variant databases.
The variants shown are described using the
NM_001034853.1
NM_000328.2
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
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Operator
Column type
Example
Matches
Text
Arg
all entries containing 'Arg'
space
Text
Arg Ser
all entries containing 'Arg' and 'Ser'
|
Text
Arg|Ser
all entries containing 'Arg' or 'Ser'
!
Text
!fs
all entries not containing 'fs'
^
Text
^p.(Arg
all entries beginning with 'p.(Arg'
$
Text
Ser)$
all entries ending with 'Ser)'
=""
Text
=""
all entries with this field empty
=""
Text
="p.0"
all entries exactly matching 'p.0'
!=""
Text
!=""
all entries with this field not empty
!=""
Text
!="p.0"
all entries not exactly matching 'p.0?'
combination
Text
*|Ter !fs
all entries containing '*' or 'Ter' but not containing 'fs'
Date
2020
all entries matching the year 2020
|
Date
2020-03|2020-04
all entries matching March or April, 2020
!
Date
!2020-03
all entries not matching March, 2020
<
Date
<2020
all entries before the year 2020
<=
Date
<=2020-06
all entries in or before June, 2020
>
Date
>2020-06
all entries after June, 2020
>=
Date
>=2020-06-15
all entries on or after June 15th, 2020
combination
Date
2019|2020 <2020-03
all entries in 2019 or 2020, and before March, 2020
Numeric
23
all entries exactly matching 23
|
Numeric
23|24
all entries exactly matching 23 or 24
!
Numeric
!23
all entries not exactly matching 23
<
Numeric
<23
all entries lower than 23
<=
Numeric
<=23
all entries lower than, or equal to, 23
>
Numeric
>23
all entries higher than 23
>=
Numeric
>=23
all entries higher than, or equal to, 23
combination
Numeric
>=20 <30 !23
all entries with values from 20 to 29, but not equal to 23
Some more advanced examples:
Example
Matches
Asian
all entries containing 'Asian', 'asian', including 'Caucasian', 'caucasian', etc.
Asian !Caucasian
all entries containing 'Asian' but not containing 'Caucasian'
Asian|African !Caucasian
all entries containing 'Asian' or 'African', but not containing 'Caucasian'
"South Asian"
all entries containing 'South Asian', but not containing 'South East Asian'
To sort on a certain column, click on the column header or on the arrows. If that column is already selected to sort on, the sort order will be swapped. The column currently sorted on has a darker blue background color than the other columns. The up and down arrows next to the column name indicate the current sorting direction. When sorting on any field other than the default, LOVD will sort secondarily on the default sort column.
855 entries on 9 pages. Showing entries 1 - 100.
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Effect
Reported
Exon
DNA change (cDNA)
RNA change
Protein
Classification method
Clinical classification
DNA change (genomic) (hg19)
DNA change (hg38)
Published as
ISCN
DB-ID
Variant remarks
Reference
ClinVar ID
dbSNP ID
Origin
Segregation
Frequency
Re-site
VIP
Methylation
Owner
+?/.
1
10
1246-?_*1091-?
r.(?)
p.(?) deletion after exon 10
-
likely pathogenic
g.?
g.?
-
-
USP9X_000005
this mutation was found in one female carrier
PubMed: Talib 2018
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
1
-
c.(1245+1_1246-1)_(*135+?)del
r.?
p.(Glu416Ilefs*4)
-
likely pathogenic
g.?
-
c.(1245+1_1246-1)_(*135+?)del
-
USP9X_000005
-
PubMed: Panneman 2023
-
-
Unknown
-
-
-
-
-
Daan Panneman
+/.
1
-
c.(1245+1_1246-1)_*456{0}
r.?
p.?
-
pathogenic
g.(?_38128423)_(38164044_38169867)del
g.(?_38269170)_(38304791_38310614)del
del ex11-19
-
RPGR_000316
-
PubMed: Bader 2003
-
-
Germline
-
-
-
-
-
LOVD
+/.
1
1_11i
c.-80_1414+42delinsC
r.?
p.?
-
pathogenic (recessive)
g.38156495_38186700delinsG
g.38297242_38327447delinsG
del ex1-11;g.ORF15+1675A>G
-
RPGR_000191
-
PubMed: Jin 2005
-
-
Germline
-
-
-
-
-
LOVD
-/.
1
-
c.-52C>A
r.(?)
p.(=)
-
benign
g.38186672G>T
-
-
-
RPGR_000811
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/., +?/.
19
3, 8
c.?
r.(?), r.?
p.(Ala83_Pro471del), p.(Asp10_Thr103del), p.(Glu260_Pro471del), p.(Gly702fs), p.(Gly718fs), p.?,
2 more items
ACMG
likely pathogenic, pathogenic, pathogenic (dominant)
g.38163968C>T, g.38182177C>A, g.38184601_38194323delinsTACACAG, g.?
g.?
