Global Variome shared LOVD
PROM1 (prominin 1)
LOVD v.3.0 Build 30b [
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Curator:
Pascal Escher
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All variants in the PROM1 gene
This database is one of the
"Eye disease"
gene variant databases.
The variants shown are described using the NM_006017.2 transcript reference sequence.
Legend
Please note that a short description of a certain column can be displayed when you move your mouse cursor over the column's header and hold it still. Below, a more detailed description is shown per column.
Effect
: The variant's effect on the function of the gene/protein, displayed in the format 'R/C'. R is the value reported by the source (publication, submitter) and this classification may vary between records. C is the value concluded by the curator. Note that in some database the curator uses Summary records to give details on the classification of the variant.Values used: '+' indicating the variant affects function, '+?' probably affects function, '-' does not affect function, '-?' probably does not affect function, '?' effect unknown, '.' effect was not classified.
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%)
How to query this table
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Operator
Column type
Example
Matches
Text
Arg
all entries containing 'Arg'
space
Text
Arg Ser
all entries containing 'Arg' and 'Ser'
|
Text
Arg|Ser
all entries containing 'Arg' or 'Ser'
!
Text
!fs
all entries not containing 'fs'
^
Text
^p.(Arg
all entries beginning with 'p.(Arg'
$
Text
Ser)$
all entries ending with 'Ser)'
=""
Text
=""
all entries with this field empty
=""
Text
="p.0"
all entries exactly matching 'p.0'
!=""
Text
!=""
all entries with this field not empty
!=""
Text
!="p.0"
all entries not exactly matching 'p.0?'
combination
Text
*|Ter !fs
all entries containing '*' or 'Ter' but not containing 'fs'
Date
2020
all entries matching the year 2020
|
Date
2020-03|2020-04
all entries matching March or April, 2020
!
Date
!2020-03
all entries not matching March, 2020
<
Date
<2020
all entries before the year 2020
<=
Date
<=2020-06
all entries in or before June, 2020
>
Date
>2020-06
all entries after June, 2020
>=
Date
>=2020-06-15
all entries on or after June 15th, 2020
combination
Date
2019|2020 <2020-03
all entries in 2019 or 2020, and before March, 2020
Numeric
23
all entries exactly matching 23
|
Numeric
23|24
all entries exactly matching 23 or 24
!
Numeric
!23
all entries not exactly matching 23
<
Numeric
<23
all entries lower than 23
<=
Numeric
<=23
all entries lower than, or equal to, 23
>
Numeric
>23
all entries higher than 23
>=
Numeric
>=23
all entries higher than, or equal to, 23
combination
Numeric
>=20 <30 !23
all entries with values from 20 to 29, but not equal to 23
Some more advanced examples:
Example
Matches
Asian
all entries containing 'Asian', 'asian', including 'Caucasian', 'caucasian', etc.
Asian !Caucasian
all entries containing 'Asian' but not containing 'Caucasian'
Asian|African !Caucasian
all entries containing 'Asian' or 'African', but not containing 'Caucasian'
"South Asian"
all entries containing 'South Asian', but not containing 'South East Asian'
To sort on a certain column, click on the column header or on the arrows. If that column is already selected to sort on, the sort order will be swapped. The column currently sorted on has a darker blue background color than the other columns. The up and down arrows next to the column name indicate the current sorting direction. When sorting on any field other than the default, LOVD will sort secondarily on the default sort column.
744 entries on 8 pages. Showing entries 1 - 100.
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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.436>T
r.(?)
p.(Arg146*)
-
likely pathogenic
g.16035000G>A
g.16033377G>A
PROM1 c.436>T
-
PROM1_000127
heterozygous
PubMed: Strauss 2018
-
-
Unknown
?
-
-
-
-
LOVD
+?/.
-
c.630_c.630+8del
r.(?)
p.?
-
likely pathogenic
g.16026807_16026815delins13
g.16025184_16025192delins13
PROM1 c.630_c.630+8 del 9 ins 13
-
PROM1_000252
error in annotation, inserted nucleotides not mentioned; heterozygous
PubMed: Strauss 2018
-
-
Unknown
?
-
-
-
-
LOVD
+?/.
-
c.?
r.?
p.?
-
likely pathogenic
g.?
-
1984A>T (Lys662*)
-
TRAPPC11_000000
-
PubMed: Huang 2017
-
-
Germline
-
-
-
-
-
LOVD
+/.
-
c.?
r.?
p.?
-
pathogenic (dominant)
g.?
-
Arg373Cys
-
TRAPPC11_000000
-
PubMed: Daiger 2014
-
-
Germline
-
-
-
-
-
LOVD
+/.
3
c.?
r.(?)
p.?
-
pathogenic
g.16035122T>C
-
c.314A>G
-
TRAPPC11_000000
-
PubMed: _Audo-2012
-
-
Unknown
-
-
-
-
-
LOVD
?/.
-
c.?
r.spl
p.(?)
-
VUS
g.16077311_16077529dup
-
PROM1 chr4:16077311_16077529dup
-
TRAPPC11_000000
out of frame exon 1, unsolved
PubMed: Zampaglione 2020
-
-
Unknown
?
-
-
-
-
LOVD
+?/.
-
c.3G>A
r.(?)
p.(Met1?)
-
likely pathogenic
g.16077527C>T
g.16075904C>T
-
-
PROM1_000149
-
PubMed: Riera 2017
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
1
c.7dup
r.(?)
p.(Leu3Profs*28)
-
likely pathogenic
g.16077523dup
g.16075900dup
-
-
PROM1_000120
-
PubMed: Boulanger-Scemama 2015
,
PubMed: Boulanger-Scemama 2019
-
-
Germline
-
-
-
-
-
Global Variome, with Curator vacancy
-/.
-
c.15C>T
r.(?)
p.(Leu5=)
-
benign
g.16077515G>A
g.16075892G>A
-
-
PROM1_000048
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/.
-
c.15C>T
r.(=)
p.(=)
-
likely benign
g.16077515G>A
g.16075892G>A
-
-
PROM1_000048
35 heterozygous, no homozygous;
Clinindb (India)
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs10033189
Germline
-
35/2795 individuals
-
-
-
Mohammed Faruq
+?/.
-
c.22del
r.(?)
p.(Leu8Cysfs*51)
ACMG
pathogenic
g.16077510del
g.16075887del
c.22del, p.Leu8fs*
-
PROM1_000254
compound heterozygous
PubMed: Cehajic-Kapetanovic 2019
-
-
Unknown
?
-
-
-
-
LOVD
?/.
1
c.55T>A
r.(?)
p.(Ser19Thr)
-
VUS
g.16077475A>T
-
c.55T>A
-
PROM1_000231
-
PubMed: Borràs 2013
-
-
Germline
no
Novel
-
-
-
LOVD
?/.
-
c.55T>G
r.(?)
p.(Ser19Ala)
-
VUS
g.16077475A>C
g.16075852A>C
-
-
PROM1_000177
-
PubMed: Wang 2014
-
rs189108830
Germline
-
-
-
-
-
LOVD
-?/.
1
c.55T>G
r.(?)
p.(Ser19Ala)
-
likely benign
g.16077475A>C
-
c.55T>G
-
PROM1_000177
-
PubMed: González-del Pozo-2011
-
-
Germline
no
2/200 controls
-
-
-
LOVD
-/.
-
c.129C>T
r.(?)
p.(Thr43=)
-
benign
g.16077401G>A
g.16075778G>A
-
-
PROM1_000047
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/.
-
c.129C>T
r.(=)
p.(=)
-
likely benign
g.16077401G>A
g.16075778G>A
-
-
PROM1_000047
36 heterozygous, no homozygous;
Clinindb (India)
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs10033086
Germline
-
36/2795 individuals
-
-
-
Mohammed Faruq
+?/.
-
c.139del
r.(?)
p.(His47Ilefs*12)
-
likely pathogenic
g.16077393del
g.16075770del
c.139_139delC
-
PROM1_000148
-
PubMed: Huang 2018
-
-
Germline
-
-
-
-
-
LOVD
+/.
-
c.139del
r.(?)
p.(His47IlefsTer12)
-
pathogenic (recessive)
g.16077393del
g.16075770del
c.139delC
-
PROM1_000148
-
PubMed: Xu 2014
-
-
Germline
-
-
-
-
-
LOVD
+?/.
-
c.139del
r.(?)
p.(His47IlefsTer12)
-
likely pathogenic (recessive)
g.16077393del
g.16075770del
c.139delC
-
PROM1_000148
-
PubMed: Xu 2014
-
-
Germline
-
2/314 case chromosomes
-
-
-
LOVD
+?/.
1
c.139del
r.(?)
p.(His47Ilefs*12)
-
likely pathogenic (recessive)
g.16077391del
-
c.139delC
-
PROM1_000148
-
PubMed: Liu-2020
-
-
Germline
-
-
-
-
-
LOVD
+?/.
1
c.139del
r.(?)
p.(His47Ilefs*12)
-
likely pathogenic (recessive)
g.16077391del
-
c.139delC
-
PROM1_000148
-
PubMed: Liu-2020
-
-
Germline
-
-
-
-
-
LOVD
+/.
-
c.139del
r.(?)
p.(His47Ilefs*12)
ACMG
pathogenic
g.16077393del
g.16075770del
PROM1 c.[139del];[242dupA], V1: c.139delC, (p.His47IlefsTer12)
-
PROM1_000148
alleles in trans; heterozygous
PubMed: Chen 2021
-
-
Unknown
?
-
-
-
-
LOVD
+/.
-
c.139del
r.(?)
p.(His47Ilefs*12)
ACMG
pathogenic
g.16077393del
g.16075770del
PROM1 c.[139del];[139=], V1: c.139delC, (p.His47IlefsTer12)
-
PROM1_000148
heterozygous
PubMed: Chen 2021
-
-
Unknown
?
-
-
-
-
LOVD
+/.
-
c.139del
r.(?)
p.(His47Ilefs*12)
ACMG
pathogenic
g.16077393del
g.16075770del
PROM1 c.[139del];[631-15_631-10del], V2: c.139delC, (p.His47IlefsTer12)
-
PROM1_000148
alleles in trans; heterozygous
PubMed: Chen 2021
-
-
Unknown
?
-
-
-
-
LOVD
+?/.
-
c.139del
r.(?)
p.(His47IlefsTer12)
-
likely pathogenic
g.16077393del
g.16075770del
PROM1 c.139del:p.His47Ilefs*12
-
PROM1_000148
compound heterozygous
PubMed: Ragi 2019
-
rs747512450
Germline
yes
-
-
-
-
LOVD
+/.
-
c.139delC
r.(?)
p.(His47IlefsTer12)
-
pathogenic
g.16077393del
g.16075770del
PROM1 c.[139del];[242dupA]; p.(His47IlefsTer12)
-
PROM1_000148
heterozygous
PubMed: Chen 2021
-
-
Germline
yes
Taiwan Biobank: 0; GnomAD_exome_East: 0.000389; GnomAD_All: 0.0000281
-
-
-
LOVD
+/.
-
c.139delC
r.(?)
p.(His47IlefsTer12)
-
pathogenic
g.16077393del
g.16075770del
PROM1 c.[139del];[139=]; p.(His47IlefsTer12)
-
PROM1_000148
heterozygous
PubMed: Chen 2021
-
-
Germline
yes
Taiwan Biobank: 0; GnomAD_exome_East: 0.000389; GnomAD_All: 0.0000281
-
-
-
LOVD
+/.
-
c.139delC
r.(?)
p.(His47IlefsTer12)
-
pathogenic
g.16077393del
g.16075770del
PROM1 c.[139del];[631-15_631-10del]; p.(His47IlefsTer12)
-
PROM1_000148
heterozygous
PubMed: Chen 2021
-
-
Germline
yes
Taiwan Biobank: 0; GnomAD_exome_East: 0.000389; GnomAD_All: 0.0000281
-
-
-
LOVD
-?/.
-
c.152C>T
r.(?)
p.(Pro51Leu)
-
likely benign
g.16077378G>A
-
PROM1(NM_001145847.1):c.152C>T (p.P51L)
-
PROM1_000241
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/.
-
c.155T>C
r.(?)
p.(Ile52Thr)
-
likely pathogenic
g.16077375A>G
g.16075752A>G
c.155T>C; p.I52T
-
PROM1_000183
-
PubMed: Kersten 2018
-
-
Germline/De novo (untested)
-
-
-
-
-
LOVD
?/.
-
c.158G>A
r.(?)
p.(Gly53Asp)
-
VUS
g.16077372C>T
g.16075749C>T
-
-
PROM1_000078
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
rs755064227
Germline
-
2/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
?/.
2
c.158G>A
r.(?)
p.(Gly53Asp)
-
VUS
g.16077372C>T
g.16075749C>T
PROM1 c.158G>A, p.Gly53Asp
-
PROM1_000078
heterozygous
PubMed: Fujinami 2020
-
-
Germline
yes
-
-
-
-
LOVD
+/.
-
c.181A>G
r.(?)
p.(Ile61Val)
-
pathogenic (dominant)
g.16077349T>C
g.16075726T>C
-
-
PROM1_000171
-
PubMed: Consugar 2015
-
-
Germline
yes
-
-
-
-
LOVD
?/.
-
c.199C>G
r.(?)
p.(Gln67Glu)
ACMG
VUS
g.16077331G>C
g.16075708G>C
PROM1 c.[199C>G];[199=], V1: c.199C>G, (p.Gln67Glu)
-
PROM1_000229
heterozygous
PubMed: Chen 2021
-
-
Unknown
?
-
-
-
-
LOVD
?/.
-
c.199C>G
r.(?)
p.(Gln67Glu)
-
VUS
g.16077331G>C
g.16075708G>C
PROM1 c.[199C>G];[199=]; p.(Gln67Glu)
-
PROM1_000229
heterozygous
PubMed: Chen 2021
-
-
Germline
yes
Taiwan Biobank: 0; GnomAD_exome_East: 0.000279; GnomAD_All: 0.0000202
-
-
-
LOVD
+/.
1
c.199C>T
r.(?)
p.(Gln67*)
-
pathogenic (recessive)
g.16077331G>A
g.16075708G>A
-
-
PROM1_000131
-
PubMed: Birtel 2018
-
-
Germline
-
-
-
-
-
LOVD
+?/.
-
c.199C>T
r.(?)
p.(Gln67*)
ACMG
pathogenic
g.16077331G>A
g.16075708G>A
PROM1 c.199C>T, p.Gln67*
-
PROM1_000131
homozygous
PubMed: Cehajic-Kapetanovic 2019
-
-
Unknown
?
-
-
-
-
LOVD
+/.
-
c.199C>T
r.(?)
p.(Gln67Ter)
ACMG
pathogenic
g.16077331G>A
g.16075708G>A
-
-
PROM1_000131
ACMG PM2, PVS1, PP5
PubMed: Weisschuh 2024
987418
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
-
c.206G>A
r.(?)
p.(Arg69His)
-
likely benign
g.16077324C>T
g.16075701C>T
PROM1(NM_001145847.1):c.206G>A (p.R69H), PROM1(NM_006017.3):c.206G>A (p.(Arg69His))
-
PROM1_000035
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
-
c.206G>A
r.(?)
p.(Arg69His)
-
likely benign
g.16077324C>T
-
PROM1(NM_001145847.1):c.206G>A (p.R69H), PROM1(NM_006017.3):c.206G>A (p.(Arg69His))
-
PROM1_000035
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
-
c.220+1G>C
r.spl
p.?
ACMG
pathogenic
g.16077309C>G
-
-
-
PROM1_000269
-
Villafuerte-de la Cruz RA, et al., 2023. Submitted
ClinVar-7898
-
Germline
yes
-
-
-
-
Rocio Villafuerte-de la Cruz
+/.
-
c.220+1G>T
r.spl?
p.?
-
pathogenic
g.16077309C>A
g.16075686C>A
PROM1(NM_001145847.2):c.220+1G>T
-
PROM1_000108
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
-
c.221-20T>G
r.221_276del
p.Asp74Alafs*14
-
pathogenic (recessive)
g.16040644A>C
g.16039021A>C
-
-
PROM1_000275
-
PubMed: Zeuli 2024
-
-
Germline
-
-
-
-
-
Susanne Roosing
+/.
-
c.221-20T>G
r.221_276del
p.Asp74Alafs*14
-
pathogenic (recessive)
g.16040644A>C
g.16039021A>C
-
-
PROM1_000275
-
PubMed: Zeuli 2024
-
-
Germline
-
-
-
-
-
Susanne Roosing
+?/.
-
c.223A>G
r.(?)
p.(Thr75Ala)
-
likely pathogenic
g.16040622T>C
g.16038999T>C
PROM1 c.223A>G, p.T75A
-
PROM1_000215
compound heterozygous
PubMed: Jauregui 2020
-
-
Unknown
?
-
-
-
-
LOVD
+/.
-
c.242dup
r.(?)
p.(Lys82Glufs*4)
ACMG
pathogenic
g.16040603dup
g.16038980dup
PROM1 c.[139del];[242dupA], V2: c.242dupA, (p.Lys82GlufsTer4)
-
PROM1_000228
alleles in trans; heterozygous
PubMed: Chen 2021
-
-
Unknown
?
-
-
-
-
LOVD
+/.
-
c.242dupA
r.(?)
p.(Lys82GlufsTer4)
-
pathogenic
g.16040603dup
g.16038980dup
PROM1 c.[139del];[242dupA]; p.(Lys82GlufsTer4)
-
PROM1_000228
heterozygous
PubMed: Chen 2021
-
-
Germline
yes
Taiwan Biobank: 0; GnomAD_exome_East: 0; GnomAD_All: 0
-
-
-
LOVD
+/.
-
c.262dup
r.(?)
p.(Ile88Asnfs*2)
-
pathogenic
g.16040586dup
g.16038963dup
-
-
PROM1_000046
-
PubMed: Haer-Wigman 2017
-
-
Germline
-
-
-
-
-
LOVD
+/.
-
c.262dup
r.(?)
p.(Ile88Asnfs*2)
-
pathogenic
g.16040586dup
g.16038963dup
-
-
PROM1_000046
-
PubMed: Haer-Wigman 2017
-
-
Germline
-
-
-
-
-
LOVD
-/.
-
c.276+15G>A
r.(=)
p.(=)
-
benign
g.16040554C>T
g.16038931C>T
PROM1(NM_001145847.2):c.276+15G>A
-
PROM1_000097
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
-
c.277-1G>A
r.spl
p.?
-
pathogenic (recessive)
g.16037385C>T
g.16035762C>T
-
-
PROM1_000169
-
PubMed: Zaneveld 2015
-
-
Germline
-
-
-
-
-
LOVD
+?/.
-
c.303G>C
r.(?)
p.(Lys101Asn)
ACMG
likely pathogenic
g.16037358C>G
g.16035735C>G
PROM1 c.[303G>C];[303=], V1: c.303G>C, (p.Lys101Asn)
-
PROM1_000227
heterozygous
PubMed: Chen 2021
-
-
Unknown
?
-
-
-
-
LOVD
+?/.
-
c.303G>C
r.(?)
p.(Lys101Asn)
-
likely pathogenic
g.16037358C>G
g.16035735C>G
PROM1 c.[303G>C];[303=]; p.(Lys101Asn)
-
PROM1_000227
heterozygous
PubMed: Chen 2021
-
-
Germline
yes
Taiwan Biobank: 0; GnomAD_exome_East: 0; GnomAD_All: 0
-
-
-
LOVD
+?/.
-
c.303+1G>A
r.spl
p.?
-
likely pathogenic (recessive)
g.16037357C>T
g.16035734C>T
-
-
PROM1_000134
-
PubMed: Bryant 2018
-
rs777673930
Germline
-
-
-
-
-
LOVD
+?/.
3i
c.303+1G>A
r.spl
p.(?)
-
likely pathogenic
g.16037357C>T
g.16035734C>T
c.303+1G>A, Splicing
-
PROM1_000134
solved, heterozygous
PubMed: Del Pozo-Valero
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
3i
c.303+1G>A
r.spl
p.(?)
-
likely pathogenic
g.16037357C>T
g.16035734C>T
c.303+1G>A, Splicing
-
PROM1_000134
solved, heterozygous
PubMed: Del Pozo-Valero
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
3i
c.303+1G>A
r.spl
p.(?)
-
likely pathogenic
g.16037357C>T
g.16035734C>T
c.303+1G>A, Splicing
-
PROM1_000134
solved, heterozygous
PubMed: Del Pozo-Valero
-
-
Germline
?
-
-
-
-
LOVD
+?/.
-
c.303+1G>A
r.spl
p.(?)
-
likely pathogenic
g.16037357C>T
g.16035734C>T
c.303+1G>A, r.spl
-
PROM1_000134
different transcript, ENST00000447510: 70: heterozygous
PubMed: Wolock 2019
-
-
Germline
-
-
-
-
-
Anna Tracewska
+/.
-
c.303+1G>A
r.spl?
p.?
-
pathogenic
g.16037357C>T
-
-
-
PROM1_000134
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/.
3i
c.303+2T>C
r.spl
p.(?)
-
likely pathogenic
g.16037356A>G
g.16035733A>G
c.303+2T>C, Splicing
-
PROM1_000205
solved, homozygous
PubMed: Del Pozo-Valero
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
-
c.303+2T>C
r.spl
p.(?)
-
likely pathogenic
g.16037356A>G
g.16035733A>G
c.303+2T>C, r.spl
-
PROM1_000205
different transcript, ENST00000447510: 70: heterozygous
PubMed: Wolock 2019
-
-
Germline
-
-
-
-
-
Anna Tracewska
+?/.
3i
c.303+2T>C
r.(?)
p.(?)
-
likely pathogenic
g.16037356A>G
g.16035733A>G
PROM1 IVS3 c.303+2T>C p.(?), IVS3 c.303+2T>C p.(?)
-
PROM1_000205
homozygous
PubMed: Martin Merida 2019
-
-
Germline
yes
-
-
-
-
LOVD
-/.
-
c.303+6G>A
r.(=)
p.(=)
-
benign
g.16037352C>T
g.16035729C>T
PROM1(NM_001145847.2):c.277-2220G>A
-
PROM1_000034
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-/.
-
c.303+6G>A
r.(=)
p.(=)
-
benign
g.16037352C>T
g.16035729C>T
PROM1(NM_001145847.2):c.277-2220G>A
-
PROM1_000034
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
4
c.314A>G
r.(?)
p.(Tyr105Cys)
-
VUS
g.16035122T>C
g.16033499T>C
c.314A>G, p.Tyr105Cys
-
TRAPPC11_000000
unsolved
PubMed: Del Pozo-Valero
-
-
Germline
no
-
-
-
-
LOVD
+?/.
5
c.334T>C
r.(?)
p.(Cys112Arg)
-
likely pathogenic
g.16035102A>G
g.16033479A>G
PROM1 c.334T>C, p.Cys112Arg
-
PROM1_000253
heterozygous
PubMed: Fujinami 2020
-
-
Germline
yes
-
-
-
-
LOVD
?/.
-
c.376G>A
r.(?)
p.(Val126Met)
-
VUS
g.16035060C>T
g.16033437C>T
PROM1(NM_001145847.2):c.349G>A (p.V117M)
-
PROM1_000096
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
3
c.376G>A
r.(?)
p.(Val292Met)
-
VUS
g.16035060C>T
-
p.V126M
-
PROM1_000096
-
PubMed: Matsui 2015
-
-
Germline
-
-
-
-
-
LOVD
+/.
-
c.380G>A
r.(?)
p.(Gly127Glu)
-
pathogenic (recessive)
g.16035056C>T
g.16033433C>T
-
-
PROM1_000157
-
PubMed: Tiwari 2016
-
-
Germline
-
-
-
-
-
LOVD
+?/.
3
c.380G>A
r.(?)
p.(Cys127Tyr)
-
likely pathogenic
g.16035056C>T
-
c.380G>A
-
PROM1_000157
-
PubMed: Maggi_2021
-
-
Germline
-
-
-
-
-
LOVD
+?/.
3
c.380G>A
r.(?)
p.(Cys127Tyr)
-
likely pathogenic
g.16035056C>T
-
c.380G>A
-
PROM1_000157
-
PubMed: Maggi_2021
-
-
Germline
-
-
-
-
-
LOVD
+?/?
-
c.400C>T
r.(?)
p.(Arg134Cys)
-
pathogenic (maternal)
g.16035036G>A
g.16033413G>A
PROM1, c.400C > T, p.(Arg134Cys)
-
PROM1_000197
-
PubMed: Surl 2020
-
-
Germline
yes
-
-
-
-
Anna Tracewska
+?/.
-
c.400C>T
r.(?)
p.(Arg134Cys)
-
likely pathogenic
g.16035036G>A
g.16033413G>A
c.400C>T, r.(?)
-
PROM1_000197
different transcript, ENST00000447510: 70: heterozygous
PubMed: Wolock 2019
-
-
Germline
-
-
-
-
-
Anna Tracewska
?/.
-
c.421G>A
r.(?)
p.(Gly141Arg)
-
VUS
g.16035015C>T
g.16033392C>T
-
-
PROM1_000095
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
-
c.430C>T
r.(?)
p.(His144Tyr)
-
VUS
g.16035006G>A
g.16033383G>A
PROM1(NM_001145847.1):c.403C>T (p.H135Y)
-
PROM1_000112
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
-
c.436C>T
r.(?)
p.(Arg146*)
-
pathogenic (recessive)
g.16035000G>A
-
4:16035000G>A ENST00000510224.1:c.436C>T (Arg146Ter)
-
PROM1_000127
-
PubMed: Carss 2017
-
-
Germline
-
-
-
-
-
LOVD
+/.
4
c.436C>T
r.(?)
p.(Arg146*)
-
pathogenic (recessive)
g.16035000G>A
g.16033377G>A
-
-
PROM1_000127
-
PubMed: Birtel 2018
-
-
Germline
-
-
-
-
-
LOVD
+?/.
-
c.436C>T
r.(?)
p.(Arg146Ter)
-
likely pathogenic
g.16035000G>A
g.16033377G>A
-
-
PROM1_000127
-
PubMed: Huang 2015
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
4,12
c.436C>T
r.(?)
p.(Arg146*)
-
likely pathogenic
g.16035000G>A
g.16033377G>A
PROM1 c.436C>T c.1354dup, p.Arg146* p.Tyr452Leufs*13
-
PROM1_000127
heterozygous
PubMed: Gliem 2020
-
-
Unknown
?
-
-
-
-
LOVD
+?/.
-
c.436C>T
r.(?)
p.(Arg146*)
ACMG
pathogenic
g.16035000G>A
g.16033377G>A
c.436C>T, p.Arg146*
-
PROM1_000127
compound heterozygous
PubMed: Cehajic-Kapetanovic 2019
-
-
Unknown
?
-
-
-
-
LOVD
+/.
-
c.436C>T
r.(?)
p.(Arg146*)
ACMG
pathogenic
g.16035000G>A
-
-
-
PROM1_000127
-
Villafuerte-de la Cruz RA, et al., 2023. Submitted
ClinVar-1065650
rs780697796
Germline
yes
-
-
-
-
Rocio Villafuerte-de la Cruz
?/.
4
c.437G>A
r.(?)
p.(Arg146Gln)
-
VUS
g.16034999C>T
g.16033376C>T
c.437G>A, p. Arg146Gln
-
PROM1_000204
unsolved
PubMed: Del Pozo-Valero
-
-
Germline
no
-
-
-
-
LOVD
?/.
-
c.437G>A
r.(?)
p.(Arg146Gln)
ACMG
VUS
g.16034999C>T
g.16033376C>T
-
-
PROM1_000204
ACMG PM2; no variant 2nd chromosome
PubMed: Weisschuh 2024
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
6
c.439C>T
r.spl?
p.(Gln147*)
ACMG
pathogenic
g.16034997G>A
g.16033374G>A
-
-
PROM1_000179
-
Tracewska 2021, MolVis in press
-
-
Germline
yes
0 (in-house database, ~5000 samples)
-
-
-
LOVD
+/.
-
c.442A>T
r.(?)
p.(Lys148*)
-
likely pathogenic (recessive)
g.16034994T>A
-
-
-
PROM1_000121
-
PubMed: Jinda 2014
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
-
c.442A>T
r.(?)
p.(Lys148*)
-
likely pathogenic (recessive)
g.16034994T>A
g.16033371T>A
-
-
PROM1_000121
-
PubMed: Jinda 2017
-
-
Germline
-
-
-
-
-
LOVD
?/.
-
c.454C>T
r.(?)
p.(Pro152Ser)
-
VUS
g.16034982G>A
-
PROM1(NM_006017.3):c.454C>T (p.P152S)
-
PROM1_000240
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+?/.
-
c.485del
r.(?)
p.(Leu162Argfs*3)
-
likely pathogenic
g.16034951del
g.16033328del
c.485_485delT
-
PROM1_000147
-
PubMed: Huang 2018
-
-
Germline
-
-
-
-
-
LOVD
-?/.
-
c.489G>A
r.(?)
p.(Leu163=)
-
likely benign
g.16034947C>T
g.16033324C>T
PROM1(NM_001145847.2):c.462G>A (p.L154=)
-
PROM1_000094
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
4i
c.510-1G>A
r.spl
p.?
-
pathogenic
g.16026936C>T
g.16025313C>T
-
-
PROM1_000007
-
-
-
-
Unknown
-
-
-
-
-
Feng Wang
+/.
3i
c.510-1G>A
r.(?)
p.?
-
pathogenic
g.16026936C>T
-
c.510-1G>A
-
PROM1_000007
-
PubMed: Wang-2014
-
-
Unknown
-
-
-
-
-
LOVD
+?/.
3i
c.510-1G>A
r.spl?
p.(?)
-
likely pathogenic
g.16026936C>T
-
c.510-1G>A
-
PROM1_000007
-
PubMed: Panneman 2023
-
-
Unknown
-
-
-
-
-
Daan Panneman
+?/.
6
c.524A>C
r.(?)
p.(Tyr175Ser)
-
likely pathogenic
g.16026921T>G
g.16025298T>G
-
-
PROM1_000006
predicted to affect function, but insufficient evidence for definite conclusion
PubMed: Neveling 2012
-
-
Germline
no
-
-
-
-
Kornelia Neveling
+?/.
4
c.544C>T
r.(?)
p.(Gln182*)
-
likely pathogenic
g.16026901G>A
-
c.544C>T
-
PROM1_000264
-
PubMed: Panneman 2023
-
-
Unknown
-
-
-
-
-
Daan Panneman
?/.
-
c.604C>G
r.(?)
p.(Arg202Gly)
-
VUS
g.16026841G>C
g.16025218G>C
PROM1(NM_001145847.1):c.577C>G (p.R193G), PROM1(NM_001145847.2):c.577C>G (p.R193G), PROM1(NM_006017.3):c.604C>G (p.(Arg202Gly))
-
PROM1_000093
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/.
-
c.604C>G
r.(?)
p.(Arg202Gly)
-
likely benign
g.16026841G>C
-
PROM1(NM_001145847.1):c.577C>G (p.R193G), PROM1(NM_001145847.2):c.577C>G (p.R193G), PROM1(NM_006017.3):c.604C>G (p.(Arg202Gly))
-
PROM1_000093
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/.
-
c.604C>G
r.(?)
p.(Arg202Gly)
-
likely pathogenic
g.16026841G>C
g.16025218G>C
-
-
PROM1_000093
-
PubMed: Patel 2016
-
-
Germline
-
-
-
-
-
LOVD
?/.
5
c.604C>G
r.(?)
p.(Arg202Gly)
-
VUS
g.16026841G>C
g.16025218G>C
c.604C>G, p.Arg202Gly
-
PROM1_000093
unsolved
PubMed: Del Pozo-Valero
-
-
Germline
?
-
-
-
-
LOVD
?/.
5
c.604C>G
r.(?)
p.(Arg202Gly)
-
VUS
g.16026841G>C
g.16025218G>C
c.604C>G, p.Arg202Gly
-
PROM1_000093
unsolved
PubMed: Del Pozo-Valero
-
-
Germline
?
-
-
-
-
LOVD
?/.
-
c.604C>G
r.(?)
p.(Arg202Gly)
ACMG
VUS
g.16026841G>C
g.16025218G>C
-
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PROM1_000093
ACMG PP5, BP6
PubMed: Weisschuh 2024
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Germline
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Johan den Dunnen
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