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.(855+1_856-1)_(1374+1_1375-1) |
r.spl |
p.(?) |
- |
likely pathogenic |
g.? |
g.? |
NM_015629, exons9_13deletion, |
- |
NPHS1_000138 |
- |
PubMed: Ezquerra-Inchausti 2018 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.(855+1_856-1)_(1374+1_1375-1) |
r.spl |
p.(?) |
- |
likely pathogenic |
g.? |
g.? |
NM_015629, exons9_13deletion, |
- |
NPHS1_000138 |
- |
PubMed: Ezquerra-Inchausti 2018 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
_1_14_ |
c.-396_*287{0} |
r.0? |
p.0? |
- |
likely pathogenic |
g.(?_54618790)_(54635150_?)del |
g.(?_54115410)_(54131719_?)del |
del entire gene |
- |
PRPF31_000125 |
- |
PubMed: Stone 2017 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+?/. |
_1_14_ |
c.-396_*287{0} |
r.0? |
p.0? |
- |
likely pathogenic |
g.(?_54618790)_(54635150_?)del |
g.(?_54115410)_(54131719_?)del |
del entire gene |
- |
PRPF31_000125 |
- |
PubMed: Stone 2017 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+?/. |
_1_14_ |
c.-396_*287{0} |
r.0? |
p.0? |
- |
likely pathogenic |
g.(?_54618790)_(54635150_?)del |
g.(?_54115410)_(54131719_?)del |
del entire gene |
- |
PRPF31_000125 |
- |
PubMed: Stone 2017 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+/. |
- |
c.946-1G>? |
r.spl |
p.? |
- |
pathogenic (dominant) |
g.54631447G>? |
g.54128072G>? |
c.946-1G>? |
- |
PRPF31_000144 |
- |
PubMed: Daiger 2014 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+/. |
_1_1i |
c.-16646_-9+285del |
r.0? |
p.0? |
- |
pathogenic (dominant) |
g.54602540_54619462del |
g.54099160_54116082del |
- |
- |
PRPF31_000351 |
- |
PubMed: Wen 2023 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Johan den Dunnen |
+?/. |
_1_3_ |
c.(?_-397)_(238+1_239-1)del |
r.(?) |
p.(?) |
ACMG |
likely pathogenic |
g.? |
g.? |
deletion of exons 1-3 |
- |
NPHS1_000138 |
Heterozygous |
PubMed: Birtel 2018 |
- |
- |
De novo |
yes |
- |
- |
- |
- |
LOVD |
+/. |
_1_5_ |
c.(?_-397)_(420+1_421-1)del |
r.(?) |
p.(?) |
ACMG |
pathogenic |
g.? |
g.? |
deletion of exons 1-5 |
- |
NPHS1_000138 |
Heterozygous |
PubMed: Birtel 2018 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
_3_4_ |
c.(?_-397)_(420+1_421-1)del |
r.(?) |
p.(?) |
ACMG |
likely pathogenic |
g.? |
g.? |
deletion of exons 1-5, |
- |
NPHS1_000138 |
Heterozygous |
PubMed: Birtel 2018 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.(?_-397)_(945+1_946-1)del |
r.spl |
p.(?) |
- |
likely pathogenic |
g.? |
g.? |
PRPF31, variant 1 :Deletion exon 1-10 |
- |
NPHS1_000138 |
solved, heterozygous |
PubMed: Weisschuh 2020 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.(?_-397)_(945+1_946-1)del |
r.spl |
p.(?) |
- |
likely pathogenic |
g.? |
g.? |
PRPF31, variant 1 :Deletion exon 1-10 |
- |
NPHS1_000138 |
solved, heterozygous |
PubMed: Weisschuh 2020 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+/. |
_1_3_ |
c.(?_-396-1)_(238+1_239-1)del |
r.(?) |
p.(?) |
ACMG |
pathogenic |
g.? |
g.? |
PRPF31 19kb+ del exons 1-3, p.(?) |
- |
NPHS1_000138 |
- |
PubMed: Xiao-2021 |
- |
- |
Unknown |
yes |
- |
- |
- |
- |
LOVD |
+/. |
1_5i |
c.(?_-396-1)_(420+1_?)del |
r.spl? |
p.? |
- |
pathogenic |
g.54618789_54625974del |
- |
Deletion of exons 15 |
- |
PRPF31_000183 |
- |
PubMed: Eisenberger-2013 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+/. |
_1_14_ |