2236_2237delCT (Glu746Argfs*23), 523+1G>T, 6.4kb deletion IVS15+1.5kb and IVS15+7.9kb (IVS15a+70),
14 more items
-
RPGR_000000, USP9X_000005
ACMG PM2, PVS1, PP5, variant description makes no sense,
2 more items
PubMed: Daiger 2014
,
PubMed: Huang 2017
,
PubMed: Jacobson 2014
,
PubMed: Koyanagi 2020
,
7 more items
-
-
Germline, Unknown
?, yes
0/600 controls, 1/2420 IRD families, 2/2420 IRD families
-
-
-
Global Variome, with Curator vacancy
,
Johan den Dunnen
+/.
1
-
c.154+3_6delTAGT
r.spl
p.?
-
pathogenic
g.38182648_38182651del
g.38323395_38323398del
RPGR c.154+3_6delTAGT
-
RPGR_000799
in vitro splicing assay: exon 2 skipping; hemizygous
PubMed: Kortum 2021
-
-
Germline
yes
-
-
-
-
LOVD
-?/.
1
-
c.9G>A
r.(?)
p.(Glu3=)
-
likely benign
g.38186612C>T
-
RPGR(NM_001034853.1):c.9G>A (p.E3=)
-
RPGR_000158
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/.
15
1
c.27del
r.(?)
p.(Asp10Ilefs*58)
-
likely pathogenic
g.38186596del
g.38327343del
-
-
RPGR_000801
this mutation was found in N female carriers (n families): 15 (1)
PubMed: Talib 2018
-
-
Germline
yes
-
-
-
-
LOVD
+/.
5
1i
c.28+1G>A
r.(?), r.spl, r.spl?
p.?
-
pathogenic, pathogenic (dominant), pathogenic (recessive)
g.38186592C>T
g.38327339C>T
28+1G>A, c.28+1G>A (Spl), IVS1+1G>A
-
RPGR_000314
-
PubMed: Prokisch 2007
,
PubMed: Sharon 2000
,
PubMed: Sharon 2003
,
PubMed: Zahid 2013
,
PubMed: Zito 1999
,
1 more item
-
-
Germline
-
-
-
-
-
LOVD
+/.
1
-
c.28+1G>C
r.spl
p.(?)
ACMG
pathogenic
g.38186592C>G
g.38327339C>G
RPGR c.28+1G>C, p.(?)
-
RPGR_000643
-
PubMed: Jespersgaar 2019
-
-
Germline
?
-
-
-
-
LOVD
+?/.
2
1i
c.28+2T>C
r.(?), r.spl?
p.(?)
-
likely pathogenic
g.38186591A>G
g.38327338A>G
c.28+2T>C
-
RPGR_000800
this mutation was found in one female carrier
PubMed: Panneman 2023
,
PubMed: Talib 2018
-
-
Germline, Unknown
yes
-
-
-
-
Daan Panneman
+/.
1
-
c.28+2T>G
r.spl?
p.?
-
pathogenic
g.38186591A>C
-
c.28+2T>G
-
RPGR_000621
-
PubMed: Branham-2012
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/.
4
1i
c.28+5G>A
r.(?), r.spl?
p.(?), p.?
-
likely pathogenic, pathogenic, pathogenic (recessive)
g.38186588C>T
g.38327335C>T
c.28+5G>A, IVS1+5G>A, RPGR p.(?)
-
RPGR_000104
1 more item
PubMed: Foote-2019
,
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
,
PubMed: Koyanagi 2020
,
1 more item
-
rs62638626
Germline, Unknown
?
1/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
-/., -?/.
6
1i
c.29-15G>A
r.(=), r.(?), r.spl?
p.(=), p.?
-
benign, likely benign
g.38182792C>T
g.38323539C>T
c.29-15G>A, IVS1-15A>G, IVS1-15G>A
-
RPGR_000137
VKGL data sharing initiative Nederland
PubMed: Buraczynska 1997
,
PubMed: Miano 1999
,
PubMed: Neidhardt 2008
,
PubMed: Neidhardt 2009
-
-
CLASSIFICATION record, Germline
-
0.017
-
-
-
VKGL-NL_Nijmegen
+/., +?/.
2
1i
c.29-2A>T
r.spl, r.spl?
p.?
ACMG
likely pathogenic (maternal), pathogenic
g.38182779T>A
g.38323526T>A
c.29-2A>T, NM_000328.2:c.29-2A>T, NP_000319.1:p.?, NC_000023.10:g.38182779T>A
-
RPGR_000601
different transcript (NM_000328.2) in publication
PubMed: Liu-2020
,
PubMed: Wang 2018
-
-
Germline
?
-
-
-
-
LOVD
+?/.
2
-
c.29-2_30del
r.spl?
p.?
