Global Variome shared LOVD
F12 (coagulation factor XII (Hageman factor))
LOVD v.3.0 Build 30b [
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Curator:
Christian Drouet
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All variants in the F12 gene
The variants shown are described using the NM_000505.3 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.
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%)
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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'
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150 entries on 2 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
-?/.
_1
c.-4811A>G
r.(=)
p.(=)
-
likely benign
g.176841339T>C
g.177414338T>C
46 C/C, -4811A>G
-
F12_000051
-
PubMed: Chou 2022
,
Journal: Chou 2022
-
rs2545801
Germline
-
-
-
-
-
Johan den Dunnen
?/.
-
c.-62C>T
r.(?)
p.(=)
-
VUS
g.176836590G>A
g.177409589G>A
F12(NM_000505.4):c.-62C>T
-
F12_000025
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/.
-
c.-62C>T
r.(?)
p.(=)
-
likely benign
g.176836590G>A
-
F12(NM_000505.4):c.-62C>T
-
F12_000025
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+/.
-
c.-62C>T
r.(=)
p.(=)
-
likely pathogenic (recessive)
g.176836590G>A
g.177409589G>A
c.[-62C>T];[-62C>T]
-
F12_000025
Homozygous carrier of c.-62C>T variant displays a very low FXII activity
Journal: Demidova 2023
ClinVar-VCV000369463.4
-
Germline
-
-
-
-
-
Christian Drouet
-?/.
-
c.-57G>C
r.(?)
p.(=)
-
likely benign
g.176836585C>G
g.177409584C>G
F12(NM_000505.4):c.-57G>C
-
F12_000024
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/.
-
c.-57G>C
r.(?)
p.(=)
-
likely benign
g.176836585C>G
g.177409584C>G
F12(NM_000505.4):c.-57G>C
-
F12_000024
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+/.
-
c.-57G>C
r.(=)
p.(=)
-
likely pathogenic (recessive)
g.176836585C>G
g.177409584C>G
c.[-57G>C];[-57G>C]
-
F12_000024
Homozygous proband plasma samples displays a very low FXII activity
Journal: Demidova 2023
ClinVar-VCV000369462.10
rs41309132
Germline
no
0.00220
-
-
-
Christian Drouet
-/.
-
c.-4T>C
r.(?)
p.(=)
-
benign
g.176836532A>G
g.177409531A>G
F12(NM_000505.4):c.-4T>C
-
F12_000010
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-?/.
-
c.-4T>C
r.(?)
p.(=)
-
likely benign
g.176836532A>G
g.177409531A>G
F12(NM_000505.4):c.-4T>C
-
F12_000010
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/-
1
c.-4T>C
r.(?)
p.(=)
ACMG
association
g.176836532A>G
g.177409531A>G
‐46C/T
-
F12_000010
c.-4T>C, also recognised as F12‐46C/T, creates a new up-stream translational start codon, thereby attenuating formation of the authentic FXII protein; the variant, alias Kozak polymorphism, has been shown to modify the course of HAE-C1-INH; variant is overrepresented in a Spanish HAE-FXII cohort and significantly influences the degree of kallikrein-kinin system activation and the clinical severity c.-4T>C also decreases FXII activity when associated with F12 variants c.-62C>T, c.-57G>C, and strongly with c.1532-1G>A
PubMed: Corvillo 2020
,
Journal: Corvillo 2020
Journal: Demidova 2023
ClinVar-VCV000001167.14
rs1801020
Germline
no
0.34803
-
-
-
Christian Drouet
+/+
1
c.1A>G
r.?
p.(Arg2Tyr)
ACMG
likely pathogenic
g.176836528T>C
g.177409536T>C
c.[1A>G];[1A>G]
-
F12_000058
Proband homozygous for the c.1A>G variant; his father, mother, sister and son are all heterozygous. Predicted harmful by SIFT online software.
Journal: Ji 2023
-
-
Germline
yes
-
-
-
-
Christian Drouet
-?/.
2i
c.115+6T>G
r.(=)
p.(=)
-
likely benign
g.176836041A>C
g.177409040A>C
F12(NM_000505.3):c.115+6T>G
-
F12_000028
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
2i
c.116-224T>C
r.(=)
p.(=)
-
likely benign
g.176833286A>G
-
-
-
F12_000071
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/+
3
c.181T>C
r.(?)
p.(Cys61Arg)
-
likely pathogenic
g.176832997A>G
g.177406042A>G
-
-
F12_000066
-
Journal: Han 2015
-
-
Germline
-
-
-
-
-
Christian Drouet
?/.
-
c.218G>C
r.(?)
p.(Cys73Ser)
-
VUS
g.176832804C>G
-
F12(NM_000505.3):c.218G>C (p.C73S)
-
F12_000034
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/.
4
c.218G>C
r.(?)
p.(Cys73Ser)
-
likely pathogenic
g.176832804C>G
-
F12(NM_000505.3):c.218G>C (p.C73S)
-
F12_000034
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
?/.
4
c.223A>G
r.(?)
p.(Thr75Ala)
-
VUS
g.176832799T>C
-
F12(NM_000505.4):c.223A>G (p.T75A)
-
F12_000072
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+?/+?
5
c.303_304del
r.(?)
p.(His101Glnfs*36)
ACMG
likely pathogenic
g.176832417_176832418del
g.177405416_177405417del
-
-
F12_000077
Attributed in ClinVar by Juno Genomics, Hangzhou China as a Factor XII deficiency disease variant. Symptoms of proband fully resolved following lanadelumab, consistent with bradykinin-pathway modulation. The father is also carrier but asymptomatic. Considered as likely pathogenic - PVS1 + PM2 -
PubMed: Gao 2025
,
Journal: Gao 2025
ClinVar-SCV005416608.2
-
Germline
-
-
-
-
-
Christian Drouet
+?/+?
5
c.346G>A
r.(?)
p.(Gly116Ser)
-
likely pathogenic (recessive)
g.176832375C>T
g.177405374C>T
c.[346G>A];[1583C>A]
-
F12_000063
Compound heterozygous variant with c.1583C>A
Journal: Cheng 2023
-
-
Germline
yes
-
-
-
-
Christian Drouet
-?/.
-
c.348C>A
r.(?)
p.(Gly116=)
-
likely benign
g.176832373G>T
g.177405372G>T
F12(NM_000505.3):c.348C>A (p.G116=)
-
F12_000022
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/.
-
c.398-12C>T
r.(=)
p.(=)
-
benign
g.176832198G>A
g.177405197G>A
-
-
F12_000021
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/+?
6
c.405C>A
r.(?)
p.(Cys135*)
-
likely pathogenic (recessive)
g.176832179G>T
g.177405178G>T
c.[405C>A];[405C>A]
-
F12_000061
-
Journal: Kwon 2010
-
-
Germline
-
-
-
-
-
Christian Drouet
-?/.
-
c.418C>G
r.(?)
p.(Leu140Val)
-
likely benign
g.176832166G>C
g.177405165G>C
F12(NM_000505.3):c.418C>G (p.L140V)
-
F12_000020
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-?/.
-
c.418C>G
r.(?)
p.(Leu140Val)
-
likely benign
g.176832166G>C
g.177405165G>C
F12(NM_000505.3):c.418C>G (p.L140V)
-
F12_000020
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/.
-
c.418C>G
r.(?)
p.(Leu140Val)
-
likely benign
g.176832166G>C
-
F12(NM_000505.3):c.418C>G (p.L140V)
-
F12_000020
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
6
c.418C>G
r.(?)
p.(Leu140Val)
-
likely benign
g.176832166G>C
g.177405165G>C
-
-
F12_000020
Conflicting interpretation of pathogenicity -1. VUS as introduced by GeneDx, Gaithersburg MA -2. Likely benign as introduced by CeGaT Center for Human Genetics Tübingen, Germany. Criteria applied: BP4, BS1 -3. Benign as introduced by CeMIA, Larissa Greece
-
ClinVar-SCV003842612.1
rs35515200
SUMMARY record
-
0.00247 (gnomAD)
-
-
-
Christian Drouet
+?/.
6i
c.529+4A>G
r.spl?
p.?
-
VUS
g.176832051T>C
-
-
-
F12_000076
-
Journal: Dias de Castro 2024
-
-
Germline
yes
-
-
-
-
Christian Drouet
-?/.
-
c.552C>T
r.(?)
p.(Leu184=)
-
likely benign
g.176831893G>A
-
F12(NM_000505.3):c.552C>T (p.L184=)
-
F12_000046
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/+
7
c.566G>C
r.(?)
p.(Cys189Ser)
-
likely pathogenic
g.176831879C>G
g.177404878C>G
-
-
F12_000068
-
Journal: Han 2015
-
-
Germline
-
-
-
-
-
Christian Drouet
+/.
7
c.615del
r.(?)
p.(Gly206Glufs*45)
-
likely pathogenic
g.176831831del
g.177404830del
-
-
F12_000055
-
Journal: Demidova 2023
-
-
Germline
-
-
-
-
-
Christian Drouet
-/.
7
c.619G>C
r.(?)
p.(Ala207Pro)
-
benign
g.176831826C>G
g.177404825C>G
F12(NM_000505.4):c.619G>C (p.A207P)
-
F12_000005
VKGL data sharing initiative Nederland
Journal: Pechnikova 2023
Journal: Shamanaev 2025
ClinVar-SCV001441476.1
rs17876030
CLASSIFICATION record
no
0.034661
-
-
-
VKGL-NL_Groningen
-/.
-
c.619G>C
r.(?)
p.(Ala207Pro)
-
benign
g.176831826C>G
g.177404825C>G
F12(NM_000505.4):c.619G>C (p.A207P)
-
F12_000005
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/-?
7
c.619G>C
r.(?)
p.(Ala207Pro)
-
benign
g.176831826C>G
g.177404825C>G
-
-
F12_000005
-
Journal: Kiss 2013
ClinVar-RCV000264675.9
rs17876030
Germline
-
-
-
-
-
Christian Drouet
-/.
-
c.711C>T
r.(?)
p.(Pro237=)
-
benign
g.176831589G>A
g.177404588G>A
-
-
F12_000009
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/.
-
c.711C>T
r.(=)
p.(=)
-
benign
g.176831589G>A
g.177404588G>A
-
-
F12_000009
15 heterozygous, no homozygous;
Clinindb (India)
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs17876047
Germline
-
15/2794 individuals
-
-
-
Mohammed Faruq
?/.
8
c.721T>G
r.(?)
p.(Trp241Gly)
-
VUS
g.176831579A>C
-
-
-
F12_000043
-
Journal: Matsukuma 2011
-
-
Germline
-
-
-
-
-
Christian Drouet
-/.
-
c.756C>T
r.(?)
p.(Ala252=)
-
benign
g.176831544G>A
g.177404543G>A
-
-
F12_000019
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
9
c.827G>A
r.(?)
p.(Trp276*)
-
VUS
g.176831388C>T
g.177404387C>T
-
-
F12_000001
-
PubMed: Bosch 2015
,
Journal: Bosch 2015
-
-
Germline
-
-
-
-
-
Danielle Bosch
+/.
-
c.827G>A
r.(?)
p.(Trp276*)
-
pathogenic
g.176831388C>T
-
F12(NM_000505.4):c.827G>A (p.W276*)
-
F12_000001
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-?/.
9
c.849C>T
r.(?)
p.(Asp283=)
-
likely benign
g.176831366G>A
g.177404365G>A
F12(NM_000505.3):c.849C>T (p.D283=)
-
F12_000027
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/+
9
c.859T>A
r.(?)
p.(Trp287Arg)
-
pathogenic
g.176831356A>T
g.177404359A>T
-
-
F12_000040
p.(Trp287Arg) variant affects the kringle domain of factor XII. Spontaneously active p.(Trp287Arg) variant product in the recombinantly production medium; prone to intracellular autoactivation and accelerating FXII activation and overriding C1-INH control function.
Journal: Hofman 2020
Journal: Scheffel 2020
-
-
Germline
yes
-
-
-
-
Christian Drouet
+?/+?
9
c.894_911dup
r.(?)
p.(Gln300_Thr305dup)
ACMG
pathogenic
g.176831306_176831323dup
g.177404305_177404322dup
c.892_909dup
-
F12_000036
No functional evidence in the report for this variation. Incomplete penetrance: 3 symptomatic individuals within 6 carriers of c.894_911dup variant. In-frame duplication of 6 residues (Gln300_Thr305dup) within the Pro-rich region of the Kringle domain of factor XII. The c.894_911dup variant has been introduced in ClinVar as pathogenic by the lab of MM Nöthen, Institute of Human Genetics, University Hospital Bonn Germany.
Journal: Kiss 2013
PubMed: Kiss 2013
ClinVar-SCV000502993.2
rs774034606
Germline
yes
1/149302
-
-
-
Christian Drouet
-?/.
9
c.914C>T
r.(?)
p.(Thr305Ile)
-
likely benign
g.176831301G>A
-
F12(NM_000505.3):c.914C>T (p.T305I)
-
F12_000047
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
?/.
-
c.922T>A
r.(?)
p.(Ser308Thr)
-
VUS
g.176831293A>T
-
F12(NM_000505.3):c.922T>A (p.S308T)
-
F12_000035
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/+
9_9i
c.971_1018+24del
r.?
p.(Lys324_Ala340delinsThr)
ACMG
pathogenic
g.176831175_176831246del
g.177404174_177404245del
-
-
F12_000037
A Brazilian male carrier is presenting with a HAE phenotype triggered by ACEi intake (Veronez 2018). The F12 c.971_1018+24del72 variant is predicted to result in an in-frame deletion p.(Lys324_Ala340delinsThr).
Journal: Bork 2011
Journal: Bork 2014
Journal: Veronez 2017
Journal: Veronez 2018
Journal: Veronez 2021
ClinVar-VCV000441533.1
rs1554097246
Germline
yes
0.0001 (ALFA project)
-
-
-
Christian Drouet
+/+
9_9i
c.971_1018+24del
r.?
p.(Lys324_Ala340delinsThr)
ACMG
pathogenic
g.176831175_176831246del
g.177404174_177404245del
-
-
F12_000037
The F12 c.971_1018+24del72 variant is predicted to result in an in-frame deletion p.(Lys324_Ala340delinsThr). No analysis of transcripts available.
Journal: Bork 2011
ClinVar-VCV000441533.1
rs1554097246
Germline
yes
-
-
-
-
Christian Drouet
+/+
9_9i
c.971_1018+24del
r.?
p.(Lys324_Ala340delinsThr)
ACMG
pathogenic
g.176831173_176831244del
g.177404174_177404245del
-
-
F12_000037
Two independent Brazilian families have been shown as carrying a g.177404174_177404245del variant. The F12 c.971_1018+24del variant is predicted to result in an in-frame deletion p.(Lys324_Ala340delinsThr). No analysis of transcripts available.
Journal: Moreno 2015
Journal: Veronez 2018
Journal: Gabriel 2025
ClinVar-SCV005350560.1
rs1554097246
Germline
yes
-
-
-
-
Christian Drouet
+/+
9
c.983C>A
r.(?)
p.(Thr328Lys)
-
pathogenic
g.176831232G>T
g.177404231G>T
F12(NM_000505.3):c.983C>A (p.T328K), F12(NM_000505.4):c.983C>A (p.T328K)
-
F12_000008
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/+
9
c.983C>A
r.(?)
p.(Thr328Lys)
ACMG
pathogenic
g.176831232G>T
g.177404231G>T
-
-
F12_000008
The variant is considered pathogenic according to ACMG Guidelines with following criteria: PS3, PS4, PM1, PM5, PP1.
Journal: Charignon 2018
ClinVar-000001169
rs118204456
Germline
yes
0.000013 (gnomAD)
-
-
-
Christian Drouet
+/.
9
c.983C>A
r.(?)
p.(Thr328Lys)
-
pathogenic
g.176831232G>T
g.177404231G>T
F12(NM_000505.3):c.983C>A (p.T328K), F12(NM_000505.4):c.983C>A (p.T328K)
-
F12_000008
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+/+
9
c.983C>A
r.(?)
p.(Thr328Lys)
ACMG
pathogenic
g.176831232G>T
g.177404231G>T
-
-
F12_000008
Numerous Brazilian pedigrees have been recorded as carrying a c.983C>A variant, with 94 families and 176 heterozygous patients. Incomplete prenetrance: 79 asymptomatic relatives within 180 carriers of a c.983C>A variant. The c.983C>A variant meets the ACMG criteria to be characterized pathogenic PP1, PM1, PM5, PS3, PS4_Mod
Journal: Veronez 2018
Journal: Veronez 2021
Journal: Gabriel 2025
ClinVar-VCV000001169.8
rs118204456
Germline
yes
0.000004 (GnomAD_exome)
-
-
-
Christian Drouet
+/+
9
c.983C>A
r.(?)
p.(Thr328Lys)
ACMG
pathogenic
g.176831232G>T
g.177404231G>T
c.[983C>A];[983C>A]
-
F12_000008
Two Brazilian pedigrees have been recorded as segregating a c.983 C>A;(p.Thr328Lys) variant, with 1 homozygous carrier, one male and one female, respectively in each family. Fourteen affected individuals. Homozygosity for c.983C>A variant lead to develop disease symptoms in males - heterozygous male carriers normally do not develop disease symptoms - and a more severe disease phenotype in females compared to patients heterozygous for the F12 c.983 C>A variant. According to ACMG Guidelines - Criteria PS3, PS4, PM1, PM5, PP1 - the variant is considered pathogenic.
Journal: Grumach 2016
Journal: Stieber 2014
ClinVar-VCV000001169.8
rs118204456
Germline
yes
0.000013 (gnomAD)
-
-
-
Christian Drouet
+/+
9
c.983C>A
r.(?)
p.(Thr328Lys)
ACMG
pathogenic
g.176831232G>T
g.177404231G>T
-
-
F12_000008
Nine families and 23 affected individuals Important Italian cohort from that has been assessed a prevalence of 1:1.4 × E6 for HAE-FXII. The c.983C>A variant meets the ACMG criteria to be characterized pathogenic PP1, PM1, PM5, PS3, PS4_Mod
Journal: Bova 2020
ClinVar-VCV000001169.8
rs118204456
Germline
yes
0.000013 (gnomAD)
-
-
-
Christian Drouet
+/.
9
c.983C>A
r.(?)
p.(Thr328Lys)
ACMG
pathogenic
g.176831232G>T
g.177404231G>T
c.1032C>A
-
F12_000008
Four independent German kindreds have been found as carrying a c.983C>A variant. According to ACMG Guidelines - Criteria PS3, PS4, PM1, PM5, PP1 - the variant is considered pathogenic.
Journal: Dewald 2006
ClinVar-VCV000001169.8
rs118204456
Germline
yes
0.000013 (gnomAD)
-
-
-
Christian Drouet
+/+
9
c.983C>A
r.(?)
p.(Thr328Lys)
ACMG
pathogenic
g.176831232G>T
g.177404231G>T
-
-
F12_000008
First Australian case with HAE-FXII. The c.983C>A variant meets the ACMG criteria to be characterized pathogenic PP1, PM1, PM5, PS3, PS4_Mod
Journal: Bell 2008
ClinVar-SCV001441472.2
rs118204456
Germline
yes
0.000013 (gnomAD)
-
-
-
Christian Drouet
+/+
9
c.983C>A
r.(?)
p.(Thr328Lys)
ACMG
pathogenic
g.176831232G>T
g.177404231G>T
c.1032C>A
-
F12_000008
The first French pedigree affected by HAE-F12. Incomplete penetrance: 8/13 affected individuals. Haplotype analyses with use of SNPs at the F12 locus provided evidence that the French family and 3 of the German families reported by Dewald and Bork (2006) shared a common founder. Plasma displays a gain-of-function of kallikrein-kinin system; p.(Thr328Lys) exhibits a lower glycosylation, with subsequent increased autoactivation of zymogen F12. Thr to Lys transition has consequence on the protein’s folding and conformation, with an open or relaxed conformation facilitating the access of kallikrein and plasmin and exposing cryptic proteolytic targets for thrombin that are normally concealed and not accessible in the compact conformation of FXII. The c.983C>A variant meets the ACMG criteria to be characterized pathogenic PP1, PM1, PM5, PS3, PS4_Mod.
Journal: Martin 2001
Journal: Cichon 2006
Journal: Björkvist 2015
Journal: de Maat 2016
ClinVar-VCV000001169.8
rs118204456
Germline
yes
0.000013 (gnomAD)
-
-
-
Christian Drouet
+/+
9
c.983C>A
r.(?)
p.(Thr328Lys)
ACMG
pathogenic
g.176831232G>T
g.177404231G>T
-
-
F12_000008
3 female individuals have been shown as affected. A single male carrier of c.983C>A variant is shown asymptomatic. The c.983C>A variant meets the ACMG criteria to be characterized pathogenic PP1, PM1, PM5, PS3, PS4_Mod
Journal: Prieto 2009
ClinVar-VCV000001169.8
rs118204456
Germline
yes
0.000013 (gnomAD)
-
-
-
Christian Drouet
+/+
9
c.983C>A
r.(?)
p.(Thr328Lys)
ACMG
pathogenic
g.176831232G>T
g.177404231G>T
-
-
F12_000008
A single Canadian pedigree with 6 affected female individuals. Affected females have polymorphisms associated with lower levels of both APP and ACE, the major enzymes responsible for bradykinin catabolism. Three patients also carry the A allele of SNP rs3788853 in the XPNPEP2 gene, which may have contributed to the phenotype, making this observation the first one with multiple genes that might contribute to estrogen-dependent or estrogen associated HAE-F12. But this allele is also found in 10 unaffected family members. The c.983C>A variant meets the ACMG criteria to be characterized pathogenic PP1, PM1, PM5, PS3, PS4_Mod
Journal: Duan 2009
ClinVar-SCV001441472.2
rs118204456
Germline
yes
0.000013 (gnomAD)
-
-
-
Christian Drouet
+/+
9
c.983C>A
r.(?)
p.(Thr328Lys)
ACMG
pathogenic
g.176831232G>T
g.177404231G>T
-
-
F12_000008
The c.983C>A variant meets the ACMG criteria to be characterized pathogenic PP1, PM1, PM5, PS3, PS4_Mod
Journal: Bork 2009
Journal: Bork 2023
-
-
Germline
yes
-
-
-
-
Christian Drouet
+/.
9
c.983C>A
r.(?)
p.(Thr328Lys)
ACMG
pathogenic
g.176831232G>T
g.177404231G>T
-
-
F12_000008
A single French pedigree with women and man. C1Inh function drops of 10% to 40% compared to normal in female individuals, in line with the cleavage of the serpin from 105 to 95 kd without development of serpin-protease association, suggesting p.(Met328Lys) beyond the control of C1-INH. According to ACMG Guidelines - Criteria PS3, PS4, PM1, PM5, PP1 - the variant is considered pathogenic.
Journal: Martin 2007
ClinVar-VCV000001169
rs118204456
Germline
yes
0.000013 (gnomAD)
-
-
-
Christian Drouet
+/+
9
c.983C>A
r.(?)
p.(Thr328Lys)
ACMG
pathogenic
g.176831232G>T
g.177404231G>T
-
-
F12_000008
Nine families with 29 affected individuals. Symptomatic individuals have been found as carriers of a c.-2399C variant of XPNPEP2 gene, and a DD or ID polymporphism of ACE gene.
Journal: Piñero-Saavedra 2016
ClinVar-VCV000001169.8
rs118204456
Germline/De novo (untested)
yes
0.000004 (GnomAD_exome)
-
-
-
Christian Drouet
+/+
9
c.983C>A
r.(?)
p.(Thr328Lys)
ACMG
pathogenic
g.176831232G>T
g.177404231G>T
-
-
F12_000008
The c.983C>A variant meets the ACMG criteria to be characterized pathogenic PP1, PM1, PM5, PS3, PS4_Mod
Journal: Moreno 2015
ClinVar-VCV000001169.8
rs118204456
Germline
yes
0.000013 (gnomAD)
-
-
-
Christian Drouet
+/+
9
c.983C>A
r.(?)
p.(Thr328Lys)
ACMG
pathogenic
g.176831232G>T
g.177404231G>T
-
-
F12_000008
Four independent kindreds have been found as carrying a c.983C>A variant. Family 1, n=5 (female) Family 2, n=7 (female) Family 3, n=1 (female) Family 4, n=2 (female) All carriers also carry the same combined SNPs already described by Cichon 2006, demonstrating a common ancestor. The c.983C>A variant meets the ACMG criteria to be characterized pathogenic PP1, PM1, PM5, PS3, PS4_Mod
Journal: Firinu 2015
ClinVar-VCV000001170.4
rs118204456
Germline
yes
-
-
-
-
Christian Drouet
+/.
9
c.983C>A
r.(?)
p.(Thr328Lys)
ACMG
pathogenic
g.176831232G>T
g.177404231G>T
-
-
F12_000008
Thirteen kindreds from North-Western Spain have been found as carrying a c.983C>A variant, with 29 affected individuals. According to ACMG Guidelines - Criteria PS3, PS4, PM1, PM5, PP1 - the variant is considered pathogenic.
Journal: Marcos 2012
ClinVar-SCV001441472.2
rs118204456
Germline
yes
0.000013 (gnomAD)
-
-
-
Christian Drouet
+/+
9
c.983C>A
r.(?)
p.(Thr328Lys)
ACMG
pathogenic
g.176831232G>T
g.177404231G>T
-
-
F12_000008
The c.983C>A variant meets the ACMG criteria to be considered pathogenic PP1, PM1, PM5, PS3, PS4_Mod
Journal: Baeza 2011
ClinVar-SCV001441472.2
rs118204456
Germline
yes
0.000013 (gnomAD)
-
-
-
Christian Drouet
+/+
9
c.983C>A
r.(?)
p.(Thr328Lys)
ACMG
pathogenic
g.176831232G>T
g.177404231G>T
-
-
F12_000008
Two families with 5 affected female individuals; 3 are presenting with severe obstetrical complications. According to ACMG Guidelines - Criteria PS3, PS4, PM1, PM5, PP1 - the variant is considered pathogenic.
Journal: Picone 2010
ClinVar-SCV001441472.2
rs118204456
Germline
yes
0.000013 (gnomAD)
-
-
-
Christian Drouet
+/+
9
c.983C>A
r.(?)
p.(Thr328Lys)
ACMG
pathogenic
g.176831232G>T
g.177404231G>T
-
-
F12_000008
A single Italian pedigree with 3 affected female individuals. The patients also carry the same combination of SNPs already observed in the first description by Cichon et al 2006, demonstrating c983C>A as a common ancestral variant among affected individuals in western Europe. The c.983C>A variant meets the ACMG criteria to be considered pathogenic PP1, PM1, PM5, PS3, PS4_Mod.
Journal: Nagy 2009
ClinVar-SCV001441472.2
rs118204456
Germline
yes
0.000013 (gnomAD)
-
-
-
Christian Drouet
+/+
9
c.983C>A
r.(?)
p.(Thr328Lys)
ACMG
pathogenic
g.176831232G>T
g.177404231G>T
-
-
F12_000008
A Spanish kindred has been shown as carrying a c.983C>A variant, with 2 affected individuals. According to ACMG Guidelines - Criteria PS3, PS4, PM1, PM5, PP1 - the variant is considered pathogenic.
Journal: Veronez 2019
ClinVar-SCV001441472.2
rs118204456
Germline
yes
0.000013 (gnomAD)
-
-
-
Christian Drouet
+/+
9
c.983C>A
r.(?)
p.(Thr328Lys)
ACMG
pathogenic
g.176831232G>T
g.177404231G>T
-
-
F12_000008
24 French pedigrees have been shown as carrying a c.983C>A variant, with 37/57 affected individuals. Family origin: 12/24 from Northern Africa. The c.983C>A variant meets the ACMG criteria to be characterized pathogenic PP1, PM1, PM5, PS3, PS4_Mod.
Journal: Deroux 2016
ClinVar-VCV000001169
rs118204456
Germline
yes
0.000013 (gnomAD)
-
-
-
Christian Drouet
+/.
9
c.983C>A
r.(?)
p.(Thr328Lys)
ACMG
pathogenic
g.176831232G>T
g.177404231G>T
-
-
F12_000008
According to ACMG Guidelines - Criteria PS3, PS4, PM1, PM5, PP1 -the variant is considered pathogenic.
Journal: Mendoza-Alvarez 2022
ClinVar-SCV001441472.2
rs118204456
Germline
yes
0.000013 (gnomAD)
-
-
-
Christian Drouet
+/+
9
c.983C>A
r.(?)
p.(Thr328Lys)
-
pathogenic
g.176831232G>T
g.177404231G>T
F12(NM_000505.3):c.983C>A (p.T328K), F12(NM_000505.4):c.983C>A (p.T328K)
-
F12_000008
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/+
9
c.983C>A
r.(?)
p.(Thr328Lys)
ACMG
pathogenic
g.176831232G>T
g.177404231G>T
-
-
F12_000008
Isolated HAE-nC1-INH individuals carrying a c.983C>A variant (10 families): Occurrence of only one symptomatic patient per family, who had no family history of angioedema but who had symptom-free relatives carrying the same HAE-linked c.983C>A variant. The c.983C>A variant meets the ACMG criteria to be characterized pathogenic PP1, PM1, PM5, PS3, PS4_Mod
Journal: Bork 2023
ClinVar-000001169
rs118204456
Germline
yes
0.00001
-
-
-
Christian Drouet
+/+
9
c.983C>A
r.(?)
p.(Thr328Lys)
ACMG
pathogenic
g.176831232G>T
g.177404231G>T
-
-
F12_000008
The c.983C>A variant meets the ACMG criteria to be characterized pathogenic PP1, PM1, PM5, PS3, PS4_Mod
Journal: Hentges 2009
ClinVar-SCV001441472.2
rs118204456
Germline
yes
0.000013 (gnomAD)
-
-
-
Christian Drouet
+/+
9
c.983C>A
r.(?)
p.(Thr328Lys)
ACMG
pathogenic
g.176831232G>T
g.177404231G>T
-
-
F12_000008
The c.983C>A variant meets the ACMG criteria to be characterized as pathogenic PP1, PM1, PM5, PS3, PS4_Mod
Journal: Gomez-Traseira 2010
ClinVar-SCV001441472.2
rs118204456
Germline
yes
0.000013 (gnomAD)
-
-
-
Christian Drouet
+/+
9
c.983C>A
r.(?)
p.(Thr328Lys)
ACMG
pathogenic
g.176831232G>T
g.177404231G>T
-
-
F12_000008
The c.983C>A variant meets the ACMG criteria to be characterized pathogenic PP1, PM1, PM5, PS3, PS4_Mod
Journal: Mansi 2015
ClinVar-SCV002240385.3
rs118204456
Germline
yes
-
-
-
-
Christian Drouet
+/+
9
c.983C>A
r.(?)
p.(Thr328Lys)
ACMG
pathogenic
g.176831232G>T
g.177404231G>T
-
-
F12_000008
-
Journal: Stieber 2015
ClinVar-SCV001441472.1
rs118204456
Germline
yes
0.000004271
-
-
-
Christian Drouet
+/+
9
c.983C>A
r.(?)
p.(Thr328Lys)
ACMG
pathogenic
g.176831232G>T
g.177404231G>T
-
-
F12_000008
The c.983C>A variant meets the ACMG criteria to be characterized pathogenic PP1, PM1, PM5, PS3, PS4_Mod
Journal: Bork 2017
ClinVar-SCV001441472.2
rs118204456
Germline
yes
0.000013 (gnomAD)
-
-
-
Christian Drouet
+/+
9
c.983C>A
r.(?)
p.(Thr328Lys)
ACMG
pathogenic
g.176831232G>T
g.177404231G>T
-
-
F12_000008
According to ACMG Guidelines - Criteria PS3, PS4, PM1, PM5, PP1 - the variant is considered pathogenic.
Journal: Lochbaum 2023
ClinVar-SCV001441472.2
rs118204456
Germline
yes
0.000013 (gnomAD)
-
-
-
Christian Drouet
+/+
9
c.983C>A
r.(?)
p.(Thr328Lys)
ACMG
pathogenic
g.176831232G>T
g.177404231G>T
-
-
F12_000008
Clinical presentation: Recurrent edema of the limbs, lips, face, abdomen, lasting approx. five days. The c.983C>A variant meets the ACMG criteria to be characterized pathogenic PP1, PM1, PM5, PS3, PS4_Mod
Journal: Lochbaum 2023
-
rs118204456
Germline
yes
0.000004
-
-
-
Christian Drouet
+/+
9
c.983C>A
r.(?)
p.(Thr328Lys)
ACMG
pathogenic
g.176831232G>T
g.177404231G>T
-
-
F12_000008
A Portugese population with 38 carriers of a c.983C>A variant; 11 adult carriers have been shown still asymptomatic. The c.983C>A variant meets the ACMG criteria to be characterized pathogenic PP1, PM1, PM5, PS3, PS4_Mod
Journal: Dias de Castro 2024
ClinVar-SCV002240385.3
rs118204456
Germline
yes
0.000004
-
-
-
Christian Drouet
+/+
9
c.983C>A
r.(?)
p.(Thr328Lys)
ACMG
pathogenic
g.176831232G>T
g.177404231G>T
-
-
F12_000008
The c.983C>A variant meets the ACMG criteria to be characterized pathogenic PP1, PM1, PM5, PS3, PS4_Mod
Journal: Saddouk 2024
ClinVar-SCV001441472.2
rs11820445
Germline
yes
0.000013 (gnomAD)
-
-
-
Christian Drouet
+/+
9
c.983C>A
r.(?)
p.(Thr328Lys)
ACMG
pathogenic
g.176831232G>T
g.177404231G>T
-
-
F12_000008
According to ACMG Guidelines - Criteria PS3, PS4, PM1, PM5, PP1 - the variant is considered pathogenic.
Journal: Lekhlit 2025
ClinVar-SCV001441472.2
rs118204456
Germline
yes
0.000013 (gnomAD)
-
-
-
Christian Drouet
+/+
9
c.983C>A
r.(?)
p.(Thr328Lys)
ACMG
pathogenic
g.176831232G>T
g.177404231G>T
p.Thr328Lys
-
F12_000008
According to ACMG Guidelines - Criteria PS3, PS4, PM1, PM5, PP1 - the variant is considered pathogenic.
Journal: Veronez 2019
ClinVar-SCV001441472.2
rs118204456
Germline
yes
0.000013 (gnomAD)
-
-
-
Christian Drouet
+/+
9
c.983C>A
r.(?)
p.(Thr328Lys)
ACMG
pathogenic
g.176831232G>T
g.177404231G>T
-
-
F12_000008
First report of a Mexican family with 12 carriers - 8 women and 4 men, including an asymptomatic male carrier. According to ACMG Guidelines - Criteria PS3, PS4, PM1, PM5, PP1 - the variant is considered pathogenic.
Journal: Contreras-Verduzco 2023
ClinVar-SCV001441472.2
rs118204456
Germline
yes
0.000013 (gnomAD)
-
-
-
Christian Drouet
+/+
9
c.983C>A
r.(?)
p.(Thr328Lys)
ACMG
pathogenic
g.176831232G>T
g.177404231G>T
-
-
F12_000008
According to ACMG Guidelines - Criteria PS3, PS4, PM1, PM5, PP1 -the variant is considered pathogenic.
Journal: García Robledo 2025
ClinVar-SCV001441472.2
rs118204456
Germline
yes
0.000013 (gnomAD)
-
-
-
Christian Drouet
+/+
9
c.983C>A
r.(?)
p.(Thr328Lys)
ACMG
pathogenic
g.176831232G>T
g.177404231G>T
-
-
F12_000008
First identification of a compound heterozygous HAEnC1INH proband with F12 and MEFV genes: c.[NM_000505.3:c.983C>A];[[NM_000243.3:c.2080A>G];[NM_000243.3:c.2080A>G]]. According to ACMG Guidelines - Criteria PS3, PS4, PM1, PM5, PP1 - the variant c.983C>A is considered pathogenic
Journal: Durut 2025
ClinVar-SCV001441472
rs118204456
Germline
yes
0.000013 (gnomAD)
-
-
-
Christian Drouet
+/+
9
c.983C>A
r.(?)
p.(Thr328Lys)
ACMG
pathogenic
g.176831232G>T
g.177404231G>T
-
-
F12_000008
Probably the 37 affected individual carrying a NM_000505.3:c.983C>A and recorded by Deroux 2016 have been included in this study. According to ACMG Guidelines - Criteria PS3, PS4, PM1, PM5, PP1 - the variant is considered pathogenic.
Journal: Bocquet 2025
ClinVar-SCV001441472.2
rs118204456
Germline
yes
0.000013 (gnomAD)
-
-
-
Christian Drouet
+/+
9
c.983C>A
r.(?)
p.(Thr328Lys)
ACMG
pathogenic
g.176831232G>T
g.177404231G>T
-
-
F12_000008
Pathogenic variant transmitted through a sperm donor. Asymptomatic brother and male relative. The variant is considered pathogenic according to ACMG Guidelines with following criteria: PS3, PS4, PM1, PM5, PP1.
Journal: Marquès 2026
ClinVar-VCV000001169.8
rs118204456
Germline
no
0.000013 (gnomAD)
-
-
-
Christian Drouet
+/.
9
c.983C>G
r.(?)
p.(Thr328Arg)
ACMG
pathogenic
g.176831232G>C
g.177404231G>C
c.1032C>G
-
F12_000039
The first c.983C>G variant that has never been found
Journal: Dewald 2006
VCV000001170.4
rs118204456
Germline
yes
-
-
-
-
Christian Drouet
-?/.
9
c.984G>C
r.(?)
p.(Thr328=)
-
likely benign
g.176831231C>G
g.177404230C>G
F12(NM_000505.3):c.984G>C (p.T328=)
-
F12_000017
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
-
c.1018+12G>C
r.(=)
p.(=)
-
likely benign
g.176831185C>G
-
F12(NM_000505.3):c.1018+12G>C
-
F12_000065
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-?/.
-
c.1018+13G>C
r.(=)
p.(=)
-
likely benign
g.176831184C>G
g.177404183C>G
F12(NM_000505.3):c.1018+13G>C
-
F12_000015
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-/.
-
c.1018+19del
r.(=)
p.(=)
-
benign
g.176831186del
g.177404185del
F12(NM_000505.4):c.1018+19delG
-
F12_000004
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-?/.
-
c.1018+19dup
r.(=)
p.(=)
-
likely benign
g.176831186dup
g.177404185dup
F12(NM_000505.3):c.1018+19dupG
-
F12_000016
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-?/.
-
c.1018+19G>A
r.(=)
p.(=)
-
likely benign
g.176831178C>T
g.177404177C>T
-
-
F12_000007
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/.
-
c.1019-5C>T
r.spl?
p.?
-
likely benign
g.176831096G>A
-
F12(NM_000505.4):c.1019-5C>T
-
F12_000032
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+/.
9i
c.1019-2A>C
r.spl?
p.?
-
pathogenic
g.176831093T>G
-
F12(NM_000505.4):c.1019-2A>C
-
F12_000045
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-?/.
-
c.1025C>T
r.(?)
p.(Pro342Leu)
-
likely benign
g.176831085G>A
-
F12(NM_000505.3):c.1025C>T (p.P342L)
-
F12_000030
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-?/.
-
c.1027G>C
r.(?)
p.(Ala343Pro)
-
likely benign
g.176831083C>G
g.177404082C>G
F12(NM_000505.3):c.1027G>C (p.A343P), F12(NM_000505.4):c.1027G>C (p.A343P)
-
F12_000014
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
-
c.1027G>C
r.(?)
p.(Ala343Pro)
-
likely benign
g.176831083C>G
g.177404082C>G
-
-
F12_000014
classification based on frequency in 305 unrelated individuals
PubMed: Le 2019
-
-
Germline
-
frequency 0.023
-
-
-
Global Variome, with Curator vacancy
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