Legend
Please note that a short description of a certain column can be displayed when you move your mouse cursor over the column's header and hold it still. Below, a more detailed description is shown per column.
Effect: The variant's effect on the function of the gene/protein, displayed in the format 'R/C'. R is the value reported by the source (publication, submitter) and this classification may vary between records. C is the value concluded by the curator. Note that in some database the curator uses Summary records to give details on the classification of the variant.Values used: '+' indicating the variant affects function, '+?' probably affects function, '-' does not affect function, '-?' probably does not affect function, '?' effect unknown, '.' effect was not classified.
Exon: number of exon/intron containing variant; 2 = exon 2, 12i = intron 12, 2i_7i = from intron 2 to intron 7, 8i_9 = intron 8/exon 9 boundary, _1 = 5' to exon 1, 18_ = 3' of exon 18, _1_18_ = encompassing the entire 18-exon gene
DNA change (cDNA): description of variant at DNA level, based on a coding DNA reference sequence (following HGVS recommendations); e.g. c.123C>T, c.123_145del, c.123_126dup. For deletions/duplications extending beyond the reference transcript resp. {0}/{2} is used to replace del/dup. Extent of the deletion/duplication should be specified using the genomic description (g.). "-" indicates the variant described on genomic level does not affect the coding DNA reference sequence.
RNA change: description of variant at RNA level (following HGVS recommendations).
- r.123c>u
- r.? = unknown
- r.(?) = RNA not analysed but probably transcribed copy of DNA variant
- r.spl? = RNA not analysed but variant probably affects splicing
- r.(spl?) = RNA not analysed but variant may affect splicing
- r.0? = change expected to abolish transcription
Protein: description of variant at protein level (following HGVS recommendations).
- p.(Arg345Pro) = change predicted from DNA (RNA not analysed)
- p.Arg345Pro = change derived from RNA analysis
- p.? = unknown effect
- p.0? = probably no protein produced
Allele: On which allele is the variant located? Does not necessarily imply inheritance! 'Paternal' (confirmed or inferred), 'Maternal' (confirmed or inferred), 'Parent #1' or #2 for compound heterozygosity without having screened the parents, 'Unknown' for heterozygosity without having screened the parents, 'Both' for homozygozity.
Classification method: The method used for the clinical classification of this variant.
All options:
- ACMG
- ACGS
- EAHAD-CFDB
- ENIGMA
- IARC
- InSiGHT
- kConFab
- other
Clinical classification: Clinical classification of variant, preferably based on standardised criteria (e.g. ACMG), directed on the clinical consequences as published/submitted, indicated using an enriched system including inheritance: e.g. pathogenic, pathogenic (dominant), pathogenic (recessive), pathogenic (!), pathogenic (maternal), pathogenic (paternal). Standard inheritance is covered by dominant/recessive, imprinting by maternal/paternal. A '!' warns for exceptional circumstances to be explained in the 'Remarks' field (low penetrance, variants pathogenic in heterozygous state only, hypomorphic/hypermorphic variants, protective variants, etc.). Non-disease consequences (e.g. drug metabolism (pharmacogenetics), risk factor, blood group, tasting bitter) are indicated using additions to the benign classification; benign (dominant), benign (recessive), benign (!), etc. The value 'association' is used for variants associated with a phenotype and 'NA' for variants from in vitro/in silico records. NOTE: classification may differ from the opinion of the curator as given in a variant SUMMARY-record or the 'Functional effect concluded'). NOTE: pathogenic/likely pathogenic should go together with "variant (probably) affects function" In ClassFunctional.
All options:
- pathogenic
- pathogenic (dominant)
- pathogenic (recessive)
- pathogenic (!)
- pathogenic (maternal)
- pathogenic (paternal)
- likely pathogenic