-
likely pathogenic
g.38182776_38182779del
g.38323523_38323526del
RPGR(NM_001034853.2):c.29-2_30delAGAT
-
RPGR_000128
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
,
VKGL-NL_AMC
+/., +?/.
4
1i
c.29-1G>T
r.spl
p.(?), p.?
ACMG
likely pathogenic, pathogenic
g.38182778C>A
g.38323525C>A
c.29-1G>T, Splice, RPGR c.29-1G>T, splice site, RPGR, variant 1: c.29-1G>T/p.?
-
RPGR_000642
ACMG PM2, PVS1_STRONG, PP5_STRONG, Hemizygous, heterozygous, solved, hemizygous
PubMed: Birtel 2018
,
PubMed: Gliem 2020
,
PubMed: Weisschuh 2020
,
PubMed: Weisschuh 2024
813233
-
Germline, Germline/De novo (untested), Unknown
?
-
-
-
-
Johan den Dunnen
+/.
1
1i_3i
c.(28+1_29-1)_(247+1_248-1)del
r.?
p.?
-
pathogenic
g.(38180343_38182105)_(38182778_38186592)del
g.(38321090_38322852)_(38323525_38327339)del
del ex2-3
-
RPGR_000181
-
PubMed: Zahid 2013
-
-
Germline
-
-
-
-
-
LOVD
+/.
1
2_5
c.29_469del
r.(?)
p.(Asp10_Thr156del)
-
pathogenic
g.38178082_38182777del
-
c.29_469del
-
RPGR_000708
-
PubMed: Khateb-2016
-
-
Germline
-
-
-
-
-
LOVD
+/.
1
-
c.35del
r.(?)
p.(Gly12Valfs*56)
-
pathogenic
g.38182773del
g.38323520del
92delG
-
RPGR_000457
-
PubMed: Bader 2003
-
-
Germline
-
-
-
-
-
LOVD
?/.
1
-
c.68C>A
r.(?)
p.(Ala23Asp)
-
VUS
g.38182738G>T
-
RPGR(NM_001034853.1):c.68C>A (p.A23D)
-
RPGR_000648
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
2
c.89delT
r.(?)
p.(Phe30Serfs*38)
-
pathogenic
g.38182718del
g.38323465del
c.89delT
-
RPGR_000492
-
PubMed: Bernardis 2016
-
-
De novo
-
-
-
-
-
LOVD
+?/.
1
-
c.92G>A
r.(?)
p.(Trp31Ter)
-
likely pathogenic
g.38182714C>T
g.38323461C>T
-
-
RPGR_000582
-
PubMed: Wang 2014
-
-
Germline
-
-
-
-
-
LOVD
+?/.
1
-
c.93G>A
r.(?)
p.(Trp31*)
-
likely pathogenic
g.38182713C>T
g.38323460C>T
-
-
RPGR_000481
-
PubMed: Stone 2017
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/.
11
2
c.101del
r.(?)
p.(Asn34Metfs*34)
-
likely pathogenic, pathogenic
g.38182709del
g.38323456del
160delA, c.101del (p.N34Mfs*34)
-
RPGR_000313
hemizygous
PubMed: Fahim 2011
,
PubMed: Guevara-Fujita 2001
-
-
Germline, Germline/De novo (untested)
?
-
-
-
-
LOVD
+/.
1
2
c.101_102insA
r.(?)
p.(Asn34Lysfs*2)
-
pathogenic
g.38182704_38182705insT
-
c.101_102insA
-
RPGR_000743
-
PubMed: Colombo-2020
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
3
2
c.112del
r.(?)
p.(Val38Tyrfs*30)
-
likely pathogenic
g.38182694del
g.38323441del
RPGR, variant 1: c.112del/p.V38Yfs*30
-
RPGR_000714
solved, hemizygous, this mutation was found in one female carrier
PubMed: Talib 2018
,
PubMed: Weisschuh 2020
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
1
-
c.122C>A
r.(?)
p.(Ser41*)
-
likely pathogenic
g.38182684G>T
g.38323431G>T
RPGR c.122C>A, p.Ser41Ter
-
RPGR_000676
-
PubMed: Zampaglione 2020
-
-
Unknown
?
-
-
-
-
LOVD
+/., +?/.
2
2
c.122C>G
r.(?)
p.(Ser41*)
-
likely pathogenic, pathogenic (dominant)
g.38182684G>C
g.38323431G>C
c.122C>G
-
RPGR_000456
-
PubMed: Zhou 2018
,
PubMed: Zhou-2011
-
-
Germline, Unknown
-
-
-
-
-
LOVD
+/.
1
-
c.126T>G
r.(?)
p.(Cys42Trp)
-
pathogenic
g.38182680A>C
-
X:38182680A>C ENST00000378505.2:c.126T>G (Cys42Trp)
-
RPGR_000455
-
PubMed: Carss 2017
-
-
Germline
-
-
-
-
-
LOVD
+/., ?/.
2
-
c.127G>A
r.(?)
p.(Gly43Arg)
-
pathogenic, VUS
g.38182679C>T
g.38323426C>T
186G>A, RPGR c.127G>A, p.Gly43Arg
-
RPGR_000454
-
PubMed: Sharon 2000
,
PubMed: Sharon 2003
,
PubMed: Zampaglione 2020
-
-
Germline, Unknown
?
-
-
-
-
LOVD
+/.
1
-
c.128G>A
r.(?)
p.(Gly43Glu)
-
pathogenic
g.38182678C>T
g.38323425C>T
187G>A
-
RPGR_000453
-
PubMed: Sharon 2000
,
PubMed: Sharon 2003
-
-
Germline
-
-
-
-
-
LOVD
+?/.
1
-
c.133G>T
r.(?)
p.(Glu45*)
-
likely pathogenic
g.38182673C>A
g.38323420C>A
RPGR, variant 1: c.133G>T/p.E45*
-
RPGR_000713
solved, hemizygous
PubMed: Weisschuh 2020
-
-
Unknown
?
-
-
-
-
LOVD
+?/.
1
-
c.139del
r.(?)
p.(Ser47Leufs*21)
ACMG
likely pathogenic
g.38182668del
g.38323415del
-
-
RPGR_000144
-
-
-
-
Germline
-
-
-
-
-
Sandro Banfi
+/.
1
-
c.139_140insTCTGC
r.(?)
p.(Ser47PhefsTer23)
-
pathogenic
g.38182666_38182667insGCAGA
g.38323413_38323414insGCAGA
-
-
RPGR_000532
not in 458 control chromosomes
PubMed: Sun 2015
-
-
Germline
-
1/442 chromosomes
-
-
-
LOVD
-/., -?/.
3
2
c.141T>G
r.(?)
p.(Ser47=)
-
benign, likely benign
g.38182665A>C
g.38323412A>C
200T>G, RPGR(NM_001034853.1):c.141T>G (p.S47=), RPGR(NM_001034853.2):c.141T>G (p.S47=)
-
RPGR_000057
VKGL data sharing initiative Nederland
PubMed: Miano 1999
-
-
CLASSIFICATION record, Germline
-
0.043
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
-/., -?/., ?/.
4
-
c.153C>T
r.(?)
p.(Thr51=)
-
benign, likely benign, VUS
g.38182653G>A
g.38323400G>A
212C>T, RPGR(NM_001034853.1):c.153C>T (p.T51=)
-
RPGR_000056
VKGL data sharing initiative Nederland
PubMed: Breuer 2002
,
PubMed: Wang 2014
-
rs201242851
CLASSIFICATION record, Germline
-
0.005
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Nijmegen
+/., +?/.
10
2
c.154G>A
r.(?), r.29_154del, r.spl
p.(Gly52Arg), p.?
ACMG
likely pathogenic, pathogenic, pathogenic (recessive)
g.38182652C>T
g.38323399C>T
213G>A (G52R), c.154G>A, Gly52Arg
-
RPGR_000103
-
Tracewska 2021, MolVis in press,
PubMed: Bernardis 2016
,
PubMed: Colombo-2020
,
PubMed: Demirci 2004
,
4 more items
-
-
Germline
yes
0 (in-house database, ~5000 samples), 1/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
+/.
4
2
c.154G>T
r.(?), r.spl
p.(Gly52*)
-
pathogenic, pathogenic (recessive)
g.38182652C>A
g.38323399C>A
213G>T, c.154G>T (p.Gly52X)
-
RPGR_000312
-
PubMed: Branham-2012
,
PubMed: Buraczynska 1997
,
PubMed: Jacobson 1997
,
1 more item
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/.
5
2i
c.154+1G>A
r.(?), r.spl
p.(?), p.?
-
likely pathogenic, pathogenic (recessive)
g.38182651C>T
g.38323398C>T
IVS2+1G>A, RPGR, variant 1: c.154+1G>A/p.?
-
RPGR_000311
solved, hemizygous
PubMed: Koenekoop 2003
,
PubMed: Weisschuh 2020
-
-
Germline, Unknown
?, yes
-
-
-
-
LOVD
+/.
1
2i
c.154+3del4
r.spl?
p.?
-
pathogenic
g.38182649del
-
c.154+3del4
-
RPGR_000569
-
PubMed: Neidhardt 2008
-
-
Germline
-
-
-
-
-
LOVD
+?/.
1
-
c.154+4A>G
r.spl?
p.(?)
-
likely pathogenic
g.2975943G>C
g.3118421G>C
c.6408C>G; p.Asn2136Lys
-
RPGR_000001
Known high myopia gene; heterozygous variant
PubMed: Wan 2018
-
rs778164827
Unknown
?
-
-
-
-
LOVD
?/.
1
-
c.154+5G>A
r.spl?
p.?
-
VUS
g.38182647C>T
g.38323394C>T
-
-
RPGR_000136
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
2
2i
c.155-2A>G
r.154_155ins[154+1_155-3;gg], r.spl?
p.?
-
pathogenic, pathogenic (recessive)
g.38182200T>C
-
c.155-2A>G, IVS2-2A>G
-
RPGR_000193
-
PubMed: Branham-2012
,
PubMed: Miano 1999
-
-
Germline
-
-
-
-
-
LOVD
+?/.
1
-
c.155-1G>A
r.spl
p.(?)
ACMG
likely pathogenic
g.38182199C>T
g.38322946C>T
RPGR c.155-1G>A, p.(?)
-
RPGR_000641
-
PubMed: Jespersgaar 2019
-
-
Germline
?
-
-
-
-
LOVD
+/.
1
-
c.155-1G>T
r.spl?
p.?
-
pathogenic
g.38182199C>A
-
-
-
RPGR_000157
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/.
1
-
c.173T>A
r.(?)
p.(Met58Lys)
-
likely pathogenic
g.38182180A>T
g.38322927A>T
-
-
RPGR_000480
-
PubMed: Stone 2017
-
-
Germline
-
-
-
-
-
LOVD
+/.
1
3
c.173_175delinsG
r.(?)
p.(Met58Serfs*4)
-
pathogenic
g.38182178_38182180delinsC
-
c.173delT;177delT (p.Met58fs)
-
RPGR_000620
-
PubMed: Branham-2012
-
-
Germline
-
-
-
-
-
LOVD
+?/.
1
-
c.178G>A
r.(?)
p.(Gly60Ser)
-
likely pathogenic
g.38182175C>T
g.38322922C>T
-
-
RPGR_000452
-
PubMed: Taylor 2017
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/., ?/.
9
3
c.179G>T
r.(?)
p.(Gly60Val)
-
likely pathogenic, pathogenic, pathogenic (recessive), VUS
g.38182174C>A
g.38322921C>A
238G>T, RPGR(NM_001034853.2):c.179G>T (p.G60V)
-
RPGR_000055
VKGL data sharing initiative Nederland
PubMed: Buraczynska 1997
,
PubMed: Fishman 1998
,
PubMed: Sharon 2000
,
PubMed: Sharon 2003
,
1 more item
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
VKGL-NL_AMC
+/.
1
3
c.194del
r.(?)
p.(Gly65Valfs*3)
-
pathogenic (recessive)
g.38182162del
g.38322909del
194delG
-
RPGR_000310
-
PubMed: Shu 2007
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/., ?/.
6
3
c.194G>A
r.(?)
p.(Gly65Asp)
-
likely pathogenic, pathogenic, VUS
g.38182159C>T
g.38322906C>T
c.194G>A, c.194G>A (p.G65D), c.194G>A (p.Gly65Asp), RPGR c.194G>A, p.Gly65Asp
-
RPGR_000596
hemizygous
PubMed: Bowne 2011
,
PubMed: Churchill-2013
,
PubMed: Fahim 2011
,
PubMed: Zampaglione 2020
-
-
Germline, Germline/De novo (untested), Unknown
?
-
-
-
-
LOVD
+/.
1
3
c.194G>T
r.(?)
p.(Gly65Val)
ACMG
pathogenic
g.38182159C>A
g.38322906C>A
c.194G>T, p.Gly65Val
-
RPGR_000523
Hemizygous
PubMed: Birtel 2018
-
-
Germline
?
-
-
-
-
LOVD
+/.
1
-
c.196C>T
r.(?)
p.(Gln66*)
-
pathogenic
g.38182157G>A
-
-
-
RPGR_000825
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
2
-
c.197A>G
r.(?)
p.(Gln66Arg)
-
pathogenic
g.38182156T>C
g.38322903T>C
NM_000328.2 :c.197A>G (Gln66Arg), NM_000328.2:c.197A>G (Gln66Arg)
-
RPGR_000479
-
PubMed: Wang 2017
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/.
3
3
c.202G>A
r.(?)
p.(Gly68Arg)
-
likely pathogenic, pathogenic (recessive)
g.38182151C>T
g.38322898C>T
c.202G>A, RPGR c.202G>A, p.G68R
-
RPGR_000309
hemizygous
PubMed: Jauregui 2020
,
PubMed: Panneman 2023
,
PubMed: Park 2013
-
-
Germline, Unknown
?
-
-
-
-
Daan Panneman
+?/.
1
-
c.202G>C
r.(?)
p.(Gly68Arg)
ACMG
likely pathogenic
g.38182151C>G
-
-
-
RPGR_000177
-
PubMed: Sharon 2019
-
-
Germline
-
1/2420 IRD families
-
-
-
Global Variome, with Curator vacancy
+/.
1
3
c.212C>G
r.(?)
p.(Ser71*)
-
pathogenic (recessive)
g.38182141G>C
g.38322888G>C
-
-
RPGR_000308
-
PubMed: Tocino 2019
-
-
Germline
-
-
-
-
-
LOVD
+/.
1
3
c.215del
r.(?)
p.(Lys72Serfs*13)
-
pathogenic (!)
g.38182140del
g.38322887del
215delA
-
RPGR_000307
-
PubMed: Nanda 2018
-
-
Germline
-
-
-
-
-
LOVD
+?/.
1
3
c.219del
r.(?)
p.(Ala74Profs*11)
-
likely pathogenic
g.38182134del
g.38322881del
c.219del (p.A74Pfs*11)
-
RPGR_000798
hemizygous
PubMed: Fahim 2011
-
-
Germline/De novo (untested)
?
-
-
-
-
LOVD
-/., -?/.
2
-
c.222C>T
r.(?)
p.(Ala74=)
-
benign, likely benign
g.38182131G>A
g.38322878G>A
RPGR(NM_001034853.1):c.222C>T (p.A74=), RPGR(NM_001034853.2):c.222C>T (p.A74=)
-
RPGR_000054
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
+?/., -/., -?/.
3
3
c.223A>G
r.(?)
p.(Ile75Val)
-
benign, likely benign, likely pathogenic
g.38182130T>C
g.38322877T>C
282A>G;844C>G p.(Ile75Val;p.(Ala262Gly), c.223A>G
-
RPGR_000010
predicted benign
PubMed: Buraczynska 1997
,
PubMed: Neveling 2012
,
PubMed: Song-2011
-
-
Germline, Unknown
-
-
-
-
-
Kornelia Neveling
-/.
1
3
c.227G>T
r.(?)
p.(Ser76Ile)
-
benign
g.38182126C>A
g.38322873C>A
286G>T
-
RPGR_000306
-
PubMed: Zito 1999
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/.
2
3
c.237_238del
r.(?)
p.(Val81Glnfs*6)
ACMG
likely pathogenic, pathogenic (recessive)
g.38182115_38182116del
g.38322862_38322863del
296-297delAT
-
RPGR_000148
-
PubMed: Miano 1999
-
-
Germline
-
-
-
-
-
Sandro Banfi
+?/.
1
-
c.247G>T
r.(?)
p.(Ala83Ser)
-
likely pathogenic
g.38182106C>A
g.38322853C>A
RPGR, variant 1: c.247G>T/p.A83S
-
RPGR_000712
possibly solved, hemizygous
PubMed: Weisschuh 2020
-
-
Unknown
?
-
-
-
-
LOVD
+?/.
1
-
c.247+1G>A
r.spl?
p.?
ACMG
likely pathogenic
g.38182105C>T
g.38322852C>T
-
-
RPGR_000150
-
-
-
-
Germline
-
-
-
-
-
Sandro Banfi
+/.
6
-
c.247+2T>A
r.spl
p.?
ACMG
pathogenic
g.38182104A>T
g.38322851A>T
RPGR c.247+2T>A, p.?
-
RPGR_000718
-
PubMed: Rodriguez Munoz 2021
-
-
Germline
yes
-
-
-
-
LOVD
+/., +?/.
2
4i
c.247+2T>C
r.spl
p.(?), p.?
-
likely pathogenic, pathogenic
g.38182104A>G
g.38322851A>G
c.247+2T>C; -
-
RPGR_000491
-
PubMed: Huang 2018
,
PubMed: Ivanova 2018
-
-
Germline
?
-
-
-
-
LOVD
+/.
2
-
c.247+5G>A
r.spl
p.?
-
pathogenic
g.38182101C>T
g.38322848C>T
RPGR c.247+5G>A
-
RPGR_000797
in vitro splicing assay: exon 3 skipping; hemizygous,
1 more item
PubMed: Kortum 2021
-
-
Germline
yes
-
-
-
-
LOVD
+/., +?/.
2
3
c.248-28_248-10del
r.(?), r.spl?
p.(?), p.?
-
likely pathogenic, pathogenic
g.38180356_38180374del
g.38321103_38321121del
-
-
RPGR_000072
this mutation was found in one female carrier
PubMed: Haer-Wigman 2017
,
PubMed: Talib 2018
-
-
Germline
yes
-
-
-
-
LOVD
+/.
1
-
c.248-6T>A
r.spl
p.?
-
pathogenic
g.38180348A>T
g.38321095A>T
IVS3-6T>A
-
RPGR_000451
-
PubMed: Sharon 2000
,
PubMed: Sharon 2003
-
-
Germline
-
-
-
-
-
LOVD
+/.
2
3i
c.248-2A>G
r.spl, r.spl?
p.(?), p.?
ACMG
pathogenic
g.38180344T>C
g.38321091T>C
c.248-2A>G, RPGR c.248-2A>G, p.(?)
-
RPGR_000595
-
PubMed: Jespersgaar 2019
,
PubMed: _Audo-2012
-
-
Germline, Unknown
?
-
-
-
-
LOVD
+/.
1
3i
c.248-1G>C
r.spl?
p.?
-
pathogenic
g.38180343C>G
-
c.248-1G>C
-
RPGR_000742
-
PubMed: Colombo-2020
-
-
Germline
yes
-
-
-
-
LOVD
+/.
1
3i
c.248-1G>T
r.spl?
p.?
-
pathogenic
g.38180343C>A
-
c.248-1G>T
-
RPGR_000619
-
PubMed: Wang-2013
-
-
Unknown
-
-
-
-
-
LOVD
+/.
1
3i_13
c.(?_248-1)_(*1091+1_?)del
r.(?)
p.?
-
pathogenic (dominant)
g.38143701_38180343del
-
delexon415 (Truncated)
-
RPGR_000570
-
PubMed: Prokisch 2007
-
-
Germline
-
-
-
-
-
LOVD
+/.
1
4_11
c.248_1414del
r.(?)
p.(Ala83_Pro471del)
-
pathogenic
g.38156537_38180342del
-
c.248_1414del
-
RPGR_000703
-
PubMed: Khateb-2016
-
-
Germline
-
-
-
-
-
LOVD
+?/.
1
-
c.255del
r.(?)
p.(Lys85Asnfs*5)
-
likely pathogenic
g.38180338del
g.38321085del
RPGR, variant 1: c.255del/p.K85Nfs*5
-
RPGR_000711
solved, hemizygous
PubMed: Weisschuh 2020
-
-
Unknown
?
-
-
-
-
LOVD
+/.
1
-
c.259G>T
r.(?)
p.(Glu87*)
ACMG
pathogenic
g.38180331C>A
-
-
-
RPGR_000176
-
PubMed: Sharon 2019
-
-
Germline
-
9/2420 IRD families
-
-
-
Global Variome, with Curator vacancy
+/.
1
4
c.274_277dup
r.(?)
p.(Ala93Glyfs*24)
-
pathogenic (recessive)
g.38180313_38180316dup
g.38321060_38321063dup
333_336dup4
-
RPGR_000305
-
Chung ARVO2003
-
-
Germline
-
-
-
-
-
LOVD
+/.
1
-
c.283G>A
r.(?)
p.(Gly95Arg)
-
pathogenic
g.38180307C>T
g.38321054C>T
-
-
RPGR_000490
-
PubMed: Vincent 2017
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/.
2
4
c.284G>A
r.(?)
p.(Gly95Glu)
ACMG
likely pathogenic, pathogenic
g.38180306C>T
g.38321053C>T
RPGR c.284G>A, p.(G95E)
-
RPGR_000478
-
PubMed: Huang 2017
,
PubMed: Xiao-2021
-
-
Germline, Unknown
yes
-
-
-
-
LOVD
+?/.
1
-
c.292C>G
r.(?)
p.(His98Asp)
-
likely pathogenic
g.38180298G>C
-
-
-
RPGR_000628
-
-
-
-
CLASSIFICATION record
-
-
-
-
-
MobiDetails
+/.
2
4
c.294C>A
r.(?)
p.(His98Gln)
-
pathogenic, pathogenic (recessive)
g.38180296G>T
g.38321043G>T
353C>A
-
RPGR_000303
-
PubMed: Meindl 1996
,
PubMed: Bader 2003
,
PubMed: Vervoort 2000
-
-
Germline
-
-
-
-
-
LOVD
+/.
1
-
c.295A>G
r.(?)
p.(Thr99Ala)
-
pathogenic
g.38180295T>C
g.38321042T>C
-
-
RPGR_000494
-
PubMed: Carrigan 2016
-
-
Germline
-
-
-
-
-
LOVD
+/.
1
4
c.295_296dup
r.(?)
p.(Leu100Profs*34)
-
pathogenic (recessive)
g.38180297_38180298dup
g.38321044_38321045dup
295-296insCA
-
RPGR_000304
-
PubMed: Weleber 1997
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/.
2
4
c.296C>A
r.(?)
p.(Thr99Asn)
-
likely pathogenic, pathogenic (recessive)
g.38180294G>T
g.38321041G>T
355C>A, RPGR, variant 1: c.296C>A/p.T99N
-
RPGR_000302
solved, hemizygous
PubMed: Miano 1999
,
PubMed: Weisschuh 2020
-
-
Germline, Unknown
?
-
-
-
-
LOVD
+/., +?/.
2
4
c.296C>T
r.(?)
p.(Thr99Ile)
-
likely pathogenic, pathogenic
g.38180294G>A
g.38321041G>A
c.296C>T (p.T99I)
-
RPGR_000102
hemizygous
PubMed: Fahim 2011
,
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
-
Germline, Germline/De novo (untested)
?
1/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
+/.
1
4
c.297_306del
r.(?)
p.(Leu100Glnfs*30)
-
pathogenic
g.38180284_38180293del
-
c.297_306del (p.Leu100Glnfs*30)
-
RPGR_000618
-
PubMed: Churchill-2013
-
-
Germline
-
-
-
-
-
LOVD
+?/., ?/.
3
4
c.299T>C
r.(?)
p.(Leu100Pro)
-
likely pathogenic, VUS
g.38180291A>G
g.38321038A>G
c.299T>C, RPGR, variant 1: c.299T>C/p.L100P
-
RPGR_000710
possibly solved, hemizygous
PubMed: Colombo-2020
,
PubMed: Weisschuh 2020
-
rs1064797366
Germline, Unknown
?
-
-
-
-
LOVD
+?/.
2
4i
c.301_310+3del
r.spl
p.(Val101Lysfs*29)
-
likely pathogenic
g.38180277_38180289del
g.38321024_38321036del
c.301_IVS4+3del
-
RPGR_000796
hemizygous
PubMed: Fahim 2011
-
-
Germline/De novo (untested)
?
-
-
-
-
LOVD
+/., +?/.
7
-
c.310+1G>A
r.spl
p.(?), p.?
-
likely pathogenic, pathogenic, pathogenic (recessive)
g.38180279C>T
g.38321026C>T
IVS4+1G>A, RPGR, variant 1: c.310+1G>A/p.?
-
RPGR_000450
solved, hemizygous
PubMed: Sharon 2000
,
PubMed: Sharon 2003
,
PubMed: Wang 2015
,
PubMed: Weisschuh 2020
,
PubMed: Xu 2014
-
-
Germline, Germline/De novo (untested)
yes
-
-
-
-
LOVD
+/.
2
-
c.310+1G>C
r.spl
p.?
-
pathogenic
g.38180279C>G
g.38321026C>G
IVS4+1G>C
-
RPGR_000449
-
PubMed: Breuer 2002
,
PubMed: Sharon 2000
,
PubMed: Sharon 2003
-
-
Germline
-
-
-
-
-
LOVD
+?/.
1
-
c.310+1G>T
r.spl?
p.?
ACMG
VUS
g.38180279C>A
-
-
-
RPGR_000589
-
-
-
-
Germline/De novo (untested)
-
-
-
-
-
Jinu Han
+/., +?/.
4
4i
c.310+3A>G
r.248_310del, r.spl, r.spl?
p.?
ACMG
likely pathogenic, likely pathogenic (maternal), pathogenic, pathogenic (recessive)
g.38180277T>C
g.38321024T>C
c.310+3A>G, IVS4+3A>G, NM_000328.2:c.310+3A>G, NP_000319.1:p.?, NC_000023.10:g.38180277T>C
-
RPGR_000301
different transcript (NM_000328.2) in publication, hemizygous
PubMed: Buraczynska 1997
,
PubMed: Fahim 2011
,
PubMed: Liu-2020
,
PubMed: Wang 2018
-
-
Germline, Germline/De novo (untested)
?
-
-
-
-
LOVD
-/.
1
-
c.310+10T>C
r.(=)
p.(=)
-
benign
g.38180270A>G
g.38321017A>G
RPGR(NM_001034853.2):c.310+10T>C
-
RPGR_000053
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
1
4i
c.311-9del13
r.spl?
p.?
-
pathogenic
g.38178249del
-
c.311-9del13
-
RPGR_000568
-
PubMed: Neidhardt 2008
-
-
Germline
-
-
-
-
-
LOVD
+?/.
1
5
c.329C>A
r.(?)
p.(Ala110Glu)
-
likely pathogenic
g.38178222G>T
g.38318969G>T
-
-
RPGR_000795
this mutation was found in one female carrier
PubMed: Talib 2018
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
1
-
c.334G>T
r.(?)
p.(Gly112Cys)
-
likely pathogenic
g.38178217C>A
g.38318964C>A
RPGR c.334G>T , p.(Gly112Cys)
-
RPGR_000567
hemizygous, also present in affected mother
PubMed: Avela 2019
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
1
5
c.334_336del
r.(?)
p.(Glu778_799delinsGlu)
-
likely pathogenic
g.38178216_38178218del
g.38318963_38318965del
-
-
RPGR_000794
this mutation was found in one female carrier
PubMed: Talib 2018
-
-
Germline/De novo (untested)
?
-
-
-
-
LOVD
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