Global Variome shared LOVD
PRSS1 (protease, serine, 1 (trypsin 1))
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Unique variants in the PRSS1 gene
The variants shown are described using the NM_002769.4 transcript reference sequence.
Legend
Please note that a short description of a certain column can be displayed when you move your mouse cursor over the column's header and hold it still. Below, a more detailed description is shown per column.
Effect
: The variant's effect on the function of the gene/protein, displayed in the format 'R/C'. R is the value reported by the source (publication, submitter) and this classification may vary between records. C is the value concluded by the curator. Note that in some database the curator uses Summary records to give details on the classification of the variant.Values used: '+' indicating the variant affects function, '+?' probably affects function, '-' does not affect function, '-?' probably does not affect function, '?' effect unknown, '.' effect was not classified.
Reported
: The number of times this variant has been reported in the database.
Exon
: number of exon/intron containing variant; 2 = exon 2, 12i = intron 12, 2i_7i = from intron 2 to intron 7, 8i_9 = intron 8/exon 9 boundary, _1 = 5' to exon 1, 18_ = 3' of exon 18, _1_18_ = encompassing the entire 18-exon gene
DNA change (cDNA)
: description of variant at DNA level, based on a coding DNA reference sequence (following HGVS recommendations); e.g. c.123C>T, c.123_145del, c.123_126dup. For deletions/duplications extending beyond the reference transcript resp. {0}/{2} is used to replace del/dup. Extent of the deletion/duplication should be specified using the genomic description (g.). "-" indicates the variant described on genomic level does not affect the coding DNA reference sequence.
RNA change
: description of variant at RNA level (following HGVS recommendations).
r.123c>u
r.? = unknown
r.(?) = RNA not analysed but probably transcribed copy of DNA variant
r.spl? = RNA not analysed but variant probably affects splicing
r.(spl?) = RNA not analysed but variant may affect splicing
r.0? = change expected to abolish transcription
Protein
: description of variant at protein level (following HGVS recommendations).
p.(Arg345Pro) = change predicted from DNA (RNA not analysed)
p.Arg345Pro = change derived from RNA analysis
p.? = unknown effect
p.0? = probably no protein produced
Classification method
: The method used for the clinical classification of this variant.
All options:
ACMG
ACGS
EAHAD-CFDB
ENIGMA
IARC
InSiGHT
kConFab
other
Clinical classification
: Clinical classification of variant, preferably based on standardised criteria (e.g. ACMG), directed on the clinical consequences as published/submitted, indicated using an enriched system including inheritance: e.g. pathogenic, pathogenic (dominant), pathogenic (recessive), pathogenic (!), pathogenic (maternal), pathogenic (paternal). Standard inheritance is covered by dominant/recessive, imprinting by maternal/paternal. A '!' warns for exceptional circumstances to be explained in the 'Remarks' field (low penetrance, variants pathogenic in heterozygous state only, hypomorphic/hypermorphic variants, protective variants, etc.). Non-disease consequences (e.g. drug metabolism (pharmacogenetics), risk factor, blood group, tasting bitter) are indicated using additions to the benign classification; benign (dominant), benign (recessive), benign (!), etc. The value 'association' is used for variants associated with a phenotype and 'NA' for variants from in vitro/in silico records. NOTE: classification may differ from the opinion of the curator as given in a variant SUMMARY-record or the 'Functional effect concluded'). NOTE: pathogenic/likely pathogenic should go together with "variant (probably) affects function" In ClassFunctional.
All options:
pathogenic
pathogenic (dominant)
pathogenic (recessive)
pathogenic (!)
pathogenic (maternal)
pathogenic (paternal)
likely pathogenic
likely pathogenic (dominant)
likely pathogenic (recessive)
likely pathogenic (!)
likely pathogenic (maternal)
likely pathogenic (paternal)
VUS
VUS (!)
likely benign
likely benign (dominant)
likely benign (recessive)
likely benign (!)
likely benign (maternal)
likely benign (paternal)
benign
benign (dominant)
benign (recessive)
benign (!)
benign (maternal)
benign (paternal)
conflicting
association
NA
DNA change (genomic) (hg19)
: HGVS description of variant at DNA level, based on the genomic (chromosomal) DNA reference sequence; e.g. g.12345678C>T, g.12345679del, g.12345678_12345890dup
DNA change (hg38)
: HGVS description of variant at DNA level, based on the hg38 genomic (chromosomal) eference sequence; e.g. g.12345678C>T, g.12345679del, g.12345678_12345890dup
Published as
: listed only when different from "DNA change"; variant as reported originally (e.g. 521delT). Variants seen in animal models, tested in vitro, predicted from RNA analysis, etc. are described between brackets like c.(456C>G)
ISCN
: description of the variant according to ISCN nomenclature
DB-ID
: database ID of variant, grouping multiple observations of the same variant together, starting with the HGNC gene symbol, followed by an underscore (_) and a six digit number (e.g. DMD_012345). _000000 is used for variants where DNA was not analysed (change predicted from RNA analysis), variants seen in animal models or variants not seen in humans but functionally tested in vitro
Variant remarks
: remarks regarding variant described, e.g. germline mosaicism in mother, 345 kb deletion, muscle RNA analysed, not in 200 control chromosomes tested, on founder haplotype, etc.
Reference
: publication describing the variant submitted, incl. links to OMIM, PubMed or other source, e.g. "den Dunnen ASHG2003 P2346"
ClinVar ID
: ID of variant in ClinVar database
dbSNP ID
: the dbSNP ID
Origin
: Origin of variant/record: Germline = in all cells, De novo = in all cells, but not in either parent, Germline/De novo (untested) = in all cells, parents not tested (use only when De novo is likely, e.g. isolated/sporadic cases with dominant disease), Somatic = present in a subset of cells, but not in either parent, Uniparental disomy = from parental disomy (maternal or paternal), CLASSIFICATION record = submitter only sharing variant classification (note another report may share Individual data), SUMMARY record = master summary record from curator (may link to another database), In vitro (cloned) = data resulting from in vitro functional assays, animal model = data from animal model, Artefact = false positive variant call, DUPLICATE record = variant already described on another chromosome (e.g. unbalanced translocation, duplicating transposition, 2nd fusion transcript, etc.)
All options:
Germline
De novo
Germline/De novo (untested)
Somatic
Uniparental disomy
Uniparental disomy, maternal allele
Uniparental disomy, paternal allele
CLASSIFICATION record
SUMMARY record
In vitro (cloned)
In silico
animal model
Artefact
DUPLICATE record
Unknown
Not applicable
Segregation
: Indicates whether the variant segregates with the phenotype (yes), does not segregate with the phenotype (no) or segregation is unknown (?)
All options:
? = unknown
yes = segregates with phenotype
no = does not segregate with phenotype
- = not applicable
Frequency
: frequency in which the variant was found; e.g 5/760 chromosomes (in 5 of 760 chromosomes tested), 1/33 patients (in 1 of 33 patients analysed in study), 0.05 controls (in 5% of control cases tested)
Re-site
: restriction enzyme recognition site created (+) or destroyed (-); e.g. BglII+;BamHI-
VIP
: variant VIP-status was requested for matchmaking - need collaboration(s) to crack the case - please contact the submitter/curator. NOTE: to get VIP status ask the curator.
Methylation
: result of methylation test; GOM (gain of methylation), LOM (loss of methylation), 30% (30% methylated). NOTE: when several tests were done mention the method as well (e.g. MS-PCR 75%)
How to query this table
All list views have search fields which can be used to search data. You can search for a complete word or you can search for a part of a search term. If you enclose two or more words in double quotes, LOVD will search for the combination of those words only exactly in the order you specify. Note that search terms are case-insensitive and that wildcards such as * are treated as normal text! For all options, like "and", "or", and "not" searches, or searching for prefixes or suffixes, see the table below.
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.
72 entries on 1 page. Showing entries 1 - 72.
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Legend
How to query
Effect
Reported
Exon
DNA change (cDNA)
RNA change
Protein
Classification method
Clinical classification
DNA change (genomic) (hg19)
DNA change (hg38)
Published as
ISCN
DB-ID
Variant remarks
Reference
ClinVar ID
dbSNP ID
Origin
Segregation
Frequency
Re-site
VIP
Methylation
Owner
-/.
1
-
c.30G>T
r.(?)
p.(Val10=)
-
benign
g.142457365G>T
-
PRSS1(NM_002769.4):c.30G>T (p.(=))
-
PRSS1_000012
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
-
c.38C>T
r.(?)
p.(Ala13Val)
-
VUS
g.142457373C>T
-
-
-
PRSS1_000089
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/.
1
-
c.40C>G
r.(?)
p.(Leu14Val)
-
benign
g.142457375C>G
g.142749524C>G
PRSS1(NM_002769.5):c.40C>G (p.L14V)
-
PRSS1_000013
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-?/.
1
-
c.40+7A>T
r.(=)
p.(=)
-
likely benign
g.142457382A>T
g.142749531A>T
PRSS1(NM_002769.4):c.40+7A>T
-
PRSS1_000014
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.41-166G>T
r.spl?
p.(?)
ACMG
likely benign
g.142458240G>T
g.142750389G>T
-
-
PRSS1_000081
-
-
-
rs182172370
Germline
-
-
-
-
-
Hasan Bas
-?/.
1
-
c.44C>G
r.(?)
p.(Ala15Gly)
-
likely benign
g.142458409C>G
g.142750558C>G
PRSS1(NM_002769.4):c.44C>G (p.(Ala15Gly))
-
PRSS1_000048
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/., +?/.
4
2
c.47C>T
r.(?)
p.(Ala16Val)
-
likely pathogenic, pathogenic
g.142458412C>T
g.142750561C>T
A16V, p.A16V
-
PRSS1_000044
The variant was reported at the protein level only
PubMed: Chen 2001
,
Journal: Chen 2001
,
PubMed: Rosendahl 2013
,
Journal: Rosendahl 2013
ClinVar-38363
rs202003805
CLASSIFICATION record, Germline
?
14/660 cases, 2/221 cases
-
-
-
MobiDetails
,
Hasan Bas
+?/.
1
2
c.49C>A
r.(?)
p.(Pro17Thr)
-
likely pathogenic
g.142458414C>A
g.142750563C>A
-
-
PRSS1_000062
Authors suggest similar biochemical effect as known pathogenic variant, p.A16V
PubMed: Németh 2017
,
Journal: Németh 2017
-
-
De novo
-
-
-
-
-
Hasan Bas
-/., -?/.
2
-
c.49C>T
r.(?)
p.(Pro17Ser)
-
benign, likely benign
g.142458414C>T
g.142750563C>T
PRSS1(NM_002769.4):c.49C>T (p.P17S, p.(Pro17Ser))
-
PRSS1_000016
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
+/.
1
2
c.56A>C
r.(?)
p.(Asp19Ala)
-
pathogenic
g.142458421A>C
g.142750570A>C
D19A
-
PRSS1_000082
-
-
-
-
Germline
?
-
-
-
-
Hasan Bas
+/.
1
2
c.62A>C
r.(?)
p.(Asp21Ala)
ACMG
pathogenic
g.142458427A>C
g.142750576A>C
-
-
PRSS1_000063
-
-
-
-
Germline
?
-
-
-
-
Hasan Bas
+/.
2
2
c.68A>G
r.(?)
p.(Lys23Arg)
-
pathogenic
g.142458433A>G
g.142750582A>G
K23R
-
PRSS1_000083
The variant was reported at the protein level only
PubMed: Jalaly 2017
,
Journal: Jalaly 2017
ClinVar-11878
rs111033567
Germline
?
1/59 "an unexplained first episode of AP <35 years of age" cases,
1 more item
-
-
-
Hasan Bas
+/., +?/.
4
2
c.86A>C
r.(?)
p.(Asn29Thr)
-
likely pathogenic, pathogenic
g.142458451A>C
g.142750600A>C
p.N29T, PRSS1(NM_002769.5):c.86A>C (p.N29T)
-
PRSS1_000008
The variant was reported at the protein level only, VKGL data sharing initiative Nederland
PubMed: Murali 2021
,
Journal: Murali 2021
,
PubMed: Rosendahl 2013
,
Journal: Rosendahl 2013
,
1 more item
ClinVar-38366
rs111033566
CLASSIFICATION record, Germline, Unknown
?
1/189 kindreds, 1/660 cases
-
-
-
Johan den Dunnen
,
VKGL-NL_Groningen
,
Hasan Bas
+/., ?/.
6
2
c.86A>T
r.(?)
p.(Asn29Ile)
-
pathogenic, VUS
g.142458451A>T
g.142750600A>T
c.86A>T (N29I), N29I, p.N29I
-
PRSS1_000006
in combination withc.592-8C>T + c.592-11C>T (IVS4),
1 more item
PubMed: Murali 2021
,
Journal: Murali 2021
,
PubMed: Rosendahl 2013
,
Journal: Rosendahl 2013
,
2 more items
ClinVar-11877
rs111033566
Germline
?
1/109 cases, 1/189 kindreds, 8/660 cases
-
-
-
Johan den Dunnen
,
Andreas Laner
,
Hasan Bas
,
Jeanette Yuen
?/.
1
2
c.107C>G
r.(?)
p.(Pro36Arg)
-
VUS
g.142458472C>G
g.142750621C>G
P36R
-
PRSS1_000071
-
PubMed: Chen 2001
,
Journal: Chen 2001
ClinVar-459181
rs769459903
Germline
-
1/221 cases
-
-
-
Hasan Bas
+?/.
1
2
c.116T>C
r.(?)
p.(Val39Ala)
-
likely pathogenic
g.142458481T>C
g.142750630T>C
-
-
PRSS1_000073
-
PubMed: Arduino 2005
,
Journal: Arduino 2005
-
rs397507439
Germline
yes
-
-
-
-
Hasan Bas
-?/.
1
-
c.121C>T
r.(?)
p.(Leu41=)
-
likely benign
g.142458486C>T
g.142750635C>T
PRSS1(NM_002769.4):c.121C>T (p.L41=)
-
PRSS1_000017
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.146G>T
r.(?)
p.(Gly49Val)
-
VUS
g.142458511G>T
g.142750660G>T
PRSS1(NM_002769.4):c.146G>T (p.G49V)
-
PRSS1_000049
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/., ?/.
2
-
c.161A>G
r.(?)
p.(Asn54Ser)
-
likely benign, VUS
g.142458526A>G
-
PRSS1(NM_002769.5):c.161A>G (p.N54S), PRSS2(NM_001303414.1):c.161A>G (p.N54S)
-
PRSS1_000020
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
,
VKGL-NL_Utrecht
?/.
1
-
c.200+1G>A
r.spl?
p.?
-
VUS
g.142458566G>A
-
PRSS1(NM_002769.5):c.200+1G>A
-
PRSS1_000026
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-?/.
1
-
c.200+13C>T
r.(=)
p.(=)
-
likely benign
g.142458578C>T
g.142750727C>T
-
-
PRSS1_000056
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/.
1
-
c.200+44C>G
r.(=)
p.(=)
-
benign
g.142458609C>G
g.142750758C>G
-
-
PRSS1_000007
-
-
-
-
Germline
-
-
-
-
-
Andreas Laner
-/.
1
-
c.200+45T>G
r.(=)
p.(=)
-
benign
g.142458610T>G
g.142750759T>G
-
-
PRSS1_000001
-
-
-
-
Germline
-
-
-
-
-
Andreas Laner
-/.
1
-
c.200+402A>T
r.(=)
p.(=)
-
benign
g.142458967A>T
g.142751116A>T
PRSS1(NM_002769.5):c.200+402A>T
-
PRSS1_000027
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-/.
1
-
c.200+407T>C
r.(=)
p.(=)
-
benign
g.142458972T>C
g.142751121T>C
PRSS1(NM_002769.5):c.200+407T>C
-
PRSS1_000028
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-/.
1
-
c.202C>T
r.(?)
p.(Arg68Cys)
-
benign
g.142459626C>T
g.142751775C>T
PRSS1(NM_002769.4):c.202C>T (p.R68C)
-
PRSS1_000029
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.234C>T
r.(?)
p.(Ile78=)
-
likely benign
g.142459658C>T
g.142751807C>T
PRSS1(NM_002769.4):c.234C>T (p.I78=)
-
PRSS1_000050
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
3
3
c.235G>A
r.(?)
p.(Glu79Lys)
-
VUS
g.142459659G>A
g.142751808G>A
E79K
-
PRSS1_000057
VKGL data sharing initiative Nederland,
1 more item
PubMed: Chen 2001
Journal: Chen 2001
,
PubMed: Chen 2001
,
Journal: Chen 2001
ClinVar- 11880
,
ClinVar-11880
rs111033564
CLASSIFICATION record, Germline
-
1/221 cases, 2/400 controls
-
-
-
VKGL-NL_Nijmegen
,
Hasan Bas
?/.
1
3
c.248G>A
r.(?)
p.(Gly83Glu)
-
VUS
g.142459672G>A
g.142751821G>A
G83E
-
PRSS1_000070
-
PubMed: Chen 2001
,
Journal: Chen 2001
-
-
Germline
-
1/221 cases
-
-
-
Hasan Bas
?/.
1
3
c.276G>T
r.(?)
p.(Lys92Asn)
-
VUS
g.142459700G>T
g.142751849G>T
K92N
-
PRSS1_000069
-
PubMed: Chen 2001
,
Journal: Chen 2001
-
-
Germline
?
1/221 cases
-
-
-
Hasan Bas
+?/., ?/.
2
3
c.298G>C
r.(?)
p.(Asp100His)
-
likely pathogenic, VUS
g.142459722G>C
g.142751871G>C
-
-
PRSS1_000074
-
PubMed: Hamoir 2013
,
Journal: Hamoir 2013
,
PubMed: Tautermann 2001
,
Journal: Tautermann 2001
-
-
Germline
?
1/109 cases, 1/351 cases
-
-
-
Hasan Bas
?/.
4
3
c.310C>G
r.(?)
p.(Leu104Val)
ACMG
VUS
g.142459734C>G
g.142751883C>G
310C>G
-
PRSS1_000064
variant also found in 2 healthy male relatives
PubMed: Gou 2013
,
PubMed: Gou 2013
,
Journal: Gou 2013
-
-
Germline
?
-
-
-
-
Hasan Bas
+?/.
3
3
c.311T>C
r.(?)
p.(Leu104Pro)
-
likely pathogenic
g.142459735T>C
g.142751884T>C
-
-
PRSS1_000075
-
PubMed: Enea 2019
,
Journal: Enea 2019
,
PubMed: Sofia 2016
,
Journal: Sofia 2016
ClinVar-440201
rs1554499091
Germline
?, yes
1/80 cases
-
-
-
Hasan Bas
+/., +?/.
4
3
c.346C>T
r.(?)
p.(Arg116Cys)
-
likely pathogenic, pathogenic
g.142459770C>T
g.142751919C>T
p.R116C, R116C
-
PRSS1_000076
The variant was reported at the protein level only
PubMed: Rosendahl 2013
,
Journal: Rosendahl 2013
,
PubMed: Tautermann 2001
,
Journal: Tautermann 2001
ClinVar-29923
rs387906698
Germline
?
1/109 cases, 2/660 cases
-
-
-
Hasan Bas
+/., +?/.
5
3
c.364C>T
r.(?)
p.(Arg122Cys)
ACMG
likely pathogenic, pathogenic
g.142459788C>T
g.142751937C>T
p.R122C, PRSS1(NM_002769.5):c.364C>T (p.R122C)
-
PRSS1_000002
The variant was reported at the protein level only, VKGL data sharing initiative Nederland
PubMed: Rosendahl 2013
,
Journal: Rosendahl 2013
ClinVar-11883
rs111033568
CLASSIFICATION record, Germline
?
5/660
-
-
-
Andreas Laner
,
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
,
Hasan Bas
+/., +?/.
18
2, 3
c.365G>A
r.(?)
p.(Arg122His)
ACMG
likely pathogenic, pathogenic, pathogenic (dominant)
g.142459789G>A
g.142751938G>A
p.R122H, R117H, R122H, R122R/H,
1 more item
-
PRSS1_000003
The variant was reported at the protein level only, VKGL data sharing initiative Nederland
PubMed: Ellison 2020
,
Journal: Ellison 2020
,
PubMed: Marfil-Garza 2021
,
Journal: Marfil-Garza 2021
,
5 more items
ClinVar-11876
rs111033565
CLASSIFICATION record, Germline
?, yes
1/100 individuals, 19/128 cases, 2/109 cases, 2/80 cases, 25/660 cases
-
-
-
Andreas Laner
,
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
,
Hasan Bas
+/.
1
3
c.365_366delinsAT
r.(?)
p.(Arg122His)
-
likely pathogenic
g.142459789_142459790delinsAT
g.142751938_142751939delinsAT
CGC>CAT R122H
-
PRSS1_000072
-
PubMed: Chen 2001
,
Journal: Chen 2001
ClinVar-11882
rs267606982
Germline
-
1/221 cases
-
-
-
Hasan Bas
+?/.
1
3
c.367G>A
r.(?)
p.(Val123Met)
-
VUS
g.142459791G>A
g.142751940G>A
V123M
-
PRSS1_000068
-
PubMed: Chen 2001
,
Journal: Chen 2001
ClinVar-411136
rs144403091
Germline
-
1/221 cases
-
-
-
Hasan Bas
?/.
1
3
c.371C>T
r.(?)
p.(Ser124Phe)
-
VUS
g.142459795C>T
g.142751944C>T
p.S124F
-
PRSS1_000077
The variant was reported at the protein level only
PubMed: Rosendahl 2013
,
Journal: Rosendahl 2013
-
rs1484849588
Germline
?
1/660 cases
-
-
-
Hasan Bas
-?/.
1
-
c.400G>A
r.(?)
p.(Ala134Thr)
-
likely benign
g.142459824G>A
g.142751973G>A
PRSS1(NM_002769.4):c.400G>A (p.A134T)
-
PRSS1_000051
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/.
2
3
c.416G>T
r.(?)
p.(Cys139Phe)
-
likely pathogenic
g.142459840G>T
g.142751989G>T
-
-
PRSS1_000078
-
PubMed: Sofia 2016
,
Journal: Sofia 2016
ClinVar-618848
rs768853338
Germline
?
2/80 cases
-
-
-
Hasan Bas
-?/.
1
-
c.417C>T
r.(=)
p.(=)
-
likely benign
g.142459841C>T
-
-
-
PRSS1_000067
-
PubMed: Gou 2013
,
Journal: Gou 2013
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.447C>T
r.(?)
p.(Ser149=)
-
likely benign
g.142459871C>T
g.142752020C>T
PRSS1(NM_002769.4):c.447C>T (p.S149=)
-
PRSS1_000030
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.454G>A
r.(?)
p.(Ala152Thr)
-
VUS
g.142459878G>A
-
-
-
PRSS1_000088
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/.
1
-
c.455-21T>A
r.(?), r.spl?
p.(=), p.(?)
ACMG
benign
g.142460261T>A
g.142752410T>A
-
-
PRSS1_000084
-
-
-
rs201825825
Germline
-
-
-
-
-
Hasan Bas
-/.
6
4
c.486T>C
r.(=), r.(?)
p.(Asp162=)
ACMG
benign
g.142460313T>C
g.142752462T>C
GAC>GAT (D162D), PRSS1(NM_002769.5):c.486T>C (p.(Asp162=), p.D162=)
-
PRSS1_000053
The authors reported the variant as a "risk variant haplotype in homozygotes",
1 more item
PubMed: Ellison 2020
,
Journal: Ellison 2020
,
PubMed: Tautermann 2001
,
Journal: Tautermann 2001
ClinVar-239391
rs6666
CLASSIFICATION record, Germline
?
44/109 cases, 6.10E−01 in 100 individuals
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Groningen
,
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
,
Hasan Bas
+?/.
1
4
c.487G>A
r.(?)
p.(Ala163Thr)
-
likely pathogenic
g.142460314G>A
g.142752463G>A
-
-
PRSS1_000085
-
PubMed: Sofia 2016
,
Journal: Sofia 2016
ClinVar-825253
rs557691366
Germline
?
1/80 cases
-
-
-
Hasan Bas
-/., ?/.
2
-
c.508A>G
r.(?)
p.(Lys170Glu)
-
benign, VUS
g.142460335A>G
g.142752484A>G
PRSS1(NM_002769.5):c.508A>G (p.K170E)
-
PRSS1_000045
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
,
VKGL-NL_Utrecht
+?/.
2
4
c.541A>G
r.(?)
p.(Ser181Gly)
-
likely pathogenic
g.142460368A>G
g.142752517A>G
-
-
PRSS1_000086
The variant was also present in the healthy mother of the proband
PubMed: Corleto 2010
,
Journal: Corleto 2010
ClinVar-571921
rs376907511
Germline
?
-
-
-
-
Hasan Bas
-/.
2
-
c.542G>A
r.(?)
p.(Ser181Asn)
-
benign
g.142460369G>A
g.142752518G>A
PRSS1(NM_002769.5):c.542G>A (p.S181N)
-
PRSS1_000033
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
,
VKGL-NL_Utrecht
-?/.
1
-
c.547A>G
r.(?)
p.(Met183Val)
-
likely benign
g.142460374A>G
g.142752523A>G
PRSS1(NM_002769.4):c.547A>G (p.(Met183Val))
-
PRSS1_000034
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
1
-
c.552C>T
r.(?)
p.(Phe184=)
-
likely benign
g.142460379C>T
g.142752528C>T
PRSS1(NM_002769.4):c.552C>T (p.F184=)
-
PRSS1_000035
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.561C>T
r.(?)
p.(Gly187=)
-
likely benign
g.142460388C>T
g.142752537C>T
PRSS1(NM_002769.4):c.561C>T (p.G187=)
-
PRSS1_000036
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/.
1
4
c.568G>A
r.(?)
p.(Glu190Lys)
-
likely pathogenic
g.142460395G>A
g.142752544G>A
-
-
PRSS1_000065
-
PubMed: Jancsó 2019
,
Journal: Jancsó 2019
-
-
Germline
?
-
-
-
-
Hasan Bas
-?/.
1
-
c.591+64G>T
r.(=)
p.(=)
-
likely benign
g.142460482G>T
-
PRSS1:c.591+64G>T
-
PRSS1_000058
-
PubMed: Maranhao 2015
-
-
Germline
-
12/25 families
-
-
-
LOVD
-?/.
1
-
c.591+77A>C
r.(=)
p.(=)
-
likely benign
g.142460495A>C
-
PRSS1:c.591+77A>C
-
PRSS1_000059
-
PubMed: Maranhao 2015
-
-
Germline
-
15/25 families
-
-
-
LOVD
-?/.
1
-
c.591+85T>C
r.(=)
p.(=)
-
likely benign
g.142460503T>C
-
PRSS1:c.591+85T>C
-
PRSS1_000060
-
PubMed: Maranhao 2015
-
-
Germline
-
9/25 families
-
-
-
LOVD
?/.
1
4i
c.592-79G>A
r.spl?
p.(?)
-
VUS
g.142460640G>A
g.142752789G>A
IVS4-79G>A
-
PRSS1_000079
-
PubMed: Tautermann 2001
,
Journal: Tautermann 2001
ClinVar-1167462
rs531271210
Germline
?
1/109 cases
-
-
-
Hasan Bas
?/.
1
4i
c.592-78G>A
r.spl?
p.(?)
-
VUS
g.142460641G>A
g.142752790G>A
IVS-78G>A
-
PRSS1_000080
-
PubMed: Tautermann 2001
,
Journal: Tautermann 2001
-
rs1337286040
Germline
?
1/109 cases
-
-
-
Hasan Bas
?/.
1
-
c.592-24C>T
r.(=)
p.(=)
-
VUS
g.142460695C>T
g.142752844C>T
-
-
PRSS1_000004
-
-
-
rs192452846
Germline
-
-
-
-
-
Andreas Laner
+?/-?, -/.
2
-
c.592-11C>T
r.(=)
p.(=)
-
benign, benign (!)
g.142460708C>T
g.142752857C>T
-
-
PRSS1_000005
1 more item
PubMed: Sofia 2016
,
Journal: Sofia 2016
ClinVar-258800
rs183791770
Germline
?
2/80 cases
-
-
-
Andreas Laner
,
Hasan Bas
-?/.
1
-
c.592-9T>C
r.(=)
p.(=)
-
likely benign
g.142460710T>C
g.142752859T>C
PRSS1(NM_002769.4):c.592-9T>C
-
PRSS1_000038
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/-?, -?/.
2
-
c.592-8C>T
r.(=)
p.(=)
-
benign (!), likely benign
g.142460711C>T
g.142752860C>T
PRSS1(NM_002769.4):c.592-8C>T
-
PRSS1_000039
VKGL data sharing initiative Nederland,
1 more item
PubMed: Sofia 2016
,
Journal: Sofia 2016
ClinVar-416610
rs200381474
CLASSIFICATION record, Germline
?
2/80 cases
-
-
-
VKGL-NL_Rotterdam
,
Hasan Bas
?/.
2
-
c.592-4C>T
r.(?), r.spl?
p.(=), p.?
ACMG
VUS
g.142460715C>T
g.142752864C>T
-
-
PRSS1_000066
-
-
-
rs375342697
Germline
?
-
-
-
-
Hasan Bas
+?/.
5
5
c.623G>C
r.(?)
p.(Gly208Ala)
-
likely pathogenic
g.142460750G>C
g.142752899G>C
G208A/G
-
PRSS1_000087
The variant was reported at the protein level only
PubMed: Saito 2016
,
Journal: Saito 2016
ClinVar-258802
rs189270875
Germline
?
7/128 cases
-
-
-
Hasan Bas
-?/.
1
-
c.637G>A
r.(?)
p.(Val213Ile)
-
likely benign
g.142460764G>A
g.142752913G>A
PRSS1(NM_002769.4):c.637G>A (p.V213I)
-
PRSS1_000041
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.651T>C
r.(?)
p.(Gly217=)
-
likely benign
g.142460778T>C
g.142752927T>C
PRSS1(NM_002769.4):c.651T>C (p.G217=)
-
PRSS1_000054
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.652G>T
r.(?)
p.(Asp218Tyr)
-
likely benign
g.142460779G>T
g.142752928G>T
PRSS1(NM_002769.4):c.652G>T (p.(Asp218Tyr))
-
PRSS1_000042
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
1
-
c.654T>C
r.(?)
p.(Asp218=)
-
likely benign
g.142460781T>C
g.142752930T>C
PRSS1(NM_002769.4):c.654T>C (p.D218=)
-
PRSS1_000055
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
2
-
c.674A>G
r.(?)
p.(Lys225Arg)
-
likely benign
g.142460801A>G
g.142752950A>G
PRSS1(NM_002769.4):c.674A>G (p.K225R, p.(Lys225Arg))
-
PRSS1_000047
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
-?/.
1
-
c.675G>T
r.(?)
p.(Lys225Asn)
-
likely benign
g.142460802G>T
-
PRSS1(NM_002769.4):c.675G>T (p.K225N), PRSS2(NM_001303414.1):c.717G>T (p.R239S)
-
PRSS1_000061
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/.
4
5
c.738T>C
r.(=), r.(?)
p.(Asn246=), p.(Asn46=)
ACMG
benign
g.142460865T>C
g.142753014T>C
AAC>AAT (N246N), N246N, PRSS1(NM_002769.5):c.738T>C (p.N246=)
-
PRSS1_000043
The authors reported the variant as a "risk variant haplotype in homozygotes",
1 more item
PubMed: Ellison 2020
,
Journal: Ellison 2020
,
PubMed: Tautermann 2001
,
Journal: Tautermann 2001
ClinVar-258803
rs6667
CLASSIFICATION record, Germline
?
44/109 cases, 6.45E−01 in 100 individuals
-
-
-
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
,
Hasan Bas
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