- likely pathogenic (dominant)
- likely pathogenic (recessive)
- likely pathogenic (!)
- likely pathogenic (maternal)
- likely pathogenic (paternal)
- VUS
- VUS (!)
- likely benign
- likely benign (dominant)
- likely benign (recessive)
- likely benign (!)
- likely benign (maternal)
- likely benign (paternal)
- benign
- benign (dominant)
- benign (recessive)
- benign (!)
- benign (maternal)
- benign (paternal)
- conflicting
- association
- NA
DNA change (genomic) (hg19): HGVS description of variant at DNA level, based on the genomic (chromosomal) DNA reference sequence; e.g. g.12345678C>T, g.12345679del, g.12345678_12345890dup
DNA change (hg38): HGVS description of variant at DNA level, based on the hg38 genomic (chromosomal) eference sequence; e.g. g.12345678C>T, g.12345679del, g.12345678_12345890dup
Published as: listed only when different from "DNA change"; variant as reported originally (e.g. 521delT). Variants seen in animal models, tested in vitro, predicted from RNA analysis, etc. are described between brackets like c.(456C>G)
ISCN: description of the variant according to ISCN nomenclature
DB-ID: database ID of variant, grouping multiple observations of the same variant together, starting with the HGNC gene symbol, followed by an underscore (_) and a six digit number (e.g. DMD_012345). _000000 is used for variants where DNA was not analysed (change predicted from RNA analysis), variants seen in animal models or variants not seen in humans but functionally tested in vitro
Variant remarks: remarks regarding variant described, e.g. germline mosaicism in mother, 345 kb deletion, muscle RNA analysed, not in 200 control chromosomes tested, on founder haplotype, etc.
Reference: publication describing the variant submitted, incl. links to OMIM, PubMed or other source, e.g. "den Dunnen ASHG2003 P2346"
ClinVar ID: ID of variant in ClinVar database
dbSNP ID: the dbSNP ID
Origin: Origin of variant/record: Germline = in all cells, De novo = in all cells, but not in either parent, Germline/De novo (untested) = in all cells, parents not tested (use only when De novo is likely, e.g. isolated/sporadic cases with dominant disease), Somatic = present in a subset of cells, but not in either parent, Uniparental disomy = from parental disomy (maternal or paternal), CLASSIFICATION record = submitter only sharing variant classification (note another report may share Individual data), SUMMARY record = master summary record from curator (may link to another database), In vitro (cloned) = data resulting from in vitro functional assays, animal model = data from animal model, Artefact = false positive variant call, DUPLICATE record = variant already described on another chromosome (e.g. unbalanced translocation, duplicating transposition, 2nd fusion transcript, etc.)
All options:
- Germline
- De novo
- Germline/De novo (untested)
- Somatic
- Uniparental disomy
- Uniparental disomy, maternal allele
- Uniparental disomy, paternal allele
- CLASSIFICATION record
- SUMMARY record
- In vitro (cloned)
- In silico
- animal model
- Artefact
- DUPLICATE record
- Unknown
- Not applicable
Segregation: Indicates whether the variant segregates with the phenotype (yes), does not segregate with the phenotype (no) or segregation is unknown (?)
All options:
- ? = unknown
- yes = segregates with phenotype
- no = does not segregate with phenotype
- - = not applicable
Frequency: frequency in which the variant was found; e.g 5/760 chromosomes (in 5 of 760 chromosomes tested), 1/33 patients (in 1 of 33 patients analysed in study), 0.05 controls (in 5% of control cases tested)
Re-site: restriction enzyme recognition site created (+) or destroyed (-); e.g. BglII+;BamHI-
VIP: variant VIP-status was requested for matchmaking - need collaboration(s) to crack the case - please contact the submitter/curator.
NOTE: to get VIP status ask the curator.
Methylation: result of methylation test; GOM (gain of methylation), LOM (loss of methylation), 30% (30% methylated). NOTE: when several tests were done mention the method as well (e.g. MS-PCR 75%)