c.(?_-396-1)_(*287_?)del |
r.(?) |
p.(?) |
ACMG |
pathogenic |
g.? |
g.? |
PRPF31 18kb+ del exons 1-14, p.(?) |
- |
NPHS1_000138 |
- |
PubMed: Xiao-2021 |
- |
- |
Unknown |
yes |
- |
- |
- |
- |
LOVD |
+/. |
_1_14_ |
c.(?_-396-1)_(*287_?)del |
r.(?) |
p.(?) |
ACMG |
pathogenic |
g.? |
g.? |
PRPF31 18kb+ del exons 1-14, p.(?) |
- |
NPHS1_000138 |
- |
PubMed: Xiao-2021 |
- |
- |
Unknown |
yes |
- |
- |
- |
- |
LOVD |
+/. |
_1_14_ |
c.(?_-396-1)_(*287_?)del |
r.(?) |
p.(?) |
ACMG |
pathogenic |
g.? |
g.? |
PRPF31 18kb+ del exons 1-14, p.(?) |
- |
NPHS1_000138 |
- |
PubMed: Xiao-2021 |
- |
- |
Unknown |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
1_14 |
c.-396-1_*287+1del |
r.spl? |
p.? |
- |
likely pathogenic (dominant) |
g.54618789_54635151del |
- |
E1-14del |
- |
PRPF31_000156 |
- |
PubMed: Liu-2020 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+/. |
1_14 |
c.0 |
r.spl? |
p.? |
- |
pathogenic |
g.54618789_54635151del |
- |
Deletion of exons 114 |
- |
PRPF31_000156 |
- |
PubMed: Eisenberger-2013 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.-396_-131del |
r.0? |
p.(?) |
- |
likely pathogenic |
g.54610320_54619055del |
g.54115410_54115675del |
PRPF31 chr19:54610320_54619055del |
- |
PRPF31_000217 |
NDUFA3, TFPT, PRPF31 partial promoter deletion, range 13203-16857 bp in various techniques, heterozygous |
PubMed: Zampaglione 2020 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.-396_-131del |
r.0? |
p.(?) |
- |
likely pathogenic |
g.54610320_54619055del |
g.54115410_54115675del |
PRPF31 chr19:54610320_54619055del |
- |
PRPF31_000217 |
NDUFA3, TFPT, PRPF31 partial promoter deletion, range 14117-15168 bp in various techniques, heterozygous |
PubMed: Zampaglione 2020 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
+/. |
1_11 |
c.(?_-396)_(1146+1_1147-1)del |
r.? |
p.? |
- |
pathogenic (!) |
g.(?_54618790)_(54631753_54632431)del |
- |
59 kb genomic deletion including the PRPF31 |
- |
PRPF31_000274 |
novel genomic deletion including almost the entire PRPF31 linked to 19q13.42 (breakpoints in intron 11 of the PRPF31 gene and in LOC441864 (ref|NT_011109.15|Hs19_11266)); variant shows an inheritance pattern with incomplete penetrance |
PubMed: Golovleva-2010, PubMed: Köhn 2009 |
- |
- |
Germline |
yes |
0/20 simplex RP cases or 0/94 healthy controls |
- |
- |
- |
LOVD |
+/. |
1_11 |
c.(?_-396)_(1146+1_1147-1)del |
r.? |
p.? |
- |
pathogenic (!) |
g.(?_54618790)_(54631753_54632431)del |
- |
59 kb genomic deletion including the PRPF31 |
- |
PRPF31_000274 |
novel genomic deletion including almost the entire PRPF31 linked to 19q13.42 (breakpoints in intron 11 of the PRPF31 gene and in LOC441864 (ref|NT_011109.15|Hs19_11266)); variant shows an inheritance pattern with incomplete penetrance |
PubMed: Golovleva-2010, PubMed: Köhn 2009 |
- |
- |
Germline |
yes |
0/20 simplex RP cases or 0/94 healthy controls |
- |
- |
- |
LOVD |
+?/. |
- |
c.(?_-396)_(1146+1_1147-1)del |
r.? |
p.? |
- |
likely pathogenic |
g.? |
- |
59 kb genomic deletion (intron 11 of the PRPF31 gene and in LOC441864) |
- |
NPHS1_000138 |
- |
PubMed: Ko?hn 2009 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.(?_-396)_(1146+1_1147-1)del |
r.? |
p.? |
- |
likely pathogenic |
g.? |
- |
59 kb genomic deletion (intron 11 of the PRPF31 gene and in LOC441864) |
- |
NPHS1_000138 |
- |
PubMed: Ko?hn 2009 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+/. |
- |
c.(?_-396)_(1148-9_?)del |
r.? |
p.? |
- |
pathogenic |
g.? |
- |
~30kb deletion |
- |
NPHS1_000138 |
- |
PubMed: Abu Safieh 2006 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+/. |
- |
c.(?_-396)_(1148-9_?)del |
r.? |
p.? |
- |
pathogenic |
g.? |
- |
~30kb deletion |
- |
NPHS1_000138 |
- |
PubMed: Abu Safieh 2006 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+/. |
- |
c.(?_-396)_(1148-9_?)del |
r.? |
p.? |
- |
pathogenic |
g.? |
- |
~30kb deletion |
- |
NPHS1_000138 |
- |
PubMed: Abu Safieh 2006 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+/. |
- |
c.(?_-396)_(1148-9_?)del |
r.? |
p.? |
- |
pathogenic |
g.? |
- |
~30kb deletion |
- |
NPHS1_000138 |
- |
PubMed: Abu Safieh 2006 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
1 |
c.-358_-131del |
r.spl |
p.(?) |
- |
likely pathogenic |
g.54618828_54619055del |
g.54115448_54115675del |
- |
- |
PRPF31_000158 |
- |
PubMed: Martin-Merida 2018 |
- |
- |
Germline |
? |
1/258 |
- |
- |
- |
LOVD |
+/. |
1 |
c.-358_-131del |
r.(?) |
p.? |
- |
pathogenic (dominant) |
g.54618828_54619055del |
g.54115448_54115675del |
PRPF31 del chr19:54618828-54619055, deletion of PRPF31 (E1) |
- |
PRPF31_000158 |
heterozygous |
PubMed: Martin-Merida 2017 |
- |
- |
Germline/De novo (untested) |
? |
- |
- |
- |
- |
LOVD |
?/. |
- |
c.-349del |
r.? |
p.? |
ACMG |
VUS |
g.54618837del |
g.54115457del |
- |
- |
PRPF31_000332 |
ACMG PM2 |
PubMed: Weisschuh 2024 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
c.-339delC |
r.(?) |
p.(=) |
- |
pathogenic (dominant) |
g.54618847del |
g.54115467del |
54618847delC |
- |
PRPF31_000126 |
not in 192 controls |
PubMed: Coussa 2015 |
- |
- |
Germline |
- |
1/60 cases |
- |
- |
- |
LOVD |
+?/. |
- |
c.-118_-9+39delins640 |
r.(?) |
p.? |
- |
likely pathogenic (dominant) |
g.54619068_54619216delins640 |
g.54115688_54115836delins640 |
PRPF31 exon 1 indel: 149 bp deleted/640 bp inserted |
- |
PRPF31_000284 |
heterozygous; insertion of 640 nucleotides from OSCAR gene in reverse complement, hg 17: 59,292,594–59,291,955 (Sullivan et al., 200 |
PubMed: Kiser 2019 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.-118_-9+39delins641 |
r.(?) |
p.? |
- |
likely pathogenic (dominant) |
g.54619068_54619216delins641 |
g.54115688_54115836delins641 |
PRPF31 exon 1 indel: 149 bp deleted/640 bp inserted |
- |
PRPF31_000285 |
heterozygous; insertion of 640 nucleotides from OSCAR gene in reverse complement, hg 17: 59,292,594–59,291,955 (Sullivan et al., 200 |
PubMed: Kiser 2019 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.-118_-9+39delins642 |
r.(?) |
p.? |
- |
likely pathogenic (dominant) |
g.54619068_54619216delins642 |
g.54115688_54115836delins642 |
PRPF31 exon 1 indel: 149 bp deleted/640 bp inserted |
- |
PRPF31_000286 |
heterozygous; insertion of 640 nucleotides from OSCAR gene in reverse complement, hg 17: 59,292,594–59,291,955 (Sullivan et al., 200 |
PubMed: Kiser 2019 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.-118_-9+39delins643 |
r.(?) |
p.? |
- |
likely pathogenic (dominant) |
g.54619068_54619216delins643 |
g.54115688_54115836delins643 |
PRPF31 exon 1 indel: 149 bp deleted/640 bp inserted |
- |
PRPF31_000287 |
heterozygous; insertion of 640 nucleotides from OSCAR gene in reverse complement, hg 17: 59,292,594–59,291,955 (Sullivan et al., 200 |
PubMed: Kiser 2019 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.-118_-9+39delins644 |
r.(?) |
p.? |
- |
likely pathogenic (dominant) |
g.54619068_54619216delins644 |
g.54115688_54115836delins644 |
PRPF31 exon 1 indel: 149 bp deleted/640 bp inserted |
- |
PRPF31_000288 |
heterozygous; insertion of 640 nucleotides from OSCAR gene in reverse complement, hg 17: 59,292,594–59,291,955 (Sullivan et al., 200 |
PubMed: Kiser 2019 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
+/. |
- |
c.-53_-9del |
r.? |
p.0? |
ACMG |
pathogenic |
g.54113356-54116922del |
- |
c.-53_-9del |
- |
PRPF31_000066 |
- |
PubMed: Sharon 2019 |
- |
- |
Germline |
- |
2/2420 IRD families |
- |
- |
- |
Global Variome, with Curator vacancy |
+/. |
_1_13i |
c.(?_-52)_(1375-490_?)del |
r.0? |
p.0? |
- |
pathogenic (dominant) |
g.(?_54619134)_(54634248_?)del |
- |
- |
- |
PRPF31_000129 |
- |
PubMed: Almoguera 2015 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
-/. |
1 |
c.-39+14A>G |
r.spl? |
p.? |
- |
benign (dominant) |
g.54619161A>G |
- |
c.-39+14A>G |
- |
PRPF31_000206 |
- |
PubMed: Anasagasti-2013 |
- |
rs4806711 |
Germline |
yes |
0.21 |
- |
- |
- |
LOVD |
-/. |
1 |
c.-39+14A>G |
r.(=) |
p.(=) |
- |
benign (dominant) |
g.54619161A>G |
- |
c.-39+14A>G |
- |
PRPF31_000206 |
- |
PubMed: Anasagasti-2013 |
- |
rs4806711 |
Germline |
yes |
0.21 |
- |
- |
- |
LOVD |
?/. |
- |
c.-23G>T |
r.(=) |
p.(=) |
- |
VUS |
g.54619163G>T |
- |
g.54619163G>T |
- |
PRPF31_000354 |
- |
PubMed: Anasagasti-2013 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+/. |
- |
c.-9+1G>A |
r.spl |
p.? |
- |
pathogenic |
g.54619178G>A |
g.54115798G>A |
- |
- |
PRPF31_000030 |
- |
- |
- |
- |
Germline |
- |
- |
- |
- |
- |
Marta de Castro-Miró |
+?/. |
1i |
c.-9+1G>T |
r.spl? |
p.? |
- |
likely pathogenic |
g.54619178G>T |
- |
IVS1+1G>T (isoform 1) |
- |
PRPF31_000295 |
0/200 controls and 0/18 unaffected family members |
PubMed: Liu 2008 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
1i |
c.-9+1G>T |
r.spl? |
p.? |
- |
likely pathogenic |
g.54619178G>T |
- |
IVS1+1G>T (isoform 2) |
- |
PRPF31_000295 |
0/200 controls and 0/18 unaffected family members |
PubMed: Liu 2008 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
-?/. |
- |
c.-9+3G>A |
r.spl? |
p.? |
- |
likely benign |
g.54619180G>A |
- |
PRPF31(NM_015629.3):c.-9+3G>A |
- |
PRPF31_000082 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
+/. |
1i_4 |
c.-9+906_247del |
r.? |
p.? |
ACMG |
pathogenic (dominant) |
g.54620083_54625247del |
g.54116703_54121868del |
- |
- |
PRPF31_000329 |
- |
PubMed: De Bruijn 2023 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Suzanne de Bruijn |
+/. |
1i_5i |
c.-8-133_421-27dup |
r.? |
p.? |
ACMG |
pathogenic |
g.54621518_54626806dup |
g.54118138_54123427dup |
dup ex2-5 |
- |
PRPF31_000006 |
2 different variants segregating in family |
PubMed: de Castro-Miró 2016 |
- |
- |
Germline |
yes |
7/9 patients in family |
- |
- |
- |
Marta de Castro-Miró |
+?/. |
_2_5_ |
c.-8-45_421-88dup |
r.spl |
p.(?) |
- |
likely pathogenic |
g.54621606_54626745dup |
g.54118226_54123366dup |
- |
- |
PRPF31_000159 |
- |
PubMed: Martin-Merida 2018 |
- |
- |
Germline |
? |
1/258 |
- |
- |
- |
LOVD |
+/. |
_2_5_ |
c.-8-45_421-88dup |
r.(?) |
p.? |
- |
pathogenic (dominant) |
g.54621606_54626745dup |
g.54118226_54123366dup |
PRPF31 dup chr19:54621606-54626745, duplication of PRPF31 (E2 to E5 |
- |
PRPF31_000159 |
heterozygous |
PubMed: Martin-Merida 2017 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+/. |
_2_5_ |
c.-8-45_421-88dup |
r.(?) |
p.? |
- |
pathogenic (dominant) |
g.54621606_54626745dup |
g.54118226_54123366dup |
PRPF31 dup chr19:54621606-54626745, duplication of PRPF31 (E2 to E5 |
- |
PRPF31_000159 |
heterozygous |
PubMed: Martin-Merida 2017 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.(-9+1_-8-1)_(177+1_178-1)del |
r.spl |
p.(?) |
- |
likely pathogenic |
g.? |
g.? |
PRPF31, variant 1 :Deletion exon 2-3 |
- |
NPHS1_000138 |
solved, heterozygous |
PubMed: Weisschuh 2020 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
+/. |
_2_3_ |
c.(-9+1_-8-1)_(238+1_239-1)del |
r.(?) |
p.(?) |
ACMG |
pathogenic |
g.? |
g.? |
PRPF31 452bp+ del exons 2-3, p.(?) |
- |
NPHS1_000138 |
- |
PubMed: Xiao-2021 |
- |
- |
Unknown |
yes |
- |
- |
- |
- |
LOVD |
+/. |
_2_3 |
c.(-9+1_-8-1)_(238+1_239-1)del |
r.(?) |
p.(?) |
ACMG |
pathogenic |
g.? |
g.? |
PRPF31 452bp+ del exons 2-3, p.(?) |
- |
NPHS1_000138 |
- |
PubMed: Xiao-2021 |
- |
- |
Unknown |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
1i_3i |
c.-8-1_238+1del |
r.spl? |
p.? |
- |
likely pathogenic (dominant) |
g.54621650_54622014del |
- |
E2-3del |
- |
PRPF31_000259 |
- |
PubMed: Liu-2020 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+/. |
1i_3i |
c.-8-1_238+1del |
r.spl? |
p.? |
- |
pathogenic (dominant) |
g.54621650_54622014del |
- |
Gross deletion including exon 2 and 3*** |
- |
PRPF31_000259 |
- |
PubMed: Numa-2020 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+/. |
_2_8_ |
c.(-9+1_-8-1)_(855+1_856-1)del |
r.(?) |
p.(?) |
ACMG |
pathogenic |
g.? |
g.? |
PRPF31 7.5kb+ del exons 2-8, p.(?) |
- |
NPHS1_000138 |
- |
PubMed: Xiao-2021 |
- |
- |
Unknown |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
1i_13i |
c.-8-1_1374+1del |
r.spl? |
p.? |
- |
likely pathogenic (dominant) |
g.54621650_54632746del |
- |
E2-13del |
- |
PRPF31_000260 |
- |
PubMed: Liu-2020 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+?/. |
1i_14 |
c.-8-1_*287+1del |
r.spl? |
p.? |
- |
likely pathogenic (dominant) |
g.54621650_54635151del |
- |
E2-14del |
- |
PRPF31_000261 |
- |
PubMed: Liu-2020 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+?/. |
2 |
c.-3_7del |
r.? |
p.? |
- |
likely pathogenic |
g.54621656_54621665del |
- |
c.-3_7del |
- |
PRPF31_000184 |
- |
PubMed: Sullivan-2013 |
- |
- |
Unknown |
- |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.-3_7del |
r.(?) |
p.? |
- |
likely pathogenic (dominant) |
g.54621656_54621665del |
g.54118276_54118285del |
PRPF31 c.-3_7del |
- |
PRPF31_000184 |
heterozygous |
PubMed: Kiser 2019 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.-3_7del |
r.(?) |
p.? |
- |
likely pathogenic (dominant) |
g.54621656_54621665del |
g.54118276_54118285del |
PRPF31 c.-3_7del |
- |
PRPF31_000184 |
heterozygous |
PubMed: Kiser 2019 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.-3_7del |
r.(?) |
p.? |
- |
likely pathogenic (dominant) |
g.54621656_54621665del |
g.54118276_54118285del |
PRPF31 c.-3_7del |
- |
PRPF31_000184 |
heterozygous |
PubMed: Kiser 2019 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.(?_-1)_(238+1_239-1)del |
r.(?) |
p.(?) |
ACMG |
likely pathogenic |
g.? |
g.? |
PRPF31 c.(?_-1) _(238+1_239-1)del, IMPDH1 c.(?_-1) _(*1_?)del |
- |
NPHS1_000138 |
- |
PubMed: Jespersgaar 2019 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.(?_-1)_(*1_?)del |
r.(?) |
p.(?) |
ACMG |
likely pathogenic |
g.? |
g.? |
PRPF31 c.(?_-1) _(*1_?)del |
- |
NPHS1_000138 |
- |
PubMed: Jespersgaar 2019 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
+/. |
- |
c.(?_-1)_(*1_?)del |
r.(?) |
p.(?) |
ACMG |
pathogenic |
g.? |
g.? |
PRPF31 c.(?_-1) _(*1_?)del |
- |
NPHS1_000138 |
- |
PubMed: Jespersgaar 2019 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
+?/. |
_1 |
- |
r.(?) |
p.(?) |
- |
likely pathogenic |
g.54606209_54614642del |
g.54102829_54111262del |
chr19:54606209_54614642del (c.-12977_-4544del) |
- |
PRPF31_000343 |
given range does not contain PRPF31 |
PubMed: Zampaglione 2020 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
?/. |
1i_8i |
c.(-9+1_-8-1)_(855+1_856-1){2} |
r.? |
p.? |
- |
VUS |
g.(54619178_54621650)_(54628036_54629902)dup |
- |
chr19:54621654–54628040dup |
- |
PRPF31_000231 |
- |
PubMed: Ellingsford 2018 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+/. |
1i_5i |
c.(-9+1_-8-1)_(c.420+1_421-1)del |
r.spl? |
p.? |
ACMG |
pathogenic |
g.(54619178_54621650)_(54625974_54626832)del |
- |
del ex 2-5 |
- |
PRPF31_000007 |
2 different variants seggregating in family |
PubMed: de Castro-Miró 2016 |
- |
- |
Germline |
yes |
2/9 patients in family |
- |
- |
- |
Marta de Castro-Miró |
+?/. |
14_14_ |
c.*19_*287{0} |
r.? |
p.? |
- |
likely pathogenic |
g.54634882_54637087del |
g.54131451_54133656del |
chr19:54634882_54637087del |
- |
PRPF31_000344 |
- |
PubMed: Zampaglione 2020 |
- |
- |
Germline/De novo (untested) |
? |
- |
- |
- |
- |
LOVD |
+?/. |
_1_14 |
c.-396_(*19_?){0} |
r.0 |
p.0 |
- |
likely pathogenic |
g.(?_54604319)_(54634882_?)del |
g.(?_54100939)_(54131451_?)del |
chr19:54604319_54634882del |
- |
PRPF31_000346 |
OSCAR, NDUFA3, TFPT, PRPF31 |
PubMed: Zampaglione 2020 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
+?/. |
_1_14_ |
c.-396_*287{0} |
r.0 |
p.0 |
- |
likely pathogenic |
g.54600232_54637087del |
g.54096852_54133656del |
chr19:54600232_54637087del |
- |
PRPF31_000125 |
deletion incl. OSCAR, NDUFA3, TFPT, PRPF31 |
PubMed: Zampaglione 2020 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
+?/. |
_1_14_ |
c.-396_*287{0} |
r.0 |
p.0 |
- |
likely pathogenic |
g.54600232_54637087del |
g.54096852_54133656del |
chr19:54600232_54637087del |
- |
PRPF31_000125 |
deletion incl. OSCAR, NDUFA3, TFPT, PRPF31 |
PubMed: Zampaglione 2020 |
- |
- |
Germline/De novo (untested) |
? |
- |
- |
- |
- |
LOVD |
+/. |
_1_14_ |
c.-396_*287{0} |
r.0 |
p.0 |
- |
pathogenic (dominant) |
g.54609771_54636572del |
g.54106454_54133135del |
- |
- |
PRPF31_000328 |
- |
PubMed: Fadaie 2021, PubMed: De Bruijn 2023 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Suzanne de Bruijn |
+/. |
_1_3i |
c.-396_239-98{0} |
r.0? |
p.0? |
- |
pathogenic (dominant) |
g.54618460_54625141del |
g.54115080_54121762del |
-726_239-98del |
- |
PRPF31_000331 |
- |
PubMed: de Bruijn 2023 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+?/. |
_1_8 |
c.-396_735{0} |
r.0? |
p.0? |
- |
likely pathogenic |
g.54600232_54627915del |
g.54096852_54124536del |
chr19:54600232_54627915del |
- |
PRPF31_000345 |
OSCAR, NDUFA3, TFPT, PRPF31 |
PubMed: Zampaglione 2020 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
+?/. |
_1_14_ |
c.0 |
r.0 |
p.0 |
- |
likely pathogenic |
g.54602946_54635178del |
g.54099655_54131887del |
- |
- |
PRPF31_000156 |
deletion encompassing OSCAR (exon 1-2), NDUFA3, TFPT, PRPF31 |
PubMed: Martin-Merida 2018 |
- |
- |
Germline |
? |
1/258 |
- |
- |
- |
LOVD |
+?/. |
_1_14_ |
c.0 |
r.0 |
p.0 |
- |
likely pathogenic |
g.54602946_54632693del |
g.54099655_54129402del |
- |
- |
PRPF31_000156 |
deletion encompassing OSCAR (exon 1-2), NDUFA3, TFPT, PRPF31 |
PubMed: Martin-Merida 2018 |
- |
- |
Germline |
? |
1/258 |
- |
- |
- |
LOVD |
+?/. |
_1_10_ |
c.0 |
r.0 |
p.0 |
- |
likely pathogenic (dominant) |
g.54577171_54630008del |
g.54073923_54126760del |
chr19:54577171_54630008del |
- |
PRPF31_000156 |
Heterozygous |
PubMed: Cho 2020 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
+/. |
- |
c.0? |
r.0? |
p.0? |
- |
pathogenic (dominant) |
g.? |
g.? |
PRPF31 deletion from the beginning of the gene to the benginning of reversely oriented OSCAR gene (the estimated size of a wild-type allele is 19,825 bp along with ~220 bp insertion |
- |
NPHS1_000138 |
heterozygous; breakpoints sequenced, but no precise locus described |
PubMed: Dong 2013 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+/. |
- |
c.0? |
r.0? |
p.0? |
- |
pathogenic (dominant) |
g.? |
g.? |
PRPF31 deletion from the beginning of the gene to the benginning of reversely oriented OSCAR gene (the estimated size of a wild-type allele is 19,825 bp along with ~220 bp insertion |
- |
NPHS1_000138 |
heterozygous; breakpoints sequenced, but no precise locus described |
PubMed: Dong 2013 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+/. |
- |
c.0? |
r.0? |
p.0? |
- |
pathogenic (dominant) |
g.? |
g.? |
PRPF31 deletion from the beginning of the gene to the benginning of reversely oriented OSCAR gene (the estimated size of a wild-type allele is 19,825 bp along with ~220 bp insertion |
- |
NPHS1_000138 |
heterozygous; breakpoints sequenced, but no precise locus described |
PubMed: Dong 2013 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+/. |
- |
c.0? |
r.0? |
p.0? |
- |
pathogenic (dominant) |
g.? |
g.? |
PRPF31 deletion from the beginning of the gene to the benginning of reversely oriented OSCAR gene (the estimated size of a wild-type allele is 19,825 bp along with ~220 bp insertion |
- |
NPHS1_000138 |
heterozygous; breakpoints sequenced, but no precise locus described |
PubMed: Dong 2013 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+/. |
- |
c.0? |
r.0? |
p.0? |
- |
pathogenic (dominant) |
g.? |
g.? |
PRPF31 deletion from the beginning of the gene to the benginning of reversely oriented OSCAR gene (the estimated size of a wild-type allele is 19,825 bp along with ~220 bp insertion |
- |
NPHS1_000138 |
heterozygous; breakpoints sequenced, but no precise locus described |
PubMed: Dong 2013 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+/. |
- |
c.0? |
r.0? |
p.0? |
- |
pathogenic (dominant) |
g.? |
g.? |
PRPF31 deletion from the beginning of the gene to the benginning of reversely oriented OSCAR gene (the estimated size of a wild-type allele is 19,825 bp along with ~220 bp insertion |
- |
NPHS1_000138 |
heterozygous; breakpoints sequenced, but no precise locus described |
PubMed: Dong 2013 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+/. |
- |
c.0? |
r.0? |
p.0? |
- |
pathogenic (dominant) |
g.? |
g.? |
PRPF31 deletion from the beginning of the gene to the benginning of reversely oriented OSCAR gene (the estimated size of a wild-type allele is 19,825 bp along with ~220 bp insertion |
- |
NPHS1_000138 |
heterozygous; breakpoints sequenced, but no precise locus described |
PubMed: Dong 2013 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+/. |
- |
c.0? |
r.0? |
p.0? |
- |
pathogenic (dominant) |
g.? |
g.? |
PRPF31 deletion from the beginning of the gene to the benginning of reversely oriented OSCAR gene (the estimated size of a wild-type allele is 19,825 bp along with ~220 bp insertion |
- |
NPHS1_000138 |
heterozygous; breakpoints sequenced, but no precise locus described |
PubMed: Dong 2013 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
4i_14_ |
c.322+115_*287{0} |
r.? |
p.? |
- |
likely pathogenic |
g.54625437_54637087del |
g.54122058_54133656del |
hr19:54625437-54637087 |
- |
PRPF31_000342 |
- |
PubMed: Zampaglione 2020 |
- |
- |
Germline/De novo (untested) |
? |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.? |
r.? |
p.? |
- |
likely pathogenic (dominant) |
g.? |
- |
del ex4-13 |
- |
NPHS1_000138 |
- |
PubMed: Weisschuh 2016 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+?/. |
10i |
c.? |
r.? |
p.? |
- |
likely pathogenic (dominant) |
g.? |
- |
1081+19del17bp |
- |
NPHS1_000138 |
not in 100 controls |
PubMed: Yang 2015 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+/. |
- |
c.? |
r.? |
p.? |
- |
pathogenic (dominant) |
g.? |
- |
c.946-1G>C |
- |
NPHS1_000138 |
- |
PubMed: Daiger 2014 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+/. |
- |
c.? |
r.? |
p.? |
- |
pathogenic (dominant) |
g.? |
- |
PRPF31 Gly272Va |
- |
NPHS1_000138 |
- |
PubMed: Daiger 2014 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
?/. |
- |
c.? |
r.(?) |
p.? |
- |
NA |
g.? |
- |
p.R293 |
- |
NPHS1_000138 |
- |
PubMed: Tanackovic 2011 |
- |
- |
In vitro (cloned) |
- |
- |
- |
- |
- |
LOVD |
?/. |
- |
c.? |
r.(?) |
p.? |
- |
NA |
g.? |
- |
p.R372 |
- |
NPHS1_000138 |
- |
PubMed: Tanackovic 2011 |
- |
- |
In vitro (cloned) |
- |
- |
- |
- |
- |
LOVD |
+/. |
- |
c.? |
r.(?) |
p.? |
- |
pathogenic |
g.? |
- |
p.Thr494Met |
- |
NPHS1_000138 |
- |
PubMed: Blanco-Kelly-2012 |
- |
- |
Unknown |
yes |
- |
- |
- |
- |
LOVD |
+/. |
- |
c.? |
r.(?) |
p.? |
- |
pathogenic |
g.? |
- |
p.PRPF31-E183_ins33bp |
- |
NPHS1_000138 |
- |
PubMed: Schorderet-2013 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.? |
r.(?) |
p.(?) |
- |
likely pathogenic |
g.? |
g.? |
whole gene heterozygous deletion detected by comparative genomic hybridization |
- |
NPHS1_000138 |
- |
PubMed: Hariri 2018 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
+?/. |
_1_ |
c.? |
r.spl |
p.(?) |
- |
likely pathogenic |
g.68679654G>A |
g.68645751G>A |
c.160+1G>A |
- |
PRPF31_000214 |
Heterozygous deletion in PRPF31 exon 1 and 5′ (putative promoter) regi |
PubMed: Shakhmantsir 2020 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.? |
r.0? |
p.0? |
- |
likely pathogenic |
g.? |
g.? |
PRPF31, variant 1 :Deletion entire gene |
- |
NPHS1_000138 |
solved, heterozygous |
PubMed: Weisschuh 2020 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |