Global Variome shared LOVD
RPGRIP1 (retinitis pigmentosa GTPase regulator inte...)
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Unique variants in the RPGRIP1 gene
This database is one of the
"Eye disease"
gene variant databases.
The variants shown are described using the NM_020366.3 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.
332 entries on 4 pages. Showing entries 1 - 100.
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Effect
Reported
Exon
DNA change (cDNA)
RNA change
Protein
Classification method
Clinical classification
DNA change (genomic) (hg19)
DNA change (hg38)
Published as
ISCN
DB-ID
Variant remarks
Reference
ClinVar ID
dbSNP ID
Origin
Segregation
Frequency
Re-site
VIP
Methylation
Owner
?/.
1
-
c.G2398G>A
r.(?)
p.(Glu800Lys)
-
VUS
g.21794020G>A
g.21325861G>A
G2398G>A
-
RPGRIP1_000007
-
PubMed: Wang 2015
-
-
Germline
-
-
-
-
-
LOVD
+/.
3
_1
-
r.?
p.?
-
likely pathogenic (recessive), pathogenic (recessive)
g.21744306_21748424del, g.21744993_21749459del
g.21276147_21280265del, g.21276834_21281300del
-
-
RPGRIP1_000303
microdeletion involving exon 1 of RPGRIP1 (NM_020366.4),
1 more item
Torii 2023, submitted
-
-
Germline
yes
-
-
-
-
Kaoruko Torii
+/.
1
_1_3i
c.-115_218+2395{0}
r.0?
p.0?
-
pathogenic (recessive)
g.21745430_21765363delinsN[330]
g.21277271_21297204delinsN[330]
-
-
RPGRIP1_000302
microdeletion involving exon 1 to 3
Torii 2023, submitted
-
-
Germline
yes
-
-
-
-
Kaoruko Torii
+/.
1
_1_2i
c.-1_(218+600_218+800){2}
r.?
p.?
-
pathogenic (recessive)
g.(21754036_21754236)_(21763568_21763768)del
g.(21285877_21286077)_(21295409_21295609)del
dup ex1-2
-
RPGRIP1_000236
deleterious duplication 2kb 5' of exon 1 to 0.7kb in intron 2
PubMed: Jamshidi 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., +?/., -/.
10
8, _1_22_
c.?
r.(?), r.0?, r.?
p.0?, p.?, p.D876G, p.G746E, p.R852Q, p.R890X, p.V857fs
-
benign, likely pathogenic, likely pathogenic (recessive), pathogenic
g.21780085G>C, g.21784106_21791536del, g.?
g.?
1033G?C, 630del (H198Tfs*50), chr14:g.21784106_21791536del, RPGRIP1 ex1-22del, RPGRIP1 p.D876G,
5 more items
-
CRYM_000000, IGF1R_000000, MYH2_000008, SERPINA1_000009, SMCHD1_000000
deletion exons 1-22, no breakpoints known; homozygous, heterozygous,
1 more item
PubMed: Ge 2015
,
PubMed: Huang 2017
,
PubMed: Li-2009
,
PubMed: Roepman 2005
,
PubMed: Turro 2020
,
1 more item
-
-
Germline, Germline/De novo (untested), In vitro (cloned)
?, yes
-
-
-
-
LOVD
-?/.
1
-
c.50T>C
r.(?)
p.(Ile17Thr)
-
likely benign
g.21756185T>C
-
RPGRIP1(NM_020366.4):c.50T>C (p.I17T)
-
RPGRIP1_000286
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+?/.
1
-
c.86-3T>G
r.spl
p.?
-
likely pathogenic (recessive)
g.21762833T>G
g.21294674T>G
-
-
RPGRIP1_000127
-
PubMed: DiIorio 2017
-
-
Germline
-
-
-
-
-
LOVD
+?/.
2
1i
c.86-1G>A
r.spl
p.?
-
likely pathogenic
g.21762835G>A
g.21294676G>A
-
-
RPGRIP1_000246
no variant 2nd chromosome
PubMed: Zou 2021
-
-
Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.86G>T
r.(?)
p.(Gly29Val)
-
VUS
g.21762836G>T
g.21294677G>T
RPGRIP1(NM_020366.3):c.86G>T (p.G29V)
-
RPGRIP1_000016
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
5
-
c.95A>T
r.(?)
p.(Met32Leu)
-
VUS
g.21762845T>A
g.21294686T>A
RPGRIP1 c.95T>A, M32L
-
RPGRIP1_000017
error in annotation, M32L is actually caused by c.95A>T and not c.95T>A; heterozygous
PubMed: Fernandez-Martinez 2011
-
-
Unknown
?
-
-
-
-
LOVD
-/., -?/.
3
-
c.95T>A
r.(?)
p.(Met32Lys)
-
benign, likely benign
g.21762845T>A
g.21294686T>A
RPGRIP1(NM_020366.3):c.95T>A (p.M32K), RPGRIP1(NM_020366.4):c.95T>A (p.M32K)
-
RPGRIP1_000017
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
,
VKGL-NL_AMC
?/.
1
-
c.116G>A
r.(?)
p.(Ser39Asn)
-
VUS
g.21762866G>A
-
RPGRIP1(NM_020366.4):c.116G>A (p.(Ser39Asn))
-
RPGRIP1_000322
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
-
c.127C>T
r.(?)
p.(Arg43Trp)
-
VUS
g.21762877C>T
-
RPGRIP1(NM_020366.4):c.127C>T (p.(Arg43Trp))
-
RPGRIP1_000323
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-/.
1
-
c.153T>C
r.(?)
p.(Phe51=)
-
benign
g.21762903T>C
g.21294744T>C
RPGRIP1(NM_020366.4):c.153T>C (p.F51=)
-
RPGRIP1_000096
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/., +?/.
8
2
c.154C>T
r.(?)
p.(Arg52*), p.Arg52*
ACMG
likely pathogenic, likely pathogenic (recessive), pathogenic
g.21762904C>T
g.21294745C>T
c.154C>T, RPGRIP1 c.154C>T, p.Arg52*, RPGRIP1 NM_020366: g.6807C>T, c.154C>T, p.R52X
-
RPGRIP1_000003
compound heterozygous
PubMed: Abu-Safieh-2013
,
PubMed: Huang 2017
,
PubMed: Liu-2020
,
PubMed: Xu 2020
,
PubMed: Zenteno 2020
-
-
Germline
yes
1/143 cases
-
-
-
Johan den Dunnen
,
Leen Abu Safieh
+/.
1
-
c.176del
r.(?)
p.(Leu59TrpfsTer2)
-
pathogenic
g.21762926del
g.21294767del
RPGRIP1(NM_020366.4):c.176delT (p.L59Wfs*2)
-
RPGRIP1_000074
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/., +?/.
2
-
c.194G>A
r.(?)
p.(Trp65*)
ACMG
likely pathogenic, pathogenic
g.21762944G>A
g.21294785G>A
RPGRIP1 Trp65Ter,
1 more item
-
RPGRIP1_000205
no nucleotide annotation, extrapolated from sequence; heterozygous,
1 more item
PubMed: Dryja 2001
,
PubMed: Jespersgaar 2019
-
-
Germline
?, yes
-
-
-
-
LOVD
+?/.
1
2i
c.218+2T>A
r.spl?
p.(?)
-
likely pathogenic
g.21762970T>A
-
c.218+2T>A
-
RPGRIP1_000291
-
PubMed: Panneman 2023
-
-
Unknown
-
-
-
-
-
Daan Panneman
?/.
1
-
c.218+6T>C
r.(=)
p.(=)
-
VUS
g.21762974T>C
-
RPGRIP1(NM_020366.3):c.218+6T>C
-
RPGRIP1_000119
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/.
1
-
c.218+13C>G
r.(=)
p.(=)
-
benign
g.21762981C>G
g.21294822C>G
RPGRIP1(NM_020366.4):c.218+13C>G
-
RPGRIP1_000018
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
1
1i_2i
c.218+2563_218+2564ins[85+1939_218+2415;218+2551_218+2563]
r.?
p.?
-
pathogenic (recessive)
g.21765531_21765532ins[21758159_21765383;21765519_21765531]
g.21297372_21297373ins[21290000_21297224;21297360_21297372]
-
-
RPGRIP1_000241
-
PubMed: Jamshidi 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
3
c.242T>A
r.(?)
p.(Leu81*)
-
pathogenic
g.21769148T>A
-
242T>A
-
RPGRIP1_000190
-
PubMed: li 2011
-
-
Germline
-
1/87 cases; 0/96 controls
-
-
-
LOVD
+/., -/.
2
-
c.256C>T
r.(?)
p.(Arg86Trp)
-
benign, pathogenic
g.21769162C>T
g.21301003C>T
RPGRIP1(NM_020366.4):c.256C>T (p.R86W)
-
RPGRIP1_000133
VKGL data sharing initiative Nederland
PubMed: Costa 2017
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
VKGL-NL_AMC
+?/., ?/.
2
-
c.268G>A
r.(?)
p.(Val90Ile)
ACMG
likely pathogenic, VUS
g.21769174G>A
g.21301015G>A
RPGRIP1, variant 1: c.2662C>T/p.R888* , variant 2: c.268G>A/p.V90I
-
RPGRIP1_000217
ACMG PM2, PP5, BP4, possibly solved, compound heterozygous
PubMed: Weisschuh 2020
,
PubMed: Weisschuh 2024
-
-
Germline, Unknown
?
-
-
-
-
Johan den Dunnen
-/.
1
3
c.286_287insATA
r.(?)
p.(Pro96delinsHisThr)
-
benign
g.21769192_21769193insATA
-
IVS6-16ˆ-15insATA
-
RPGRIP1_000141
-
PubMed: Booij 2005
-
-
Germline
-
-
-
-
-
Julia Lopez
-/., -?/.
3
-
c.287C>A
r.(?)
p.(Pro96Gln)
-
benign, likely benign
g.21769193C>A
g.21301034C>A
RPGRIP1(NM_020366.3):c.287C>A (p.P96Q)
-
RPGRIP1_000019
209 heterozygous;
Clinindb (India)
, 5 homozygous;
Clinindb (India)
,
1 more item
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs1040904
CLASSIFICATION record, Germline
-
209/2795 individuals, 5/2795 individuals
-
-
-
VKGL-NL_Rotterdam
,
Mohammed Faruq
?/.
1
-
c.320_322del
r.(?)
p.(Ala107del)
-
VUS
g.21769226_21769228del
g.21301067_21301069del
c.320_322delCGG
-
RPGRIP1_000176
-
PubMed: Wang 2014
-
-
Germline
-
-
-
-
-
LOVD
+?/.
1
-
c.331C>T
r.(?)
p.(Gln111Ter)
ACMG
likely pathogenic (recessive)
g.21769237C>T
g.21301078C>T
-
-
RPGRIP1_000304
ACMG PM2, PVS1
PubMed: Weisschuh 2024
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.353G>A
r.(?)
p.(Arg118His)
-
VUS
g.21769259G>A
g.21301100G>A
RPGRIP1(NM_020366.4):c.353G>A (p.R118H)
-
RPGRIP1_000075
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
3
3
c.358C>T
r.(?)
p.(Gln120*)
-
pathogenic
g.21769264C>T
-
358C>T
-
RPGRIP1_000191
-
PubMed: li 2011
-
-
Germline
-
3/87 cases; 0/96 controls
-
-
-
LOVD
+/.
2
-
c.367C>T
r.(?)
p.(Arg123*), p.(Arg123Ter)
ACMG
pathogenic
g.21769273C>T
g.21301114C>T
RPGRIP1 NM_020366: g.13176C>T, c.367C>T, p.R123X
-
RPGRIP1_000162
single heterozygous
PubMed: Wang 2015
,
PubMed: Xu 2020
-
-
Germline, Unknown
?
-
-
-
-
LOVD
-?/.
1
-
c.372G>A
r.(?)
p.(Leu124=)
-
likely benign
g.21769278G>A
-
RPGRIP1(NM_020366.3):c.372G>A (p.L124=)
-
RPGRIP1_000120
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.376G>C
r.(?)
p.(Gly126Arg)
-
likely benign
g.21769282G>C
g.21301123G>C
RPGRIP1(NM_020366.4):c.376G>C (p.G126R)
-
RPGRIP1_000020
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
1
-
c.403A>C
r.(?)
p.(Ser135Arg)
-
VUS
g.21769309A>G
g.21301150A>G
RPGRIP1 c.403A>G, S135R
-
RPGRIP1_000263
1 more item
PubMed: Fernandez-Martinez 2011
-
-
Unknown
?
-
-
-
-
LOVD
+?/.
1
3
c.416C>T
r.(?)
p.(Ala139Val)
-
likely pathogenic
g.21769322C>T
g.21301163C>T
-
-
RPGRIP1_000247
no variant 2nd chromosome
PubMed: Zou 2021
-
-
Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.420del
r.(?)
p.(Gln140Hisfs*30)
-
likely pathogenic
g.21769326del
g.21301167del
RPGRIP1 c.[420delG]
-
RPGRIP1_000282
homozygous
PubMed: Abouzeid 2016
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
1
3
c.430C>T
r.(?)
p.(Gln144*)
-
likely pathogenic
g.21769336C>T
-
c.430C>T
-
RPGRIP1_000292
-
PubMed: Panneman 2023
-
-
Unknown
-
-
-
-
-
Daan Panneman
-/.
2
-
c.450C>G
r.(?)
p.(Leu150=)
-
benign
g.21769356C>G
g.21301197C>G
RPGRIP1(NM_020366.3):c.450C>G (p.L150=), RPGRIP1(NM_020366.4):c.450C>G (p.L150=)
-
RPGRIP1_000021
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
-?/.
1
-
c.453C>T
r.(?)
p.(His151=)
-
likely benign
g.21769359C>T
-
RPGRIP1(NM_020366.3):c.453C>T (p.H151=)
-
RPGRIP1_000121
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/.
1
3
c.473C>T
r.(?)
p.(Pro158Leu)
-
likely pathogenic
g.21769379C>T
g.21301220C>T
-
-
RPGRIP1_000248
no variant 2nd chromosome
PubMed: Zou 2021
-
-
Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.487del
r.(?)
p.(Arg163GlyfsTer7)
-
likely pathogenic
g.21769393del
g.21301234del
-
-
RPGRIP1_000160
-
PubMed: Patel 2016
-
-
Germline
-
-
-
-
-
LOVD
+/.
2
-
c.490+1G>T
r.spl
p.?
-
pathogenic
g.21769397G>T
g.21301238G>T
-
-
RPGRIP1_000163
-
PubMed: Wang 2015
-
-
Germline
-
-
-
-
-
LOVD
?/.
1
3i
c.491-11T>A
r.(=)
p.(?)
-
VUS
g.21770636T>A
-
c.491-11T>A
-
RPGRIP1_000293
-
PubMed: Panneman 2023
-
-
Unknown
-
-
-
-
-
Daan Panneman
-/., -?/.
2
-
c.491-3T>C
r.spl?
p.?
-
benign, likely benign
g.21770644T>C
g.21302485T>C
RPGRIP1(NM_020366.3):c.491-3T>C, RPGRIP1(NM_020366.4):c.491-3T>C
-
RPGRIP1_000076
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
+?/.
2
-
c.511del
r.(?)
p.(Tyr171Thrfs*19)
-
likely pathogenic
g.21770667del
g.21302508del
RPGRIP1 delT511
-
RPGRIP1_000264
obsolete annotation, extrapolated from sequence; heterozygous,
1 more item
PubMed: Gerber 2001
-
-
Germline
yes
-
-
-
-
LOVD
-/.
2
4
c.525A>G
r.(=), r.(?)
p.(=), p.(Pro175=)
-
benign
g.21770681A>G
g.21302522A>G
525A>G, RPGRIP1(NM_020366.3):c.525A>G (p.P175=)
-
RPGRIP1_000022
VKGL data sharing initiative Nederland
PubMed: Dryja 2001
;
PubMed: Booij 2005
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Julia Lopez
,
VKGL-NL_Rotterdam
+/., +?/., -/., -?/.
10
4
c.535del
r.(?)
p.(Glu179Serfs*11), p.(Glu179SerfsTer11)
-
benign, likely benign, likely pathogenic, likely pathogenic (recessive), pathogenic
g.21770691del, g.21770691delG
g.21302532del
534delG, 535delG, c.534delG p.K178fs, c.535delG, c.[535delG];[535delG],
1 more item
-
RPGRIP1_000023, RPGRIP1_000192
VKGL data sharing initiative Nederland
PubMed: Chen-2013
,
PubMed: Huang 2016
,
PubMed: li 2011
,
PubMed: Wang 2015
,
PubMed: Wang 2016
-
-
CLASSIFICATION record, Germline, Unknown
-
0/384 controls, 1/87 cases; 0/96 controls
-
-
-
Johan den Dunnen
,
VKGL-NL_Rotterdam
+/.
3
-
c.535delG
r.(?)
p.(Glu179Serfs*11)
ACMG
pathogenic
g.21770691del
g.21302532del
RPGRIP1 NM_020366: g.14594delG, c.535delG, p.E179Sfs11
-
RPGRIP1_000023
-
PubMed: Xu 2020
-
-
Germline
yes
-
-
-
-
LOVD
-/.
1
4
c.560G>T
r.(?)
p.(Gly187Val)
-
benign
g.21770716G>T
-
560G?T
-
RPGRIP1_000177
-
PubMed: Li-2009
-
-
Germline
-
-
-
-
-
LOVD
?/.
1
-
c.563A>C
r.(?)
p.(Glu188Ala)
-
VUS
g.21770719A>C
g.21302560A>C
RPGRIP1(NM_020366.3):c.563A>C (p.E188A)
-
RPGRIP1_000024
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/.
1
-
c.564A>G
r.[(565_587del,491_587del)]
p.?
-
likely pathogenic
g.21770720A>G
g.21302561A>G
-
-
RPGRIP1_000134
effect on splicing predicted from mini-gene splicing assay
PubMed: Soens 2017
-
-
Germline
-
-
-
-
-
LOVD
+/., -/.
6
4
c.574A>G
r.(?)
p.(Lys192Glu)
-
benign, likely benign
g.21770730A>G
g.21302571A>G
574A>G, c.574A>G,
1 more item
-
RPGRIP1_000001
predicted benign, VKGL data sharing initiative Nederland
PubMed: Dryja 2001
;
PubMed: Booij 2005
,
PubMed: Jonsson-2013
,
PubMed: Neveling 2012
-
-
CLASSIFICATION record, Germline, Unknown
no
-
-
-
-
Julia Lopez
,
Kornelia Neveling
,
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
+?/.
1
4i
c.587+1G>C
r.spl?
p.?
-
likely pathogenic
g.21770744G>C
-
-
-
RPGRIP1_000184
-
PubMed: mckibbin-2010
-
-
Germline
-
-
-
-
-
LOVD
+?/.
1
-
c.587+2T>G
r.spl
p.?
ACMG
likely pathogenic
g.21770745T>G
g.21302586T>G
-
-
RPGRIP1_000313
-
-
-
-
Germline
yes
-
-
-
-
Li Zhang
+?/.
1
-
c.631del
r.(?)
p.(Ser211ValfsTer64)
ACMG
likely pathogenic
g.21771533del
g.21303374del
-
-
RPGRIP1_000315
no variant 2nd chromosome, case unsolved
PubMed: Hitti-Malin 2024
,
Journal: Hitti-Malin 2024
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
2
5
c.673del
r.(?)
p.(His225Thrfs*50), p.(His225ThrfsTer50)
-
likely pathogenic
g.21771575del
g.21303416del
c.673delC
-
RPGRIP1_000107
no variant 2nd chromosome
PubMed: Holtan 2020
,
PubMed: Zou 2021
-
-
Germline, Germline/De novo (untested)
-
2/899 cases
-
-
-
Global Variome, with Curator vacancy
,
Johan den Dunnen
-?/.
1
-
c.675C>T
r.(?)
p.(His225=)
-
likely benign
g.21771577C>T
g.21303418C>T
RPGRIP1(NM_020366.3):c.675C>T (p.H225=)
-
RPGRIP1_000097
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/., +?/.
2
-
c.711del
r.(?)
p.(Lys239Serfs*36)
-
likely pathogenic, pathogenic (recessive)
g.21771613del
g.21303454del
711delA, 711_711del1
-
RPGRIP1_000135
-
PubMed: Jamshidi 2019
,
PubMed: Stone 2017
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.711delA
r.(?)
p.(Lys239Serfs*36)
ACMG
pathogenic
g.21771613del
g.21303454del
1 more item
-
RPGRIP1_000135
heterozygous, ACMG classified, novel (Table 2)
PubMed: Hull 2020
-
-
Germline
?
-
-
-
-
LOVD
+/., +?/.
2
-
c.767C>G
r.(?)
p.(Ser256*)
-
likely pathogenic (recessive), pathogenic (recessive)
g.21771669C>G
g.21303510C>G
-
-
RPGRIP1_000128
-
PubMed: Bryant 2018
,
PubMed: Jamshidi 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
5
c.775T>C
r.(?)
p.(Cys259Arg)
-
likely pathogenic
g.21771677T>C
g.21303518T>C
-
-
RPGRIP1_000249
no variant 2nd chromosome
PubMed: Zou 2021
-
-
Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.783G>A
r.(?)
p.(Gln261=)
-
likely benign
g.21771685G>A
g.21303526G>A
RPGRIP1(NM_020366.4):c.783G>A (p.Q261=)
-
RPGRIP1_000077
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/., +?/.
7
5
c.799C>T
r.(?)
p.(Arg267*), p.(Arg267Ter)
ACMG
likely pathogenic (recessive), pathogenic, pathogenic (recessive)
g.21771701C>T
g.21303542C>T
799C>T, c.799C>T, RPGRIP1(NM_020366.4):c.799C>T (p.(Arg267*))
-
RPGRIP1_000026
heterozygous, causative variant, VKGL data sharing initiative Nederland
PubMed: Haer-Wigman 2017
,
PubMed: Hosono 2018
, Torii 2023, submitted,
PubMed: li 2011
,
2 more items
-
-
CLASSIFICATION record, Germline
yes
1/87 cases; 0/96 controls
-
-
-
Johan den Dunnen
,
VKGL-NL_Leiden
,
VKGL-NL_Nijmegen
,
Kaoruko Torii
?/.
1
-
c.800G>A
r.(?)
p.(Arg267Gln)
ACMG
VUS
g.21771702G>A
g.21303543G>A
RPGRIP1 c.800G>A; p.Arg267GIn
-
RPGRIP1_000223
heterozygous
PubMed: Sallum 2020
-
-
Unknown
?
-
-
-
-
LOVD
+/., +?/.
5
5i
c.800+1G>A
r.spl, r.spl?
p.(?), p.?
ACMG
likely pathogenic, pathogenic, pathogenic (recessive)
g.21771703G>A
g.21303544G>A
allele 1: c.800+1G>A/p.?, allele 2: c.2718dup/p.N907*, c.800+1G>A, RPGRIP1 c.800+1G>A; p.?,
1 more item
-
RPGRIP1_000206
ACMG PM2, PVS1_STRONG, PP5_STRONG, heterozygous, homozygous, solved, compound heterozygous
PubMed: Panneman 2023
,
PubMed: Sallum 2020
,
PubMed: Weisschuh 2018
,
PubMed: Weisschuh 2020
,
1 more item
-
-
Germline, Unknown
?, yes
-
-
-
-
Johan den Dunnen
,
Daan Panneman
+?/.
1
-
c.801-25_843del
r.spl
p.(?)
ACMG
likely pathogenic
g.21775865_21775932del
g.21307706_21307773del
allele 1: c.1303A>T/p.K435*, allele 2: c.801-25_c.843del
-
RPGRIP1_000207
heterozygous
PubMed: Weisschuh 2018
-
-
Germline
yes
-
-
-
-
LOVD
-?/., ?/.
2
-
c.808A>G
r.(?)
p.(Ile270Val)
-
likely benign, VUS
g.21775897A>G
g.21307738A>G
RPGRIP1(NM_020366.3):c.808A>G (p.I270V), RPGRIP1(NM_020366.4):c.808A>G (p.I270V)
-
RPGRIP1_000027
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
+/.
1
-
c.832C>T
r.(?)
p.(Arg278Ter)
-
pathogenic (recessive)
g.21775921C>T
g.21307762C>T
-
-
RPGRIP1_000309
-
PubMed: Zeuli 2024
-
-
Germline
-
-
-
-
-
Susanne Roosing
+/., +?/.
2
-
c.832del
r.(?)
p.(Arg278Aspfs*15)
ACMG
likely pathogenic, pathogenic
g.21775921del
g.21307762del
RPGRIP1 c.832del, p.(Arg278Aspfs*15),
1 more item
-
RPGRIP1_000224
heterozygous, ACMG classified, novel (Table 2), heterozygous, probably non-causal incidental finding
PubMed: Hull 2020
,
PubMed: Zhu 2022
-
-
Germline, Germline/De novo (untested)
?
-
-
-
-
LOVD
-?/.
1
-
c.860A>G
r.(?)
p.(Asn287Ser)
-
likely benign
g.21775949A>G
g.21307790A>G
RPGRIP1(NM_020366.4):c.860A>G (p.N287S)
-
RPGRIP1_000028
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
1
-
c.871_872ins[N[(300_400)];854_871]
r.?
p.(Val291Glyfs*32)
ACMG
pathogenic (recessive)
g.21775960_21775961ins[N[(300_400)];21775943_21775960]
g.21307801_21307802ins[N[(300_400)];21307784_21307801]
-
-
RPGRIP1_000299
AluY insertion with an 18-bp target site duplication
Torii 2023, submitted
-
-
Germline
yes
-
-
-
-
Kaoruko Torii
+/., +?/.
3
6
c.895_896del
r.(?)
p.(Glu299Serfs*21), p.(Glu299SerfsTer21)
-
likely pathogenic, pathogenic
g.21775984_21775985del
g.21307825_21307826del
c.895_896del
-
RPGRIP1_000013
-
PubMed: de Castro-Miró 2014
,
PubMed: de Castro-Miró-2014
,
PubMed: Riera 2017
-
-
Germline
yes
-
-
-
-
Marta de Castro-Miró
+/.
1
-
c.895_896delGA
r.(?)
p.(Glu299Serfs*21)
-
pathogenic (recessive)
g.21775984_21775985delGA
g.21307825_21307826del
895_896delGA
-
RPGRIP1_000237
-
PubMed: Jamshidi 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.901C>T
r.(?)
p.(Gln301Ter)
-
pathogenic
g.21775990C>T
g.21307831C>T
RPGRIP1(NM_020366.4):c.901C>T (p.Q301*)
-
RPGRIP1_000078
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
1
11i
c.903_906+17del
r.spl
p.(Glu302Hisfs*13)
-
pathogenic
g.21775992_21776012del
g.21307833_21307853del
RPGRIP1 c.900_906+14delTCAAGAGGTGAGTTGCCATCA
-
RPGRIP1_000283
double homozygous
PubMed: Tallapaka 2019
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
1
-
c.906+2T>G
r.spl
p.(?)
-
likely pathogenic
g.21775997T>G
g.21307838T>G
RPGRIP1, variant 1: c.906+2T>G/p.? , variant 2: c.1612-3C>A/p.?
-
RPGRIP1_000218
possibly solved, compound heterozygous
PubMed: Weisschuh 2020
-
-
Unknown
?
-
-
-
-
LOVD
-/.
1
-
c.906+15_906+17del
r.(=)
p.(=)
-
benign
g.21776010_21776012del
g.21307851_21307853del
RPGRIP1(NM_020366.4):c.906+15_906+17delTCA
-
RPGRIP1_000029
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/.
1
6i
c.907-154_907-153del
r.(=)
p.(=)
-
benign
g.21778589_21778590del
-
IVS6-153_-154delGG
-
RPGRIP1_000142
-
PubMed: Booij 2005
-
-
Germline
-
-
-
-
-
Julia Lopez
+/.
1
6i
c.907-17_907-15delTAA
r.(=)
p.(=)
-
likely benign
g.21778726_21778728delTAA
-
c.907-17_907-15delTAA
-
RPGRIP1_000199
-
PubMed: Jonsson-2013
-
-
Unknown
-
-
-
-
-
LOVD
-/.
2
-
c.907-16_907-14del
r.(=)
p.(=)
-
benign
g.21778727_21778729del
g.21310568_21310570del
RPGRIP1(NM_020366.3):c.907-16_907-14delAAT, RPGRIP1(NM_020366.4):c.907-16_907-14delAAT
-
RPGRIP1_000030
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
,
VKGL-NL_Utrecht
+/.
1
-
c.930+1G>A
r.spl
p.?
-
pathogenic (recessive)
g.21778767G>A
g.21310608G>A
-
-
RPGRIP1_000171
-
PubMed: Maria 2015
-
-
Germline
-
-
-
-
-
LOVD
-/., ?/.
2
-
c.930+3A>G
r.spl?
p.(?), p.?
ACMG
benign, VUS
g.21778769A>G
g.21310610A>G
RPGRIP1(NM_020366.4):c.930+3A>G, RPGRIP1:NM_020366 c.930+3A>G, p.?
-
RPGRIP1_000031
heterozygous, individual unsolved, causality of variants unknown,
1 more item
PubMed: Rodriguez-Munoz 2020
-
-
CLASSIFICATION record, Germline
?
-
-
-
-
VKGL-NL_AMC
-?/.
1
-
c.930+4_930+7del
r.spl?
p.?
-
likely benign
g.21778770_21778773del
-
RPGRIP1(NM_020366.3):c.930+4_930+7delCTTA
-
RPGRIP1_000287
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/.
1
-
c.930+77A>G
r.spl
p.?
-
pathogenic (recessive)
g.21778843A>G
g.21310684A>G
-
-
RPGRIP1_000310
-
PubMed: Perrault 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
8
c.932del
r.(?)
p.(Asn311Ilefs*5)
-
pathogenic
g.21779984del
g.21311825del
931delA
-
RPGRIP1_000012
-
PubMed: Li 2017
-
-
Germline
yes
-
-
-
-
James Hejtmancik
+/.
1
-
c.934dup
r.(?)
p.(Gln312Profs*9)
-
pathogenic (recessive)
g.21779986dup
g.21311827dup
934dupC
-
RPGRIP1_000243
-
PubMed: Zou 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.953C>T
r.(?)
p.(Ala318Val)
-
VUS
g.21780005C>T
g.21311846C>T
RPGRIP1 c.953C>T, A318V
-
RPGRIP1_000265
heterozygous
PubMed: Fernandez-Martinez 2011
-
-
Unknown
?
-
-
-
-
LOVD
+?/.
1
-
c.964_967dup
r.(?)
p.(Leu323ProfsTer7)
-
likely pathogenic
g.21780016_21780019dup
g.21311857_21311860dup
c.961_962insCCCT p.A321fs
-
RPGRIP1_000145
-
PubMed: Wang 2016
-
-
Germline
-
-
-
-
-
LOVD
+?/.
1
8
c.1015A>G
r.(?)
p.(Lys339Glu)
-
likely pathogenic
g.21780067A>G
g.21311908A>G
-
-
RPGRIP1_000250
no variant 2nd chromosome
PubMed: Zou 2021
-
-
Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.1078-9C>A
r.(=)
p.(=)
-
likely benign
g.21780583C>A
-
RPGRIP1(NM_020366.4):c.1078-9C>A
-
RPGRIP1_000307
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+?/.
9
_10_18_
c.(1077+1_1078-1)_(2895+1_2896-1)del
r.spl
p.(?)
ACMG
likely pathogenic
g.?
g.?
RPGRIP1 Exon 10-18 deletion; p.*, RPGRIP1 Exon 10-18 deletion; p.?
-
SERPINA1_000009
heterozygous, homozygous
PubMed: Sallum 2020
-
-
Unknown
?
-
-
-
-
LOVD
+/., +?/.
2
-
c.1087_1090del
r.(?)
p.(Arg363Leufs*11)
-
likely pathogenic (recessive), pathogenic (recessive)
g.21780601_21780604del
g.21312442_21312445del
1084_1087del
-
RPGRIP1_000129
-
PubMed: Bryant 2018
,
PubMed: Jamshidi 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.1088G>C
r.(?)
p.(Arg363Thr)
-
VUS
g.21780602G>C
g.21312443G>C
RPGRIP1 c.1088G>C, R363T
-
RPGRIP1_000266
heterozygous
PubMed: Fernandez-Martinez 2011
-
-
Unknown
?
-
-
-
-
LOVD
+/.
2
9
c.1089_1090dup
r.(?)
p.(Val364Glufs*12)
-
pathogenic
g.21780603_21780604dup
-
c.1083_1084insGA
-
RPGRIP1_000200
-
PubMed: Wang-2013
-
-
Unknown
-
-
-
-
-
LOVD
+/., +?/., ?/.
33
9
c.1107del
r.(?)
p.(Glu370Asnfs*5), p.(Glu370AsnfsTer5)
ACMG
likely pathogenic, likely pathogenic (recessive), pathogenic, VUS
g.21780621del
g.21312462del
1107delA, c.1107del, c.1107delA, RPGRIP1 c.1107del p.(Glu370Asnfs *5), RPGRIP1 Lys342(1-bp del),
3 more items
-
RPGRIP1_000006
homozygous, no nucleotide annotation, extrapolated from sequence; homozygous,
2 more items
PubMed: Abu-Safieh-2013
,
PubMed: Alfares 2018
,
PubMed: Dryja 2001
,
PubMed: Eisenberger-2013
,
6 more items
-
rs61751266
CLASSIFICATION record, Germline, Germline/De novo (untested), Unknown
?, yes
frequency in 1500 in-house samples: 0
-
-
-
Johan den Dunnen
,
Leen Abu Safieh
,
VKGL-NL_AMC
+/., +?/.
4
-
c.1111C>T
r.(?)
p.(Arg371*)
ACMG
likely pathogenic, pathogenic
g.21780625C>T
g.21312466C>T
c.1111C>T, p.(Arg371*), RPGRIP1 NM_020366: g.24528C>T, c.1111C>T, p.R371X,
1 more item
-
RPGRIP1_000210
compound heterozygous, solved, compound heterozygous
PubMed: Wang 2019
,
PubMed: Weisschuh 2020
,
PubMed: Xu 2020
-
-
Germline
?, yes
-
-
-
-
LOVD
+/.
1
-
c.1113del
r.(?)
p.(Lys372Asnfs*3)
-
pathogenic (recessive)
g.21780627del
-
14:21780626GA>G ENST00000400017.2:c.1116delA (Lys372AsnfsTer3)
-
RPGRIP1_000114
-
PubMed: Carss 2017
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/.
3
9
c.1116del
r.(?)
p.(Lys372Asnfs*3)
ACMG
likely pathogenic, pathogenic, pathogenic (recessive)
g.21780630del
g.21312471del
1116delA, RPGRIP1 c.1116delA, p.Lys372AsnfsTer3
-
RPGRIP1_000072
ACMG PVS1, PM2, PP4, heterozygous
PubMed: Turro 2020
,
PubMed: Zenteno 2020
-
-
Germline, Germline/De novo (untested)
?
1/143 cases
-
-
-
Johan den Dunnen
,
Juan Carlos Zenteno
-?/.
1
-
c.1128C>A
r.(?)
p.(Asp376Glu)
-
likely benign
g.21780642C>A
g.21312483C>A
RPGRIP1(NM_020366.3):c.1128C>A (p.D376E)
-
RPGRIP1_000098
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/.
1
9
c.1148_1151del
r.(?)
p.(Glu383Alafs*19)
-
likely pathogenic
g.21780662_21780665del
-
c.1148_1151del (p.(Glu383Alafs*19))
-
RPGRIP1_000231
-
PubMed: SkorczykWerner-2020
-
-
Germline
-
-
-
-
-
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