 Effect
|

 Exon
|

 DNA change (cDNA)
|

 RNA change
|

 Protein
|

 Classification method
|

 Clinical classification
|

 DNA change (genomic) (hg19)
|

 DNA change (hg38)
|

 Published as
|

 ISCN
|

 DB-ID
|
 Variant remarks
|

 Reference
|

 ClinVar ID
|

 dbSNP ID
|

 Origin
|

 Segregation
|

 Frequency
|

 Re-site
|

 VIP
|

 Methylation
|

 Owner
|
| +?/. |
- |
c.-446_*2878del |
r.spl |
p.(?) |
- |
likely pathogenic |
g.46696090_46742082del |
g.46836655_46882647del |
RP2 chrX:46696090_46742082del |
- |
RP2_000156 |
size 45992 bp, hemizygous |
PubMed: Zampaglione 2020 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
| +/. |
1_2i |
c.(?_-189)_(768+1_769-1)del |
r.spl? |
p.? |
- |
pathogenic (recessive) |
g.46696347_46713577del |
- |
- |
- |
RP2_000046 |
Variant Error [EMISMATCH/EUNCERTAIN]: This transcript variant has an error. Please fix this entry and then remove this message. |
- |
- |
- |
Germline |
- |
- |
- |
- |
- |
Juan Carlos Zenteno |
| +?/. |
1_5 |
c.0 |
r.0 |
p.0 |
- |
likely pathogenic |
g.(?_46696347_46741793_?)del |
- |
All gen deletion |
- |
RP2_000155 |
- |
PubMed: de Castro-Miró-2014 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +?/. |
- |
c.-2C>T |
r.spl |
p.(?) |
- |
likely pathogenic |
g.46696534C>T |
g.46837099C>T |
c.-2C>T |
- |
RP2_000001 |
heterozygous |
PubMed: Gao 2019 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
| +?/. |
- |
c.-2C>T |
r.spl |
p.(?) |
- |
likely pathogenic |
g.46696534C>T |
g.46837099C>T |
c.-2C>T |
- |
RP2_000001 |
heterozygous |
PubMed: Gao 2019 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
| +?/. |
- |
c.(?_-1)_(102+1_103-1)del |
r.? |
p.? |
ACMG |
VUS |
g.(?_46696535)_(46696638_46712910)del |
g.(?_46837100)_(46837203_46853475)del |
1-?_102+?del |
- |
RP2_000138 |
- |
- |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Jinu Han |
| +/. |
_1_5_ |
c.(?_-1)_(*1_?)del |
r.0 |
p.0 |
- |
pathogenic |
g.(?_46696535)_(46739205_?)del |
- |
all gene deletion |
- |
RP2_000010 |
- |
PubMed: de Castro-Miró 2014 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Marta de Castro-Miró |
| +/. |
_1_1i |
c.-189_(102+1_103-1){0} |
r.0? |
p.0? |
- |
pathogenic |
g.(?_46696347)_(46696638_46712910)del |
g.(?_46836912)_(46837203_46853475)del |
- |
- |
RP2_000066 |
- |
PubMed: Bader 2003 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
| +/. |
_1_2i |
c.-189_(768+1_769-1){0} |
r.0? |
p.0? |
ACMG |
pathogenic |
g.(?_46696347)_(46713577_46719422)del |
g.(?_46836912)_(46854142_46859987)del |
del exon 1-2 |
- |
RP2_000064 |
ACMG PVS1, PM2, PP1, PP4 |
PubMed: Zenteno 2020 |
- |
- |
Germline |
- |
1/143 cases |
- |
- |
- |
Johan den Dunnen |
| +?/. |
- |
c.? |
r.? |
p.? |
- |
likely pathogenic |
g.? |
- |
del ex2 |
- |
USP9X_000005 |
- |
PubMed: Stone 2017 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +?/. |
- |
c.? |
r.? |
p.? |
- |
likely pathogenic |
g.? |
- |
del ex2 |
- |
USP9X_000005 |
- |
PubMed: Stone 2017 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +/. |
- |
c.? |
r.(?) |
p.? |
- |
pathogenic (dominant) |
g.? |
- |
del15.2kb_incl_ex4 (Truncated) |
- |
USP9X_000005 |
- |
PubMed: Prokisch 2007 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +/. |
- |
c.? |
r.? |
p.? |
- |
pathogenic (dominant) |
g.? |
- |
del ex4-flanking |
- |
USP9X_000005 |
- |
PubMed: Daiger 2014 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +/. |
- |
c.? |
r.(?) |
p.? |
- |
pathogenic |
g.? |
- |
delEX04-flanking |
- |
USP9X_000005 |
- |
PubMed: Churchill-2013 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +/. |
- |
c.? |
r.(?) |
p.? |
- |
pathogenic |
g.? |
- |
p.RP2-E20X |
- |
USP9X_000005 |
- |
PubMed: Schorderet-2013 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
| -/. |
2 |
c.? |
r.(?) |
p.? |
- |
benign |
g.46712913A>T |
- |
105A>T |
- |
USP9X_000005 |
- |
PubMed: Thiseton-2000 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +?/. |
- |
c.? |
r.? |
p.? |
ACMG |
likely pathogenic |
g.46634266_46989306del |
- |
- |
- |
USP9X_000005 |
ACMG PM2, PVS1 |
PubMed: Weisschuh 2024 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
| +/. |
- |
c.? |
r.? |
p.? |
ACMG |
pathogenic |
g.(46737528_46737831)_46745341delins[NC_000002.11:g.(158406216_158406544)_158695305;GTTG] |
- |
- |
- |
USP9X_000005 |
ACMG PM2, PVS1, PP5 |
PubMed: Weisschuh 2024 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
| +/. |
- |
c.? |
r.? |
p.? |
ACMG |
pathogenic |
g.46694894_46708019del |
- |
- |
- |
USP9X_000005 |
ACMG PM2, PVS1, PP5 |
PubMed: Weisschuh 2024 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
| +?/. |
1 |
c.1A>G |
r.(?) |
p.(Met1?) |
- |
pathogenic (recessive) |
g.46696536A>G |
g.46837101A>G |
- |
- |
RP2_000047 |
- |
- |
- |
- |
Germline |
- |
- |
- |
- |
- |
Juan Carlos Zenteno |
| +/. |
- |
c.1A>G |
r.(?) |
p.(Met1?) |
ACMG |
pathogenic |
g.46696536A>G |
g.46837101A>G |
- |
- |
RP2_000047 |
ACMG PVS1, PS*, PM2, PP4 |
PubMed: Zenteno 2020 |
- |
- |
Germline |
- |
1/143 cases |
- |
- |
- |
Johan den Dunnen |
| +?/. |
1 |
c.1A>G |
r.(?) |
p.(Met1?) |
- |
likely pathogenic |
g.46696536A>G |
g.46837101A>G |
- |
- |
RP2_000047 |
- |
PubMed: Martin-Merida 2018 |
- |
- |
Germline |
yes |
1/258 |
- |
- |
- |
LOVD |
| +?/. |
- |
c.2T>C |
r.(?) |
p.(Met1?) |
- |
likely pathogenic |
g.46696537T>C |
g.46837102T>C |
- |
- |
RP2_000113 |
- |
PubMed: Patel 2016 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +?/. |
- |
c.5G>T |
r.(?) |
p.(Gly2Val) |
- |
likely pathogenic (recessive) |
g.46696540G>T |
g.46837105G>T |
- |
- |
RP2_000080 |
- |
PubMed: Zhou 2018 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +/. |
1 |
c.5G>T |
r.(?) |
p.(Gly2Val) |
- |
pathogenic |
g.46696540G>T |
- |
c.5G>T |
- |
RP2_000080 |
- |
PubMed: Pelletier 2007 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Julia Lopez |
| +?/. |
1 |
c.5G>T |
r.(?) |
p.(Gly2Val) |
- |
likely pathogenic |
g.46696540G>T |
- |
c.5G>T |
- |
RP2_000080 |
- |
PubMed: Zhou-2011 |
- |
- |
Unknown |
- |
- |
- |
- |
- |
LOVD |
| +/. |
1 |
c.8G>C |
r.(?) |
p.(Cys3Ser) |
- |
pathogenic |
g.46696543G>C |
- |
c.8G>C (p.Cys3Ser) |
- |
RP2_000140 |
- |
PubMed: Branham-2012 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +/. |
1 |
c.8G>C |
r.(?) |
p.(Cys3Ser) |
- |
pathogenic |
g.46696543G>C |
- |
c.8TGC>TCC |
- |
RP2_000140 |
- |
PubMed: Jayasundra-2010 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +?/. |
- |
c.8G>C |
r.(?) |
p.(Cys3Ser) |
- |
likely pathogenic |
g.46696543G>C |
- |
RP2(NM_006915.2):c.8G>C (p.(Cys3Ser)) |
- |
RP2_000140 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Leiden |
| +/. |
- |
c.8G>C |
r.(?) |
p.(Cys3Ser) |
- |
pathogenic |
g.46696543G>C |
g.46837108G>C |
- |
- |
RP2_000140 |
variants reported seperately, unknown if mono-allelic or bi-allelic |
PubMed: Retterer 2016 |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
| +/. |
- |
c.9_11del |
r.(?) |
p.(Phe5del) |
- |
pathogenic |
g.46696544_46696546del |
- |
X:46696543GCTT>G ENST00000218340.3:c.14_16delTCT (Phe5del) |
- |
RP2_000081 |
- |
PubMed: Carss 2017 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +?/. |
- |
c.14_16del |
r.(?) |
p.(Phe5del) |
- |
likely pathogenic |
g.46696549_46696551del |
g.46837114_46837116del |
c.9_11delCTT |
- |
RP2_000093 |
- |
PubMed: Huang 2018 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +/. |
- |
c.14_16del |
r.(?) |
p.(Phe5del) |
- |
pathogenic (dominant) |
g.46696549_46696551del |
g.46837114_46837116del |
c.9_11del |
- |
RP2_000093 |
- |
PubMed: Fernandez-San Jose 2015 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +/. |
1 |
c.14_16del |
r.(?) |
p.(Phe5del) |
- |
pathogenic |
g.46696548_46696550del |
- |
c.13_15del3 |
- |
RP2_000093 |
- |
PubMed: Neidhardt 2008 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +?/. |
1 |
c.14_16del |
r.(?) |
p.(Phe5del) |
- |
likely pathogenic |
g.46696549_46696551del |
g.46837114_46837116del |
- |
- |
RP2_000093 |
- |
PubMed: Martin-Merida 2018 |
- |
- |
Germline |
yes |
1/258 |
- |
- |
- |
LOVD |
| +?/. |
- |
c.14_16del |
r.(?) |
p.(Phe5del) |
- |
likely pathogenic |
g.46696549_46696551del |
g.46837114_46837116del |
RP2 c.14_16delTCT, p.Phe5del |
- |
RP2_000093 |
hemizygous |
PubMed: Turro 2020 |
- |
- |
Germline/De novo (untested) |
? |
- |
- |
- |
- |
LOVD |
| +?/. |
1 |
c.14_16del |
r.(?) |
p.(Phe5del) |
- |
likely pathogenic (maternal) |
g.46696549_46696551del |
- |
c.9_11delCTT |
- |
RP2_000093 |
- |
PubMed: Liu-2020 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +?/. |
- |
c.14_16del |
r.(?) |
p.(Phe5del) |
ACMG |
likely pathogenic |
g.46696549_46696551del |
g.46837114_46837116del |
RP2 c.[14_16del];[0], V1: c.14_16delTCT, (p.Phe5del) |
- |
RP2_000093 |
hemizygous |
PubMed: Chen 2021 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
| +/. |
- |
c.14_16del |
r.(?) |
p.(Phe5del) |
ACMG |
pathogenic |
g.46696549_46696551del |
g.46837114_46837116del |
- |
- |
RP2_000093 |
ACMG PM2, PM4, PP5_STRONG, PS4_MODERATE |
PubMed: Weisschuh 2024 |
437942 |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
| +/. |
- |
c.14_16del |
r.(?) |
p.(Phe5del) |
ACMG |
pathogenic |
g.46696549_46696551del |
g.46837114_46837116del |
- |
- |
RP2_000093 |
ACMG PM2, PM4, PP5_STRONG, PS4_MODERATE |
PubMed: Weisschuh 2024 |
437942 |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
| +/. |
- |
c.14_16del |
r.(?) |
p.(Phe5del) |
ACMG |
pathogenic |
g.46696549_46696551del |
g.46837114_46837116del |
- |
- |
RP2_000093 |
ACMG PM2, PM4, PP5_STRONG, PS4_MODERATE |
PubMed: Weisschuh 2024 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
| +/. |
- |
c.14_16del |
r.(?) |
p.(Phe5del) |
- |
pathogenic |
g.46696549_46696551del |
g.46837114_46837116del |
14_16delTCT |
- |
RP2_000093 |
- |
PubMed: Midgley 2024 |
- |
rs1556313414 |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
| +?/. |
- |
c.14_16delTCT |
r.(?) |
p.(Phe5del) |
- |
likely pathogenic |
g.46696549_46696551del |
g.46837114_46837116del |
RP2 c.[14_16del];[0]; p.(Phe5del) |
- |
RP2_000093 |
hemizygous |
PubMed: Chen 2021 |
- |
- |
Germline |
yes |
Taiwan Biobank: 0; GnomAD_exome_East: 0; GnomAD_All: 0 |
- |
- |
- |
LOVD |
| +/. |
1 |
c.16_18del |
r.(?) |
p.(Ser6del) |
- |
pathogenic |
g.46696551_46696553del |
g.46837116_46837118del |
TCCdel Ser6del |
- |
RP2_000023 |
- |
PubMed: Schwahn 1998, OMIM:var0001 |
- |
rs137852284 |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
| +/. |
- |
c.16_18del |
r.(?) |
p.(Ser6del) |
- |
pathogenic (recessive) |
g.46696551_46696553del |
- |
delSer6 |
- |
RP2_000023 |
- |
PubMed: Rosenberg 1999 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Johan den Dunnen |
| +/. |
1 |
c.16_18del |
r.(?) |
p.(Ser6del) |
- |
pathogenic (dominant) |
g.46696551_46696553del |
- |
c.16_18del (p.S6del) |
- |
RP2_000023 |
- |
PubMed: Prokisch 2007 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +/. |
1 |
c.16_18del |
r.(?) |
p.(Ser6del) |
- |
pathogenic (dominant) |
g.46696551_46696553del |
- |
c.16_18del (p.S6del) |
- |
RP2_000023 |
- |
PubMed: Prokisch 2007 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +/. |
1 |
c.16_18del |
r.(?) |
p.(Ser6del) |
- |
pathogenic (dominant) |
g.46696551_46696553del |
- |
c.16_18del (p.S6del) |
- |
RP2_000023 |
- |
PubMed: Prokisch 2007 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +/. |
1 |
c.16_18del |
r.(?) |
p.(Ser6del) |
- |
pathogenic (dominant) |
g.46696551_46696553del |
- |
c.16_18del (p.S6del) |
- |
RP2_000023 |
- |
PubMed: Prokisch 2007 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +/. |
- |
c.16_18del |
r.(?) |
p.(Ser6del) |
ACMG |
pathogenic |
g.46696551_46696553del |
g.46837116_46837118del |
RP2 c.16_18del, p.(Ser6del), RP1 c.1126C>T, p.(Arg376*) |
- |
RP2_000023 |
- |
PubMed: Jespersgaar 2019 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
| +/. |
- |
c.16_18del |
r.(?) |
p.(Ser6del) |
ACMG |
pathogenic |
g.46696551_46696553del |
g.46837116_46837118del |
RP2 c.16_18del, p.(Ser6del) |
- |
RP2_000023 |
- |
PubMed: Jespersgaar 2019 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
| +?/. |
1 |
c.22A>T |
r.(?) |
p.(Arg8*) |
- |
likely pathogenic (maternal) |
g.46696557A>T |
- |
c.22A>T |
- |
RP2_000169 |
- |
PubMed: Liu-2020 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +/. |
1 |
c.28A>T |
r.(?) |
p.(Lys10*) |
- |
pathogenic (recessive) |
g.46696563A>T |
- |
c.28A>T:p.K10X |
- |
RP2_000174 |
- |
PubMed: Numa-2020 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +?/. |
- |
c.40del |
r.(?) |
p.(Glu14Serfs*32) |
- |
likely pathogenic |
g.46696575del |
g.46837140del |
RP2, variant 1: c.40del/p.E14Sfs*32 |
- |
RP2_000159 |
solved, hemizygous |
PubMed: Weisschuh 2020 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
| +/. |
- |
c.43del |
r.(?) |
p.(Ser15Argfs*31) |
- |
pathogenic |
g.46696578del |
- |
X:46696577GT>G ENST00000218340.3:c.43delT (Ser15ArgfsTer31) |
- |
RP2_000082 |
- |
PubMed: Carss 2017 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +?/. |
- |
c.43del |
r.(?) |
p.(Ser15Argfs*31) |
- |
likely pathogenic |
g.46696578del |
g.46837143del |
RP2 c.43delT, p.Ser15ArgfsTer31 |
- |
RP2_000082 |
hemizygous |
PubMed: Turro 2020 |
- |
- |
Germline/De novo (untested) |
? |
- |
- |
- |
- |
LOVD |
| +/. |
1_5 |
c.46_*62del |
r.(?) |
p.(Arg16*) |
- |
pathogenic |
g.46696581_46739266del |
- |
c.(?_46)_( * 62_?) |
- |
RP2_000101 |
- |
PubMed: Pelletier 2007 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Julia Lopez |
| +/. |
- |
c.49C>T |
r.(?) |
p.(Pro17Ser) |
- |
pathogenic (recessive) |
g.46696584C>T |
g.46837149C>T |
- |
- |
RP2_000117 |
- |
PubMed: Xu 2014 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
LOVD |
| +?/. |
- |
c.49C>T |
r.(?) |
p.(Pro17Ser) |
ACMG |
likely pathogenic |
g.46696584C>T |
g.46837149C>T |
RP2 c.49C>T, p.(Pro17Ser) |
- |
RP2_000117 |
- |
PubMed: Jespersgaar 2019 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
| ?/. |
- |
c.58G>C |
r.(?) |
p.(Glu20Gln) |
ACMG |
VUS |
g.46696593G>C |
g.46837158G>C |
- |
- |
RP2_000208 |
ACMG PM2 |
PubMed: Weisschuh 2024 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
| +?/. |
- |
c.58G>T |
r.(?) |
p.(Glu20*) |
- |
likely pathogenic |
g.46696593G>T |
g.46837158G>T |
RP2, variant 1: c.58G>T/p.E20* |
- |
RP2_000160 |
solved, hemizygous |
PubMed: Weisschuh 2020 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
| +?/. |
- |
c.58G>T |
r.(?) |
p.(Glu20*) |
- |
likely pathogenic |
g.46696593G>T |
g.46837158G>T |
RP2, variant 1: c.58G>T/p.E20* |
- |
RP2_000160 |
solved, hemizygous |
PubMed: Weisschuh 2020 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
| +?/. |
1 |
c.58G>T |
r.(?) |
p.(Glu20*) |
- |
likely pathogenic (maternal) |
g.46696593G>T |
- |
c.58G>T |
- |
RP2_000160 |
- |
PubMed: Liu-2020 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +?/. |
1 |
c.58G>T |
r.(?) |
p.(Glu20*) |
- |
likely pathogenic (maternal) |
g.46696593G>T |
- |
c.58G>T |
- |
RP2_000160 |
- |
PubMed: Liu-2020 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +/. |
1 |
c.76C>T |
r.(?) |
p.(Gln26*) |
- |
pathogenic |
g.46696611C>T |
g.46837176C>T |
CAG>TAG codon26 |
- |
RP2_000024 |
- |
PubMed: Schwahn 1998, OMIM:var0002 |
- |
rs104894925 |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
| +/. |
- |
c.76C>T |
r.(?) |
p.(Gln26*) |
- |
pathogenic (!) |
g.46696611C>T |
- |
Gln26stop |
- |
RP2_000024 |
- |
PubMed: Rosenberg 1999 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Johan den Dunnen |
| +/. |
1 |
c.76C>T |
r.(?) |
p.(Gln26*) |
- |
pathogenic (dominant) |
g.46696611C>T |
- |
c.76C>T (p.Q26X) |
- |
RP2_000024 |
- |
PubMed: Prokisch 2007 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +/. |
1 |
c.76C>T |
r.(?) |
p.(Gln26*) |
- |
pathogenic (dominant) |
g.46696611C>T |
- |
c.76C>T (p.Q26X) |
- |
RP2_000024 |
- |
PubMed: Prokisch 2007 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +/. |
1 |
c.76_77dup |
r.(?) |
p.(Gln26Hisfs*21) |
- |
pathogenic |
g.46696611_46696612dup |
g.46837176_46837177dup |
77/78insCA |
- |
RP2_000029 |
- |
PubMed: Mears 1999 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Johan den Dunnen |
| +/. |
1 |
c.76_77dup |
r.(?) |
p.(Gln26Hisfs*21) |
- |
pathogenic |
g.46696611_46696612dup |
- |
c.77insCA |
- |
RP2_000029 |
- |
PubMed: Jayasundra-2010 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +/. |
1 |
c.81C>G |
r.(?) |
p.(Tyr27*) |
- |
pathogenic |
g.46696616C>G |
g.46837181C>G |
82C>G |
- |
RP2_000067 |
- |
PubMed: Breuer 2002 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
| +/. |
- |
c.81C>G |
r.(?) |
p.(Tyr27*) |
- |
pathogenic |
g.46696616C>G |
- |
82C>G |
- |
RP2_000067 |
- |
PubMed: Andreasson 2003 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Johan den Dunnen |
| +/. |
- |
c.87G>A |
r.(?) |
p.(Trp29*) |
- |
pathogenic |
g.46696622G>A |
g.46837187G>A |
- |
- |
RP2_000038 |
- |
PubMed: Koyanagi 2019, Journal: Koyanagi 2019 |
- |
- |
Germline |
- |
1/1203 cases with retinitis pigmentosa |
- |
- |
- |
Yoshito Koyanagi |
| ?/. |
- |
c.89A>G |
r.(?) |
p.(Asp30Gly) |
- |
VUS |
g.46696624A>G |
g.46837189A>G |
RP2(NM_006915.2):c.89A>G (p.(Asp30Gly)), RP2(NM_006915.3):c.89A>G (p.D30G) |
- |
RP2_000011 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_AMC |
| -?/. |
- |
c.89A>G |
r.(?) |
p.(Asp30Gly) |
- |
likely benign |
g.46696624A>G |
g.46837189A>G |
RP2(NM_006915.2):c.89A>G (p.(Asp30Gly)), RP2(NM_006915.3):c.89A>G (p.D30G) |
- |
RP2_000011 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Leiden |
| +/. |
- |
c.97G>T |
r.(?) |
p.(Glu33*) |
ACMG |
pathogenic |
g.46696632G>T |
g.46837197G>T |
RP2 c.[97G>T];[0], V1: c.97G>T, (p.Glu33Ter) |
- |
RP2_000173 |
hemizygous |
PubMed: Chen 2021 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
| +/. |
- |
c.97G>T |
r.(?) |
p.(Glu33Ter) |
- |
pathogenic |
g.46696632G>T |
g.46837197G>T |
RP2 c.[97G>T];[0]; p.(Glu33Ter) |
- |
RP2_000173 |
hemizygous |
PubMed: Chen 2021 |
- |
- |
Germline |
yes |
Taiwan Biobank: 0; GnomAD_exome_East: 0; GnomAD_All: 0 |
- |
- |
- |
LOVD |
| +?/. |
- |
c.100_102del |
r.(?) |
p.(Lys34del) |
ACMG |
VUS |
g.46696635_46696637del |
.46837200_46837202del |
- |
- |
RP2_000137 |
- |
- |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Jinu Han |
| +?/. |
- |
c.102G>A |
r.spl |
p.? |
- |
likely pathogenic |
g.46696637G>A |
g.46837202G>A |
- |
- |
RP2_000087 |
effect on splicing predicted from mini-gene splicing assay |
PubMed: Soens 2017 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +/. |
- |
c.102G>A |
r.(=) |
p.(=) |
ACMG |
likely pathogenic |
g.46696637G>A |
- |
- |
- |
RP2_000087 |
- |
Villafuerte-de la Cruz RA, et al., 2023. Submitted |
ClinVar-1348552 |
rs1556313552 |
Germline |
yes |
- |
- |
- |
- |
Rocio Villafuerte-de la Cruz |
| +?/. |
- |
c.102+1G>A |
r.spl |
p.? |
- |
likely pathogenic |
g.46696638G>A |
g.46837203G>A |
- |
- |
RP2_000098 |
- |
PubMed: Ellingford 2016 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +/. |
1i |
c.102+1G>A |
r.spl? |
p.? |
- |
pathogenic |
g.46696638G>A |
- |
IVS1+1G>A |
- |
RP2_000098 |
- |
PubMed: Jayasundra-2010 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +?/. |
- |
c.102+1G>A |
r.spl |
p.? |
- |
likely pathogenic |
g.46696638G>A |
g.46837203G>A |
c.102+1G>A, p.? |
- |
RP2_000098 |
hemizygous |
PubMed: Kurata 2019 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
| +?/. |
- |
c.102+1G>A |
r.spl |
p.? |
- |
likely pathogenic |
g.46696638G>A |
g.46837203G>A |
c.102+1G>A, p.? |
- |
RP2_000098 |
heterozygous |
PubMed: Kurata 2019 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
| +/. |
- |
c.102+1G>T |
r.spl |
p.? |
ACMG |
pathogenic |
g.46696638G>T |
- |
- |
- |
RP2_000058 |
- |
PubMed: Sharon 2019 |
- |
- |
Germline |
- |
1/2420 IRD families |
- |
- |
- |
Global Variome, with Curator vacancy |
| +?/. |
- |
c.102+3A>C |
r.spl |
p.? |
- |
likely pathogenic |
g.46696640A>C |
g.46837205A>C |
- |
- |
RP2_000088 |
effect on splicing predicted from mini-gene splicing assay |
PubMed: Soens 2017 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +/. |
1i |
c.102+3A>G |
r.(?) |
p.? |
- |
pathogenic |
g.46696640A>G |
g.46837205A>G |
IVS1+3A>G |
- |
RP2_000068 |
- |
PubMed: Breuer 2002 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
| +/. |
1i |
c.102+3A>G |
r.spl? |
p.? |
- |
pathogenic |
g.46696640A>G |
- |
IVS1+3A>G |
- |
RP2_000068 |
- |
PubMed: Jayasundra-2010 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +/. |
1i |
c.102+3A>T |
r.spl |
p.? |
- |
pathogenic (recessive) |
g.46696640A>T |
g.46837205A>T |
IVS3+3A>T |
- |
RP2_000069 |
- |
PubMed: Sharon 2000, PubMed: Sharon 2003 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +/. |
1i |
c.102+146_102+147ins[AF148856.1inv;102+133_102+146] |
r.0 |
p.0 |
- |
pathogenic |
g.46696783_46696784ins[AF148856.1inv;46696770_46696783] |
- |
intron 1 L1 insertion |
- |
RP2_000028 |
positional cloning; 5.8kb L1-insertion flanked by 14-bp target site duplication |
PubMed: Schwahn 1998 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
| +?/. |
1i |
c.103-299_103-190delinsG |
r.(=) |
p.(=) |
- |
likely pathogenic |
g.46712612_46712721delinsG |
- |
c.103-299_190delinsG |
- |
RP2_000176 |
- |
Dickson 2019 (https://www.hilarispublisher.com/open-access/novel-emrp2em-gene-deletion-in-a-patient-with-bilateral-retinitis-pigmentosa-insights-of-technical-challenges-of-next-ge.pdf) |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +/. |
1i |
c.103-2A>G |
r.spl? |
p.? |
- |
pathogenic |
g.46712909A>G |
- |
103-2A>G |
- |
RP2_000177 |
- |
PubMed: Miano-2001 |
- |
- |
Germline |
yes |
0/120 controls |
- |
- |
- |
LOVD |
| +/. |
1i |
c.103-2del |
r.spl? |
p.? |
- |
pathogenic |
g.46712909del |
- |
c.103-2del |
- |
RP2_000170 |
- |
PubMed: Colombo-2020 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +?/. |
2_5 |
c.103_1053del |
r.? |
p.? |
- |
likely pathogenic |
g.46712911_46739204del |
- |
(c.103_1053del, p.Val35_Ile350del) |
- |
RP2_000133 |
- |
PubMed: Ji-2010 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +?/. |
2 |
c.111_112insC |
r.(?) |
p.(Lys38Glnfs*11) |
- |
likely pathogenic |
g.46712919_46712920insC |
- |
c.111insC (p.Pro37fsX48) |
- |
RP2_000178 |
- |
PubMed: De Lin-2014 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +?/. |
2 |
c.111_112insC |
r.(?) |
p.(Lys38Glnfs*11) |
- |
likely pathogenic |
g.46712919_46712920insC |
- |
c.111insC (p.Pro37fsX48) |
- |
RP2_000178 |
- |
PubMed: De Lin-2014 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
| +/. |
- |
c.115G>A |
r.(?) |
p.(Asp39Asn) |
- |
pathogenic (recessive) |
g.46712923G>A |
g.46853488G>A |
- |
- |
RP2_000118 |
- |
PubMed: Xu 2014 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
LOVD |
| ?/. |
- |
c.127A>G |
r.(?) |
p.(Ser43Gly) |
- |
VUS |
g.46712935A>G |
g.46853500A>G |
- |
- |
RP2_000039 |
- |
PubMed: Koyanagi 2019, Journal: Koyanagi 2019 |
- |
rs201111874 |
Germline |
- |
1/1204 cases with retinitis pigmentosa |
- |
- |
- |
Yoshito Koyanagi |
| ?/. |
2 |
c.127A>G |
r.(?) |
p.(Ser43Gly) |
- |
VUS |
g.46712935A>G |
- |
c.127A>G |
- |
RP2_000039 |
- |
PubMed: Panneman 2023 |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Daan Panneman |
| ?/. |
- |
c.155G>A |
r.(?) |
p.(Arg52His) |
- |
VUS |
g.46712963G>A |
g.46853528G>A |
RP2(NM_006915.2):c.155G>A (p.R52H) |
- |
RP2_000049 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |