Global Variome shared LOVD
PRPF31 (PRP31 pre-mRNA processing factor 31 homolog...)
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Global Variome, with Curator vacancy
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Unique variants in the PRPF31 gene
This database is one of the
"Eye disease"
gene variant databases.
The variants shown are described using the NM_015629.3 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%)
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!fs
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^
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^p.(Arg
all entries beginning with 'p.(Arg'
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=""
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combination
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Date
2020
all entries matching the year 2020
|
Date
2020-03|2020-04
all entries matching March or April, 2020
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Date
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<
Date
<2020
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<=
Date
<=2020-06
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>
Date
>2020-06
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>=
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
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Numeric
<=23
all entries lower than, or equal to, 23
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Numeric
>23
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Numeric
>=23
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combination
Numeric
>=20 <30 !23
all entries with values from 20 to 29, but not equal to 23
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all entries containing 'South Asian', but not containing 'South East Asian'
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390 entries on 4 pages. Showing entries 1 - 100.
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Effect
Reported
Exon
DNA change (cDNA)
RNA change
Protein
Classification method
Clinical classification
DNA change (genomic) (hg19)
DNA change (hg38)
Published as
ISCN
DB-ID
Variant remarks
Reference
ClinVar ID
dbSNP ID
Origin
Segregation
Frequency
Re-site
VIP
Methylation
Owner
+?/.
2
-
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
+?/.
3
_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
-
c.946-1G>?
r.spl
p.?
-
pathogenic (dominant)
g.54631447G>?
g.54128072G>?
c.946-1G>?
-
PRPF31_000144
-
PubMed: Daiger 2014
-
-
Germline
-
-
-
-
-
LOVD
+/.
1
_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
_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
+/., +?/.
2
_1_5_, _3_4_
c.(?_-397)_(420+1_421-1)del
r.(?)
p.(?)
ACMG
likely pathogenic, pathogenic
g.?
g.?
deletion of exons 1-5, deletion of exons 1-5,
-
NPHS1_000138
Heterozygous
PubMed: Birtel 2018
-
-
Germline
?, yes
-
-
-
-
LOVD
+?/.
2
-
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
_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
1_5i
c.(?_-396-1)_(420+1_?)del
r.spl?
p.?
-
pathogenic
g.54618789_54625974del
-
Deletion of exons 1–5
-
PRPF31_000183
-
PubMed: Eisenberger-2013
-
-
Germline
-
-
-
-
-
LOVD
+/.
3
_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
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
1_14
c.0
r.spl?
p.?
-
pathogenic
g.54618789_54635151del
-
Deletion of exons 1–14
-
PRPF31_000156
-
PubMed: Eisenberger-2013
-
-
Germline
-
-
-
-
-
LOVD
+?/.
2
-
c.-396_-131del
r.0?
p.(?)
-
likely pathogenic
g.54610320_54619055del
g.54115410_54115675del
PRPF31 chr19:54610320_54619055del
-
PRPF31_000217
2 more items
PubMed: Zampaglione 2020
-
-
Unknown
?
-
-
-
-
LOVD
+/., +?/.
4
1_11
c.(?_-396)_(1146+1_1147-1)del
r.?
p.?
-
likely pathogenic, pathogenic (!)
g.(?_54618790)_(54631753_54632431)del, g.?
-
59 kb genomic deletion (intron 11 of the PRPF31 gene and in LOC441864),
1 more item
-
NPHS1_000138, PRPF31_000274
1 more item
PubMed: Golovleva-2010
,
PubMed: Köhn 2009
,
PubMed: Ko?hn 2009
-
-
Germline
yes
0/20 simplex RP cases or 0/94 healthy controls
-
-
-
LOVD
+/.
4
-
c.(?_-396)_(1148-9_?)del
r.?
p.?
-
pathogenic
g.?
-
~30kb deletion
-
NPHS1_000138
-
PubMed: Abu Safieh 2006
-
-
Germline
yes
-
-
-
-
LOVD
+/., +?/.
2
1
c.-358_-131del
r.(?), r.spl
p.(?), p.?
-
likely pathogenic, pathogenic (dominant)
g.54618828_54619055del
g.54115448_54115675del
PRPF31 del chr19:54618828-54619055, deletion of PRPF31 (E1)
-
PRPF31_000158
heterozygous
PubMed: Martin-Merida 2017
,
PubMed: Martin-Merida 2018
-
-
Germline, Germline/De novo (untested)
?
1/258
-
-
-
LOVD
?/.
1
-
c.-349del
r.?
p.?
ACMG
VUS
g.54618837del
g.54115457del
-
-
PRPF31_000332
ACMG PM2
PubMed: Weisschuh 2024
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
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
+?/.
1
-
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
1 more item
PubMed: Kiser 2019
-
-
Germline
?
-
-
-
-
LOVD
+?/.
1
-
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
1 more item
PubMed: Kiser 2019
-
-
Germline
?
-
-
-
-
LOVD
+?/.
1
-
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
1 more item
PubMed: Kiser 2019
-
-
Germline
?
-
-
-
-
LOVD
+?/.
1
-
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
1 more item
PubMed: Kiser 2019
-
-
Germline
?
-
-
-
-
LOVD
+?/.
1
-
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
1 more item
PubMed: Kiser 2019
-
-
Germline
?
-
-
-
-
LOVD
+/.
1
-
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
_1_13i
c.(?_-52)_(1375-490_?)del
r.0?
p.0?
-
pathogenic (dominant)
g.(?_54619134)_(54634248_?)del
-
-
-
PRPF31_000129
-
PubMed: Almoguera 2015
-
-
Germline
-
-
-
-
-
LOVD
-/.
2
1
c.-39+14A>G
r.(=), r.spl?
p.(=), p.?
-
benign (dominant)
g.54619161A>G
-
c.-39+14A>G
-
PRPF31_000206
-
PubMed: Anasagasti-2013
-
rs4806711
Germline
yes
0.21
-
-
-
LOVD
?/.
1
-
c.-23G>T
r.(=)
p.(=)
-
VUS
g.54619163G>T
-
g.54619163G>T
-
PRPF31_000354
-
PubMed: Anasagasti-2013
-
-
Germline
yes
-
-
-
-
LOVD
+/.
1
-
c.-9+1G>A
r.spl
p.?
-
pathogenic
g.54619178G>A
g.54115798G>A
-
-
PRPF31_000030
-
-
-
-
Germline
-
-
-
-
-
Marta de Castro-Miró
+?/.
2
1i
c.-9+1G>T
r.spl?
p.?
-
likely pathogenic
g.54619178G>T
-
IVS1+1G>T (isoform 1), IVS1+1G>T (isoform 2)
-
PRPF31_000295
0/200 controls and 0/18 unaffected family members
PubMed: Liu 2008
-
-
Germline
yes
-
-
-
-
LOVD
-?/.
1
-
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
+/.
1
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
+/.
1
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
+/., +?/.
3
_2_5_
c.-8-45_421-88dup
r.(?), r.spl
p.(?), p.?
-
likely pathogenic, 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
,
PubMed: Martin-Merida 2018
-
-
Germline
?, yes
1/258
-
-
-
LOVD
+?/.
1
-
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
_2_3, _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
1i_3i
c.-8-1_238+1del
r.spl?
p.?
-
likely pathogenic (dominant), pathogenic (dominant)
g.54621650_54622014del
-
E2-3del, Gross deletion including exon 2 and 3***
-
PRPF31_000259
-
PubMed: Liu-2020
,
PubMed: Numa-2020
-
-
Germline
-
-
-
-
-
LOVD
+/.
1
_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
+?/.
1
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
+?/.
1
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
+?/.
4
2
c.-3_7del
r.(?), r.?
p.?
-
likely pathogenic, likely pathogenic (dominant)
g.54621656_54621665del
g.54118276_54118285del
c.-3_7del, PRPF31 c.-3_7del
-
PRPF31_000184
heterozygous
PubMed: Kiser 2019
,
PubMed: Sullivan-2013
-
-
Germline, Unknown
?
-
-
-
-
LOVD
+?/.
1
-
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
+/., +?/.
2
-
c.(?_-1)_(*1_?)del
r.(?)
p.(?)
ACMG
likely pathogenic, pathogenic
g.?
g.?
PRPF31 c.(?_-1) _(*1_?)del
-
NPHS1_000138
-
PubMed: Jespersgaar 2019
-
-
Germline
?
-
-
-
-
LOVD
+?/.
1
_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
?/.
1
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
+/.
1
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
+?/.
1
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
_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
+/., +?/.
3
_1_14_
c.-396_*287{0}
r.0
p.0
-
likely pathogenic, pathogenic (dominant)
g.54600232_54637087del, g.54609771_54636572del
g.54096852_54133656del, g.54106454_54133135del
chr19:54600232_54637087del
-
PRPF31_000125, PRPF31_000328
deletion incl. OSCAR, NDUFA3, TFPT, PRPF31
PubMed: Fadaie 2021
,
PubMed: De Bruijn 2023
,
PubMed: Zampaglione 2020
-
-
Germline, Germline/De novo (untested)
?, yes
-
-
-
-
Suzanne de Bruijn
+/.
1
_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
_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
+/., +?/.
11
_1_10_, _1_14_
c.0, c.0?
r.0, r.0?
p.0, p.0?
-
likely pathogenic, likely pathogenic (dominant), pathogenic (dominant)
g.54577171_54630008del, g.54602946_54632693del, g.54602946_54635178del, g.?
g.54073923_54126760del, g.54099655_54129402del, g.54099655_54131887del, g.?
chr19:54577171_54630008del,
1 more item
-
NPHS1_000138, PRPF31_000156
deletion encompassing OSCAR (exon 1-2), NDUFA3, TFPT, PRPF31, Heterozygous,
1 more item
PubMed: Cho 2020
,
PubMed: Dong 2013
,
PubMed: Martin-Merida 2018
-
-
Germline, Unknown
?, yes
1/258
-
-
-
LOVD
+?/.
1
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
+/., +?/., ?/.
27
10i, _1_, _1_14_, _1_2_
c.?
r.(?), r.0?, r.?, r.spl
p.(?), p.0?, p.?
ACMG
likely pathogenic, likely pathogenic (dominant), NA, pathogenic, pathogenic (dominant)
g.54602638_54636555del, g.54602946_54632693del, g.54602946_54635178del, g.54615301_54637092del, g.?
,
7 more items
g.54099655_54129402del, g.54099655_54131887del, g.54116329_54132413del, g.68645751G>A, g.?
1081+19del17bp, c.160+1G>A, c.946-1G>C, chr19:g.54622548_54633842del, del ex4-13, p.R293, p.R372,
10 more items
-
NPHS1_000138, PRPF31_000156, PRPF31_000214
ACMG PM2, PVS1, ACMG PM2, PVS1, PP5, heterozygous, not in 100 controls, solved, heterozygous,
1 more item
PubMed: Aleman 2009
,
PubMed: Blanco-Kelly-2012
,
PubMed: Daiger 2014
,
PubMed: Hariri 2018
,
10 more items
na
-
Germline, Germline/De novo (untested), In vitro (cloned), Unknown
0, ?, yes
-
-
-
-
Johan den Dunnen
+/., +?/.
2
-
c.1A>T
r.(?), r.?
p.(Met1?), p.?
-
likely pathogenic, pathogenic (dominant)
g.54621659A>T
g.54118279A>T
19:54621659A>T ENST00000321030.4:c.1A>T (Met1?), PRPF31 c.1A>T, p.Met1?
-
PRPF31_000078
heterozygous
PubMed: Carss 2017
,
PubMed: Turro 2020
-
-
Germline, Germline/De novo (untested)
?
-
-
-
-
LOVD
+/., +?/.
2
-
c.3G>A
r.(?)
p.0?
ACMG
likely pathogenic (dominant), pathogenic (dominant)
g.54621661G>A
g.54118281G>A
Met1Ile
-
PRPF31_000060
-
PubMed: Karali 2019
,
Journal: Karali 2019
-
-
Germline
yes
-
-
-
-
Sandro Banfi
-/.
1
2
c.5C>G
r.(?)
p.(Ser2Cys)
-
benign
g.54621663C>G
-
c.5C G
-
PRPF31_000145
-
PubMed: Lim-2009
-
-
Unknown
no
-
-
-
-
LOVD
+/., ?/.
2
-
c.18G>C
r.(?)
p.(Glu6Asp)
-
pathogenic (dominant), VUS
g.54621676G>C
g.54118296G>C
-
-
PRPF31_000131
VKGL data sharing initiative Nederland
PubMed: Van Huet 2015
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
VKGL-NL_Nijmegen
+?/.
1
2
c.19_20insA
r.(?)
p.(Leu7Hisfs*4)
-
likely pathogenic
g.54621677_54621678insA
-
c.19_20insA
-
PRPF31_000185
-
PubMed: Sullivan-2013
-
-
Unknown
-
-
-
-
-
LOVD
+/.
3
2
c.34G>T
r.(?)
p.(Glu12*), p.(Glu12Ter)
ACMG
pathogenic, pathogenic (dominant)
g.54621692G>T
g.54118312G>T
-
-
PRPF31_000023
VKGL data sharing initiative Nederland
PubMed: Van Cauwenbergh 2017
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
2
c.46G>T
r.(?)
p.(Glu16*)
ACMG
pathogenic
g.54621704G>T
g.54118324G>T
PRPF31 c.46G > T, p.(E16*)
-
PRPF31_000275
heterozygous
PubMed: Wang 2022
-
-
Germline/De novo (untested)
?
-
-
-
-
LOVD
+?/.
1
2
c.55del
r.(?)
p.(Glu19Lysfs*46)
-
likely pathogenic
g.54621713del
g.54118333del
-
-
PRPF31_000160
-
PubMed: Martin-Merida 2018
-
-
Germline
?
1/258
-
-
-
LOVD
+/.
12
2
c.59_65del
r.(?)
p.(Gly20Alafs*43)
-
pathogenic
g.54621717_54621723del
-
c.59_65del7 (p.Gly20AlafsX43)
-
PRPF31_000296
0/100 healthy controls from the same ethnic group
PubMed: Saini 2012
-
-
Germline
no, yes
-
-
-
-
LOVD
+?/.
2
-
c.77_78insC
r.(?)
p.(Glu26AspfsTer32)
-
likely pathogenic
g.54621735_54621736insC
g.54118355_54118356insC
PRPF31(NM_015629.4):c.77_78insC (p.E26Dfs*32)
-
PRPF31_000063
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
,
VKGL-NL_AMC
+?/.
1
2
c.79G>T
r.(?)
p.(Glu27*)
-
likely pathogenic
g.54621737G>T
-
c.79G>T
-
PRPF31_000297
-
PubMed: Waseem 2007
-
-
Germline
-
-
-
-
-
LOVD
+?/.
3
-
c.81_84del
r.(?)
p.(Glu28Serfs*36)
-
likely pathogenic
g.54621739_54621742del
g.54118359_54118362del
PRPF31, variant 1: c.81_84del/p.E28Sfs*36
-
PRPF31_000232
solved, heterozygous
PubMed: Weisschuh 2020
-
-
Germline, Unknown
?, yes
-
-
-
-
LOVD
+?/.
3
-
c.96_102del
r.(?)
p.(Ile32Metfs*31)
-
likely pathogenic
g.54621754_54621760del
g.54118374_54118380del
PRPF31, variant 1: c.96_102del/p.I23Mfs*31
-
PRPF31_000233
1 more item
PubMed: Weisschuh 2020
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
1
-
c.118C>T
r.(?)
p.(Gln40*)
-
likely pathogenic
g.54621776C>T
g.54118396C>T
PRPF31 c.118C>T, p.Gln40Ter
-
PRPF31_000218
heterozygous
PubMed: Zampaglione 2020
-
-
Unknown
?
-
-
-
-
LOVD
?/.
1
-
c.121C>G
r.(?)
p.(Leu41Val)
-
VUS
g.54621779C>G
g.54118399C>G
-
-
PRPF31_000117
-
PubMed: Ellingford 2016
-
-
Germline
-
-
-
-
-
LOVD
-/.
1
2
c.138T>C
r.(=)
p.(=)
-
benign
g.54621796T>C
-
c.138T C
-
PRPF31_000146
-
PubMed: Lim-2009
-
-
Unknown
-
-
-
-
-
LOVD
+/.
1
-
c.172A>T
r.(?)
p.(Lys58Ter)
ACMG
pathogenic (dominant)
g.54621830A>T
g.54118450A>T
c.A172T
-
PRPF31_000114
-
PubMed: Zhang 2016
-
-
Germline
-
-
-
-
-
LOVD
+?/.
2
-
c.176_177insTA
r.(?)
p.(Met59Ilefs*7)
-
likely pathogenic
g.54621834_54621835insTA
g.54118454_54118455insTA
PRPF31, variant 1: c.176_177insTA/p.M59Ifs*7
-
PRPF31_000234
solved, heterozygous
PubMed: Weisschuh 2020
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
1
2i
c.177+1del
r.spl?
p.?
-
likely pathogenic
g.54621836delG
-
IVS2+1delG
-
PRPF31_000166
-
PubMed: RioFrio 2008
-
-
Germline
-
-
-
-
-
LOVD
?/.
1
2i
c.177+1delG
r.spl?
p.?
-
NA
g.54621836delG
-
IVS2+1delG
-
PRPF31_000166
-
PubMed: Tanackovic 2011
-
-
In vitro (cloned)
-
-
-
-
-
LOVD
+/.
1
2i
c.177+1G>A
r.spl?
p.?
-
pathogenic
g.54621836G>A
-
IVS2+1G>A
-
PRPF31_000098
-
PubMed: Sullivan 2006
-
-
Germline
yes
-
-
-
-
Julia Lopez
-?/.
1
-
c.178-19G>C
r.(=)
p.(=)
-
likely benign
g.54621934G>C
-
PRPF31(NM_015629.4):c.178-19G>C
-
PRPF31_000327
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/., +?/.
2
2i
c.178-2A>G
r.spl
p.(?), p.?
ACMG
likely pathogenic, pathogenic
g.54621951A>G
g.54118571A>G
PRPF31 c.178-2A > G, p.?, PRPF31, variant 1: c.178-2A>G/p.?
-
PRPF31_000235
heterozygous, solved, heterozygous
PubMed: Wang 2022
,
PubMed: Weisschuh 2020
-
-
Germline/De novo (untested), Unknown
?
-
-
-
-
LOVD
+/., +?/.
3
-
c.178-1G>A
r.spl?
p.?
-
likely pathogenic, pathogenic
g.54621952G>A
g.54118572G>A
PRPF31(NM_015629.4):c.178-1G>A
-
PRPF31_000008
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
,
VKGL-NL_VUmc
,
VKGL-NL_AMC
+/.
5
3
c.180dup
r.(?)
p.(Ala61Cysfs*2)
ACMG
pathogenic
g.54621955dup
g.54118575dup
PRPF31 c.180dup, p.(A61Cfs*2)
-
PRPF31_000276
heterozygous
PubMed: Wang 2022
-
-
Germline, Germline/De novo (untested)
?, yes
-
-
-
-
LOVD
?/.
1
-
c.182C>G
r.(?)
p.(Ala61Gly)
ACMG
VUS
g.54621957C>G
g.54118577C>G
PRPF31:NM_015629 c.C182G, p.A61G
-
PRPF31_000215
heterozygous, individual unsolved, causality of variants unknown
PubMed: Rodriguez-Munoz 2020
-
-
Germline
?
-
-
-
-
LOVD
?/.
1
3
c.194T>A
r.(?)
p.(Met65Lys)
-
VUS
g.54621969T>A
-
p.Met65Lys
-
PRPF31_000207
-
PubMed: Anasagasti-2013
-
rs145505952
Germline
yes
-
-
-
-
LOVD
+/., +?/.
2
3
c.196_197del
r.(?)
p.(Lys66Aspfs*2)
-
likely pathogenic, pathogenic
g.54621971_54621972del
-
c.196_197delAA
-
PRPF31_000298
-
PubMed: Xu 2012
,
PubMed: Yang 2020
,
PubMed: Xu 2012
-
-
Germline
yes
-
-
-
-
LOVD
+/.
1
-
c.201del
r.(?)
p.(Ile67MetfsTer14)
-
pathogenic
g.54621976del
g.54118596del
201delT
-
PRPF31_000138
-
PubMed: Watson 2014
-
-
Germline
-
-
-
-
-
LOVD
+?/.
1
-
c.210T>G
r.(?)
p.(Tyr70*)
-
likely pathogenic
g.54621985T>G
g.54118605T>G
-
-
PRPF31_000099
-
PubMed: Huang 2018
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/.
3
3
c.217A>T
r.(?)
p.(Lys73*), p.(Lys73Ter)
ACMG
likely pathogenic, pathogenic, pathogenic (dominant)
g.54621992A>T
g.54118612A>T
c.217A>T
-
PRPF31_000087
ACMG PM2, PVS1, PP5
PubMed: Eisenberger-2013
,
PubMed: Stone 2017
,
PubMed: Weisschuh 2024
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., +?/.
7
3
c.220C>T
r.(?)
p.(Gln74*)
ACMG
likely pathogenic (dominant), pathogenic, pathogenic (dominant)
g.54621995C>T
g.54118615C>T
c.220C>T, PRPF31 c.220C>T, p.(Gln74*), PRPF31 c.220C>T, p.(Q74*)
-
PRPF31_000088
heterozygous, incomplete penetrance in the family
PubMed: Dan 2020
,
PubMed: Kiser 2019
,
PubMed: Van Cauwenbergh 2017
,
PubMed: Wang 2017
,
2 more items
-
-
Germline, Germline/De novo (untested), Unknown
?, yes
-
-
-
-
LOVD
+?/.
1
-
c.238+1G>A
r.spl
p.?
-
likely pathogenic
g.54622014G>A
g.54118634G>A
-
-
PRPF31_000100
-
PubMed: Bravo-Gil 2017
-
-
Germline
-
-
-
-
-
Nereida Bravo Gil
+/.
1
-
c.238+1G>T
r.spl?
p.?
-
pathogenic
g.54622014G>T
g.54118634G>T
PRPF31(NM_015629.4):c.238+1G>T
-
PRPF31_000009
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
2
-
c.238+515_1375-917del
r.?
p.?
ACMG
pathogenic (dominant)
g.54622528_54633821del
g.54119148_54130390del
-
-
PRPF31_000333
ACMG PM2, PVS1, PP5
PubMed: Weisschuh 2024
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.238+705_1375-727del
r.?
p.?
ACMG
likely pathogenic (dominant)
g.54622718_54634011del
g.54119338_54130580del
-
-
PRPF31_000334
ACMG PM2, PVS1
PubMed: Weisschuh 2024
-
-
Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.239-620_1375-747del
r.?
p.?
ACMG
pathogenic (dominant)
g.54624619_54633991del
g.54121240_54130560del
-
-
PRPF31_000335
ACMG PM2, PVS1, PP5
PubMed: Weisschuh 2024
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.239-329_1374+963del
r.spl
p.(?)
-
likely pathogenic
g.54624910_54633708del
g.54121531_54130333del
PRPF31 chr19:54624910_54633708del
-
PRPF31_000219
range 8798-15168 bp in various techniques, heterozygous
PubMed: Zampaglione 2020
-
-
Unknown
?
-
-
-
-
LOVD
-?/.
1
-
c.239-12G>A
r.(=)
p.(=)
-
likely benign
g.54625227G>A
-
PRPF31(NM_015629.4):c.239-12G>A
-
PRPF31_000330
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/., +?/.
2
3i
c.239-2A>G
r.spl, r.spl?
p.?
ACMG
likely pathogenic (dominant), pathogenic (dominant)
g.54625237A>G
g.54121858A>G
c.239-2A>G
-
PRPF31_000262
ACMG PM2, PVS1_STRONG, PP5_STRONG
PubMed: Liu-2020
,
PubMed: Weisschuh 2024
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
3i
c.239-1G>A
r.spl, r.spl?
p.?
ACMG
pathogenic
g.54625238G>A
g.54121859G>A
PRPF31 c.239-1G > A, p.?
-
PRPF31_000024
heterozygous, VKGL data sharing initiative Nederland
PubMed: Wang 2022
-
-
CLASSIFICATION record, Germline/De novo (untested)
?
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
-
c.(238+1_239-1)_(420+1_421-1)dup
r.(?)
p.(?)
ACMG
pathogenic
g.?
g.?
PRPF31 c.(238+1_239-1)_(420+1_421-1)dup
-
NPHS1_000138
-
PubMed: Jespersgaar 2019
-
-
Germline
?
-
-
-
-
LOVD
+?/.
13
-
c.(238+1_239-1)_(1374+1_1375-1)del
r.?, r.spl
p.(?), p.(Val80Glyfs*16)
-
likely pathogenic
g.?
g.?
c.(238+1_239-1)_(1374+1_1375-1)del, PRPF31, variant 1 :Deletion exon 4-13
-
NPHS1_000138
solved, heterozygous
PubMed: Panneman 2023
,
PubMed: Weisschuh 2020
-
-
Germline, Unknown
?, yes
-
-
-
-
Daan Panneman
+?/.
2
-
c.240_241insT
r.(?)
p.(Met81Tyrfs*9)
ACMG
likely pathogenic
g.54625240_54625241insT
g.54121861_54121862insT
PRPF31 c.240_24insT, p.Met81TyrfsTer9
-
PRPF31_000299
error in annotation c.240_24insT should be c.240_241insT, heterozygous, probably causal
PubMed: Zhu 2022
-
-
Germline/De novo (untested)
?
-
-
-
-
LOVD
+/.
1
4
c.245_246del
r.(?)
p.(Gly82Alafs*7)
-
pathogenic
g.54625245_54625246del
-
c.245_246del
-
PRPF31_000323
-
PubMed: Panneman 2023
-
-
Unknown
-
-
-
-
-
Daan Panneman
?/.
1
-
c.247C>G
r.(?)
p.(Pro83Ala)
-
VUS
g.54625247C>G
g.54121868C>G
PRPF31(NM_015629.4):c.247C>G (p.P83A)
-
PRPF31_000042
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
1
-
c.251T>C
r.(?)
p.(Val84Ala)
-
VUS
g.54625251T>C
g.54121872T>C
-
-
PRPF31_000031
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
rs369049017
Germline
-
3/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
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