Global Variome shared LOVD
PSEN1 (presenilin 1)
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Unique variants in the PSEN1 gene
The variants shown are described using the NM_000021.3 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%)
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.
229 entries on 3 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
-?/.
1
-
c.21G>A
r.(?)
p.(Pro7=)
-
likely benign
g.73614748G>A
g.73148040G>A
PSEN1(NM_000021.3):c.21G>A (p.P7=)
-
PSEN1_000415
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/?
1
4
c.(96T>C)
r.(?)
p.(=)
-
VUS
g.72707266T>C
g.72240558T>C
-
-
PSEN1_000209
Observed in 1 AD patient. /r/Silent point mutation in coding region
-
-
-
Unknown
-
-
-
-
-
Marc Cruts
-/-, -?/., ?/.
4
4
c.104G>A
r.(?)
p.(Arg35Gln)
-
benign, likely benign, VUS
g.73637521G>A
g.73170813G>A
PSEN1(NM_000021.3):c.104G>A (p.R35Q)
-
PSEN1_000204
;VKGL data sharing initiative Nederland,
1 more item
-
-
rs63750592
CLASSIFICATION record, Germline, Unknown
no
-
-
-
-
Zafar Iqbal
,
VKGL-NL_Rotterdam
,
Marc Cruts
-?/.
1
-
c.118_120del
r.(?)
p.(Asp40del)
-
likely benign
g.73637535_73637537del
-
PSEN1(NM_000021.4):c.118_120del (p.(Asp40del))
-
PSEN1_000426
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
-
c.124C>T
r.(?)
p.(Arg42Trp)
-
VUS
g.73637541C>T
-
PSEN1(NM_000021.4):c.124C>T (p.(Arg42Trp))
-
PSEN1_000427
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
2
-
c.234C>T
r.(?)
p.(Gly78=)
-
likely benign
g.73637651C>T
g.73170943C>T
PSEN1(NM_000021.3):c.234C>T (p.G78=), PSEN1(NM_000021.4):c.234C>T (p.G78=)
-
PSEN1_000416
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_VUmc
+/+, +/.
10
4
c.236C>T
r.(?)
p.(Ala79Val)
-
pathogenic
g.73637653C>T
g.73170945C>T
PSEN1(NM_000021.3):c.236C>T (p.A79V), PSEN1(NM_000021.4):c.236C>T (p.A79V)
-
PSEN1_000215
Point mutation in coding region predicting an amino acid substitution,
1 more item
-
-
rs63749824
CLASSIFICATION record, Unknown
no, yes
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_VUmc
,
Marc Cruts
+/+
1
4
c.244G>C
r.(?)
p.(Val82Leu)
-
pathogenic
g.73637661G>C
g.73170953G>C
-
-
PSEN1_000216
Point mutation in coding region predicting an amino acid substitution
-
-
rs63749967
Unknown
no
-
-
-
-
Marc Cruts
+/+
1
4
c.247_252del
r.(?)
p.(Ile83_Met84del)
-
pathogenic
g.73637664_73637669del
g.73170956_73170961del
-
-
PSEN1_000217
Hexanucleotide deletion resulting in deletion of 2 amino acids
-
-
rs63750307
Unknown
no
-
-
-
-
Marc Cruts
+/+
1
4
c.(254T>C)
r.(?)
p.(Leu85Pro)
-
pathogenic
g.73637671T>C
g.73170963T>C
-
-
PSEN1_000218
Point mutation in coding region predicting an amino acid substitution
-
-
rs63750599
Unknown
no
-
-
-
-
Marc Cruts
+/+
1
4
c.(265G>T)
r.(?)
p.(Val89Leu)
-
pathogenic
g.73637682G>T
g.73170974G>T
-
-
PSEN1_000219
Point mutation in coding region predicting an amino acid substitution
-
-
rs63750815
Unknown
yes
-
-
-
-
Marc Cruts
+/+
2
4
c.(275G>C)
r.(?)
p.(Cys92Ser)
-
pathogenic
g.73637692G>C
g.73170984G>C
-
-
PSEN1_000220
Point mutation in coding region predicting an amino acid substitution
-
-
rs63751141
Unknown
no
-
-
-
-
Marc Cruts
+/+
1
4
c.(280G>A)
r.(?)
p.(Val94Met)
-
pathogenic
g.73637697G>A
g.73170989G>A
-
-
PSEN1_000221
Point mutation in coding region predicting an amino acid substitution
-
-
rs63750831
Unknown
no
-
-
-
-
Marc Cruts
+/+
1
4
c.286G>T
r.(?)
p.(Val96Phe)
-
pathogenic
g.73637703G>T
g.73170995G>T
-
-
PSEN1_000222
Point mutation in coding region predicting an amino acid substitution
-
-
rs63750601
Unknown
yes
-
-
-
-
Marc Cruts
+/+
1
4
c.(289G>T)
r.(?)
p.(Val97Leu)
-
pathogenic
g.73637706G>T
g.73170998G>T
-
-
PSEN1_000223
Point mutation in coding region predicting an amino acid substitution
-
-
rs63750852
Unknown
yes
-
-
-
-
Marc Cruts
+/+
1
4
c.(313T>A)
r.(?)
p.(Phe105Ile)
-
pathogenic
g.73637730T>A
g.73171022T>A
-
-
PSEN1_000224
Point mutation in coding region predicting an amino acid substitution
-
-
rs63750325
Unknown
no
-
-
-
-
Marc Cruts
+/+
1
4
c.(313T>G)
r.(?)
p.(Phe105Val)
-
pathogenic
g.73637730T>G
g.73171022T>G
-
-
PSEN1_000225
Point mutation in coding region predicting an amino acid substitution
-
-
-
Unknown
no
-
-
-
-
Marc Cruts
+/+
1
4
c.(315T>G)
r.(?)
p.(Phe105Leu)
-
pathogenic
g.73637732T>G
g.73171024T>G
-
-
PSEN1_000226
Point mutation in coding region predicting an amino acid substitution
-
-
rs63750321
Unknown
no
-
-
-
-
Marc Cruts
+?/.
1
-
c.337C>T
r.(?)
p.(Leu113=)
-
likely pathogenic
g.73637754C>T
g.73171046C>T
PSEN1 L109L
-
PSEN1_000425
-
PubMed: Miszalski-Jamka 2017
-
-
Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
+/+
1
4
c.(338T>A)
r.(?)
p.(Leu113Gln)
-
pathogenic
g.73637755T>A
g.73171047T>A
-
-
PSEN1_000228
Point mutation in coding region predicting an amino acid substitution
-
-
rs63751399
Unknown
no
-
-
-
-
Marc Cruts
+/+
1
4
c.338T>C
r.(?)
p.(Leu113Pro)
-
pathogenic
g.73637755T>C
g.73171047T>C
-
-
PSEN1_000227
Point mutation in coding region predicting an amino acid substitution
-
-
rs63751399
Unknown
yes
-
-
-
-
Marc Cruts
+/+, +/.
10
4
c.338+1del
r.spl?, r.[338_339insTAC;170_338del;88_338del]
0, p.?
-
pathogenic
g.73637756del
g.73171048del
PSEN1(NM_000021.4):c.338+1delG
-
PSEN1_000229
VKGL data sharing initiative Nederland,
1 more item
-
-
rs63751475
CLASSIFICATION record, Unknown
no, yes
-
-
-
-
VKGL-NL_VUmc
,
Marc Cruts
+/+
4
5
c.343T>C
r.(?)
p.(Tyr115His)
-
pathogenic
g.73640278T>C
g.73173570T>C
-
-
PSEN1_000230
Point mutation in coding region predicting an amino acid substitution
-
-
rs63749962
Unknown
no
-
-
-
-
Marc Cruts
+/+, +/.
5
5
c.(344A>G), c.344A>G
r.(?)
p.(Tyr115Cys)
-
pathogenic
g.73640279A>G
g.73173571A>G
PSEN1(NM_000021.3):c.344A>G (p.Y115C)
-
PSEN1_000231
Point mutation in coding region predicting an amino acid substitution,
1 more item
-
-
rs63750450
CLASSIFICATION record, Unknown
no, yes
-
-
-
-
VKGL-NL_Rotterdam
,
Marc Cruts
+/+
4
5
c.347C>A
r.(?)
p.(Thr116Asn)
-
pathogenic
g.73640282C>A
g.73173574C>A
-
-
PSEN1_000232
Point mutation in coding region predicting an amino acid substitution
-
-
rs63750730
Unknown
no, yes
-
-
-
-
Marc Cruts
+/+
2
5
c.347C>T
r.(?)
p.(Thr116Ile)
-
pathogenic
g.73640282C>T
g.73173574C>T
-
-
PSEN1_000233
Point mutation in coding region predicting an amino acid substitution
-
-
rs63750730
Unknown
no
-
-
-
-
Marc Cruts
+/+
2
5
c.(349C>G)
r.(?)
p.(Pro117Ala)
-
pathogenic
g.73640284C>G
g.73173576C>G
-
-
PSEN1_000235
Point mutation in coding region predicting an amino acid substitution
-
-
-
Unknown
yes
-
-
-
-
Marc Cruts
+/+
1
5
c.(349C>T)
r.(?)
p.(Pro117Ser)
-
pathogenic
g.73640284C>T
g.73173576C>T
-
-
PSEN1_000234
Point mutation in coding region predicting an amino acid substitution
-
-
rs63750550
Unknown
yes
-
-
-
-
Marc Cruts
+/+
2
5
c.(350C>G)
r.(?)
p.(Pro117Arg)
-
pathogenic
g.73640285C>G
g.73173577C>G
-
-
PSEN1_000237
Point mutation in coding region predicting an amino acid substitution
-
-
rs63749805
Unknown
no
-
-
-
-
Marc Cruts
+/+
2
5
c.(350C>T)
r.(?)
p.(Pro117Leu)
-
pathogenic
g.73640285C>T
g.73173577C>T
-
-
PSEN1_000236
Point mutation in coding region predicting an amino acid substitution
-
-
rs63749805
Unknown
no, yes
-
-
-
-
Marc Cruts
+/+
2
5
c.(358G>A)
r.(?)
p.(Glu120Lys)
-
pathogenic
g.73640293G>A
g.73173585G>A
-
-
PSEN1_000238
Point mutation in coding region predicting an amino acid substitution
-
-
rs63750800
Unknown
no, yes
-
-
-
-
Marc Cruts
+/+
1
5
c.(359A>G)
r.(?)
p.(Glu120Gly)
-
pathogenic
g.73640294A>G
g.73173586A>G
-
-
PSEN1_000239
Point mutation in coding region predicting an amino acid substitution
-
-
-
Unknown
yes
-
-
-
-
Marc Cruts
+/+
3
5
c.(360A>C)
r.(?)
p.(Glu120Asp)
-
pathogenic
g.73640295A>C
g.73173587A>C
-
-
PSEN1_000241
Point mutation in coding region predicting an amino acid substitution
-
-
rs63751272
Unknown
no, yes
-
-
-
-
Marc Cruts
+/+
1
5
c.(360A>T)
r.(?)
p.(Glu120Asp)
-
pathogenic
g.73640295A>T
g.73173587A>T
-
-
PSEN1_000240
Point mutation in coding region predicting an amino acid substitution
-
-
rs63751272
Unknown
yes
-
-
-
-
Marc Cruts
+/+
1
5
c.(367G>A)
r.(?)
p.(Glu123Lys)
-
pathogenic
g.73640302G>A
g.73173594G>A
-
-
PSEN1_000242
Point mutation in coding region predicting an amino acid substitution
-
-
rs63750378
Unknown
yes
-
-
-
-
Marc Cruts
+/+
1
5
c.(403A>G)
r.(?)
p.(Asn135Asp)
-
pathogenic
g.73640338A>G
g.73173630A>G
-
-
PSEN1_000243
Point mutation in coding region predicting an amino acid substitution
-
-
rs63750353
Unknown
no
-
-
-
-
Marc Cruts
+/+, ?/.
3
5
c.(404A>G), c.404A>G
r.(?)
p.(Asn135Ser)
-
pathogenic, VUS
g.73640339A>G
g.73173631A>G
-
-
PSEN1_000244
Point mutation in coding region predicting an amino acid substitution
PubMed: Ganapathy 2019
ClinVar-RCV000084303.1
rs63751278
Germline, Unknown
yes
-
-
-
-
Johan den Dunnen
,
Marc Cruts
+/+
1
5
c.(407C>G)
r.(?)
p.(Ala136Gly)
-
pathogenic
g.73640342C>G
g.73173634C>G
-
-
PSEN1_000245
Point mutation in coding region predicting an amino acid substitution
-
-
rs41345849
Unknown
no
-
-
-
-
Marc Cruts
+/+
9
5
c.415A>G
r.(?)
p.(Met139Val)
-
pathogenic
g.73640350A>G
g.73173642A>G
-
-
PSEN1_000246
Point mutation in coding region predicting an amino acid substitution
-
-
rs63751037
Unknown
no, yes
-
-
-
-
Marc Cruts
+/+
1
5
c.(416T>A)
r.(?)
p.(Met139Lys)
-
pathogenic
g.73640351T>A
g.73173643T>A
-
-
PSEN1_000248
Point mutation in coding region predicting an amino acid substitution
-
-
rs63751106
Unknown
no
-
-
-
-
Marc Cruts
+/+
3
5
c.416T>C
r.(?)
p.(Met139Thr)
-
pathogenic
g.73640351T>C
g.73173643T>C
-
-
PSEN1_000247
Point mutation in coding region predicting an amino acid substitution
-
-
rs63751106
Unknown
no, yes
-
-
-
-
Marc Cruts
+/+
1
5
c.(417G>A)
r.(?)
p.(Met139Ile)
-
pathogenic
g.73640352G>A
g.73173644G>A
-
-
PSEN1_000249
Point mutation in coding region predicting an amino acid substitution
-
-
rs63750522
Unknown
no
-
-
-
-
Marc Cruts
+/+
1
5
c.(417G>C)
r.(?)
p.(Met139Ile)
-
pathogenic
g.73640352G>C
g.73173644G>C
-
-
PSEN1_000250
Point mutation in coding region predicting an amino acid substitution
-
-
-
Unknown
no
-
-
-
-
Marc Cruts
+?/.
1
5
c.418A>T
r.(?)
p.(Ile140Phe)
-
likely pathogenic
g.73640353A>T
g.73173645A>T
-
-
PSEN1_000208
-
-
-
-
Germline/De novo (untested)
-
-
-
-
-
Gemeinschaftspraxis für Humangenetik Dresden
+/+
1
5
c.(427A>G)
r.(?)
p.(Ile143Val)
-
pathogenic
g.73640362A>G
g.73173654A>G
-
-
PSEN1_000252
Point mutation in coding region predicting an amino acid substitution
-
-
-
Unknown
no
-
-
-
-
Marc Cruts
+/+
1
5
c.(427A>T)
r.(?)
p.(Ile143Phe)
-
pathogenic
g.73640362A>T
g.73173654A>T
-
-
PSEN1_000251
1 more item
-
-
rs63750322
Unknown
no
-
-
-
-
Marc Cruts
+/+
1
5
c.(428T>A)
r.(?)
p.(Ile143Asn)
-
pathogenic
g.73640363T>A
g.73173655T>A
-
-
PSEN1_000254
Point mutation in coding region predicting an amino acid substitution
-
-
rs63750004
Unknown
no
-
-
-
-
Marc Cruts
+/+
8
5
c.428T>C
r.(?)
p.(Ile143Thr)
-
pathogenic
g.73640363T>C
g.73173655T>C
-
-
PSEN1_000253
Point mutation in coding region predicting an amino acid substitution
-
-
rs63750004
Unknown
no, yes
-
-
-
-
Marc Cruts
+/+
1
5
c.(429T>G)
r.(?)
p.(Ile143Met)
-
pathogenic
g.73640364T>G
g.73173656T>G
-
-
PSEN1_000255
Point mutation in coding region predicting an amino acid substitution
-
-
rs63751071
Unknown
yes
-
-
-
-
Marc Cruts
+/+
14
5
c.436A>C
r.(?)
p.(Met146Leu)
-
pathogenic
g.73640371A>C
g.73173663A>C
-
-
PSEN1_000258
Point mutation in coding region predicting an amino acid substitution
-
-
rs63750306
Unknown
no, yes
-
-
-
-
Marc Cruts
+/+
4
5
c.436A>G
r.(?)
p.(Met146Val)
-
pathogenic
g.73640371A>G
g.73173663A>G
-
-
PSEN1_000257
Point mutation in coding region predicting an amino acid substitution
-
-
rs63750306
Unknown
no
-
-
-
-
Marc Cruts
+/+
2
5
c.436A>T
r.(?)
p.(Met146Leu)
-
pathogenic
g.73640371A>T
g.73173663A>T
-
-
PSEN1_000256
Point mutation in coding region predicting an amino acid substitution
-
-
rs63750306
Unknown
yes
-
-
-
-
Marc Cruts
+/+
2
5
c.(438G>A)
r.(?)
p.(Met146Ile)
-
pathogenic
g.73640373G>A
g.73173665G>A
-
-
PSEN1_000260
Point mutation in coding region predicting an amino acid substitution
-
-
rs63750391
Unknown
no, yes
-
-
-
-
Marc Cruts
+/+
1
5
c.(438G>C)
r.(?)
p.(Met146Ile)
-
pathogenic
g.73640373G>C
g.73173665G>C
-
-
PSEN1_000259
Point mutation in coding region predicting an amino acid substitution
-
-
rs63750391
Unknown
yes
-
-
-
-
Marc Cruts
+/+
1
5
c.438G>T
r.(?)
p.(Met146Ile)
-
pathogenic
g.73640373G>T
g.73173665G>T
-
-
PSEN1_000261
Point mutation in coding region predicting an amino acid substitution
-
-
rs63750391
Unknown
no
-
-
-
-
Marc Cruts
+/+
1
5
c.440C>T
r.(?)
p.(Thr147Ile)
-
pathogenic
g.73640375C>T
g.73173667C>T
-
-
PSEN1_000262
Point mutation in coding region predicting an amino acid substitution
-
-
rs63750907
Unknown
yes
-
-
-
-
Marc Cruts
?/.
1
-
c.446T>C
r.(?)
p.(Leu149Pro)
-
VUS
g.73640381T>C
g.73173673T>C
PSEN1(NM_000021.4):c.446T>C (p.L149P)
-
PSEN1_000417
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
?/.
1
-
c.451G>A
r.(?)
p.(Val151Met)
-
VUS
g.73640386G>A
g.73173678G>A
PSEN1(NM_000021.3):c.451G>A (p.V151M)
-
PSEN1_000403
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/+, +/.
5
5
c.(457C>G), c.457C>G
r.(?)
p.(Leu153Val)
-
pathogenic
g.73640392C>G
g.73173684C>G
-
-
PSEN1_000263
Point mutation in coding region predicting an amino acid substitution
-
-
rs63751441
CLASSIFICATION record, Unknown
no, yes
-
-
-
-
Marc Cruts
,
MobiDetails
+/+
1
5
c.(460T>A)
r.(?)
p.(Tyr154Asn)
-
pathogenic
g.73640395T>A
g.73173687T>A
-
-
PSEN1_000264
Point mutation in coding region predicting an amino acid substitution
-
-
rs63750588
Unknown
yes
-
-
-
-
Marc Cruts
+/+
1
5
c.(461A>G)
r.(?)
p.(Tyr154Cys)
-
pathogenic
g.73640396A>G
g.73173688A>G
-
-
PSEN1_000265
Point mutation in coding region predicting an amino acid substitution
-
-
rs63751292
Unknown
no
-
-
-
-
Marc Cruts
+/+
2
5
c.466_467insTTATAT
r.(?)
p.(Lys155_Tyr156insPheIle)
-
pathogenic
g.73640401_73640402insTTATAT
g.73173693_73173694insTTATAT
-
-
PSEN1_000266
1 more item
-
-
rs63750631
Unknown
no
-
-
-
-
Marc Cruts
+/+
1
6
c.487C>T
r.(?)
p.(His163Tyr)
-
pathogenic
g.73653567C>T
g.73186859C>T
-
-
PSEN1_000267
Point mutation in coding region predicting an amino acid substitution
-
-
rs63749885
Unknown
no
-
-
-
-
Marc Cruts
+/+
15
6
c.488A>G
r.(?)
p.(His163Arg)
-
pathogenic
g.73653568A>G
g.73186860A>G
-
-
PSEN1_000268
Point mutation in coding region predicting an amino acid substitution
-
-
rs63750590
Unknown
no, yes
-
-
-
-
Marc Cruts
+/+
1
6
c.(493T>G)
r.(?)
p.(Trp165Gly)
-
pathogenic
g.73653573T>G
g.73186865T>G
-
-
PSEN1_000269
Point mutation in coding region predicting an amino acid substitution
-
-
rs63751010
Unknown
no
-
-
-
-
Marc Cruts
+/+
1
6
c.495G>C
r.(?)
p.(Trp165Cys)
-
pathogenic
g.73653575G>C
g.73186867G>C
-
-
PSEN1_000270
Point mutation in coding region predicting an amino acid substitution
-
-
rs63751484
Unknown
no
-
-
-
-
Marc Cruts
+/+
1
6
c.(496_498del)
r.(?)
p.(Leu166del)
-
pathogenic
g.73653576_73653578del
g.73186868_73186870del
-
-
PSEN1_000271
Trinucleotide deletion predicted to result in deletion of 1 amino acid
-
-
rs63751458
Unknown
no
-
-
-
-
Marc Cruts
+/+
1
6
c.(497T>A)
r.(?)
p.(Leu166His)
-
pathogenic
g.73653577T>A
g.73186869T>A
-
-
PSEN1_000274
de novo or non-paternity?. /r/Point mutation in coding region predicting an amino acid substitution
-
-
rs63750265
Unknown
no
-
-
-
-
Marc Cruts
+/+
1
6
c.(497T>C)
r.(?)
p.(Leu166Pro)
-
pathogenic
g.73653577T>C
g.73186869T>C
-
-
PSEN1_000273
Point mutation in coding region predicting an amino acid substitution
-
-
rs63750265
Unknown
no
-
-
-
-
Marc Cruts
+/+
1
6
c.(497T>G)
r.(?)
p.(Leu166Arg)
-
pathogenic
g.73653577T>G
g.73186869T>G
-
-
PSEN1_000272
Point mutation in coding region predicting an amino acid substitution
-
-
rs63750265
Unknown
yes
-
-
-
-
Marc Cruts
+/+
1
6
c.(501_503del)
r.(?)
p.(Ile168del)
-
pathogenic
g.73653581_73653583del
g.73186873_73186875del
-
-
PSEN1_000275
Trinucleotide deletion resulting in in-frame deletion of 1 amino acid
-
-
rs63750879
Unknown
no
-
-
-
-
Marc Cruts
+/+
1
6
c.(505T>C)
r.(?)
p.(Ser169Pro)
-
pathogenic
g.73653585T>C
g.73186877T>C
-
-
PSEN1_000276
Point mutation in coding region predicting an amino acid substitution
-
-
rs63750418
Unknown
yes
-
-
-
-
Marc Cruts
+/+
2
6
c.(506C>T)
r.(?)
p.(Ser169Leu)
-
pathogenic
g.73653586C>T
g.73186878C>T
-
-
PSEN1_000277
Point mutation in coding region predicting an amino acid substitution
-
-
rs63751210
Unknown
no
-
-
-
-
Marc Cruts
+/+
1
6
c.(507_509del)
r.(?)
p.(Ser170del)
-
pathogenic
g.73653587_73653589del
g.73186879_73186881del
-
-
PSEN1_000278
Trinucleotide deletion in coding region predicting a deletion of one amino acid
-
-
-
Unknown
yes
-
-
-
-
Marc Cruts
+/+
3
6
c.(509C>T)
r.(?)
p.(Ser170Phe)
-
pathogenic
g.73653589C>T
g.73186881C>T
-
-
PSEN1_000279
Point mutation in coding region predicting an amino acid substitution
-
-
rs63750577
Unknown
no
-
-
-
-
Marc Cruts
+/+
2
6
c.(512T>C)
r.(?)
p.(Leu171Pro)
-
pathogenic
g.73653592T>C
g.73186884T>C
-
-
PSEN1_000280
Point mutation in coding region predicting an amino acid substitution
-
-
rs63750963
Unknown
no
-
-
-
-
Marc Cruts
+/+
1
6
c.518T>G
r.(?)
p.(Leu173Trp)
-
pathogenic
g.73653598T>G
g.73186890T>G
-
-
PSEN1_000281
Point mutation in coding region predicting an amino acid substitution
-
-
rs63750299
Unknown
no
-
-
-
-
Marc Cruts
+/+
1
6
c.(519G>C)
r.(?)
p.(Leu173Phe)
-
pathogenic
g.73653599G>C
g.73186891G>C
-
-
PSEN1_000282
Point mutation in coding region predicting an amino acid substitution
-
-
-
Unknown
yes
-
-
-
-
Marc Cruts
+/+
2
6
c.(520C>A)
r.(?)
p.(Leu174Met)
-
pathogenic
g.73653600C>A
g.73186892C>A
-
-
PSEN1_000283
Point mutation in coding region predicting an amino acid substitution
-
-
rs63751144
Unknown
no, yes
-
-
-
-
Marc Cruts
+/+
1
6
c.521T>G
r.(?)
p.(Leu174Arg)
-
pathogenic
g.73653601T>G
g.73186893T>G
-
-
PSEN1_000284
Point mutation in coding region predicting an amino acid substitution
-
-
rs63751025
Unknown
yes
-
-
-
-
Marc Cruts
-/?
1
6
c.(524T>C)
r.(?)
p.(Phe175Ser)
-
benign
g.72723357T>C
-
-
-
PSEN1_000210
1 more item
-
-
rs63750771
Unknown
-
-
-
-
-
Marc Cruts
+/+
2
6
c.529T>C
r.(?)
p.(Phe177Leu)
-
pathogenic
g.73653609T>C
g.73186901T>C
-
-
PSEN1_000285
Point mutation in coding region predicting an amino acid substitution
-
-
rs63749911
Unknown
no, yes
-
-
-
-
Marc Cruts
+/+, +?/.
2
6
c.530T>C
r.(?)
p.(Phe177Ser)
ACMG
likely pathogenic, pathogenic
g.73653610T>C
g.73186902T>C
-
-
PSEN1_000286
Point mutation in coding region predicting an amino acid substitution,
1 more item
-
-
rs63749806
Germline, Unknown
no
-
-
-
-
Andreas Laner
,
Marc Cruts
+/+
2
6
c.532T>C
r.(?)
p.(Ser178Pro)
-
pathogenic
g.73653612T>C
g.73186904T>C
-
-
PSEN1_000287
Point mutation in coding region predicting an amino acid substitution
-
-
rs63750155
Unknown
no
-
-
-
-
Marc Cruts
+/+
1
6
c.(548G>T)
r.(?)
p.(Gly183Val)
-
pathogenic
g.73653628G>T
g.73186920G>T
-
-
PSEN1_000288
Point mutation in coding region predicting an amino acid substitution
-
-
rs63751068
Unknown
yes
-
-
-
-
Marc Cruts
+/+
2
7
c.(552A>C)
r.(?)
p.(Glu184Asp)
-
pathogenic
g.73659355A>C
g.73192647A>C
-
-
PSEN1_000289
Point mutation in coding region predicting an amino acid substitution
-
-
rs63750311
Unknown
no
-
-
-
-
Marc Cruts
-/?
1
7
c.(572T>C)
r.(?)
p.(Val191Ala)
-
benign
g.72729128T>C
-
-
-
PSEN1_000211
1 more item
-
-
-
Unknown
-
-
-
-
-
Marc Cruts
+/+
3
7
c.616G>A
r.(?)
p.(Gly206Ser)
-
pathogenic
g.73659419G>A
g.73192711G>A
-
-
PSEN1_000290
Point mutation in coding region predicting an amino acid substitution
-
-
rs63750569
Unknown
no, yes
-
-
-
-
Marc Cruts
+/+, +/., +?/.
3
7
c.(617G>A), c.617G>A
r.(?)
p.(Gly206Asp)
ACMG
pathogenic, pathogenic (dominant)
g.73659420G>A
g.73192712G>A
PSEN1(NM_000021.3):c.617G>A (p.G206D)
-
PSEN1_000293
ACMG: PS3, PM5_STR, PS4_MOD, PM1, PM2_SUP, PP3, VKGL data sharing initiative Nederland,
1 more item
1 more item
-
rs63750082
CLASSIFICATION record, Germline, Unknown
?, yes
-
-
-
-
Andreas Laner
,
VKGL-NL_Rotterdam
,
Marc Cruts
+/+
18
7
c.617G>C
r.(?)
p.(Gly206Ala)
-
pathogenic
g.73659420G>C
g.73192712G>C
-
-
PSEN1_000291
Point mutation in coding region predicting an amino acid substitution
-
-
rs63750082
Unknown
no, yes
-
-
-
-
Marc Cruts
+/+
1
7
c.617G>T
r.(?)
p.(Gly206Val)
-
pathogenic
g.73659420G>T
g.73192712G>T
-
-
PSEN1_000292
Point mutation in coding region predicting an amino acid substitution
-
-
rs63750082
Unknown
no
-
-
-
-
Marc Cruts
+/+
1
7
c.(625G>A)
r.(?)
p.(Gly209Arg)
-
pathogenic
g.73659428G>A
g.73192720G>A
-
-
PSEN1_000294
Point mutation in coding region predicting an amino acid substitution
-
-
rs63749880
Unknown
yes
-
-
-
-
Marc Cruts
+/.
1
-
c.625G>C
r.(?)
p.(Gly209Arg)
-
pathogenic
g.73659428G>C
-
-
-
PSEN1_000422
-
-
-
-
Unknown
-
-
-
-
-
IMGAG
+/+
1
7
c.(626G>A)
r.(?)
p.(Gly209Glu)
-
pathogenic
g.73659429G>A
g.73192721G>A
-
-
PSEN1_000296
Point mutation in coding region predicting an amino acid substitution
-
-
rs63750053
Unknown
no
-
-
-
-
Marc Cruts
+/+
1
7
c.626G>T
r.(?)
p.(Gly209Val)
-
pathogenic
g.73659429G>T
g.73192721G>T
-
-
PSEN1_000295
Point mutation in coding region predicting an amino acid substitution
-
-
rs63750053
Unknown
yes
-
-
-
-
Marc Cruts
+/+
1
7
c.(635C>A)
r.(?)
p.(Ser212Tyr)
-
pathogenic
g.73659438C>A
g.73192730C>A
-
-
PSEN1_000297
Point mutation in coding region predicting an amino acid substitution
-
-
-
Unknown
yes
-
-
-
-
Marc Cruts
+/+
1
7
c.(637A>T)
r.(?)
p.(Ile213Phe)
-
pathogenic
g.73659440A>T
g.73192732A>T
-
-
PSEN1_000299
Point mutation in coding region predicting an amino acid substitution
-
-
rs63750861
Unknown
no
-
-
-
-
Marc Cruts
+/+
1
7
c.637A>C
r.(?)
p.(Ile213Leu)
-
pathogenic
g.73659440A>C
g.73192732A>C
-
-
PSEN1_000298
Point mutation in coding region predicting an amino acid substitution
-
-
rs63750861
Unknown
yes
-
-
-
-
Marc Cruts
+/+
1
7
c.638T>C
r.(?)
p.(Ile213Thr)
-
pathogenic
g.73659441T>C
g.73192733T>C
-
-
PSEN1_000300
Point mutation in coding region predicting an amino acid substitution
-
-
rs63751309
Unknown
yes
-
-
-
-
Marc Cruts
+/+
1
7
c.(640C>G)
r.(?)
p.(His214Asp)
-
pathogenic
g.73659443C>G
g.73192735C>G
-
-
PSEN1_000302
Point mutation in coding region predicting an amino acid substitution
-
-
-
Unknown
no
-
-
-
-
Marc Cruts
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